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Disease List

Cardiovascular

Stable Angina
Gen: d/t increased O2 demand. DM is the worst risk factor. HTN is most common RF.
Clinical: Substernal chest pain that is worse with exertion and better with rest or NG
Dx:
- 1st – Resting ECG: usully normal in stable angina
- 2nd – Stress: +  cardiac cath w angio (definitive test)
 Stress ECG: looking for ST depression.
 Stress Echo: wall motion tenderness.
 Pharm stress: uses IV adenoside or dypridamole or
dobutamine
- Other: Holter monitor
Tx:
- 1st – RFs reduction, ASA, B-blocker (atenolol, metoprolol), NG (PRN)
 NG adverse effects: HOTS (HA, ortho hypo, tolerance, syncope)
- 2nd – CCBs
- Other - ACEI/Diuretic if also have HF
- Surgical – PCI and CABG

Unstable Angina
Gen: O2 demand is unchanged. Supply is decreased secondary to reduced resting coronary flow. The
only difference b/w USA and NSTEMI is that NSTEMI also has elevation of cardiac enzymes
Clinical:
- Chronic angina with increasing frequency, duration, or intensity
- New onset that is severe and worsening
- Angina at rest
Dx:
-Same as stable angina. r/o MI in all pts and stabilize before attempting dx tests.
Tx:
- Hospital admission with cardiac monitoring, IV access, O2, pain control with nitrates and
morphine if nec.
- Aggressive acute medical management (no thrombolysis) ((MONA-BASH-C))
 ASA
 Clopidogrel
 Beta-blockers
 Enoxaparin
 Nitrates
 O2 if nec
 GP 2b/3a if nec (abciximab)
 Morphine (controversial cuz it can mask sx)
 Electrolyte replacement, especially K and Mg
o Cardiac cath/revascularization
 If medical therapy fails, ischemia changes persisting longer
than 48hrs
 OR coexisting hemodynamic instability, ventricular
arrhythmias, and new mitral regurg.
o After acute tx
 Continue ASA (or other antiplatelet) beta-blockers, and
nitrates
 Reduce RFs (statins, DM drugs, HTN, etc)

Variant (Prinzmetal) Angina


Gen: Transient coronary vasospasm.
Clinical: Episodes of angina at rest and a/w ventricular dysrhythmias. Classically at night
Dx: Hallmark is transient ST segment elevation during chest pain. Definitive test is coronary
angiography; spasms provoked with IV ergonovine or acetylcholine.
Tx: CCBs and nitrates. Rf lowering.

Myocardial Infarction
Clinical: Intense substernal pressure with radiation to neck, jaw, arms, or back. Commonly left side
- can also be epigastric discomfort or asx
- other sx: dyspnea, diaphoresis, weakness/fatigue, n/v, sense of impending doom, syncope
- sudden cardiac death – d/t Vfib
Dx:
- ECG:
 Anterior (LAD)
 ST elevation in V1-V4
 Q waves in V1-V4
 Posterior (Post. Descending artery from RCA)
 Large R wave in V1-V2
 ST segment depression in V1-V2
 Upright and prominent T waves in V1-V2
 Lateral (LCX)
 Q waves in I and AVL
 Inferior (RCA)****DO NOT GIVE NITRATES OR DIURETICS d/t
CV collapse
 Q waves in II, III, AVF
- Enzymes: dx gold standard for myocardial injury; drawn on admission and q8h 3x
 Troponins – most important initially
 CK-MB – reinfarction
Tx: in acute MI, aspirin, b-blocker, acei are the only agents shown to reduce mortality.
- MONA BASH C (see OME notes) for acute
- Maintenance: ASA, Beta-blocker, ACEI, statins
- Revascularization via thrombolysis (rural within 6hrs; ex is alteplase), PCI, or CABG. Best
within 90mins.
 Contraindications to thrombolytics therapy
 Trauma, previous stroke, recent surgery, dissecting
aortic aneurysn, active bleeding.
 Dual clopidogrel and ASA in stents
Complications: Many so look at individual sections for management of most
- Pump failure
- Arrhythmias
- Recurrent infarction
- Mechanical rupture
 Free wall
 IV septum
 Papillary muscle  produces MR
 Ventricular pseudoaneurysm (more likely to end up rupturing)
 Ventricular aneurysm (less likely)
- Acute pericarditis
- Dressler syndrome (aka post-MI syndrome)
 Immunologically based consisting of fever, malaise,
pericarditis, leukocytosis, and pleuritis, occurring weeks to
months after MI
 Tx: 1st – ASA. 2nd – Ibuprofen
Gen: Systolic failure common causes : ischemic heart dz, HTN resulting in cardiomyopathy, valvular
heart dz, myocarditis (post-viral). Diastolic common causes: HTN leading to hypertrophy (MCC),
valvular dz like AS, MS, and MR, and finally restrictive cardiomyopathy.
Clinical features:
- left sided s/s:
 dyspnea, orthopnea, PND, nocturnal cough, confusion,
diaphoresis and cool extremities
 displaced PMI (usually to the left) d/t cardiomegaly, pathologic
S3 gallop, S4 gallop, crackles/rales at lung base (pulm edema),
dullness to percussion and decreased tactile fremitus over
lower lung fields (pleural effusion), increased intensity of
pulmonic component of S2 (pulm HTN)
- right sided s/s:
 peripheral pitting edema, nocturia, JVD,
hepatomegaly/hepatojugular reflux, ascites, RV heave
Dx:
- CXR and ECG (not helpful for the actual dx but is uaully done anyway, cardiac enzymes, CBC,
echo (test of choice; EF <40% = systolic HF, EF >40% = diastolic EF), BNP >150
 BNP, echo (best), and cath are the better ones
- Other: Radionuclide ventriculography with tec-99, cardiac catheterization and angiography
to help find etiology.
Tx:
- Systolic dysfunction: standard tx is first ACEI and beta-blocker, then diuretic if sx. Also add
statins and ASA if CAD associated.
 RF modification: Na less than 2g/day, fluid restriction 1.5-
2.0L/day, weight loss, smoking, restrict ETOH, exercise,
monitor weight daily, annual flu and pneumococcal vaccine.
 Diuretics for sx: furosemide, thiazide
 Spironolactone for advanced stages: monitor K+ and renal
function (CI’d in renal failure)
 ACEI (1st line)– combo of diuretic and ACEI should be started in
most sx pts. ACEI reduces mortality in all stages of CHF. Should
be on even if asx. Monitor BP, K, BUN/Cr
 ARBs (-sartan) or hydralazine/isosorbide dinitrates in
blacks or if they can’t tolerate ACEI
 Beta-blockers: metoprolol, bisoprolol, and carvediol
 Also decreases mortality in post-MI HF
 Digoxin in severe pts after spironolactone hasn’t worked
 s/s of toxicity: N/V, anorexia, ectopic beats, AV block,
Afib, visual disturbances, disorientation
 Contraindicated in CHF
 Metformin – lethal lactic acidosis
 Thiazolidinediones (-zones) – fluid retention
 NSAIDs – may cause exacerbation
 Some antiarrhythmics that have negative inotropic
effects.
 ICD (defibrillator) or cardiac resynchronization therapy in
advanced cases
- Diastolic dysfunction: few options; mostly to treat sx only. No mortality benefit
 Beta blockers
 Diuretics
 Digoxin and spironolactone should NOT be used
- Acute decompensation: LMNOP (lasix, morphine, nitrates, O2, position)
 Do all tests listed first above.
Atrial Fibrillation
Gen: irregularly irregular, ventricular rate between 75-175
Causes:
- heart disease: CAD, MI, HTN, MV dz
- pericarditis and pericardial trauma (e.g. surgery)
- pulmonary dz, including PE
- hyper/hypothyroidism
- systemic illness
- stress
- excessive alcohol intake
- sick sinuc syndrome
- pheochromcytomy
Clinical features: fatigue, exertional dyspnea, palpitations, dizziness, angina, irregularly irregular
pulse, blood stasis.
Dx: ECG – irreg irreg, without P waves with a wavy baseline.
Tx:
- acute
 hemodynamically unstable – immediate electrical
cardioversion
 stable
 beta blockers for rate control
 cardioversion to sinus rhythm
 anticoagulation with an INR 2-3 goal.
- Chronic: same as acute without cardioversion. Pts under 60 don’t even need anticoag, just
beta blocker

Atrial Flutter
Gen: 250-350 atrial bpm, only a some make it through to ventricles
Causes: Heart dz (HF is MC association), rheumatic heart disease, CAD; COPD; Atrial septal defect
Dx: ECG – sawtooth at baseline with QRS every 2-3 p-wave. Best seen in the inferior leads (II, III,
aVF)
Figure 1
Tx: same as Afib

PSVT
Gen: AV nodal reentrant tachycardia
Causes: Ischemic HD, digoxin toxicity (PSVT with 2:1 block is MC association with dig), AV nodal
reentry, Atrial flutter with rapid ventricular response, AV reciprocating tachy, excessive caffeeine or
alcohol consumption.
Dx: ECG - Narrow QRS complexes without P waves (or rare P waves)
Figure 2
Tx:
- acute: vasovagal maneuvers, IV adenosine (1st line; tx of choice), IV verapamil or DC
cardioversion
 adenosine s/e: HA, flushing, SOB, chest pressure, nausea
- Prevention: Verapamil or beta-blockers; radiofrequency catheter ablation of AV node or
accessory tract

Wolff-Parkinson-White Syndrome
Gen: Accession conduction through the bundle of kent that causes premature ventricular contraction
d/t skipping AV node.
Dx: ECG – narrow complex tachy, a short PR interval, and a delta wave
Tx: Radiofrequency ablation of accessory pathway. Med options include procainamide or quinidine
Ventricular Tachycardia
Gen – 3 or more PVCs in a row at a rate of 100-250. Originates below bundle of His. Pwaves
unaffeceted, VT or Vfib causes 75% of episodes of cardiac arrest.
Causes: CAD with prior MI (MCC), active ischemia with hypotension, cardiomyopathies, congenital
defects, prolonged QT syndrome, drug toxicity
Torsades de pointes:
- rapid polymorphic form of VT that can lead to Vfib.
- Ecg shows twisting bow
- a/w prolonged QT d/t genetics or drugs (TCA, antidepressants, electrolytes,
antiarrhythmics)
- tx: IV magnesium
Clinical: palpitations, dyspnea, lightheadness, impaired consciousness, SCD, cannon A waves in the
neck (both atria and ventricle contract at the same time and its like a brief JVD)
Dx: ECG – wide and bizzare QRS complexes
Figure 3
Tx:
- Identify and treat causes
- Acute stable  IV amiodarone
- Acute unstable  DC cardioversion followed by IV amiodarone
- Nonsustained and underlying heart disease  ICD placement 1st; amiodarone 2nd.
- Nonsustained and nml heart  none

Ventricular Fibrillation
Causes: Ischemic heart dz (MCC), drugs that causes torsades, Afib + WPW
Clinical: cant read BP, absent heart sounds and pulse, unconscious
Dx: ECG – no p waves or qrs can be identified
Tx:
- Immediate defibrillation and CPR
 Intubation if indicated
 Epinephrine and repeat defibrillation
 IV amiodarone if refractory
- Successful cardioversion: maintain IV amiodarone until implantable defibs.

Sinus Bradycardia  atropine and pacemaker if persistent


- Sick sinus synrome is persistent spontaneous bradycardia that’s usually in the elderly.

AV Block
Figure 4
- 1st degree
 PR interval prolonged. QRS follows each P wave. Block is in AV
node
 Benign, no tx
- 2nd degree
 type 1 aka Wenchkebach
 progressive prolongation of PR interval until a P wave
fails to conduct (dropped QRS beat)
 block is in AV node. No tx
 type 2
 P wave fails to conduct suddenly, w/o a preceding PR
interval prolongation; therefore, the QRS drops
suddenly
 Often brogresses to complete heart block
 Site of block is within His-purkinje
 Tx is pacemaker
 Type 3 aka complete
 Absense of conduction of atrial impulses to the
ventricles; no correspondence between P and QRS (AV
dissociation
 An ectopic ventricular pacemaker maintains a
ventricular rate of 25-40 (so spread out QRS)
 Tx is pacemaker.

Dilated Cardiomyopathy
Gen – MC type of cardiomyopathy. Insult causes LV dysfunction
Causes:
- idiopathic (50%)
- CAD with prior MI is common known cause
- Toxic: ETOH, doxorubicin, adriamycin
- Metabolic: thiamine or selenium def, hypophosphatemia, uremia
- Infxn: Chaga’s sz, viral, lyme dz, HIV
- Thyroid: hypo or hyper
- Peripartum cardiomyopathy
- SLE, slceroderma
- Prolonged, uncontrolled tachycardia
- Pheochromocytoma, cocaine
- Familial/genetic
Clinical: S/S of L/R heart failure. Cardiomegaly. Associated extra sounds, murmurs, or arrhythmias.
Sudden death

Dx: ECG, CXR, and echo results consistent CHF


Tx: Similar to CHF: Dig, diuretics, vasodilators, and transplant. Remove offending agent. Consider
anticoagulation

Hypertrophic Cardiomyopathy
Gen: Most cases are autosomal dominant (mutation in sarcomeres). Main problem is diastolic
dysfunction d/t hypertrophy that elevate diastoic filling pressures, which further increase with high
HR and contractility or decrease ventricular filling. May also have dynamic outflow obstruction d/t
hypertrophy of the interventricular septum
Clinical:
- Symptoms
 Dyspnea on exertion
 Angina
 Syncope
 Palpitations and arrhythmias
 Cardiac failure and sudden death
- Signs
 Sustained PMI
 Loud S4
 Systolic ejection murmur best heard at LLSB
 Decreases with increased SVR and increased VR:
squatting, lying, straight leg raise, sustained handgrip
 Increases with valsalva and standing
 Rapidly increasing carotid pulse with two upstrokes
(bisferious pulse)
Dx: Echo established dx. Clinical and fam hx are main components
Tx:
- All should avoid strenuous exercise
- Symptomatic pts: 1st - beta-blockers (improves diastolic filling and reduces myocardial
contractility). 2nd – CCBs.
- Symptoms based off complications
- Surgery – Myomectomy of the septum in pts with severe dz

Restrictive Cardiomyopathy
Gen: infiltration causes impaired filling d/t less compliance. Less common
Causes:
- amyloidosis
- sarcoidosis
- hemochromatosis
- scleroderma
- carcinoid syndrome
- chemo or rad induced
- idiopathic
Clinical: elevated filling pressures cause dyspnea and exercise tolerance. Right-sided sx present for
the same reason
Dx:
- Echo: thickened myocardium, increased RA/LA size with normal RV/LV size.
 In amyloidosis: myocardium appears brighter or may have a
sparkled appearance
- ECG: low voltages or conduction abnormalities, arrhythmias, Afib
- Endocardial bx may be diagnostic
Tx:
- Tx underlying disorder
 Hemochromatosis – phlebotomy or deferoxamine
 Sarcoidosis – glucocorticoids
 Amyloidosis – none available
 Give Digoxin if systolic dysfunction is present (except in
amyloidosis d/t increased risk of toxicity.

Myocarditis
Causes:
- Viral: Coxsackie, parvovirus B19, HHV-6. etc
- Bacterial: GAS in rheumatic fever, Lyme dz, mycoplasma, etc
- Other: SLE, meds (sulfonamides), idiopathic
Clinical: may be asx. Or present with fatigue, fever, chest pain, pericarditis, CHF, arrhythmia, death
Classic presentation is a young male
Dx: Cardiac enzyme elevation and ESR
Tx is supportive and of the underlying cause and complications.

Acute Pericarditis
Causes: Many but think about post-viral (esp coxsackie), post-MI (dressler), post-surgery
(postpericardiotomy syndrome) for possible questions.
Complications: Pericardial effusion and cardiac tamponade
Clinical: Cardinal manifestations:
- chest pain (pleuritic, positional that’s relieved by sitting up and leaning forward.
- Look for recent illness
- pericardial friction rub
- ECG changes – diffuse ST elevation; PR depression
- Pericardial effusion w or w/o tamponade
Dx: ECG. Echo for effusion (should be done in all pts to check).
Tx:
- most cases are self-limited in 2-6wks
- treat underlying cause
- mainstay is NSAIDs (colchicine)
 2nd but should be avoided if possible is glucocorticoids.

Constrictive Pericarditis
Gen: fibrous scarring of the pericardium leading to rigidity. Diastolic dysfunction that has rapid filling
early and then halted filling (vs. tamponade where filling is impeded throughout diastole)
Clinical:
- symptoms related to fluid overload and diminished cardiac output
- signs
 JVD
 Kussmaul – JVD fails to decrease during inspiration
 Pericardial knock – corresponds to abrupt cessation of filling
 Ascites and depn edema
 May lead to tamponade
Dx:
- ECG: low voltage. Sometimes afib in advanced dz
- Echo: (best) increased pericardial thickness and sharp halt in filling
- CT/MRI showing thickening and calcifications
- Cath for pressures in filling ventricles
Tx: treat the underlying condition and symptoms. Surgical pericadiectomy for advanced.

Pericardial Effusion
Clinical – nonspecific but include muffled heart sounds, soft PMI, friction fub, dullness at left lung
base.
Dx
- Echo (best)
- CXR shows enlargement and may have “water-bottle” appearance. No pulmonary vascular
congestion.
- Pericardial fluid analysis to find cause
Tx: Don’t do cenesis unless there’s tamponade. Treat underlying cause. Small and asx  repeat echo
in 1-2 weeks

Cardiac Tamponade
Gen: accumulation of pericardial fluid; rate is more important than amount. Effusion that
mechanically impairs filling. Equalization of intracardiac and intrapericardial pressures during
diastole (RV, LV, RA, LA, pulm arter, and pericardium all equalize).
Causes:
- penetrating trauma
- iatrogenic – central-line placement, pacemaker insertion, pericardiocentesis, etc
- pericarditis
- post-MI with free wall rupture
Clinical:
- Beck triad: JVD, hypotension, muffled heart sounds
- Narrowed pulse pressure
- Pulsus paradoxus
 Exaggerated decrease in arterial pressure during inspiration
(>10mmHg)
Dx:
- Echo
Tx: Pericardiocentesis if unstable. Surgery if d/t trauma (pericardial window)

Mitral Stenosis
Gen – almost all cases are d/t rheumatic heart dz. Immune-mediated damage d/t cross-reactivity
between M protein of strep and valve tissue.
-leads to pulm congestion. Anything that increase flow exacerbates the pulm HTN.
-Long standing MS can lead to pulm HTN, RHF, and Afib.
Clinical
- exertional dyspnea, orthopnea, PND
- palpitations, chest pain
- heomptysis
- thromboembolism (afib)
- RHF s/s
- Murmur: opening snap followed by a low-pitched diastolic rumble. The closer the opening
snap is to S2, the worse the stenosis (this is not a typo). Murmur followed by loud S1.
Dx: Echo – LA enlargement; thick, calcified MV; narrow, “fish-mouth”-shaped orifice; RHF in
advanced dz.
Tx:
- Med: (for mild sx; no tx for asx)
 Diuretics for pulm congestion and edema
 Beta-blockers to decrease HR and CO
- Surgical (for severe dz)
 Percutaneous balloon valvuloplasty

Aortic Stenosis
Gen: Severe AS can lead to LV dilation with pulls the MV annulus apart, causing MR.
Causes: Calcification of tricuspid the elderly and bicuspid in the young. Rheumatic fever
Clinical:
- Sx
 Angina
 Syncope (exertional)
 HF sx
- Signs
 Murmur: harsh crescendo-decrescendo systolic murmur heard
in second right intercostal space that radiates to carotid
arteries
 Soft S2. S2 may also be single since the aortic component may
be delayed and merge into P2.
 S4
 Parvus et tardus – diminished and delated carotid upstrokes
 Sustained PMI
 Precordial thrill
Dx:
- ECG: LVH, LA abnormality
- ECHO diagnostic in most cases. LVH; thickened, immobile aortic valve and dilated aortic root
- Cardiac catheterization
 Definitive dx test but used only prior to surgery
- Other: CXR late findings – calcific aortic valve, enlarged LV/LA
Tx: Aortic valve replacement is tx of choice and indicated in all sx pts.

Aortic Regurgitation
Causes:
- Acute
 Infective endocarditis
 Trauma
 Aortic dissection
 Iatrogenic as during a failed replacement surgery
- Chronic
 Primary valvular: rheumatic fever, bicuspid aortic valve,
marfan syndrome, Ehlor-danlos, ankylosing spondylitis, SLE.
 Aortic root disease: syphilitic aortitis, osteogenesis imperfecta,
aortic dissection, Behcet syndrome (causes blood vessel
inflammation), Reiter syndrome, systemic HTN
Clinical
- Sx: HF sx, palpitations, angina, shock
- PE:
 Widened pulse pressure
 Diastolic decrescendo murmur best heard at LSB
 Corrigan (water-hammer) pulse aka bounding
 Austin flint murmur – low-pitched diastolic rumble d/t
competing flow anterograde from the LA and retrograde from
the aorta. Similar to MS murmur.
Dx:
- CXR/ECG – LVH and dilated aorta
- Echo – performed serially in chronic.
- Cardiac cath – to assess severity of AR and degree of LV dysfxn.
Tx: Conservative if asx with vasodilators etc, definitive is surgery and must perform replacement if
its an Acute AR (e.g., post-MI)

Mitral Regurgitation
Causes:
- Acute:
 Endocarditis (MC staph aureus)
 Papillary muscle rupture (from infarction) or dysfunction
(from ischemia)
 Chordae tendineae rupture
- Chronic
 MVP
 RF
 Marfan
 Cardiomyopathy
Clinical: LVH sx; Holosystolic murmur at the apex which radiates to the back or clavicular area.
Dx: Echo will show dilation of the left heart. CXR will show LHF type findings.
Tx: Med – afterload. Surgical – replacement (must be performedbefore LV function is too
compromised)

Tricuspid Regurgitation
Causes:
- usually d/t RV dilation
 LVF, RV Infarct, inferior wall MI, cor pulmonale
- Tricuspid endocarditis in IVDU
- Epstein anomaly – congenital malformation of tricuspid valve in which there is downward
displacement of the valve into the RV. Complication of Lithium in preggers and WPW syndrome.
Clinical: usually asx unless the pt developt RHF/pulm HTN. Blowing holosystolic murmur at LLSB
that intensifies with inspiration.
Dx: Echo
Tx: symptomatic. Surgical correction IF pulmonary HTN is not present.

Mitral Valve Prolapse


Gen – presence of excessive or redundant mitral leaflet tissue d/t myxomatous degeneration of MV
leaflets and/or chordae tendineae. Common in those with marfan, osteo imperfecta, EDS. MCC of MR
in developed countries.
Clinical: Key signs – systolic clicks, midsystolic rumbling murmur that increases with standing and
the valsalva maneuver and decreases with squatting (so decreases with increased VR; only other
one besides HOCM).
Dx – Echo
Tx – condition is usually benign

Rheumatic Heart Disease


Gen: RF may progress to RHD
Dx: Requires two major criteria or one major and two minor
- Major (JONES)
 migratory polyarthitis
 cardiac involvement (pericarditis, CHF, valve dz – usually MS)
 subcutaneous nodules
 erythema marginatum
 Sydenham’s chorea
- Minor
 Fever
 Elevated ESR
 Polyarthralgias
 Prior hx of RF
 Prolonged PR interval
 Evidence of preceding streptococcal infxn
Tx:
- tx strep pharyngitis with penicillin or erythromycin to prevent RF
- acute RF is tx with NSAIDs. CRP is used to monitor tx
- tx valvular pathology of RHD

Infective Endocarditis
Gen: Don’t forget HACEK group: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella.
Dx: TransEsophageal Echo is best (and classic clinical features)
Tx: Parenteral abx based on culture for 4-6 wks. If negative culture, treat with a penicillin (or vanco)
plus an AG.
Px: Indication AND certain procedures
- Qualifying cardiac indications
 Prosthetic valves
 Hx of IE
 Congenital heart dz (unrepaired or repaired in last 6mos)
 Cardiac transplant with valvulopathy
- Qualifying procedures
 Dental, bx or incision of respiratory mucosa, involvement of
infected skin or MSK tissue
- Do not give abx for native MVP/MS or routine GI/GU procedures.
Other:
- Nonbacterial Thrombotic Endocarditis (aka Marantic)
 a/w debilitating illness like met cancer.
 Sterile deposits of fibrin and platelets form on valve.
Vegetations occur and can embolize.
- Nonbacterial Verrucous Endocarditis (aka Libman-Sacks)
 Aortic valves (MC) in SLE pts
 Formation of small, warty vegetations on both sides of leaf-lets
and may present with regurgitant murmurs. Can cause emboli
 Treat underlying SLE and anticoagulate

Atrial Septal Defect (L to R)


Types: ostium secundum (MC), ostium primum, sinus venosus
Clinical:
- usually asx until middle age and presents as exertional dyspnea
- mild systolic ejection murmur at pulmonary area secondary to increased flow. Diastolic
rumble
- fixed split S2
- RVF if advanced.
Dx:
- TEE is best for dx
- CXR shows large pulm arteries and increased pulm markings
- ECG – RBB and RAD; atrial abnormalities
Complications
- Pulmonary HTN
- Eisenmenger disease: irreversible pulm HTN causing reversal and LHF and cyanosis
- RHF, AFIB, stroke from paradoxical emboli or afib
Tx: Surgery if symptomatic

Ventral Septal Defect (L to R)


Gen: MC congenital defect
Clinical:
- Harsh, blowing holosystolic murmur with thrill at 4 left intercostal space. Murmur decreases
with Valsalva and handgrip. The smaller the defect, the louder the murmur
- Sternal lift (RV enlargement)
- Increased P2 heart sound
- Possible Aortic regurg
Dx:
- Echo is best
- ECG – biventricular hypertrophy when PVR is high
- CXR – enlargemnt of the pulm artery and cardiac silhouette.
Complications
- endocarditis
- progressive aortic regurg
- heart failure
- eisenmenger
Tx: same as ASD

Coarctation of the Aorta


Gen – if it’s a woman, think Turner’s
Clinical
- HTN in UE and hypotension in LE; also well-developed in upper and underdeveloped in
lower.
- Midsystolic murmur heard best over the back.
- Sx include HA, cold extremities, claudication with exercise and leg fatigue
- Delayed femoral pulses
Dx
- ECG shows LVH
- CXR:
 Rib notching
 Aorta looks like a “3” d/t indentation of it
Complications:
- Severe HTN
- Rupture of cerebral aneurysms
- IE
- Aortic dissection
Tx: Standard tx invloves surgical decompression. Perc balloon aortoplasty is also an option.
Patent Ductus Arteriosus (L to R)
Gen: Associated with congenital rubella syndrome, high altitude, and premature births. Leading
causes of death in adults are HF and IE. Adults with PDA usually have nml pulmonary pressures
Clinical:
- Continuous “machinery murmur” at left 2nd ICS both systolic and diastolic.
- Loud P2 (pulm HTN)  RV hypertrophy  LVH
- Wide pulse pressure and bounding peripheral pulses
- LE clubbings
- Complication = reversal of shunt
Dx:
- Echo is best for dx
- CXR shows same as other L to R shunts. Additionally sometimes calcification of ductus
arteriosus.
Tx:
- If pulmonary vascular disease is absent – surgical ligation
- If severe pulmonary HTN or R-L shunt is present, do NOT correct PDA. Surgery is
contraindicated.
- Indomethacin indicated for closure. PGE1 can use to keep it open (in face of other
abnormalities like Tetralogy)

Tetralogy of Fallot (R to L)
Gen: Tetrad – 1. VSD 2. Pulmonary artery stenosis 3. RVH 4. Overriding aorta
Clinical:
- Cyanosis is the most common sx
- Sx depns on degree of RV outflow obstruction
- Tet spells: they will squat after exertion (exercise or crying) to increase SVR, which helps
shunt blood from the RV to the lungs instead of the aorta.
- Murmur is typically crescendo-decrescendo best heard at LUSB
Dx:
- Echo is best.
- EKG may show enlarged RA and RV
- Chest xray classically shows boot-shaped heart
Tx:
- O2, Morphine, Beta-blockers may be needed if pt continues to be cyanotic
- Surgical preferably before 1st year of life. MCCOD is SCD and HF
 Complications after surgery include arrhythmias, pulmonary
regurgitations, residual outflow obstruction, and HF.

Hypertensive Emergency/Urgency
HTN emergency - >220 and/or >120 in addition to end-organ damage  immediate tx with IV meds.
HTN urgency is without end organ damage tx with PO meds or decrease BP over 24hrs.
HTN emergency may lead to posterior reversible encephalopathy syndrome (PRES) – a radiographic
condition which is postulated to be caused by autoreguatory failure of cerebral vessels as well as
endothelial dysfunction.
- posterior cerebral white matter edema on MRI
- sx include insidious onset of HA, AMS, vision changes, seizures.
- Tx HTN and sx
-
Aortic Dissection
Gen: Type A (ascending)  surgical tx. Type B (descending)  medical tx.
Clinical:
- Severe, tearing/ripping/stabbing pain, typically abrupt, either in the anterior (type A) or
back/interscapular (type B) of chest.
- Diaphoresis, HTN, or hypotensive
- Pulse or BP asymmetry between limbs
- Aortic regurg (Esp type A)
- Neurologic – hemiplegia/hemianesthesia d/t obstruction of carotid.
Dx:
- CXR shows widened mediastinum
- TEE and CT are the preferred tests for acute dx. TEE is best for unstable pt cuz its
bedside.
- MRI is good but take awhile
- Aortic angiography is the actual best test for determining extent of the dissection for surgery
but its invasive.
Tx:
- Immediate medical therapy for both types
 IV beta blockers
 IV sodium nitroprusside to lower systolic BP below 120mmHg
- Type A – surgery
- Type B – medical
 IV beta blockers
 Pain control
 Unrelenting sx require surgery.

AAA
Gen – most occur between the renal arteries and iliac bifurcation. More common in men but more
likely to rupture in women.
Clinical:
- usually asx, incidental finding
- if present, pain is throbbing
- pulsatile mass on ab exam
- Grey turner sign (ecchymoses on back and flanks) and Cullen sign (ecchymoses around
umbilicus) are signs suggesting impending rupture.
- Rupture of AAA: Triad of abdominal pain, hypotension, and palpable pulsatile mass
requires emergent laparotomy. No further dx testing needed.
 Also CV collapse, syncope/near-syncope, N/V
Dx:
- Ultrasound is test of choice
- CT abdomen only if stable and preop planning.
Tx:
- Unruptured: >5cm or sx  endovascular graft placement.
- Ruptured: emergency repair. Open is gold standard but endovascular can be done too

Peripheral Vascular Disease


Gen: Sites – superficial femoral artery (MC), popliteal, aortoiliac (sx in butt and hips instead of legs).
Smoking is most important RF, DM, CAD, HLD, HTN.
Clinical:
- Intermittent is very reproducible with same distance
- Rest pain is often prominent at nigiht
- Diminished pulses, muscular atrophy, decreased hair, thick toenails, and decreased skin
temp. ischemic ulceration (usually on the toes)
Dx:
- 1st test is ABI (DM pts have false readings)
 >1.3 is very severe cuz vessels are non compressible
 claudication under .7
 rest pain under .4
- Arteriography is gold standard
Tx:
- Conservative for intermittent
 Stop smoking
 Graduated exercise
 Asa and cilostazol and other RF decrease
- Surgical for rest pain, ischemic ulcerations, and severe refractory sx. Options are angioplasty
or bypass grafting.

Acute Arterial Occlusion


Gen: usually caused by embolization. MC site of occlusion is the common femoral artery. MC source is
heart (MC AFIb).
Clinical: Six Ps – pain, pallor, polar, paralysis, paralysis, paresthesisas, pulselessness.
Dx: Arteriogram is gold standard
Tx: anticoagulation or emergent surgery (cutdown and fogarty balloon for embolectomy; bypass if
fogarty failure).
Compartment: assess for compartment syndrome

Cholesterol Embolization Syndrome


-d/t showers of cholesterol crystals from a proximal source triggered by interventions or
thrombolysis. Presents with small areas of iscemia.
- Tx is supportive. Do NOT anticoagulate.

Mycotic Aneurysm
-Aneurysm that results from damage to the aortic wall secondary to infxn. Tx IV abx and surgically
excise.

Luetic Heart
-Complication of syphilitic aortitis with retrograde extension that causes aortic regurg. Tx with IV
penicillin and surg repair.

DVT
Gen: Virchow’s triad – endothelial injury, venous stasis, hypercoagulability (inherited examples –
factor V leiden, protein C and S deficiency, AT3 deficiency).
Clincial: usually asx; calf pain
Dx: Duplex US, d-dimer
Tx: anticoagulation (heparin to warfarin for 3-6mos), tPa, IVC filter (only if absolute contraindication
or failure of anticoagulation – prevents PE not DVT)
Complications:
- PE
- Postthrombotic syndrome: chronic venous insufficiency can result in ambulatory venous
HTN -- see below. Happens in most pts.
- Phlegmasia cerulea dolens (painful, blue, swolen leg)
 Occurs in extreme cases indicating major venous obstruction
 Severe leg edema can compromise arterial supply leading to
impaired sensory and motor
 Venous thrombectomy is indicated.

Chronic Venous Insufficiency


Clinical: aching of LE, worse at end of day; relieved by elevation of legs and worsened by
recumbency; edema, pigmentation, ulcers.
Dx: clinical
Tx: leg elevation, avoid long periods of standing, elastic stocking; if ulcer develop, Unna boots, wet-to-
dry dressings

Superficial Thrombophlebitis
Gen: In UE – usually at IV infusion sites. In LE – usually a/w varicose veins
Clinical: local tenderness, erythema along the course of a superficial vein
Dx: clinical
Tx: analgesics, monitor for spread or cellulitis
Suppurative thrombophlebitis – septic phlebitis d/t IV cannula. Remove and give systemic
abx. Different than cellulitis and lymphangitis because there’s less swelling and there is a palpable,
indurated vein.

Shock
Gen: PCWP is up in cardiogenic and down in all others. CVP is down in hypovolemic only. Sx in all are
hypotension, oliguira, tachycardia, AMS.
Initial steps:
- 2 large-bore caths
- fluid bolus
- draw blood: CBC, electrolytes, renal fxn, PT/PTT
- ECG, CXR
- Continuous pulse ox
- Vasopressors if fluid fails (dopamine (esp if cardiogenic), NE or phenylephrine)
- If all else to dx – pulmonary artery catheter and/or echo
Other:
- Cardiogenic: IVF is likely to be harmful if LV pressures are elevated. Patients may need
diuretics.
- For nonhemorrhagic hypovolemic shock, blood replacement isnt necessary just crystalloid
solution with appropriate electrolyte replacement.
- Urine output measuring is best for treatment monitoring.
- SIRS is characterized by 2 or more
 Fever or hypothermia (38 or 36). Hypo usually in young and
old
 Tachypnea over 20 or PaCO2 under 32
 Tachycardia over 90
 Increased WBC over 12,000 or under 4,000 or bands over 10%
- Sepsis = SIRS + infection source suspicion
- Septic Shock = hypotension persisting despite fluids
- Multiple Organ Dysfunction Syndrome = altered organ function

Pulmonary System Diseases

COPD
Gen – leads to chronic respiratory alkalosis with metabolic alkalosis as compensation.
Dx: PFT values
- FEV1/FVC ratio is <0.70
- Fev1 is decreased
- TLC is increased
- RV is increased.
- Peak expiratory flow is low
- VC is low.
Clinical monitoring includes serial FEV1 measurement, pulse ox, exercise tolerance.
Tx:
- smoking cessation (most important): slows rate of decline but does not result in complete
reversal. Cessation and O2 therapy are the only ones to lower mortality
- Guidelines
 Mild to moderate
 Begin with anticholinergic and/or beta-agonist
 ICS may be used at lowest dose
 Theophylline may be considered after above
 Severe
 Above meds (triple inhaler therapy works combining
ICS,beta,anti-M)
 O2 is hypoxemic
 Pulm rehab
- Acute hospitalized exacerbations
 IV Steroids (plus taper) and Abx (azithro, levo, doxy), O2 to
keep SaO2 between 90-93% (not higher because you lose
respiratory drive and risks higher CO2 retention). May
progress to PPV and intubation.
- Beta blockers are generally contraindicated
- Continuous O2 when PaO2 is at 55mmHg or O2 sat is less than 88%
- Vaccination: yearly flu, pneumococcal every 5 yrs for pts over 65 or under 65 with severe
disease
Complications: Acute exacerbations, secondary polycythemia (>55% Hct in men or >47% in women),
pulmonary HTN and cor pulmonale.

Asthma
Dx:
- PFTs are required for dx
 Ratio under 0.7
 If bronchodilator results in increase in FEV1 or FVC by
at least 12%, obstruction is considered reversible.
 In asthma, methacholine or histamine decreases FEV1
by over 20%
- Pts should self monitor peak expiratory flow rate to self-adjust meds. Peak flow is also used
in the emergent setting.
Clinical: If the patient is having an attack, they should have hypocarbia cuz of the increased RR. If CO2
increases, it’s a bad sign and ventilation should be considered.
Tx/Management: Avoid beta-blockers
- Mild intermittent (sx 2 or less times per week): PRN albuterol
- Mild persistent (sx 2 or more times per week, but not everyday): low dose ICS
- Moderate persistent (daily sx; frequent exacerbations): Daily ICS + LABA (alternative to ICS
is LTA or theophylline)
- Severe persistent (everyday with limited function): daily high dose ICS and LABA. Consider
omalizumab and PO steroids.
- Cromolyn sodium used for ppx only in kids
- Acute severe: inhaled beta2 agonist, IV steroids, possible IV magnesium (3 rd line), O2,
consider abx depn on picture, intubation if nec.

Bronchiectasis
Causes: recurrent infxns, CF (MCC), Primary Ciliary Dyskinesia, AI dz, Humoral immunodeficiency.
Clinical: Chronic cough with large amounts of mucopurulent, foul-sputum. Dyspnea, hemoptysis,
recurrent/persistent PNA.
Dx:
- High-rest CT is best
- PFTs reveal obstructive pattern, Cxr abnormalities, bronchoscopy
Tx:
- Abx for acute exacerbations
- Bronchial hygeine: hydration, chest physiotherapy, inhaled bronchodilators

Cystic Fibrosis
-usually Peds but they can live till 30 now
-tx is pancreatic enzyme replacement, fat-soluble vitamin replacement, chest PT, vaccination (flu and
pneumo), abx, inhaled recombinant human deoxribonuclease (breaks down DNA in respiratory
mucus).
Lung Neoplasms
Random:
- pancoast tumor is a superior sulcus tumor. Usually squamous cell Ca. UE weakness d/t
brachial plexus invasion. a/w horner’s syndrome
- mets: MC sites are brain, bone, adrenal glands, and liver
- paraneoplastic
 SIADH – SCLC
 Ectopic ACTH – SCLC
 PTHrP – Squamous
 Hypertrophic pulmonary osteoarthropathy – adca and
squamous – severe long bone pain may be present
 Eaton-lambert syndrome – SCLC. Proximal muscle weakness
but IMPROVES on muscle use
 Digital clubbing
Staging/Dx: central lesions should be biopsied via bronchoscopy and peripheral lesions can use
transthoracic needle bx under fluorscopic or CT guidance.
- Pleural effusions should be tapped and sent to cytology.
- Pathology is required for cancer dx
- Always perform a bx for intrathoracic lymphadenopathy
Tx:
- NSCLC
 Surgery is best if theres not mets
 Radiation is an important adjunct
- SCLC
 For limited dz, combo oc chemo and radiation is used initially
 For extensive dz, chemo is used alone as initial tx. If pt
responds, then radiation is used as ppx for brain mets.
Figure 5

Mediastinal Masses – MCC is mets (usually from lung) in older pts


Common causes by location:
- Anterior mediastium (Ts)
 Thyroid
 Teratogenic tumors
 Thymoma
 Terrible lymphoma
- Middle: lung cancer, lymphoma, aneurysms, cysts, morgagni hernia
- Posterior: neurogenic tumors, esophageal masses, enteric cysts, aneurysms, bochdalek
hernia.
Clinical: usually asx. When they cause sx, its d/t to compression like cough, dysphagia, nerves etc
Dx: Chest CT is test of choice. If it looks benign and pt is asx then observation is tx.

Pleural Effusion
Gen: caused by – increased drainage of fluid into pleural space, increased production of fluid by cells
in the pleural space, or decreased drainage.
- Transudative: due to elevated hydrostatic pressure or decreased oncotic pressure
o CHF, cirrhosis, PE, nephrotic syndrome, peritoneal dialysis, hypoalbuminemia, atelectasis
- Exudative: caused by increased permeability of pleural surfaces or decreased lymphatic flow
because of damage to membranes or vasculature.
o Bacterial PNA, TB, malignancy, viral infxn, PE, collagen vascular disease.
o Meet at least one of Light’s criteria
 Pleural protein/serum protein > 0.5
 Pleural LDH/serum LDH >0.6
 LDH > 2/3rd the upper limit of nml serum LDH.
Pleural Fluid analysis
- elevated amylase: esophageal rupture, pancreatitis, malignancy
- Milk, opalescent: chylothorax (lymph in the pleural space)
- Frankly purulent: empyema (pus in space)
- Bloody: malignancy
- Exudative effusions that are primarily lymphocytic: TB
- pH < 7.2: parapneumonic effusion or epyema
Dx: CXR is initial, CT more reliable, thoracentesis is dx and tx for bigger effusions
Tx:
- Transudative
 Diuretics and sodium restriction
 Therapeutic thoracenesis if massive and causing dyspnea
- Exudative: treat underlying dz
o Parapneumonic (effusion in presence of PNA)
 Uncomplicated (effusion isnt infectious itself) – abx
 Complicated or empyema (the effusion is also infectious)
 Empyema – aggressive thoracentesis plus abx
 Chest tube drainage
 Intrapleural injection of thrombolytic agents
(streptokinase or urokinase)
 Surgical lysis of adhesions may be required.

Spontaneous Pneumothorax
Tx
- primary spontaneous
o if small and pt is asx – observation (should resolve in 10 days). Small chest tube may benefit
some
o if larger or sx – supplemental O2, needle aspiration/chest tube
- secondary spontaneous (d/t underlying lung dz) – chest tube
Tension Pneumo
Causes: mechanical ventilation w/ barotrauma, CPR, trauma
Clinical features: Hypotension, JVD/neck veins, shift of trachea away, decreased BS, hyperresonance
Tx: medical emergency
- immediate chest decompression with a large-bore need (in 2nd or 3rd ICS of midclavicular
line) followed by chest tube

Interstitial Lung Disesase – general


-PFTs: FEV1/FVC ratio is increased. All lung volumes are low. Low DLco.
-CT can usually be enough for dx. Bx to confirm.
-CXR: reticular, reticulonodular, ground-glass, honeycombing. Depn on severity
ILD – a/w granulomas
Sarcoidosis
- systemic noncaseating granulomatous diseases almost always involving lungs. Usually black
women
- Clinical features
o Consitutional
o Lungs – dry cough, dyspnea
o Skin
o Erythema nodosum, plaques, nodules
o Eyes – anterior uveitis MC
o Heart – arrhythmias, etc (uncommon but MCCOD)
o Msk – arthralgias and arthitis and bone lesions
o Neuro – bell’s palsy, optic nerve dysfunction, papilledema, peripheral neuropathy.
- Dx
o CXR – bilateral hilar adenopathy
o Elevated ACE in serum
o Hypercalciuria and hypercalcemia
o Definitive dx is noncaseating granulomas on transbronchial bx plus clinical.
o PFTs a/w ILD
- Tx: Systemic corticosteroids. Mtx if unresponsive
Histiocytosis X
- 90% of pts are smokers
- variants include eosinophilic granuloma (localized to bone or lung), and 2 systemic forms –
Letterer-Siwe dz and Hand-Schuller-Christian syndrome
- dyspnea and nonproductive cough
- steroids sometimes helpful. May need transplant
Wegener granulomatosis (WeCkner’s)
- necrotizing granulomatous vasculitis
- affects vessels of lungs, kidneys, upper airway, and sometimes other organs
- manifestations: upper and lower respiratory infxns, glomerulonephritis, and pulm nodules.
- Dx: C-ANCA increases likelihood. Gold standard is bx.
- Tx: immunosuppression (cyclophosphamide for ex) and glucocorticoids.
Churg-Strauss syndrome
- granulomatous vasculitis in pts with asthma
- presents with pulm infiltrates, rash and eosinophilia
- a/w P-ANCA
- Tx: systemic glucocorticoids

ILD – Environmental/Pneumoconiosis
Coal Worker’s pneumoconiosis – inhaltion of coal which contains carbon and silica
Asbestosis
- develops 15-20 years later
- increased risk of bronchogenic carcinoma (smoking is synergistic) more commonly, and
malignant mesothelioma.
- CXR shows hazy infiltrates with bilateral linear opacities and may show pleural plaques
- No specific tx.
Silicosis
- Localized and nodular peribronchial fibrosis (upper lobes are more common)
- Can be acute or chronic; CXR findings of egg shell calcifications
- a/w increased risk of TB
- sources include mining, stone cutting, and glass manufacturing
- tx is removal from exposure.
Berylliosis
- aerospace or nuclear industry workers
- acute disease  diffuse pneumonitis. Chronic dz  looks like sarcoidosis
- beryllium lymphocyte proliferation test is useful
- glucocorticoid tx for acute and chronic

ILD a/w Hypersensitivity


Hypersensitivity Pneumonitis
- chronic exposure can lead to ILD
- causes
o farmer’s lung – moldly hay aka spores of thermophilic actinomycetes
o bird-breeder’s lung – bird shit
o air-conditioner lungs - spores of thermophilic actinomycetes
o bagassosis – moldy sugar cane aka spores of thermophilic actinomycetes
o mushroom worker – compost aka spores of thermophilic actinomycetes
o dx by serum IgG and IgA to antigen
o acute form is flu-like. Chronic is more insidious
o tx is removal
Eosinophilic PNA – fever and peripheral eosinophilia that may be acute or

ILD - Alveolar Filling Disease


Goodpasture syndrome
- AI dz caused by IgG abs against the glomerular and alveolar basement membranes (Type 2
HSR against Type IV collagen)
- Hemorrhagic pneumonitis and glomerulonephritis. Hemoptysis and dyspnea.
- Treat with plasmapheresis, cyclophosphamide, and corticosteroids.
Pulmonary Alveolar Proteinosis
- caused by accumulation of surfactant-like protein and phospholipids in the alveoli
- dry cough, dyspnea, hypoxia, and rales
- CXR has a ground glass appearance with bilateral alveolar infiltrates that resemble a bat
shape. Lung bx required for definitive
- Tx: lung lavage and GCSF. Do NOT give steroids cuz they are at increased for infxn.

ILD – Miscellaneous
Idiopathic Pulmonary Fibrosis
- more common in men
- dx: Cxr has ground glass or honeycombed; may be normal. Definitive dx is bx. Exclude all
other causes
- treatment not really effective: O2, steroids, lung transplant
Cryptogenic organizing pneumonitis (COP)
- Similar clinical and radiographic features to infectious PNA
- Tx: steroids. (abx are not helpful)
Radiation Pneumonitis – interstitial inflammation leading to fibrosis. CT scan is best and shows
diffuse infiltrates. Tx: steroids

Acute Respiratory Failure


-General criteria for resp failure: hypoxia (PaO2 <60), hypercapnia (PCO2 >50)
-To determine cause of hypoxemia, you need: PaCO2 level, A-a gradient, response to supplemental O2
- A-a gradient is nml if hypoventilation or low inspired PO2 is the only mechanism
- If V/Q mismatch or shunting is present, then both PaCO2 and A-a gradient are elevated;
response to O2 differentiates between to mechanisms.
Figure 6

- V/Q Mismatch
 Caused by defect in either alveolar ventilation (e.g., pulmonary
edema) or perfusion (e.g., PE)
 Typically leads to hypoxia without hypercapnia
 MC mechanism of hypoxemia and responsive to
supplemental O2
- Shunting
 Little or not ventilation in perfused areas (d/t collapsed or
fluid-filled alveoli)
 Causes: atelectasis or fluid buildup (PNA or pulm edema);
congenital heart dz
 Not responsive to O2
- Hypoventilation – leads to hypercapnia with secondary hypoxemia
- Increased CO2 production (e.g., sepsis, DKA, hyperthermia)
- Diffusion impairment (e.g., ILD) causes hypoxia without hypercapnia

ARDS
Definition
- acute onset ( <1wk)
- bilateral infiltrates on chest imaging. CXR follow clinical severity
- pulmonary edema not explained by fluid level or CHF (e.g., no clinical evidence of CHF or
PCWP <18mmHg)
- abnormal PaO2/FiO2 ratio (<300. The lower, the worse)
- caused by massive intrapulmonary shunting that is not improved by 100% supplemental
O2 (requires high PEEP)
Causes
- Sepsis is most common RF (so any infection)
- Aspiration of gastric contenst
- Severe trauma, fractures, acute pancreatitis, massive transfusions, near-drowning
- Drug OD, toxic inhalation
- Intracranial HTN
- Cariopulmonary bypass
Tx:
- mechanical ventilation – High PEEP and low tidal volumes
Complications:
- permanent lung injury causing scarring and honeycomb
- barotrauma secondary to high pressure ventilation (pneumothorax)
 tracheomalacia (softening) can be caused by prolonged ET
intubation over 2 weeks so a tracheostomy is placed at 2
weeks to prevent
- nosocomial PNA
- line-associated infxns
- renal failure
- ileus, stress ulcers
- multiple organ failure
- critical illness myopathy

Pulmonary Hypertension
Def: mean pulmonary arterial pressure greater than 25mmHg at rest.
Causes:
- passive d/t resistance in the heart (e.g., CHF)
- hyperkinetic (left to right shunts like ASD or PDA)
- obstruction (PE, pulm artery stenosis)
- pulmonary vascular obliteration (collagen vasc dz)
- pulmonary vasoconstriction (eg., chronic hypoxemia, COPD, OSA)
- idiopathic primary – young women
Signs: Loud P2 and subtle lift of sternum (RV dilatation); RHF signs
Dx:
- ECG – RVH/RAD
- CXR – enlarged pulmonary arteries
- Echo – dilated pulm artery, dilatation/hypertrophy of RA and RV, abnormal movement of IV
septum
- Right heart catheterization – reveals increased PA pressure
Tx:
- usually underlying dz
- PAH – use vasoactive agents like sildenafil, oral CCBs, prostacyclins, endothelin receptor
antagonists (e.g., bosentan)
- O2 and other supportive care
- Transplant.

Cor Pulmonale
Gen: RVH with eventual RVF resulting from pulmonary HTN, secondary to pulmonary disease
Causes: usually COPD, recurrent PE, ILD, asthma, OSA, CF, pneumoconioses
Clinical:
- decrease in exercise tolerance
- cyanosis and clubbing
- signs of RVF
- parasternal lift
Dx:
- CXR: enlargement of RA, RV, and PA
- ECG: RAD, P pulmonale (peaked P waves), RVH
- Echo: RV dilatation but nml LV size and function
Tx: underlying dz and supportive care

Pulmonary Embolism
Sources:
- fat embolism – long bone fractures (AMS, dyspnea, petechiae)
- amniotic fluid embolism – during or after delivery
- air embolism – trauma to thorax, indwelling venous/arterial lines
- septic embolism – IVDU
- schistosomiasis
Random RFs – factor V Leiden, protein C/S def, AT3 def., nephrotic syndrome, pregnancy/estrogen
use
Pathophysiology: Blood flow decreases in some areas of the lung and creates dead space. Resulting in
hypoxemia and hypercapnia leading to tachypnea. Leading to low PaO2 and low PaCO2 creating
respiratory alkalosis. A-a gradient is elevated.
Dx: note – V/Q scan is used if pt has significant renal insufficiency and thus CTA is CI’d
Figure 7

Tx:
- Supplemental O2
- LMW heparin with goal of INR 2-3 bridged with warfarin or novel oral anticoagulant for
long-term. Continue for 3-6mos depn on RFs.
o This prevents new clots not existing one
o LWMH has less complications than regular IV heparin.
- Thrombolytic (treats current clot)
 Streptokinase, tPA
 Consider when pt is hemodynamically unstable or evidence of
RHF
- IVC filter if anticoagulation is CI’d

GI Diseases
-note EGD may appear as upper endoscopy

Colorectal Cancer
Dx: Colonoscope best. If found, then CT of chest, ab, pelvis + PE to determine staging. Liver MC site.
Random:
- Both UC and Crohn’s increases risk, UC is just more so (surveillance after 8years).
- Sydromes:
 FAP
 AD mutations in the APC tumor suppressor gene.
 Hundreds of polyps in colon and commonly the
duodenum. Also present elsewhere.
 100% cancer rate by 3rd or 4th decade. PPX colectomy
 Gardner
 FAP (so AD)plus oseomas, dental abnormalities,
benign soft tissue tumors, desmoid tumors, sebaceous
cysts.
 CRC 100% by about 40.
 Turcot
 AD or AR polyps plus cerebellar medulloblastoma or
glioblastoma multiforme
 Peutz-Jeghers
 AD. Single or multiple hamartomas (low malignant
potential) that may be scattered through entire GI
tract (small bowel and colon MC)
 Pigmented spots around lips, oral mucosa, face,
genitals, and palmar surfaces
 Intussusception or GI bleeding may occur
 Familial juvenile polyposis coli
 Rare, childhood numerous colon polyps. Only SMALL
risk of CRC
 Hereditarry nonpolyposis CRC – early onset CRC withOUT
polyps
 Lynch 1 – just CRC
 Lynch 2 – family cancer syndrome. Lynch 1 plus
increased number and early occurrence of other
cancers (e.g., female genital tract, skin, stomach,
pancreas, brain, breast, biliary tract). Think ECO
 CRC is the mCC of large bowel obstruction in adults.
o L side obstructs and R side bleeds (melena not hematochezia).
Rectal cancer: hematochezia, tenesmus, rectal mass and feeling of incomplete evacuation. Higher
recurrence rate

Polyps
Nonneoplastic
- hyperplastic MC
- juvenile (under 10 yo) are highly vascular and common
- inflammatory (pseudopolyps) are a/w UC
Adenomatous polyps
- tubular is MC and has smallest risk of malignancy
- tubulovillous is intermediate
- villous is greatest risk
- shape: sessile is more likely to be malignant vs pedunculated.

Diverticulosis
Dx: for diverticulosis alone – barium enema is the test of choice
Tx: high-fiber and psyllium
Complications
- Painless rectal bleeding – usually no tx is needed unless its severe and you need surgery
- Diverticulitis
 Complications
 Abscess formation that can be drained percutaneously
under CT guidance or surgery
 Colovesical fistula
 Obstruction
 Perforation – surgery immediately
 Clinical – fever, LLQ, leukocytosis
 Dx – CT ab/pelvis with contrast is test of choice
 Barium and colonoscopy are contraindicated
 Tx for uncomplicated
 Outpatient: PO abx (cipro and metro for ex
 Inpatient: IVF, NPO, IV Abx (cephalosporin plus metro
for ex)
 Tx for complicated is typically surgery.

Angiodysplasia/AV malformation/Vascular ectasia – common cause of lower GI bleeding in adults


over 60. Typically stops on its own. Dx via colonoscope. Tx if necessary can be done by colonoscopic
coagulation of the lesion or right hemicolectomy if really bad.

Acute Mesenteric Ischemia


Types:
- Arterial Embolism
 From heart
 Sx are more sudden and painful than others
- Arterial thrombosis
 Atherosclerosis
 Sx more gradual and less severe
- Nonocclusive ischemia
 Splanchnic vasconstriction 2/2 to low CO
 Usually critically ill pts
- Venous thrombosis
 Sx may be present for several days or weeks with gradual
worsening
 Hypercoagulability
Clinical: Classic – severe abdominal pain that is disproportionate to PE
- also: anorexia, vomiting, mild GI bleeding, or advanced peritoneal signs
- Intestinal infarction: shock and lactic acidosis. Check lactic acid when AMI suspected
Dx: Mesenteric angiography is test of choice. Plain ab film first to exclude other stuff.
Tx:
- support: IVF and broad-spec abx
- arterial causes: direct intra-arterial infusion of papaverine (vasodilator) during
arteriography.
 Direct intra-arterial thrombolytics if necessary for embolus.
- Heparin for venous thrombosis
- Surgery resection if peritonitis develops

Chronic Mesenteric Ischemia


-caused by atherosclerosis
-postprandial abdominal angina
-significant weight loss can occur
-dx: mesenteric arteriography.
-tx: Surgical revascularization

Ogilvie Syndrome
-sx and radio evidence of LBO but there is no mechanical obstruction usually after trauma or surgery
or serious illness or 2/2 to drugs.
-Tx: stop offending agents and supportive measures (IVF, electrolyte repletion)
-decompression is next step if above fails. Surgical decompression is last resory

Pseudomembranous Colitis – C diff


-MCC are clindamycin, ampicillin, cephalosporins
-profuse watery diarrhea with crampy abdominal pain
-complications: toxic megacolon, perf, anasarca (widespread edema), electrolyte
disturbances
-dx: c diff toxins in stool
-ab xray to r/o megacolon or perforation
-leukocytosis
Tx:
- d/c offending med
- metronidazole is drug of choice
 don’t use in infants or pregnant
- oral vanco if resistant or can’t tolerate

Chronic Volvulus
Gen: MC site is the sigmoid colon. Followed by cecum.
Clinical:
- acute onset of colicky ab pain
- obstipation, distension
- anorexia, N/V
Dx:
- pain films:
 sigmoid – omega loop sign (bent inner-tube shape)
 cecal – coffee bean sign (distension of cecum and small bowel)
- sigmoidoscopy: preferred dx and tx for sigmoid volvulus
- barium enema shows bird’s beak
Tx: sigmoidoscope decompression for sigmoid. Emergent surgery for cecal.

Cirrhosis
Gen: Fibrotic replacement of damaged hepatocytes
-Causes:
Alcohol (MCC)
Chronic Hep B and Hep C
Drugs (acetaminophen, methotrexate)
NASH
Congestion secondary to RHF
AI hepatitis
PBC
Inherited metabolic diseases (Wilson, hereditary hemochromatosis)
Alpha 1 antitrypsin def
Hepatic venoocclusive disease (after BM transplant)
-Management
1. treat underlying cause
2. avoid toxic agents
3. treat complications
4. liver transplant
5. Adding Vaccinations for hep A and B
-Complications and associated clinical features
1. Varices/Portal HTN
A. Esophageal varices - massive hematemesis or melena
ppx: nonselective beta-blocker
acute: ligation, endoscopic sclerotherapy, IV octreotide (splanchnic vasoconstriction), IV vasopressin
(alternative to octreotide)
other: TIPS and liver transplant
B. Rectal hemorrhoids
C. Caput medusa
D. Ascites – d/t portal HTN and hypoalbuminemia
Dx: Distension, shifting dullness, fluid wave. US. Paracentesis for dx and Tx
Tx: Low sodium and diuretics. Paracentesis. TIPS
2. Hepatic Encephalopathy
-Precipitants: alkalosis, hypokalemia, sedatives, GI bleeding, infxn, hypovolemia
-Clinical: AMS, asterixis, rigidity, hyperreflexia, musty odor of breath
-Tx: Lactulose (promotes excretion), rifaximin (decreases production)
3. Hepatorenal syndrome
-happens in advanced liver disease, secondary to renal hypoperfusion resulting from
vasoconstriction of renal vessels. Functional renal failure
- azotemia, oliguria, hyponatremia, hypotension, low UOP
-only tx is transplant
4. SBP
-E coli, klebsiella, strep pneumo
-ab pain, fever, rebound tenderness, vomiting, may lead to sepsis
-Dx by increased WBC/PMNs from paracentesis
-Tx: broad-spec abx (cefotaxime) until sensitivities
5. Hyperestrinism
-spider angiomas, palmar erythema, gynecomastia, testicular atrophy
6. Coagulopathy
- prolonged INR first then PTT
- Vit K ineffective
-Tx with FFP
7. HCC

Management
1. treat underlying cause
2. avoid toxic agents
3. treat complications
4. liver transplant
5. Adding Vaccinations for hep A and B
Wilson Disease
Gen: AR mutation in the ATP7B gene
Clinical features: deposition in liver, eye, CNS (EPS like parkinsonian, chorea, etc; also psych
disturbances), renal involvement (aminoaciduria, nephrocalcinosis).
Dx:
- low ceruloplasmin, elevated LFTs, liver bx
- if diagnosed, 1st degree relatives must be screened
Tx:

- d-penicillamine
- zinc
 prevents uptake of dietary copper
 given alone in presymptomatic pts and pregnant pts or given in
conjunction with d-penicillamine
- liver transplant
- monitor copper levels and liver and other signs of dz

Hemochromatosis
Gen: AR dz of iron absorption – screen siblings.. Accumulates as ferritin and hemosiderin that lead to
hydroxyl free radicals to affect:

- liver (primarily) - cirrhosis


- pancreas - DM
- heart – cardiomyopathy leading to CHF and arrhythmias
- joints – MC sites are 2nd and 3rd MCPs, hips, knees
- skin - hyperpigmentation
- thyroid - hypothyroid, gonads – hypogonadism in both sexes, hypothalamus

Dx:
- markedly elevated serum iron and ferritin
- decreased TIBC/elevated transferrin saturation
- liver bx is required to for dx
- genetic testing
Tx: repeated phlebotomies and treat any complications. Liver transplant in advanced

Hepatocellular Adenoma
-benign liver tumor usually seen in young females, OCP use, anabolic steroid use
- asymptomatic or could have RUQ pain/fullness.
- more likely to rupture than turn malignant.
- Dx: CT or US
-Tx: D/C OCPs and surgiccally resect if they don’t regress and they are large.

Cavernous Hemangiomas
-MC type of benign liver tumor. Usually small and asx. OCPs or pregnancy can increase size and cause
complications like obstruction and rupture.
-Dx: US or CT with IV contrast. Bx contraindicated d/t risk of rupture
-Tx: resect if too large or about to rupture.

Focal Nodular Hyperplasia – benign liver tumor without malignant potential in women with
reproductive age. There is NO a/w OCPs. No tx necessary usually

Hepatocellular Carcinoma (HCC)


Gen: 2 pathologic types: nonfibrolammelar (a/w HBV,HCV, and cirrhosis). Usually unresectable and
poor prognosis. Fibrolamellar (not a/w hep and cirrhosis). Adols and YAs. Resectable and better.
RFs:
-cirrhosis. Especially if a/w hep b or c
-chemical carcinogens (aflatoxin, vinyl chloride, thorotrast)
- AAT def
- hemochromatosis, wilson’s
-hepatic adenoma (10% risk)
-smoking
-glycogen storage dz type 1
Clinical:
-ab pain, systemic, liver dz
- paraneoplastic syndromes: erythrocytosis, thrombocytosis, hypercalcemia, carcinoid
syndrome, hypertrophic pulmonary osteodystrophy, hypoglycemia, high cholesterol.
Dx:
- liver bx – required
- Hep B/C serology, LFTs, coagulation tests, imaging, AFP is useful as a screening.

Hemobilia
Gen: bleeding into the duodenum from the CBD. Source can be anywhere along biliary tract
Causes: Trauma (MCC), papillary thyroid carcinoma, surgery, tumors, infxn
Clinical: UGI bleed, jaundice, and RUQ pain
Dx: Arteriogram is diagnostic.
Tx: resuscitation. Ligation or embolization may be necessary.

Hydatid Liver Cysts


Cause: infection from the tapeworm Echinococcus. Cysts MC occur in the right lobe of the liver
Tx: Surgical resection followed by Mebendazole.
Complication: Anaphylactic shock --- either from rupture or spilling contents during surgery.

Liver Abscess – MC in R lobe


Pyogenic:
- MCC is biliary tract obstruction. Other causes are spread of infxn or trauma
- Orgs: E Coli, Klebsiella, proteus, enterococcus, and anaerobes.
- Clinical: Sick as shit. With RUQ pain and jaundice.
- Dx: US/CT; elevated LFTs
- Tx: IV abx and percutaneous drainage. May require surgical drainage.
Amebic:
-MC in men, particularly, homosexual men. Transmitted through fecal-oral route
-Entamoeba histolytica
-Clinical: fever, RUQ pain, N/V hepatomegaly, diarrhea.
-Dx: First test is imaging US/CT to find absecess and then Serologic (IgG immunoassay)
establishes diagnosis. LFTs also elevated.
-Tx: IV metronidazole. Perc drainage may be necessary if large (high risk of rupture) or
refractory to treatment.

Budd-Chiari
Gen: Caused by occlusion of hepatic venous outflow leading to microbascular ischemia and
congestion resembling cirrhosis clinically.
Causes: Hypercoagulable states, myeloproliferative DOs, pregnancy, chronic inflamm, infxn, cancer,
trauma, mostly idiopathic.
Dx: Hepatic venography; serum ascites albumin gradient >1.1 g/dL
Tx: Medical usually fails. Surgery to place a stent in IVC or portacava. Transplant if progressed to
cirrhosis.

Jaundice
Figure 8
Cholelithiasis and related diseases
Stones: Black stones are usually d/t hemolysis and alcoholic cirrhosis
Cholithiasis: biliary colic. Elective surgery if symptomatic
Acute Cholecystitis: obstruction and inflammation (not infection)
- RUQ pain that is constant, Murphy’s sign, low grade fever and leukocytosis
-Dx: US that may show: thickened GB wall, pericholecystic fluid, distended GB and stones
-CT if identifying complications: gangrenous chole, perforation, emphysematous
chole, cholecystoenteric fistula with gallstone ileus, empyema of GB
-HIDA scan when US is inconclusive
-If no GB is visualzied then cholecystitis is confirmed
-Tx: Initially – IVF, NPO, IV ABX, analgesics, electrolytes.
-Followed with early cholecystectomy.
-Note: acalculous cholecystitis is usually found in severe ICU pts. Treatment is still removal
or drainage.

Choledocholithiasis
-Sx: RUQ/epigastric pain and jaundice.
-Dx: 1st – US 2ND – ERCP (Gold standard)
-Tx: ERCP

Cholangitis
- Charcot’s triad - RUQ pain, jaundice, and fever. Pentad – add AMS and septic shock
(hypotension)
-Dx: 1st – US (shows dilatation so usually good enough)
- ERCP/PTC after acute phase
-Tx: IVF and IV abx. After resolution do ERCP to decompress (sphincterotomy). Or do it
emergently if no response to abx.
-Note: Hepatic Abscess is the most dreaded complication and has a high mortality rate.

Carcinoma of the Gallbladder


-most are adenocarcinomas and in the elderly with a dismal prognosis
-a/w gallstones in most cases; other RFs include cholecystoenteric fistula and porcelain gallbladder
Primary Biliary Cirrhosis vs. Primary Sclerosing Cholangitis
Figure 9
Cholangiocarcinoma
Gen/types: Most are adenocarcinomas
- Proximal 1/3rd of CBD (aka Klatskin tumor; MC location), distal extrahepatic (best chance
at resection), intrahepatic (least common)
- PSC is the major risk factor in the U.S.; other RFs – UC, choldochal cysts, Clonorchis sinensis
infestation (Hong Konx)
Clinical: Obstructive jaundice and weight loss
Dx: Cholangiography (aka ERCP or PTC) with stenting to help with obstruction

Biliary Dyskinesia
-motor dysfunctin of the sphincter of Oddi causing biliary colic without any evidence of stones on US,
CT, ERCP
-dx: HIDA scan with CCK. Low EF = dyskinesia
-Tx: lap chole or endoscopic sphincterotomy.

Appendicitis
-Clinical diagnosis
-signs of perforation: high fever, tachycardia, marked leukocytosis, peritoneal signs, toxic
appearance)
-carcinoid tumors originate from neuroendocrine cells and secrete serotonin
- most common site is appendix (can also be found in small bowel, rectum, bronchus, kidney,
pancreas).
– excess serotonin can lead to carcinoid syndrome: cutaneous flushing, diarrhea, sweating,
wheezing abd pain, and heart valve dysfunction (tricuspid insufficiency and pulmonary stenosis)

Acute Pancreatitis
Gen: Inflammation resulting from prematurely activated pancreatic digestive enzymes that involve
pancreatic autodigestion. Can be severe (necrotizing)
Causes:
- ETOH and gallstones are equally common
- Post-ERCP
- Viral infections (mumps, Coxsackie B)
-Drugs: sulfonamides, thiazide diuretics, furosemide, estrogens, HIV meds, and others
-Postop complications
- Scorpion bites
- Pancreatic cancer
-Hypertriglyceridemia, hypercalcemia
-Uremia
-Blunt abdominal trauma (MCC in kids)
Clinical: Ab pain radiating to the back. Worse when supine and after meals. N/V and anorexia.
-Hemorrhage
-Grey turner sign: flank ecchymoses
-Cullen: periumbilical ecchymoses
-Fox sign: ecchymosis of inguinal ligament
Dx: Labs are supportive CT is confirmatory
-Lipase, LFTs, GALAW
Complications:
- Necrosis
-Sterile vs infected? Do a CT-guided percutaneous aspiration for gram stain
-If infected, then debridement and abx are indicated
- Pseudocyst
-Encapsulated fluid that appears 2-3 weeks after acute attack. Lacks true epithelial
lining. Untreated can lead to rupture, infect, obsturction, etc.
-Dx: CT
-Tx: If under 5cm –observation. If over 5cm – drain percutaneously or surgically.
- Hemorrhage (as shown by signs listed above): CT with IV contrast is study of choice
-ARDS
-Pancreatic ascites/pleural effusion
-Ascending cholangitis
-Abscess: 4-6 weeks later and less threatening then necrosis.
Tx:
-Mild: supportive. Pain control, NPO, IVF
-Severe: ICU (3 or more Ransom criteria). Early enteral nutrition in the first 72 hrs through
NJ tube. If more than 30% of pancreas is necrosed, ppx abx with Imipenam should be considered.

Chronic Pancreatitis
Gen: Persistent inflammation with fibrotic tissue replacement and alteration of pancreatic ducts
(stricture and/or dilation); eventually leads to irreversible damage and impairs endocrine/exocrine
functions. MCC is chronic alcoholism.
Clinical: same as acute but can also have steatorrhea and diabetes leading to malabsorption and
weight loss.
Dx:
- CT with calcifications plus clinical presentation is diagnostic
- ERCP is the gold standard but not routinely done.
-Amylase and lipase are NOT elevated
Complications:
- Narcotic addiction (MC)
- DM
- Malabsorption/steatorrhea (late manifestation)
-pseudocyst
-pancreatic ductal dilation
-CBD obstruction
-Vitamin B12 malabsorption
-Effusions
-Higher risk of panc carcinoma
Tx:
-Medical tx:
-Narcotics for pain
-NPO
-Panc enzymes and H2 blockers given together (enzymes decrease endogenous
secretions after meals and H2 blockers prevent enzymes from getting degraded)
-Insulin
-Alcohol abstinence
-Frequent, small-volume, low-fat meals
-Surgical: goal is to relieve pain
-pancreaticojejunostomy – decreases dilated ducts – MC procedure
-pancreatic resection

Pancreatic Cancer (painless jaundice is NOT common in pancreatic cancer)


Clinical: Ab pain (90% of pts), jaundice, wt loss, recent glucose intolerance, migratory
thrombophlebitis, and Courvosiier sign (palpable GB)
Dx:
-ERCP is best for the actual panc cancer
-Then, CT for staging
-Tumor markers: CA-19, CEA
Tx:
-Whipple procedure
-ERCP/PTC with stent for palliation if biliary obstruction is present and the tumor is
unresectable.

GI Bleeding
UGI Causes (if melena, note that dark stools can also result from bismuth, iron, spinach, charcoal, and
licorice). Side note – if BUN/Cr is elevated, its more likely UGI
-PUD
-Reflux esophagitis
-Esophageal varices
-Gastric varices, gastric erosions, duodenitis
-mallory-weiss tear
-hemobilia
-Dieulafoy’s vascular malformation – submucosal dilated arterial lesions that can cause
massive GI bleeding
-Aortoenteric fistulas – after aortic surgery/grafting – can lead to fatal hemorrhage –
perform endoscopy or surgery immediately.
-neoplasm
LGI Causes
-Diverticulosis (painless)
-angiodysplasia
-IBD
-CRC
-polyps
-ischemic colitis
-hemorrhoids, anal fissures
-small intestine (dx after r/o UGI and colon)
Tests: NG tube is often performed before anything. If bile without blood = LGI. If blood = UGI
(usually)
-Hematemesis – EGD is initial test
-Hematochezia – first r/o anorectal cause (hemorrhoids for ex). Colonoscopy is the initial
test.
-Melena – first EGD. If nothing found, then do colonoscopy
-Occult blood – Colonsocopy first. If nothing then EGD
-Radionuclotide testing can detect blood at slower flow rates but doesn’t localize
-Arteriography definitively locates (can also tx with vasoconstrictor)
-Ex lap is last resort – not responding to resuscitation

Dysphagia DDX Chart


Figure 10

Esophageal Cancer
Gen – SCC d/t smoking and drinking usually. Adca – GERD/Barrett’s
Clinical
- dysphagia (solids then liquids)
- weight loss, anorexia
- odynophagia – late in course
-hematemesis, hoarseness, CP, fistula
-aspiration PNA d/t involvement tracheobronchial tree
Dx: UGD with bx and mets workup if positive
Tx: Palliative is usual tx. Esophagectomy if low staged or chemo + radiation

Achalasia
Gen – incomplete relaxation of the LES and aperistalsis of esophagus. Increased risk of SCC
Causes – U.S.=idiopathic (MCC) and AdCa of proximal stomach. Worldwide = Chagas
Clinical – Equal difficulty swallowing solids and liquids (vs. cancer). Dysphagia, regurgitation, CP,
weight loss, recurrent pulm complications secondary to aspiration
Dx – Barium swallow  UGI endoscopy to r/o secondary causes  manometry to confirm
Tx: - antimuscarinic agent (dicyclomine) (usually unsuccessful)
- sublingual NG, nitrates, CCBs may help in early stages
- botulinum toxin during endoscopy
- force dilatation: pneumatic balloon is most effective
- surgical: “Heller myotomy” - circular muscle layer of LES is incised

Diffuse Esophageal Spasm


Gen: Nonperistaltic spontaneous contraction of several segments of esophagus. Sphincter function
is normal
Clinical: Non cardiac chest pain that mimics angina and may radiate to the jaw, arms, and back.
Dysphagia. (regurg is uncommon)
Dx: Initial – Barium swallow showing “corkscrew esophagus”. Manometry is diagnostic.
Tx: nothing really effective. Can try nitrates and CCBs and TCAs for symptoms

Esophageal Hiatal Hernias


Gen: Think of sliding and paraesophageal. Sliding = GERD. Paraesophageal = possible strangulation
and needs surgery.
Dx: barium upper GI series and EGD.
Tx: see Gen

Mallory-Weiss Syndrome/Boerhaave Syndrome


-forceful vomiting causing a tear in the mucosa at the GE junction  Mallory
-forceful vomiting causing transmural tear  Boerhaave
-hematemesis is always present and EGD is diagnostic
-most mallory tears stop bleeding without treatment
-otherwise surgery is required to oversew the tear or angiographic embolization. Acid suppression
helps healing.

Plummer-Vinson Syndrome (upper esophageal webs)


Key features: upper esophageal web causing dysphagia, iron def anemia, koilonychia (spoon-shaped
fingernails) and atrophic oral mucosa
-precursor for SCC
-Tx: esophageal dilatation; correct nutritional deficiency

Schatzki Ring (Distal esophageal webs)


-a circumferential ring in the lower esophagus that is ALWAYS accompanied by a sliding hiatal hernia
-usually d/t ingestion of alkali, acids, bleach or detergents in suicide attempts
-alkali causes full-thickness necrosis and perforation  esophagectomy
-complications: stricture formation and risk of eso cancer
-bougienage for stricture

Esophageal Diverticula
Upper 1/3rd – Zenker – underlying motility disorder – failure of the cricopharyngeal muscle to relax
during swallowing leads to increased pressure and outpouching pharyngeal constrictors – dx with
barium swallow – tx is cricopharhyngeal myotomy
Mid 1/3rd – Traction – d/t traction from contiguous mediastinal inflammation and adenopathy (like
pulmonary TB) – usually no treatment
Lower 1/3rd – Epiphrenic – underlying motility disorder – a/w DES or achalasia – dx with barium
swallow – tx esophagomyotomy

Esophageal Perforation
Causes: blunt trauma, boerhaave syndrome
Clinical: pain (severe retrosternal/chest/shoulder), SIRS sx, Hamman sign (mediastinal crunch
produced by the heart beating against air-filled tissues), pneumothorax, effusion.
Dx:
-Contrast esophagram is definitive
-CXR usually shows air in the mediastinum
Tx:
-small  IVF, NPO, IV ABX, H2 Blockers
-large/unstable  surgery within 24hrs.

Peptic Ulcer Disease


-urea breath test can be initial test. Bx is gold standard for H pylori though
-triple therapy: PPI + amoxicillin + clarithromycin for 2 weeks. Quadruple therapy: PPI, bismuth
subsalicylate, metronidazole, and tetracycline fro 1 week.
-Complications
-perforation
-acute severe ab pain, signs of peritonitis, hemodynamic instability
-upright CXR (free air under diaphragm), CT is most sensitive
-emergency surgery to close perf and vagotomy or vagotomy/pylroplasty
-can progress to sepsis and death if untreated
-gastric outlet obstruction
-N/V, epigastric fullness/early satiety, weight loss
-barium swallow and upper endoscopy; saline load test
-initially, NG suction; replace electrolyte/volume deficits; supplement nutrition;
surgery usually necessary
-most common with duodenal ulcers and prepyloric (type 3) gastric ulcers
-GI bleeding
-may be slow leading to anemia or rapid leading to shock
-stool guaic; UGD is dx and tx
-resusitation and endoscopy; surgery if nec.
-peptic ulcer disease is the MCC UGI bleed

Acute/Chronic Gastritis
- acute is very similar to PUD; relationship between eating and pain is not consistent.
-H pylori is the most common cause for acute or chronic
-Autoimmune gastritis leads to chronic atrophic gastritis with serum antiparietal and anti-intrinsic
factor antibodies (and possible development of pernicious anemia)

Gastric Cancer
Gen: The majority are AdCa. Ulcerative, polypoid, superficial spreading, linits plastica
Metastases:
-Krukenburg – ovary
-Blumer’s shelf – rectum (pelvic cul-de-sac) – can palpate on DRE
- Sister Mary Joseph – periumbilical LN
- Virchow node – supraclavicular fossa nodes
- Irish node – left axillary adenopathy
Dx: EGD with biopsy followed by ab CT for staging
Tx: surgical resection with extended LN dissection. May add chemo

Gastric Lymphoma
-Non-hodgkin’s lymphoma in the stomach that presents similar to gastric cancer
-complications include bleeding, obstruction, and perf
- same dx and tx as gastric cancer

Small Bowel Obstruction


Gen:
- Partial vs complete: partial can pass gas
- closed loop obstruction, where the lumen is occluded at 2 points, the blood supply can be
compromised (strangulation  perf, shock, etc
-causes hypochloremic, hypokalemic, metabolic alkalosis
Sx: proximal – frequent vomiting, severe pain, minimal distension.Distal – less vomiting and
significant ab distension, more obvious on xray..
Causes: 1 – adhesions. 2- incarcerated hernias. 3 – malignancy, intussusception, chrons,
carcinomatosis, and SMA syndrome
Dx: Ab plain films – dilated loops, air-fluid levels proximal to obstruction, minimal gas in colon
Tx:
-IVF and potassium
-NG decompression
-abx
-surgery (lysis of adhesion and resect any necrotic bowel) if complete obstruction,
strangulation, persistent partial.

Paralytic Ileus
CXR: ileus vs SBO  ileus will have gas all throughout the intestines.
-hypokalemia is significant cause
-failure to pass contrast medium beyond a fixed point is diagnostic
-tx: IVF, NPO, correct electrolyte, NG suction, tube placement if necessary (no surgery)

Celiac Sprue
-best antibody test is anti-TTG
-a/w vitamin deficiencies
-osteoporosis (vit d)
-easy bleeding (vit K)
-megaloblastic anemia (poor folate and B12 absorption)
-a/w dermatitis herpetiformis (papulovesicular lesion seen on the extensor surfaces)
-Dx: biopsy of duodenum shows flattening of villi
-tx: avoid gluten

Whipple disease
-inflammation secondary to infection and villi damages
-weight loss, diarrhea, joint pain and arthritis
-dx: PAS positive macrophages in the lamina propria containing non-acid-fast gram-positive bacilli
-tx: abx for up to 1-2 years

Tropical Sprue
-celiac sprue but lives in tropical region
-same clinical and endoscopy findings
-tx is abx and folic acid for 6 months or so

Crohn’s disease
Dx: endoscopy with bx – aphthous ulcers, cobblestone, pseudopolyps, skip lesions. Other pathology:
transmural inflammation, noncaseating granulomas, strictures, mesenteric fat creeping
Extraintestinal manifestations: uveitis, arthritis, ankylosing spondylitis, erythema nodosum,
pyoderma gangrenosum, aphthous oral ulcers, cholelithiasis, nephrolithiasis
Complications: fistulae, anorectal disease, SBO, malignancy (less common than UC), malabsorption of
B12 and bile acids (so therefore DEAK vitamins def and cholelithiasis)
Tx:
- Sulfasalazine (better for UC but still works as anti-inflammatory)
-metronidazole if no response to 5-ASA
- Steroids for acute exacerbations and if no response to metro
-steroids plus other immunosuppressants
-bile acid sequestrants (cholestyramine, colestipol)
-surgical for complications
-nutritional support

Ulcerative Colitis
-only involves mucosa and submucosa and is continuous
-PMNs accumulate in the crypts of the colon (crypt abscesses
-clinical: usually abdominal pain and hematochezia
-extraintestinal: same as crohn’s with emphasis on arthritis, pyoderma gangrenosum, ankylosing
spondylitis, sclerosing cholangitis
-dx: initial  stool cultures to r/o infectious causes, fecal leukocytes. Confirm with colonoscopy
-complications: IDA, hemorrhage, E+ disturbance, strictures, colon cancer, sclerosing cholangitis,
cholangiocarcinoma, toxic megacolon.
-screening colo after 8 years with disease
-Tx:
-steroids for acute exacerbatioins
-Topical Sulfasalazine is the mainstay (5-ASA is the active metabolite)
-immuosuppresive to prevent relapses if refractive
-surgery is often curative (unlike Crohns) and involves total colectomy. Used for refractory
and complicated disease.

Endocrine and Metabolic Disease

Hyperthyroidism
-causes
- Graves (diffuse toxic goiter): MCC
- AI disorder. IgG antibody binds to the TSH receptors and triggers excess synthesis.
-Young women usually. A radioiodide scan shows diffuse uptake
-Plummer disease (multinodular toxic goiter)
-hyperfunctioning areas that produce high T4 and T3 levels and decreases TSH
levelsresulting in thyroid atrophy
-Scan will have patchy appears on the scan
-Toxic thyroid adenoma
-Hashimoto thyroiditis and subacute granulomatous thyroiditis: both can cause transient
hyperthyroid.
-other (rare): postpartum thyroiditis, iodine-induced, excessive levothyroxine
-Clinical features
-classic: nervousness, insomnia, irritability, tremor, hyperactivity, sweating, heat intolerance,
weight loss, diarrhea, palpitations muscle weakness
-elderly: weight loss, weakness, Afib
-Graves: exophthalmos, pretibial myxedema, thyroid bruit, diffuse enlargement
-Subacute thyroiditis: exquisitely tender, diffusely enlarged glang (with a viral illness)
-Plummer disease and Hashimoto – bumpy, irregular
-Toxic adenoma – single nodule amongst atrophic gland.
Labs: Factors that increase TBG (and therefore total T4) include pregnancy, liver disease, OCPs, and
aspirin. TBG is decreased by anabolic steroids.
-Tx: note - for all pts on antithyroid medication, monitor leukocyte count for agranuloctosis
-Immediate control of adrenergic sx: Propranolol
-Non-pregnant with Graves disease
-start methimazole + propranolol. Taper propranolol and continue methimazole
-Pregnany with graves
-PTU is preferred
-Radioactive iodine ablation
-leads to hypothyroidism in most pts.
-consider in the elderly with Graves, pts with solitary toxic nodule, or those that are
refractory to meds.

Thyoid Storm
-rare, life-threatening complication of thyrotoxicosis look for AMS
-usually a precipitating factor: infection, DKA, or stress (surgery, childbirth for ex)
- Supportive therapy, PTU, iodine, betablockers, dexamethasone (impairs peripheral conversion to
T3 and supports adrenals)

Hypothyroidism
Random: Myxedema Coma – rare condition that presents with a depressed state of consciousness,
profound hypothermia, and respiratory depression. Thought to present after years of untreated
hypothyroidism that’s precipitated by a stress factor. Tx: medical emergency. Supportive. IV
thyroxine and hydrocortisone.
Causes:
-Primary:
-Hashimoto’s (chronic thyroiditis) MCC and a/w other AI diseases (e.g., lupus,
pernicious anemia)
- Type IV HSR: antiperoxidase abs (90%), antithyroglobulin abs, anti-
microsomal
-goiter is MC feature
-iatrogenic: surgery, radioiodine therapy, meds (lithium)
-Secondary/Tertiary: pituitary and hypothalamic disease respectively. Both have low TSH
and T4.
Clinical (other than the typical)
-menorrhagia
-hoarseness
-nonpitting edema (glycosaminoglycan in intersitial tissues)
-non tender goiter
Subclinical hypothyroidism: elevated TSH and normal T4, nonspecific hypothyroidism signs, treat if
thyroxine if symptomatic.
Labs: Other labs besides high TSH – elevated LDL and decreased HDL, mild normocytic anemia.

Thyroiditis
Subacute (viral) thyroiditis (subacute granulomatous thyroiditis)
-usually follows viral illness; a/w HLA-B35
-prodrometransient hyperthyroidismeuthyroidhypothyroid states
-painful thyroid gland
-Dx: radioiodine uptake is low. Low TSH to begin with, and high ESR
-Tx: NSAIDs for mild, corticosteroids for severe.
Subacute lymphocytic thyroiditis – transient. Similar to viral but painless
Hashimotos (see above)

Thyroid Cancer
Nodule – FNA is the initial test of choice and is reliable in all but follicular (encapsulated)
-Radioactive scans can be used when answer is indeterminate. Cold suggests malignancy.
-US  cystic and solid. Most cancer are solid
RFs:
-Head and neck radiation (during childhood)
-Gardner and Cowden syndrome for papillary cancer
-MEN 2 for medullatry.
Papillary:
-MCC. Least aggressive. Biggest RF is radiation. Lymph spread to cervical nodes commonly
-Tx: lobectomy with isthmusectomy or total
Follicular:
-hematogenous route so therefore distant mets can happen.
-may be a/w iodine def
-needs tissue biopsy to look for vascular or capsule invasion
-tx: total thyroidectomy with postop iodine ablation
-variant: Hurthle cell carcinoma – cells contain abundant cytoplasm, tightly packed
mitochondria, and oval nuclei with prominent nucleoli (orphan annie). Radioiodine resistant.
Medullary: parafollicular C cells – produces calcitonin. MEN2a + b
-total thyroidectomy with radical neck dissection when LN involvement (which is most of
the time)
Anaplastic: tx – chemoradiation and palliative surgery.

Pituitary Adenomas
Hormonal hypersecretion effects in order: Prolactin, GH, ACTH, TSH
Hypopituitarism: GH deficiency and hypogonadotropic hypogonadism are the MC problems
Clinical: Mass effect - HA and bitemporal hemianopsia
Dx: MRI is the study of choice. Also get hormone levels
Tx: Transphenoidal surgery in most pts (except prolactinomas, for which medical management
should be tried first)

Hyperprolactinoma
Gen: High levels of prolactin inhibit secretion of GnRH which leads to decreased FSH/LH and leads to
decreased production of estrogen/testosterone  hence the hypogonadotropic hypogonadism being
the MC problem.
Causes:
-Prolactinoma
-Meds (e.g., psychiatric meds, H2 blockers, metoclopramide, verapmis, estrogen)
-pregnancy
-renal failure
-suprasellar mass lesions (compressions)
-hypothyroidism
-idiopathic
Tx: 1. Treat underlying cause 2. Bromocriptine or cabergoline (less s/e) 3. Surgery

Acromegaly
-GH-secreting pituitary adenoma after epiphyseal closure (gigantism if before)
-Cardiomyopathy (hypertropic) is the MCC of death
-Non growth clinical features: DM and hyperhidrosis, parasellar manifestations, HTN, OSA
-30% also have hyperprolactinemia
Dx: IGF-1 (somatomedin C) is significantly elevated. Oral glucose suppression test fails to suppress
GH confirms if the IGF-1 level is equivocal
-MRI of pituitary
-don’t just do a random GH level
Tx: transphenoid resection
-radiation therapy if IGF levels remain elevated
-octreotide or other somatostatin analog to suppress GH secretion

Craniopharyngioma
-rathke’s pouch remnant
-suprasellar sx. May cause hyperprolactinemia, diabetes insipidus, or panhypopituitarism
-Dx by MRI (calcification of suprasellar region), treat is surgical w or w/o radiation

Hypopituitarism
Gen: loss of hormones are unpredictable but LH, FSH, and GH are usually lost first
Causes:
- hypothalamic or pit tumor MCC
- radiation, Sheehan syndrome, infiltrative (sarcoidosis, hemochromatosis), head trauma,
cavernous sinus thrombosis, surgery
Clinical:
-GH: growth failure (decreased muscle mass in adults), increased LDL
-Prl: failure to lactate
-ACTH: adrenal insufficiency
-TSH: hypothyroidism
-Gonadotropins: infertility, amenorrhea, loss of secondary sex, loss of libido
-ADH (hypothalamic lesion): DI
-MSH: decreased skin and hair pigmentation
Dx: low levels of target hormones and normal/low trophic hormones; MRI may help
Tx: replace

Diabetes Insipidus
Types:
Central DI is most common – low ADH secretion by posterior pituitary
-mostly idiopathic, trauma, things affecting hypothalamus
Nephrogenic DI
-MCC is chronic lithium use. Congenital mutation in ADH receptor gene or the
aquaporin-2 gene.
Clinical: Colorless polyuria (5-15 L a day), polydipsia, mild hypernatremia
Dx:
-Urine: low specific gravity and low osmolality indicate DI
-Serum Osmolality
-nml: 250-290 mOsm/kg
-primary polydipsia: 255-280
-DI: 290-310
-***water deprivation test is required
-Normal patients and polydipsia patients will have an increase in urine osmolality
after dehydration without further response to ADH
-Central DI pts will not have an increase in urine osmolality after dehydration but
will have a response to ADH
-Nephrogenic DI will not respond to dehydration or ADH
Tx:
Central – DDAVP. Chlorpropamide also increases ADH and enhances effect
Nephrogenic – sodium restriction and thiazide diuretics.
-depletion of sodium leads to increased reabsorption in the proximal tubules which
means that less water reaches the distal tubules, leading to decreased urine volume.

Syndrome of Inappropriate Secretion of Antidiuretic Hormone (SIADH)


Pathophysiology: Excess ADH from the posterior pituitary or an ectopic source  leading to water
retention and excretion of concentrated urine leading to hyponatremia and volume expansion 
despite the volume expansion, edema is not seen because of natriuresis occurs despite
hyponatremia (so volume expansion correction wins out)
-volume expansion increases atrial natriuretic peptide
-volume expansion leads to decrease proximal tubule Na absorption
-RAA system is inhibited
Hyponatremia pearls
-hypovolemic hyponatremia – volume contracted
-hypervolemic hypernatremia – volume expaned with edema (CHF, cirrhosis for ex)
-SIADH – volume expanded without edema
Causes: many random ass ones like neoplasms, CNS disorders, pulm disorders, etc
Clinical features: acute hyponatremia
leads to brain swelling and neuro disorders like seizures, coma, death. Chronic can be asymptomatic
or just have N/V because the brain adjusts via chronic loss of Na and K to decrease edema.
Dx: Dx of exclusion – hyponatremia, volume expansion without edema, natiuresis, hypouricemia and
low BUN, normal Cr, normal thyroid and adrenal function.
Tx:
-underlying cause
-asymptomatic ---water restriction
-symptomatic
-restrict water and NS
-hypertonic saline in severe cases but be careful cuz of CPM!!!!!
-Na replacement should not exceed 0.5mEq/L per hour

Hypopituitarism
Clinical: cardiac arrhythmias, rickets/osteomalacia, NM irritability d/t hypocalcemia (hyperreflexive,
facial nerve tap, BP cuff inflation, grand mal seizures), prolonged QT interval, cataracts
Dx: low serum calcium, high serum phosphate, serum PTH inappropriately low, low urine cAMP
Tx: IV calcium gluconate in severe cases, oral calcium in less severe
-vitamin D supplementation

Primary Hyperparathyroidism
If PTH is normal with high calcium, its still primary hyperparathyroidism
Clinical:
-Stones: nephrolithiasis, nephrocalcinosis
-bones: bone aches and pains, osteitis fibrosa cystic
-groans: muscle pain and weakness, panreatitis, PUD, gout, constipation
-Psych overtones: depression, fatigue, anorexia, sleep disturbances, anxiety, lethargy
-other: polydipsia, polyuria, HTN, shortened QT interval, weight loss
Dx:
Primary: PTH levels (note that in presence of hypercalcemia, a nml PTH is abnormal), low
phosphate, hypercalciuria, urine cAMP, chloride/phosphorus is >33 is diagnostic.
Secondary: high PTH and a low or low-nml serum calcium. d/t renal failure or vit d def
Tx: surgery. (and diuretics if severe hypercalcemia)

Cushing Syndrome
Gen: Syndrome – any cause of excess glucocorticosteroids. Disease – pituitary adenoma
Clinical:
-nonspecific: obesity, HTN, osteoporosis, DM
-more specific: easy bruising, ab striae, myopathy, and virilizing. Appearance (central
obesity, hirsutism, moon facies, buffalo hump, ab striae, lanugo hair, acne, easy bruising)
-androgen excess manifesting as masculinization in females is only seen in ACTH-depn
forms (i.e. not exogenous steroids)
-asceptic necrosis of femoral head with exogenous steroids
-infections more likely due to impaired immunity
- Pts with pituitary tumor causing cushings will also have the hyperpigmentation (elevated
ACTH to MSH) but pts with other types of cushings will NOT have hyperpigmentation
Dx
Figure 11

Tx: taper steroids or surgery

Pheochromocytoma
MEN Syndrome: AD with incomplete penetrance
1 – Parathyroid, panc, pituitary tumors
2a – medullary thyroid, pheo, hyperPTH
2b – medullary thyroid, mucosal neuromas, marafanoid, pheo
Gen – most common extra-adrenal site is the organ of Zuckerkandl (aortic bifurcation)
- arise from chromaffin cells in the adrenal medulla or sympathetic cells extra-adrenal
Lab: associated findings: hyperglycemia, HLD, hypokalemia
Dx: urine VMA, HMA followed by CT
Tx: surgical resection with early ligation of venous drainage. But first do alpha blocker
(phenoxybenzamine) followed by beta blocker

Primary Hyperaldosterone
-always suspect in a hypertensive pt with hypokalemia (and metabolic alkalosis) who is NOT on a
diuretic. Most likely an adrenal adenoma; can be carcinoma or hyperplasia
-peripheral edema will be absent
**-adenoma is cured by adrenalectomy but hyperplasia (bilateral) is NOT cured by surgery.
Dx:
-initial test: plasma alodosterone/renin. Positive if aldo:renin ratio is over 30:1
-to find cause: adrenal venous sampling for aldosterone levels – one side = unilateral. two
sides = bilateral
- imaging
Tx: adenoma = surgery. Bilateral hyperplasia = spironolactone

Adrenal Insufficiency
Gen:
-secondary to chronic steroid use is the MCC worldwide. Usually after abrupt steroid
cessation or increased need after infection or surgery. Tx with steroids
- primary: Addison’s disease (idiopathic) is MCC in industrialized world and infectious
disease (TB is MCC).
-MC clinical findings
-weight loss
-weakness/anorexia
-pigmentation
-nausea
-postural hypotension
-abdominal pain
-hypoglycemia
-hyperpigmentation in and hyperkalemia/hyponatremia fr low aldo occus in primary only
-aldosterone is reliant on the RAA system, not ACTH. So since no mineralocorticoids
are produced, blood pressure will be low and thus RAA will be inactivated.
Dx: plasma ACTH level  high? primary low? Secondary.
Tx: Primary  daily po glucocorticoid and daily fludrocortisone (mineralocorticoid)
Secondary gluco but no fludro

Congential Adrenal Hyperplasia


1_ = HTN _1 = virilization
-AR disease. Usually 21-hydroxylase deficiency
-decreased cortisol and aldosterone leads to increased ACTH causing bilateral adrenal hyperplasia
-virilization in females: female infants are born with ambiguous external genitalia but normal
female ovaries and uterus. (men have no abnormalities at birth)
-21 def can be severe and salt wasting causing hypotension (with hyponatremia and hyperkalemia
also from lack of aldo). Shows up as emesis and shock early in life
Dx for 21 def: high levels of 17-hydroxyprogesterone
Tx: Medically – cortisol and mineralocorticoid to shut off excess ACTH via negative feedback
Surgically correct genital abnormalities early in life.

Diabetes Mellitus (random)


-Type 2 has a high concordance in twins so has a much stronger genetic component
-type 1 is a/w HLA-DQ/DR
-Dx of diabetes include one of the following
-2 fasting glucose greater than 125mg
-single glucose level over 200mg with symptoms
-increased glucose level on oral glucose tolerance test
-HGBA1C > 6.5
-2 hour post-prandial over 200mg
-screen all aduls over 45 q 3yrs with fasting glucose test
-BP goal in type 2 is less than 130/80 and LDL is under 100
-most oral hypoglycemic agents are contraindicated in pregnancy, use insulin instead.
-metformin is contraindicated in patient with renal failure (creatine over 1.5 in men, 1.4 in women)
-sliding scale regular insulin is used for control for in-patient people
-definitin of microalbuminuria: 30 to 300mg/day albumin-creatinine ratio of 0.02 to 0.20
-critical to intervene before micro turns to proteinuria to prevent ESRD
-hold metformin for 48hrs after radiocontrast to prevent renal damage
-key characteristics of prolferative retinopaty are new vessel formation and scarring that can lead to
vitreal hemorrhage and retinal detachment leading to blindness. Laser photocoagulation is the tx
-CN III involvement: remember that the pupils are spared
-increased susceptibility of infection is d/t impaired WBC function, reduced blood supply and
neuropathy
-Neuropathy tx: NSAIDs, TCAs, gabapentin. Gastroparesis – metoclopramide

DKA
Gen: can occur in both types but is more common in type 1. Severe hyperglycemia, often preciptiated
by stress, leads to an osmotic diuresis resulting in hyperglycemia, ketoemia, metabolic acidosis, and
volume depletion
Clinical features
-N/V, abdominal pain
-kussmaul respiration (rapid, deep breathing)
-fruity (acetone) breath
-volume depletion is always present
-polydipsia, polyuria, polyphagia, weakness
-AMS (drowsy to coma)
Dx:
-serum glocose usually 450-850mg. (vs alcoholic KA which can be euglycemic)
-HAGMA (required)
-ketonemia (required)
-potassium: initially high because of acidosis but as insulin is given a rapid hypokalemia can
occur.
Tx: Insulin, IVF, K ppx *ensure pt is NOT hypokalemic before insulin administration
Complications – cerebral edema if glucose drops too quickly (add some glucose in IVF when
its at about 250); hyperchloremic nongap metabolic acidosis d/t large IVF

Hyperosmolar hyperglycemic nonketotic syndrome


-typically elderly type 2 diabetics; CNS findings are common (seizures for ex)
-severe dehydration and osmotic diuresis
-Labs: glucose >900mg; hyperosmalarity >320; serum pH >7.3 (no acidosis)
-tx: aggressive IVF, low-dose insulin (add some glucose in IVF when its at about 250)

Hypoglycemia
Clinical: sx occur at a blood glucose level of 40-50mg, elevated epinephrine levels causing adrenergic
sx, and there’ll be neuroglycopenic sxs (lethargy, convulsions, etc)
Labs: measure glucose, plasma insulin, C-peptide, proinsulin
Insulinoma: high insulin, low glucose, high C-peptide, high proinsulin
Exogenous insulin: high insulin, low glu, low C-peptide
Sulfonylurea: high insulin, low glu, high C-peptide, normal proinsulin
Other: anti-insulin abs, plasma and urine sulfonylurea levels
Tx: glucose obviously. Po preferred if possible. If pt is an alcoholic, give thiamine first!
Insulinoma:
Dx: 72-hour fast and do above labs
Tx: resection 80-90% cure rate

Zollinger-Ellison Syndrome (Gastrinoma)


-90% are in the gastrinoma triangle: formed by cystic duct superiorly, junction of second and third
portions of the duodenum inferiorly, and neck of the pancreas medially.
-complications: GI hemorrhage, perforation, or obstructure. Mets to the liver
-Secretin injuection test is diagnostic test of choice. (normal response is for gastrin to be inhibits, but
in ZES the gastrin increases substantially)
-Tx: high dose PPI and attempt resection

Glucagonoma
-clinical: necrotizing migratory erythema (usually below the waist), glossitis, stomatitis,
hyperglycemia
-tx: resect

Somatostatinoma – classic triad: gallstones, diabetes, steatorrhea

VIPoma – watery diarrhea (leading to dehydration, hypoK, acidosis), achlorhydria (VIP inhibits
gastric acid secretion), hyperglycemia, and hypercalcemia). Tx is resection

CNS and PNS Diseases

Ischemic Stroke
Gen:
-the most important risk factors are age and HTN.
-TIAs can involve either the carotid or the vertebrobasilar system
-Lacunar stroke – small vessel thrombotic disease is d/t thickening of vessel walls not by
thrombosis
(Subclavian Steal Syndrome:
-caused by stenosis of subclavian artery proximal to origin of vertebral artery – exercise of
left arm causes the ipsilateral vertebral to supply the subclavian instead.
-leads to decreased cerebral blood flow and symptoms of vertebrobasilar arterial
insufficiency
-BP is left arm is less than in right arm; decrease in pulse in left arm; UE claudication
-tx: surgical bypass)

Clinical:
-ACA – contralateral LE and face
-MCA – aphasia, contralateral hemiparesis
-Vertebral/basilar – ipsilateral: ataxia, diplopia, dysphagia, dysarthria, and vertigo
contralateral: homonymous hemianopsia with basilar –PCA lesions
-Lacunar
-internal capsule: pure motor hemiparesis
-pons: dysarthria, clumsy hand
-thalamus: pure sensory deficit.
Dx: 1st – CT without contrast. 2nd – MRI. Supportive – ECG, Carotid duplex but MRA is the definitive
for stenosis of the head and neck.
Tx:
-acute:
-ABCs
-tPA if within 3 hours and no obvious bleed risk.
-don’t give aspirin for the first 24 hrs
-aspirin if over 3 hrs and after 24hrs post-tpa. (or give clopidogrel or ticlopidine)
-Don’t give anticoagulants after stroke in the acute setting
-no hypertensives unless very high and/or controlling post-TPA
-goal is to prevent future strokes: CEA if symptomatic and >70% occlusion

Intracerebral hemorrhage
Gen:
-HTN (particularly a sudden increase in BP) is the MCC
-other: amyloid angiopathy, drugs (cocaine and anticoags), brain tumors, AV malformation
-usually located in the basal ganglia
Clinical: Abrupt onset of a FND that worsens steadily over 30mins-90mins, AMS, HA/vomiting, signs
of increase ICP
-pinpoint pupils: pons
-poorly reactive pupils: thalamus
-dilated pupils: putamen
Dx: Noncontrast CT
Complications: increased ICP, seizures, rebleeding, vasospasm, hydrocephalus, SIADH
Tx: Gradual BP reduction (do not cause hypotension and reduce cerebal blood flow)
-if BP is >160/105

Subarachnoid Hemorrhage
Common sites:
-junction of anterior communicating artery with anterior cerebral artery
-junction of posterior communicating artery with the internal carotid
-bifucation of the MCA
Clinical: Thunderclap HA, sudden transient LOC, vomiting, meningeal irritation, death, retinal
hemorrhages.
Dx: Noncontrast CT. LP (diagnostic) if negative – blood in the CSF and xanthochromia (fr RBC lysis)
-If papilledema is present, do NOT perform LP cuz may cause a herniation
-Once diagnosed, order a cerebral angiogram to detect site of bleeding for surgical clipping.
Complications: seizures, rebleeding, vasospasm, hydrocephalus (communicating), SIADH
Tx: Surgical consult for clipping
-stool softeners, analgesia, IVF, lower BP gradually, CCB (nifedipine) for vasospasm

Parkinson Disease
Gen: loss of dopamine-containing neurons in substantia nigra AND locus ceruleus
Tx:
-Carbidopa-Levodopa is drug of choice
-S/E: dyskinesias (chorea) after 5-7 years
-shows an “on and off” effect later in the disease
-Dopamine-receptor agonists (bromocriptine, pramipexole). Good for early on
-Selegiline: MAOB inhibitor which increases dopamine activity. Early and adjuvent
-Amantadine: mild benefir, eary disease
-Anticholinergics (trihexyphenidyl and benztropine)
-particularly helpful tremor. Don’t use in older pts or demented
-Amitriptyline
-DBS if not response or severe disease before 40
Meds that cause PD: Neuroleptic drugs (chlorpromazine, haloperidol, perphenazine, reserpine)
Shy-Drager syndrome = parkinsonian sxs + autonomic insufficiency

Progressive Supranuclear Palsy


-like PD, PSP causes progressive bradykinesia, limb rigidity, cognitive decline but does NOT cause
tremor and DOES cause ophthalmoplegia
Tourette Syndrome
- a/w with OCD
-onset before 21yo
-tx: clonidine, pimozide, haloperidol

Dementia vs. Deliruim


Figure 12

-Since pts with alzheimer’s already have low acetylcholine, you must avoid anticholinergics!
-Dementia with lewy bodies initially have visual hallucinations then EPS features and fluctuating
mental status. Very sensitive to neuroleptic agents’ side effects aka anti-psych aka anti-dopamine.
However, neuroleptics are used for hallucinations and psychotic features.
-Pick’s disease (frontotemporal dementia) has severe personality changes.
-Perform an LP in any febrile, delerious patient unless there are contraindications (e.g. cerebral
edema)

Multiple Sclerosis
Gen:
-classic location of plaques is at the angles of the lateral ventricles
-demyelination of white matter
-must have 2 episodes of symptoms for diagnosis
Clinical:
-transient sensory deficit (MC initial presentation)
-fatigue
-motor symptoms (pyramidal tract involvement)
-visual disturbances
-Optic neuritis: monocular vision loss and painful movement, central scotoma,
decreased pupillary reaction
-Internuclear ophthalmoplegia
-lesion in the MLF results in ipsilateral medial rectus palsy on attempted
lateral gaze and horizontal nystagmus of abducting eye (contralateral to lesion)
-cerebellar involvement
-loss of bladder control
-autonomic
-cerebral involvement
-neuropathic pain (commonly hyperesthesia and trigeminal neuralgia)
Dx:
-clinical: 2 episodes
-MRI
-CSF with oligoclonal bands of IgG
-abnormal evoked potentials
Tx:
Acute
-High-dose IV corticosteroids
Dmards: recombinant beta-1a and beta-1b, and glatiramer acetate can be helpful if started
early in the course
Symptomatic: baclofen or dantrolene for muscle spasticity, carbamezapine or gabapentin for
neuropathic pain

Guillain-Barre Syndrome (AIDP)


Gen:
-primarily affects motor nerves (but not only motor)
-usually preceded by a viral or mycoplasmal infxn of URI or GI (esp: campy, CMV, hep, HIV).
Clincal:
-abrupt ascending weakness/paralysis of all 4 extremities. Can lead to respiratory arrest.
-areflexia
-autonomic features (arrhytmias, tachy, hypotension) are dangerous complications
Dx: CSF – elevated protein, but normal cell count. Also decreased motor NCS
Tx: supportive  IVIG/PLEX. Never give steroids.
Fischer-variant: ophthalmoplegia, areflexia, with or without ataxia and/or weakness.

Myasthenia Gravis
Gen: Autoantibodies against nicotinic acetylcholine receptors at the NMJ. Muscles stimulated freq go
weak. Younger women and older men.
Clinical: Proximal fatigue as muscles are used more. Improved with rest. (vs. Lambert-Eaton which is
abs against presynaptic calcium channels and sxs improve with repeated stimulation)
Dx: AchR abs, EMG (decremental response), thymoma on CT, tensilon test.
Tx: AchE, thymectomy, azathioprine or cyclorsporine, plex/ivig, low threshold for intubation (FVC of
15)
Meds that exacerbate sx: aminoglycosides and tetracyclines, beta-blockers, quinidine and
procainamide and lidocaine.

Duchenne Muscular Dystrophy


Gen: XLR. No inflammation. Pseudohypertrophy of calves
Dx: CPK  DNA testing (no longer bx)
Tx: Prednisone
Becker MD is the exact same but some dystrophin is present

VHL – AD. Cavernous hemangiomas of the brain, renal angiomas, and cysts in multiple organs. a/w
RCC and pheos.

Syringomyelia
-MC a/w arnold-chiari malformation
-sx may include bilateral loss of pain and temp over the shoulder (cape-like) but preservation of
touch
-dx via MRI
-tx depns on size (surgery)

Brown-Sequard Syndrome
-spinal hemisection usually at cervical region
-usually caused by trauma/crush injury of spinal cord
-contralateral loss of pain and temp, ipsilateral hemiparesis, and ipsilateral
position/vibration

Transverse Myelitis
-usually thoracic horizontal aspect
-cause is usually unknown, can be after viral infxns, progression is rapid
-LE weakness or plegia, back pain, sensory deficits below the level of the lesion, urinary retention (or
other sphincter issues)
-Dx with MRI
-Tx: high-dose steroid

Horner’s Syndrome – Causes – idiopathic, pancoast, internal carotid dissection, brainstem stroke,
neck trauma

Vertigo
Peripheral vs Central: Peripheral is more sudden, severe, a/w N/V, intense nystagmus (vertical only),
no associated FNDs.
Central: tumor, CVA, MS, vertebrobasilar insufficiency, migraine-associated vertigo
Peripheral:
-BPV: Tx with meclizine or epley maneuver. Dx with dix-hallpike
-Meniere: vertigo, tinnitus, and hearing loss. Tx with sodium restriction and duretics
-Acute Labrynthitis: viral. Several days
-Ototoxic drugs: aminoglycosides, some loop diuretics
-acoutic neuroma (schwannoma) of 8th CN: ataxia, gait unsteadiness, nystagmus, hearing
loss, tinnitus.

Seizures
-if pt is unknown, immediate check calcium, sodium, glucose, and BUN
-status epilepticus: treat with IV diazepam
-anticonvulsants are teratogenic. Always do preg test before prescribing
Figure 13

ALS
Hallmark: upper and lower motor neuron signs
Unaffected: bowel/bladder, sensation, cognition, extraocular muscles, sexual fxn.
Dx is helped with clinical or electrical evidence of 2+ regional involvement.
Tx is mainly supportive. Riluzole is a glutamate-blocking agent that may prolong life.

Aphasia
Conduction – disturbance in repetitition
Global – all areas. Often a/w a right hemiparesis

Bell’s Palsy
-unilateral, both upper and lower parts of the face.
-steroids is the treatment, EXCEPT IF LYME IS SUSPECTED
-bilateral bell’s is found in GBS

Trigeminal Neuralgia
Dx: Clinical. MRI to r/o CPA tumor
Tx: carbamezapine

Localizing the lesion


Cerebral cortex – contralateral motor and sensory
Subcortical – hemiparesis is usually complete
Cerebellar – incoordination, intention tremor, atazia
Brainstem – specific CN findings, crossed hemiplegia (ipsi face and contra body)
Spinal – level of lesion corresponds to the sensory level
Plexus – motor and sensory involve more than one nerve; brachial and lumbosacral
Roots (radiculopathy) – pain is the key finding
Peripheral nerve – weakness is worse distally; usually asymmetric
NMJ – fatigability. Normal sensation. No atrophy
Myopathy – acquried (vs dystrophy). Symmetric if proximal. Normal reflex, normal sensation, no
fasciculation. Atrophy and diminished reflexes late in course.

Connective Tissue and Joint Diseases

SLE
Types:
-Spontaneous SLE
-Discoid lupus (skin lesions without systemic disease)
-drug-induced
-antihistone abs. no anti-sm or dsdna
-similar to SLE but does NOT affect CNS or kidneys and skin is usually not seen
-agents: hydralazine, procainamide, isoniazid, chlopromazine, methyldopa, and
quinidine
-ANA-negative lupus
-arthritis, raynauds, subacute cutaneous lupus
-serology: Ro (anti-SSA) positive, ANA negative
-risk of neonatal lupus in infants of affect women
-Neonatal lupus
-skin lesions, cardiac (AV block, transposition of great vessels), valvular and septal
defects)
-Lupus anticoagulant: clotting; recurrent miscarriages. Lupus anticoagulant positive (yes I
said anticoagulant) and/or anticardiolipin. Prolonged PTT or PT that is NOT corrected by adding
normal plasma.
-treat with long term anticoagulant (despite prolonged labs)
Dx: 4 or more present at any time
-mucocutaneous signs (each count as one)
-butteryfly rash
-photosensitivity
-oral or nasopharyngeal ulcers
-discoid rash
-arthritis
-pericarditis, pleuritis
-hematologic: basically pancytopenia
-renal disease: proteinuria >0.5g/day, cellular casts
-CNS – seizures, psychosis
-immunologic: positive LE prep, false-positive test result for syphillis (RPR), anti-dsDNA,
anti-SM Ab (either is diagnostic)
-ANA
Tx:
-avoid sun exposure
-NSAIDs for less severe sx
-steroids for acute exacerbations
-long term: Hydroxychloroquine (annual eye exam needed because of retinal toxicity)
-glomerulonephritis: cyclophosphamide

Scleroderma (diffuse and limited)


Clinical:
- Raynaud Phenomenon
-usually initial sx in both types
-sclerodactyly
-GI findings like dysphagia in both
-pulmonary is MCCOD
-pulm fibrosis is more common in diffuse
-heart and renal
-CREST is found in limited
Calcinosis of digits, raynaud, esophageal dysmotility, sclerodactyly, telangiectases
(over fingers and under nails)
Dx: almost all have ANA
-Limited = anticentromere ab
-Diffuse = antitopoisomerase ab
Tx: symptomatic

Sjogren Syndrome
Gen:
-in patients with SS, search for occult non-hodgkins lymphoma (LAD or HSM)
-primary is SS alone, secondary is with another connective tissue disease
Clinical: dry eyes, dry mouth and tooth decay, arthralgias, arthritis, fatigue, interstitial nephritis and
vasculitis
Dx: ANA and RF can be present
-Anti Ro/La aka SSA/SSB
-risk of neonatal lupus with anti ssa
-Schirmer test: filter paper inserted in eye to measure lacrimal output
-salivary gland is most accurate but no necessary
Tx: Pilocarpine or Cevmeline for ocular secretions (anti-cholinesterase inhibitors, increase ACH)
-otherwise symptomatic tx

Mixed Connective Disease


-overlap syndrome with clinical features similar to SLE, RA, systemic sclerosis, and polymyositis
-anti-U1-RNP antibodies

Rheumatoid Arthritis
-can involve any joint except DIP
-cervical spine is common at C1-2; subluxation and instability.
-all RA pts should have cervical spine xrays before any surgery
-Labs: RF (titers correlate with disease severity), anticitrullinated peptide/protein antibodies
-Dx: arthritis of 3 or more joints, elevated CRP and ESR, sx lasting at least 6 weeks, positive serum
ACPA or RF, radiographic changes consistent with RA (erosions and periarticular decalcification)
-Tx: NSAIDs, corticosteroids for exacerbation = pain relief. DMARDs for long term.
-start DMARDs immediately. They take about 6 weeks so bridge with NSAIDs and then
remove NSAIDs.
-best is MTX
-S/E: gi upset, bone marrow suppression (coadminister folinic acid)
-others: leflunomide, hydroxychloroquine, sulfasalazine, anti-TNF (etanercept, infliximab)
figure 14

Gout
Pathogenesis: PMNs phagocytize monosodium urate crystals and release inflammatory mediators
and proteolytic enzymes causing inflammation.
Increased production
-HGPRT deficiencies – Lesch-Nyhan syndrome
-PRPP overactivity
-increase cell turnover states
Decreased excretion (most cases)
-renal disease
-NSAIDs, diuretics, acidosis
-Avoid thiazide and loop diuretics
Dx: Joint aspiration and synovial fluid analysis – needle-shaped and negatively birefringent urate
crystals.
Tx:
-acute
-NSAIDs (indomethacin is usually picked)
-avoid aspirin and acetaminophen
-Colchicine if they can’t take NSAIDs
-Prednisone is 3rd line
-PPx
-wait till patient has had >2 attacks
-uricosuric drugs (probenecid, sulfinpyrazone) if 24-hr urine uric acid is under
800mg because this indicated undersecretion. Contraindicated if hx of renal stones or renal
dysfunction
-allopurinol (xanthine oxidase inhibitor) decreases uric acid synthesis. Use if
excretion is more than 800mg. never use in acute gout. Watch for SJS
Pseudogout (Calcium Pyrophosphate Deposition Disease)
-increase risk with age and OA of the joints. a/w hemochromatosis and hyperparathyroidism.
-aspirate: weakly positively birefringent, rod-shaped and rhomboid crystals.
-acute tx is same as gout. No real chronic treatment

Inclusion Body Myositis


-Insidious onset with progressive proximal AND distal weakness. Asymmetrical
-also lose deep tendon reflexes
-no real labs or antibodies

Polymyalgia Rheumatica
-elderly patients. a/w temporal arteritis
-hip and shoulder muscle pain, constitutional, joint swelling.
-Dx: ESR elevated. Clinical dx
-Tx: Corticosteroids.

Fibromyalgia
-1st line tx is amitriptyline

Ankylosing Spondylitis
-stong a/w HLA-B27. Negative RF
-bilateral sacroiliitis is a prereq for dx
-LBP and stiffness improve with activity, ethesitis, prone to spinal fractures
-other manifestations: anterior uveitis, AV heart block, aortic insufficiency are the important ones.
-complications: restrictive lung dz, cauda equina, spinal fx, osteoporosis, spondylodiscitis
-tx: NSAIDs, anti-TNF

Reactive Arthritis/Reiter Syndrome


-don’t have to have the classic triad
-common orgs: salmonella, shigella, campylobacter, chlamydia, yersinia
-asx polyarthritis
-no specific test. r/o other shit with synovial analysis
-Tx: 1st line – NSAIDs. 2nd – Sulfasalazine, azathioprine

Psoriatic Arthritis
-asx and polyarticular, sausage digits, nail pitting. Smaller joints more common.
-1st – NSAIDs. 2nd – MTX or anti-tnf

Vasculities
Large vessel – Takayasu arteritis, temporal arteritis
Med – PAN, Kawasaki, Wegner, Churg-Strauss, microscopic polyangiiitis
Small – HSP, hypersensitivity vasculitis, Behcet syndrome

Temporal/Giant Cell Arteritis


-granulomatous
-a/w polymyalgia rheumatica and aortic aneurysm and dissection
-HA, vision (ophth artery involvement), jaw
-dx: ESR. Bx confirms
-Tx: start prednisone immediately. Monitor ESR for improvement

Takayasu Arteritis
-young asian woman, decreased/absent peripher pulses, BP discrepancies, arterial bruits
-granulomatous vasculitis of aortic arch and its major branches
-Dx: arteriogram
-Tx: steroids first, treat HTN, surgery may be required to recannulate stenosed vessels.

Churg-Strauss Syndrome
-asthma, skin lesions, eosinophilia
-a/w p-ANCA
-tx: steroids

We(C)gner’s Granulomatosis
-sinusitis, pulm, renal (glomerulonephritis)
-Dx: CXR, elevated ESR, anemia, hematuria, C-anca. Lung bx confirms
-tx: cyclophosphamide and sterodis. (rituximab may work also)

Polyarteritis Nodosa
-nervous system and GI. NO pulm
-a/w hepatitis B, HIV, and drug reactions.
-PMN invasion of all layers and fibrinoid necrosis leading reduced luminal area causing ischemia
-necrosis is segmented leading to rosary sign as a result of aneurysms
-Dx: bx or mesenteric angiography. ESR elevated, P-anca may be present
-Tx: initial  steroids. Severe  add cyclophosphamide

Behcet Syndrome
-painful oral and genital ulcers, arthritis, eye involvement, CNS
-often in middle easteners
-dx is bx. Tx is steroids

Buerger Disease (Thomboangiitis Obliterans)


-young male smokers
-segmental inflammation that may lead to digit gangrene and autoamputation
-tx: smoking cessation

Hypersensitivty Vasculitis
-response to a drug, infection, or other
-skin lesions (palpable purpura, vesicles on LE)
-dx is bx. Tx is withdrawal of offender and steroids.

Renal and GU diseases

AKI – random
-whenever a pt has elevated creatinine levels, the first step is to determine the baseline Cr
-oliguria is ALWAYS found in prerenal failure
-FENa is most useful in oliguria ( <1% = prerenal; >2-3% ATN)
-rhabdo tx: IVF, mannitol, and bicarb (for hyperkalemia)
-postrenal failure: both kidneys must be obstructive for Cr to rise.
-UA:
-prerenal = hyaline casts
-muddy brown = ATN
-acute glomerulonephritis = RBC casts, protein and blood
-AIN: RBCs, WBCs with casts, eosinophils

CKD – random
-defined as >3 months
-ESRD is not defined by BUN/Cr; it is defined by lab and clinical findings of uremia.
-Uremia: Neuro dysfunction (AMS, neuropathy, seizures etc), GI (N/V, anorexia), pericarditis
(absolute indication for dialysis), and platelet dysfunction
-pts may show restless legs – pain only relieved by moving legs (neuropathic)
-correct hyperphosphatemia with calcium citrate (a phosphate binder)
-intoxications that can be dialyzed: methanol, ethylene glycol, lithium, aspirin, magnesium-containing
laxatives

Proteinuria
Nephrotic syndrome
-key features: urine protein ( >3.5g/day), hypoalbuminemia and edema, hyperlipidemia,
hypercoagulable state
-dx: dipstick  UA (fatty casts)  24 urine collection or albumin/Cr ratio

Hematuria
-defined as >3 erythrocytes/HPF on UA
-gross painless hematuria = bladder or renal cancer until proven otherwise
-if microscopic, think glomerular dz
-initial test: urine dipstick and UA
-Nephritic syndrome: hematuria, HTN, azotemia

Primary Glomerular Diseases


Minimal Change Dz
-nephrotic syndrome
-MC in kids – lymphoma has been a/w it
-fusion of foot processes on EM
-tx: steroids

Focal segmental glomerulosclerosis


-nephrotic
-more common in minorities. Hematuria and HTN often present
-resistent to steroids alone

MemBRANous glomerulonephritis
-MCC in adults
-spike and dome on EM
-can be due to Hep B and others

IgA Nephropathy
-asx recurrent hematuria/mild proteinuria. MCC of glomerular hematuria. Gross
hematuria after URI or exercise is common
-mesangial IgA and C3 on EM and IF

Hereditary Nephritis (Alport syndrome)


-XL or AD with variable penetrance
-hematuria, pyuria, proteinuria, high-frequency hearing loss without deafness,
progressive renal failure

Secondary Glomerular DOs


Diabetic nephropathy – MCC of ESRD
HTN nephropathy
Lupus
Membranoproliferative GN
-Usually due to hep c
-common a/w cryoglobulinemia
PSGN (PIGN)
-MCC of nephritic syndrome
-GAS infection of URT or impetigo
-coca-cola urine, HTN, edema, proteinuria
-self limited; tx is supportive
Goodpasture Syndrome
-triad: proliferative crescentric GN, pulm hemorrhage, and IgG antiglomerular BM
antibody.
-lung disease usually precedes renal
-linear IF pattern
-treat with plasmapheresis to remove antibodies. Cyclophosphamide and
steroids can decrease formation of new abs
Dysproteinemias – amyloidosis, light/heavy chain dz
Sickle Cell Nephropathy
HIV Nephropathy
-proteinuria, edema, and hematuria
-Histo often resembles a collapsing form of FSGS
-tx: prednisone, ACEI, and HAART
Wegner’s
PAN

Acute Interstitial Nephritis


Causes:
-acute allergic rxn to meds is MCC: pens, cephalos, sulfas, diuretics, anticoagulants,
phenytoin, rifampin, allopurinol, PPIs
-infection (esp kids): streptococcus and legionella
-collagen vascular diseases and AI diseases
Clinical: AKI with fever rash and eosinophilia; pyuria and hematuria may be present
Dx: UA – looks for eosinophils
Tx: remove agent, treat infection etc

Renal Papillary Necrosis


-most commonly a/w analgesic nephropathy (NSAIDS), diabetes, sickle cell, UT obstruction, UTI,
alcoholism, and renal transplant rejection
-Dx: excretory urogram – note change in papilla or medulla
- treat underlying cause or remove agent

Renal Tubular Acidosis – leads to NAGMA (hypochloremic)


1 (distal)
-inability to secrete H+
-urine pH >6
-hypokalemia
-nephrolithiasis and nephrocalcinosis DO occur
-tx: sodium bicarb for acidosis and phosphate salts
2(proximal)
-inability to reabsorb HCO3
-caused by fanconi’s in kids; MM in adults
-so increase HCO3 excretion
-nephrolithiasis and nephrocalcinosis DO NOT occur
-tx: underlying disease (do NOT give bicarb cuz you can reabsorb)
4 (hyperaldosteronism) – still NAGMA surprisingly
-decreased Na absorption and H and K secretion in distal tubule
-results in hyperkalemia and acidic urine (so alkalosis)
- ephrolithiasis and nephrocalcinosis rare

Hartnup Syndrome
-AR inheritence of defective neutral amino acid transporter
-tryptophan for example, causing nicotinamide def
-leads to similar features as pellagra (niacin def): dermatitis, diarrhea, ataxia, and psych disturbance
-give supplemental nicotinamide

Fanconi Syndrome
-acquired proximal tubule dysfunction that leads to defective transport of glucose, AA, Na, K, PO4, UA,
HCO3 – leading to rickets in kids, RTA 2
-treat with supplementation and hydration

ADPKD
-AD or AR will correlate with severity and age of onset
-ADPKD: pain, hematuria, infection, HTN, stones, berry aneurysm, MVP, other cysts
-Dx: US is confirmatory
-Tx: drain if symptomatic and treat complications
-ARPKD: infants usually
-hepatic fibrosis is always present
-pulmonary hypoplasia or full-on hydramnios and Potter sequence
-dx is US. If survive treat with respiratory management and renal replacement therapy

Medullary Sponge Kidney


-characterized by cystic dilation of the collecting ducts
-may present with hematuria, UTIs, or nephrolithiasis, or asx
-may be a/w hyperparathyroidism (adenoma)
-Dx: IVP
-Tx: treat stones and infections

Renal Artery Stenosis (Renovascular HTN)


Gen: MCC of secondary HTN. Stenosis causes activation of RAA. Stenosis can be d/t atherosclerosis
(MCC) or fibromuscular dysplasia in young females
Clinical: Sudden, often severe HTN that is refractory to medical management. Abdominal bruit in
fibromuscular.
Dx: Renal arteriogram is diagnostic but do not use if renal railure
-RF  MRA
Tx: Initial – revascularization with percutaneous transluminal renal angioplasty (PRTA) – best for
fibromuscular dysplasia. Surgery if unsucessful (bypass)

Sickle Cell Nephropathy


-sickling in the microvasculature leading to infarction. Occurs mostly in the renal papilla and can
lead to renal papillary necrosis, RF, and high frequencies UTIs.
-ACEI may be helpful

Nephrolithiasis
Gen:
-ureterovesicular junction is the MC site of impaction.
-diseases that precipitate: gout, crohn’s, hyperPTH, type 1 RTA
-Meds: loop diuretics, acetazolamide, antacids, chemo (Uric acid stones)
Types:
-calcium oxalate: radiodense
-uric acid: radiolucent and cannot be seen on ab radiograph – require CT, US, IVP
-urine pH under 5.5 promotes
-gout or chemo
-struvite (staghorn): radiodense rectangular prisms
-recurrent UTIs with urease-producers (Proteus, klebsiells, serratia, enterobacter)
-facilitated by alkaline urine – creates ammonium magnesium phosphate stones
-cysteine: radiolucent
- seen in the AR disease, cystinuria, hexagon-shaped crystals (UA)
Clinical: Sunden colicky flank pain that radiates to the groin and UA with hematuria. Cannot sit still.
Dx: CT without contrast aka spiral CT is gold standard for all types of stones
-renal US in preggers
Tx:
-most stones: analgesia and vigorous fluids
-Extracorporeal shock wave lithotripsy if pain isn’t controlled
-best for stones 5mm-2cm
-Percutaneous nephrolithotomy if lithotripsy fails
-best for >2cm
-prevention
-hydration and avoiding purines (red meat)
-thiazide diuretics reduce urinary calcium, esp in pts with hypercalcemia
-allopurinol in pts with UA stones

Prostate Cancer
Dx: If PSA is over 10 OR DRE is abnormal, transrectal ultrasound (TRUS) with biopsy is indicated
regardless of if the other test is normal; or if PSA has risen >0.75 per year (PSA velocity)
-carcinoma is hard, nodular, and irregular on DRE
Tx:
-disease in a pt that has less than 10 years  observation
-localized disease  radical prostatectomy
-complication: ED and urinary incontinence
-locally invasive: give radiation therapy + androgen deprivation (see below)
-Mets: reduce testosterone with
-orchiectomy if pt is non-compliant with meds
-anti-androgens (flutamide)
-leuprolide (LH releasing hormone against
-GnRH antagonists (Degarelix)

Renal Cell Carcinoma


Gen: Tumor thrombus invades renal vein or IVC and spread hematogenously to lung, liver, brain,
bone
Rfs: smoking, phenacetin analgesics, ADPKD, chronic diaylsis (multicystic dz develops), exposure to
heavy metals (mercury, cadmium), HTN
Clinical: triad – hematuria, flank/ab pain, flank/ab mass. Constitutional sx.
-paraneoplastic: EPO secreting – PV; PTHrP – hypercalcemia; renin – HTN; cortisol –
cushings; or gonadotropins – feminization or masculinization.
DX: Renal US. Then CT for staging.
Tx: radical nephrectomy (+adrenal gland is radical) for all

Bladder Cancer
Gen: 90% are transitional cell. Local excision is the most common route of spread. Likely to recur
after removal.
RFs: smoking, industrial carcinogens (aniline dye, azo dyes), long-term tx with cyclophosphamide
Clinical: painless hematuria
Dx: UA and culture to r/o infection. Then, urine cytology. Cystoscopy and bx (definitive). Then stage
Tx:
-CIS: intravesical chemo
A (lamina propria): transurethral resection
B (muscle invasion): radical cystectomy, LN dissection, removal of prostate/uterus/ovaries/
anterior vag wall, and urinary diversion (e.g., ileal conduit)
C (perivesicular fat): same as B
D (mets): cystectomy and chemo
Testicular Cancer
Gen: more common 20-35. Germ cells is most common (seminatous and non-seminomatous).
Nongermcells are usually benign.
RFs: cryptorchidism (surgical correction reduces but doesn’t eliminate risk); klinefelter
Dx: PE, US (initial), tumor markers, staging
Tx:
-if suspected based off PE and US, remove to confirm dx. Do the CT and tumor markers after
removal to compare.
-seminoma  add radiation
-nonseminomatous  add retroperitoneal LND with or w/o chemo

Penile Cancer
-a/w HSV and HPV18
-presents as exophytic mass and treatment is local excision

Testicular Torsion
-will be in a higher position comparatively plus acute pain
-surgical emergency  detorsion and orchipexia bilaterally
-if delayed beyond 6hrs, infarction may occur and you have to remove it.
-if ruled out, consider epididymitis (E coli infection in young and old; G+C in adults)

Fluids, electrolytes and Acid-Base Disorders

Maintenance fluids calculation


-100 ml/kg for first 10 kg, 50ml/kg for next 10, 20ml/kg for every 1 kg over 20
so all adults just do 1500ml + (20xkg over 20)
eg: 70 kg man: 1500 + (20x50) = 2500ml/kg/day divided by 24 = 104ml/hr

Hyponatremia
Causes/Classification:
Figure 15

-urine Na concentration less than 40mmol/L is consistent with SIADH


-note: hypothyroidism causes reduced GFR and thus hyponatremia (unknown how)
Tx:
-hypovolemic hyponatremia is obviously IVF replacement
-isotonic and hypertonic hyponatremia: tx underlying disorder
-hypotonic hyponatremia
-mild (120-130): withhold free water
-moderate (110-120): loop diuretics (given with saline to prevent renal
concentration of urine d/t high ADH)
-severe (<110): hypertonic saline to improve by 1-2 per hr
-do not increase sodium more than 8mmol/L during the first 24 hrs in order to
prevent CPM

Hypernatremia

Figure 16

Clinical: neuro sx predominate: AMS, restlessness, weakness, FNDs  confusion, seizures, coma. Sx
are worse when sodium increase was rapid. Also signs of dehydration.
Tx: like hyponatremia, don’t correct >8 in the first 24 hrs, to avoid cerebral edema
-hypovolemic hypernatremia: give isotonic fluids to restore hemodynamics then replace free
water deficit (Water deficit = TBW (1-actual Na / desired Na)
-isovolemic hypernatremia: central DI – give vasopressin. PO oral fluids or D5W
-hypervolemic hypernatremia – give diuretics and D5W. if renal failure  dialysis
Calcium
-changes in pH alter calcium binding, especially acute alkalotic states: in respiratory alkalosis, total
calcium is normal but free calcium lower because more is bound to albumin. This is why you get
symptoms (perioral numbness for ex) when hyperventilating
-Hypocalcemia
-pseudohypoparathyroidism: end organ resistance to PTH. So high PTH but low calcium. AR
disorder. a/w mental retardation and short metacarpal bones
-cardiac manifestations: arrhythmias, prolonged QT
-serum PO4 will be high in renal failure and hypoparathyroidism, but low in vit D def
-normal vitamin D levels increase both calcium and phosphate
-normal PTH levels increase calcium but decreases phosphate
-tx: if symptomatic, IV calcium gluconate. Long term – calcium carbonate and vit D
-Hypercalcemia
-milk-alkali syndrome: hypercalcemia, alkalosis, and renal impairment d/t excessive intake
of calcium and certain absorbable antacids (calcium carbonate, milk)
-cardiac manifestation: short QT
-Tx:
-1st step: IVF (1st) and diuretics
-inhibit bone resorption in patients with osteoclastic disease (e.g. malignancy) –
bisphosphonates (pamidronate) and calcitonin
-glucocorticoids if vitamin D-related (intox, granulomatous disorders) or MM are
the cause.
-hemodialysis if renal failure

Potassium – need to correct magnesium in order to correct K (whether high or low)


-serum K is affected by pH: Alkalosis leads to hypokalemia, acidosis can lead to hyperkalemia
-if HTN and hypokalemia  think excessive aldosterone
-Hypokalemia
-causes: usually GI losses (urine K less than 20) or renal losses (urine K over 20)or insulin
administration
-random: Bartter syndrome
-abx: bactrim and Amp B
-epinephrine
-hypokalemia predisposes to digoxin toxicity

Hypomagnesemia
-ETOH is a common cause
-NM and CNS hyperirritability (twitching, hyperreflexia, seizures, AMS)
-coexisting hypocalcemia is common because of decreased release of PTH and bone resistance to PTH
when Mg is low
-coexisting hypokalemia: in muscle and myocardium one affects the other
-ECG: loss of t wave (like hypoK) prolonged QT  Torsades
-PO mg for mild, IV for severe

Hypermagnesemia
-Renal failure is the MCC
-loss of DTRs is classic first sign; coma and paralysis late
-ECG: similar to hyperkalemia
-tx: IV calcium gluconate for emergent sx. Saline and furosemide. Dialysis

Hyperphosphatemia – tx is phosphate-binding antacids: aluminum hdroxide or carbonate. Dialysis if


necessary.

Acid-Base Disorder equations


1. Use PCO2 to determine acidosis/alkalosis
2. Anion Gap = Na – (HCO3 +Cl)
3. Winter’s formula: expected pCO2 = (1.5 x HCO3 + 8) +/- 2
if actual is more than expected then you have co-existing respiratory acidosis
classic example of met acid and resp acid is an asthmatic child who has a PaCO2 that
goes from abnormal to normal. Means impending resp failure required intubation
if actual is less than expected then you have co-existing respiratory alkalosis
4. Delta gap = actual – normal
if delta gap plus given HCO3 is more than 24 +/- 2, then you have a co-existing met alkalosis
if delta gap plus given HCO3 is less than 24 +/- 2, then you have a co-existing met acidosis
5. calculated osmolar gap = 2Na + glu/18 + BUN/2.8 + Ethanol/4.6
calc – serum osmolarity = osmolar gap. If over 10 then you have something else there 
methanol or ethylene glycol or isopropyl alcohol. SO doesn’t work for ethanol cuz its in the
calculation

-diarrhea causes NAGMA, but Vomiting causes met alkalosis


-first step in met alkalosis is to determine ECF volume (contraction vs expansion)
-saline-sensitive: contraction usually with co-existing hypkalemia.
-urine chloride will be low (less than 10)
-saline-insensitive: expansion usually with HTN (d/t excess mineralocorticoids from
adrenal pathology)
-urine chloride will be high (>20)
-tx with saline or aldosterone (until adrenal path fixed)
-respiratory acidosis:
-increased PaCO2  increased cerebral blood flow  increased CSF pressure, resulting in
CNS depression.

HEME/ONC Diseases

Microcytic Anemias
-retic count will typically be less than 2% cuz it’s a production problem
-IDA
-menstruation or GI typically. RDW is typically high unlike the other micro anemias
-nutritionial deficiency: kids 6mos-3years because of only human milk, adolescent women,
pregnant women
-microcytic, hypochromic RBCs on blood smear
-only consider IV iron if the cause is poor absorption or can’t tolerate oral iron
-Thalassemias
-Beta-thal
-major
-homozygous mutation, typically mediterranean people, severe anemia,
massive HSM, expansion of marrow space (xray = crew-cut of skull)
-dx: hemoglobin electrophoresis – HbF and HbA2 elevated
-blood smear may have target cells
-tx: transfusion dependent. May cause iron overload so typically given with
defuroxamine.
-alpha-thal
-HbH disease: mutation/deletion of three alpha-loci
-similar sxs and tx as beta-thal-major. Electrophoresis will show high HbH
-4 loci deletion cause hydrops fetalis
-Sideroblastic anemia
-caused by abnormality in RBC iron metabolism
-hereditary or acquired – acquired causes include drugs (chloramphenicol, INH, alcohol),
lead exposure, collagen vascular dz, and myelodysplastic dz
-labs: increased iron and ferritin and TIBC saturation is normal/elevated (distinguishes it
from IDA); ringed sideroblasts in bone m arrow
-tx: remove offending agents. Consider pyridoxine
-Anemia of chronic disease
-can be micro or normo (usually normo apparently)

Macrocytic Anemias
-B12 deficiency:
-causes: Pernicious anemia (MCC), gastrectomy, poor diet (strict vegetatians (no fish or
meat)), alcoholism, crohn’s, ileal resection, competitors (Diphyllobothrium latum and blind loop
syndrome (bacterial overgrowth))
-clinical: anemia, sore tongue, demyelination of posterior columns, incontinence, impotence,
dementia.
-Dx: peripheral blood smear showing macrocytic RBCs and hypersegmented neutrophls.
High methylmalonic acid and homocyteine (vs Folate def)
-antibodies to IF can dx pernicious anemia
-schilling test: adding IF will result in more absorbed B12 and less in urine =
pernicious anemia
-Tx parenteral once a month – cyanocobalamin
-Folate def
-similar to B12 but no neuro and methylmalonic acid wont be high

Normocytic Anemias – Production problem


-decreased retic response <2%
-Aplastic Anemia
-BM failure leading to pancytopenia
-causes: idiopathic, radiation, meds (chloramphenicol, sulfonamides, gold, carbamezapine),
viral (HPV, HCV, HBV, EBV, CMV, HIV), chemicals (benzene, insecticides)
-expected signs of pancyto. Can transform to acute leukemia
-Dx: BM bx for definitive – reveals hypocellular marrow and the absence of progenitors of all
3 hematopoietic cell lines
-Tx: BMT, transfusion if nec, underlying causes
-Anemia of chronic disease (disscussed earlier)

Normocytic Anemias – Hemolytic Anemia (HA)


Intravascular hemolysis – microangiopathic HA (e.g. DIC, mechanical valves), transfusion rxns,
infections (e.g. clostridial sepsis), paroxysmal nocturnal hemoglobinuria (PNH), IV RhoD Ig infusion
Extravascular hemolysis – Intrinsic RBC enzyme deficiency (e.g., G6PD), hemoglobinopathies (e.g.,
sickle cells, thalassemia), membrane defects (e.g. Hereditary spherocytosis), hypersplenism, IVIG
infusion, warm or cold-agglutinin autoimmune hemolyitic anemia (most cases), infections (e.g.,
Bartonella, malaria), toxins (snake bite or brown recluse spider)
-high (>2.5%) reticulocyte count because BM is responding appropriately until it can’t keep up with
hemolysis
-labs: elevated retic, LDH; decreased haptoglobin and Hgb/Hct
-Clinical: jaundice, dark urine (from hemoglobinuria, not bilirubin  indicates intravascular)
-spherocytes or helmet cells suggest extravascular
-Tx-general: underlying cause, transfusion if nec, folate supplements (folate is depleted in hemolysis)

Sickle Cell Anemia


-AR disorder. Glutamic acid replaces valine at the 6 position of the beta-chain
-Sickle cell trait is a/w isosthenuria – the inability to concentrate or dilute urine. They will have a
constant osmolality on UA.
-Clinical (other than obvious)
-high-output heart failure: many die from CHF
-aplastic crises: generally precipitated by parvo B19. Treat with transfusion
-Dx: confirmed with hemoglobin electrophoresis
-Tx:
-vaccines for encapsulated bacteria
-ppx with penicillin between 4mos-6years
-folic acid supplements d/t chronic hemolysis
-supportive management (hydration and pain control) for pain crises
-hydroxyurea: enhances Hb F
-transfusion based on clinical picture
-BMT

Hereditary Spherocytosis
-AD defect in spectrin
-Spherocytosis causes: HS, G6PD def, ABO incompatibility, hyperthermia, AIHA
-Dx: Osmotic fagility to hypotonic saline (spherocytes cant tolerate swelling and they rupture)
-negative direct coombs test
-Tx: splenectomy

Glucose-G-Phosphate dehydrogenase deficiency


-XLR (so mostly men). Leads to accumulation of H2O2 which denatures Hgb and it precipitates into a
heinz body
-Precipitants: SNP D and fava beans. Sulfonamides (i.e. bactrim), Nitrofurantoin, primaquine,
dimercaprol/dapsone and fava beans and infection
-Dx: blood smear – heinz bodies and bite cells. Measurement of G6PD levels is diagnostic
-Tx: avoid and support

Autoimmune Hemolytic Anemia (AIHA)


-Warm: more common. IgG autoantibody that binds to RBC membranes best at 37 deg and results in
extravascular hemolysis.
-splenomegaly is a common feature (sequestration)
-usually idiopathic. Can be secondary to lymphoma/leukemia, collagen vascular disease
(SLE; aka other AI diseases), drugs like alpha-methydopa
-Cold: IgM autoantibody that binds optimally at 0-5deg
-causes complement activation and intravascular hemolysis. Sequestrated in the liver.
-idiopathic (elderly) or d/t infection (mycoplasma pneumoniae or infectious
mononucleosis)
-Dx: Direct Coombs
-if coated with IgG then its warm AIHA. May have spherocytosis
-if coated with complement then cold AIHA. Cold agglutination also
-Tx: only if severe
-Warm: glucocorticoids, immunosuppression/splenectomy/RBC transfusions/folate suppl.
-Cod: avoiding cold, RBC transfusions/chemo agents, steroids do NOT work

Paroxysmal Nocturnal Hemoglobinuria (PNH)


-acquired disorder resulting in deficient glycosylphosphatidylinositol, which anchors CD55 and
CD59. that normally inhibit complement. Leads to lysis of RBC, WBC, and platelets
-Clinical
-chronic intravascular hemolysis reesulting in chronic PNH and elevated LDH
-pancytopenia
-thrombosis of venous systems can occur (e.g., hepatic veins  budd-chiari)
-ab, back, msk pain
-may evolve into aplastic, or myelodysplasia
-Dx:
-Ham test: cells are incubated in acidified serum, triggering the alternative complement
pathway leading to lysis of PNH cells
-Sugar water test: serum mixed is sucrose, hemolysis ensues
-Best: flow cytometry for CD55 59
-TX:
-Iron/folate supp
-Eculizumab (mab that inhibits complement activation)

Immune (Idiopathic) Thrombocytopenia


-autoimmune IgG coat and damage platelets, which are then removed by splenic macs
-2 forms
-acute: kids after viral infection. Spontaneously resolve
-chronic: 20-40 yo women, doesn’t spontaneously resolve
-clinical: petechiae and ecchymoses, bleeding of mucous membranes, no splenomegaly
-Dx: Plt <20,000. Rest is normal.
-tx: adrenal corticosteroids, IVIG, splenectomy in increasing order for severity.

Thrombotic Thrombocytopenic Purpura


-lack functional ADAMTS13 and this leads to ultra large vWF multimers
-microthrombi (mostly platelet thrombi) occlude small vessels leading to microangiopathic HA
(schistocytes). Emergency.
-PT and PTT normal
-TTP=HUS + fever + AMS
HUS = microangiopathic HA + thrombocytopenia + renal failure
-Tx: immediate large volume plasmapheresis
-platelet transfusions are contraindicated

Heparin-Induced Thrombocytopenia
-antibodies against heparin-platelet factor 4 complex
-few days after heparin. Platelets aggregate leading to venous thrombosis (DVT/PE)
-decrease in plt clount by 50% suggests HIT
-Dx: antiplatelet factor IV ab or serotonin release assay
-Tx: stop heparin. If needed, start thrombin inhibitor (lepirudin, argatroban, dabigatran)

Bernard-Soulier Syndrome
-AR disorder of platelet adhesion (to subendothelium) d/t def in platelet glycoprotein GPIb-IX
Glanzmann Thrombasthenia
-AR disorder in platelet aggregation d/t def in glycoprotein GPIIb-IIIa

von Willebrand Disease


-AD disorder (MCC inherited bleeding DO) resulting in def vWF and thus disrupts plt aggregation and
adhesion. It also is the typical carrier for factor VIII
-mucosal/cutaneous bleeding and menorrhagia
-Dx:
-prolonged bleeding time but nml plt count – PTT may be prolonged (cuz the FVIII
degradation) so increased PTT and increased bleeding time
-decreased titers or activity of FVIII
-reduced ristocetin-induced plt aggregation
-Tx: Desmopressin (DDAVP) 1st. FVIII concentrate 2nd
-Only clotting factor not made in the liver. Its synthesized by megakaryocytes and endothelial cells

Hemophilia A
-XLR disorder. Suspect if unsuspected hemorrhage occurs in a male pt with positive family hx
-AIDS d/t past transfusion is the MCCOD. 2nd is intracranial bleed. Common – hemarthroses
causing joint destruction
-Dx: only PTT prolonged
-Tx: analgesia and immobilization for hemarthroses. FVIII concentrate mainstay when necessary for
bleeding episodes, pre-surgery or dental work.
-Hemo B: XLR def in FIX
Disseminated Intravascular Coagulation
-Causes: infection (MCC), OB (placenta and uterus have tissue factor) – amniotic fluid emboli,
retained dead fetus, abruptio placentae, major tissue injury, malignancy, shock, rattlesnake venom
-labs are all abnormal:
-Increased: PT, PTT, TT, Fibrin split products, D-dimer, bleeding time
-decreased: fibrinogen (if it was nml/elevated then that r/o DIC), plt count
-schisotcytes
-tx: supportive; and maybe cryoprecipitate (clotting factors and fibrinogen) or FFP (clotting factors)

Inherited Hypercoagulable States


-ATIII deficiency
-AD inheritance. ATIII is normally an inhibitor of thrombin, so def leads to increased
thrombosis. Pts with disorder DO NOT respond to heparin
-Antiphospholipid antibody syndrome
-can present with thrombosis, recurrent preg loss, or thrombocytopenia (yes I said that
right – also has false PTT prolongation)
-see notes from earlier
-Protein C def
-AD. Decreased inhibition of factors V and VIII
-Protein S def
-cofactor of protein C. so same thing
-Factor V Leiden
-MC hereditary hypercoagulability disorder among whites
-Protein C unable to inactivate FV
-Prothrombin gene mutation
-Hyperhomocysteinemia.
*pts who’ve had 2 or more events should be permanently anticoagulated with warfarin

Multiple Myeloma
-proliferation of a monoclonal immunoglobulin, usually IgG or IgA
-plasma cells take over normal cells in the BM
-Signs: CRAB – calcium (hyperCa), Renal failure, Anemia, Bone lesions (lytic)
-Renal failure d/t
-myeloma nephrosis – Ig precipitation in the tubules leads to casts of Bence jones
protein
-hypercalcemia also plays a role in renal impairment.
-Recurrent infections 2/2 deprivation of normal plasma cells; MCCOD
-osteolytic lesions are d/t release of osteoclast-activating factor by the plasma cells
-Dx:
-Serum and urine protein electrophoresis (M protein)
- BM bx is essential for diagnosis and reveals at least 10% abnml plasma cells.
- other: peripheral smear shows RBCs in rouleaux formation from hyperglobulinemia
-Tx: autologous stem cell transplant if younger and early in dz.

Waldenstrom Macroglobulinemia
-proliferation of plasmacytoid lymphocytes that produce IgM paraprotein, which causes
hyperviscosity
-sx: constitutional, neuro, LAD, splenomegaly and hyperviscosity
-hyperviscosity syndrome can lead to retinal vessel dilation and hemorrhage  blindness
-Dx: IgM >5mg. Bence-jones, absence of bone lesions
-Tx for hyperviscosity – chemo and plasmapheresis

Hodgkin Lymphoma
Gen
-bimodal age: 15-30, >50
-histo: lymphocyte predominance (few Reed-sternberg (RS) cells), nodular sclerosis (bands
of collagen surrounding pools of RS cells), mixed cellularity (large number of RS cells), lymphocyte
depletion (worse prognosis)
Clinical: painless LAD that spreads by continuity from one LN to the next +/- B sx (fever, night
sweats, weight loss)
Dx: LN bx – RS cells are required
Tx: Chemo + Radiation

Non-hodgkins Lymphomas (NHL)


-Epidemiology: burkitt = africa. Adult T-cell lymphoma = Japan and the Caribbean
-Rituximab is often used in combo with CHOP (cyclophosphamide, hydroxydaunomycin
(doxorubicin), Oncovin (vinchristine), Prednisone----CDVP) chemo
-Any node over 1cm present for more than 4 weeks, not attributed to infected, should be biopsied
- Low-grade, indolent tumors are unlikely to be cured but have better 5-year prognosis. While
intermediate and high-grade tumors are more responsive to chemo and have better chance of cure if
they are treated

figure 17

Acute Leukemias – affect more immature cells


-2 types
-AML (myeloblasts)
-Adults, exposure to radiation or chemo, down’s
-variant: APL t(15;17) that often presents with pancytopenia. Can be treated with
all-trans retinoic acid and chemo
-may have skin nodules
-ALL (lymphoblasts)
-MCC malignancy in kids under 15
-most responsive to therapy
-may have testicular involvement or anterior mediastinal mass (T-ALL)
-clinical features result from decrease in non-WBCs, also HSM and LAD
-Dx: BM bx is required[
-watch for tumor lysis syndrome: medical emergency

Chronic Lymphocytic Leukemia


-MCC leukemia and typically over 60 yos
-morphologically mature lymphocytes but functionally defective (i.e. don’t differentiate into
antibody-producing plasma cells)
-nontender LAD, splenomegaly, frequent infections
-Dx: CBC: WBC = 50,000-200,000 and the rest is low
-blood smear is often diagnostic: absolute lymphocytosis (mostly mature), smudge cells

Chronic Myeloid Leukemia


-middle aged. CML follows indolent course and transforms to acute leukemia in a blast crisis
-a/w t(9;22) resulting in activated tyrosine kinase. Hence the treatment: TKIs (imatinib or other
inibs)
-constitutional sx are usually the initial sx. CML usually discovered on routine blood work (marked
leukocytosis with left shift). Can also have HSM and LAD.
-Dx:
-WBC: leukocytosis with left shift and eosinophilia
-blood smear: immature granulocytes and decreased leukocyte alk phos activity (vs.
leukomoid reaction which has increased leukocyte alk phos)

What’s my age again? ALL = less than 15. AML/CML =40-60. CLL = greater than 65
Polycythemia Vera
Gen:
-increase in RBC mass independent of EPO
-mutations in JAK2 tyrosine kinase in most cases
Clinical
-hyperviscosity: HA, dizziniess, weakness pruritis, visual impairment, dyspnea
classic: pt who complains of severe pruritis after a hot bath/shower
-thrombotic phenomena (DVT, MI, CVA)
-bleeding
-HSM and HTN
Dx:
-1st: R/O secondary polycythema
-CBC: elevated RBC count, H/H (>50)
-decreased EPO
-BM bx confirms
Tx: Repeated phlebotomy to lower Hct

Essential Thrombocytosis
-platelets over 600,000. Reactive must be r/o
-smear shows hypogranular, abnml shaped platelets
-BM bx shows increased megakaryocytes
-Jak2 mutation in ½ the pts
-tx is antiplatelet agents such anagrelide and low-dose ASA. Sometimes hydroxyurea

Infectious Diseases

Pneumonia
-CAP
-uncomplicated without significant comorbidities: azithromycin or clarithromycin
-uncomplicated with comorbidities: flouroquinolone (levo or moxi)
-hospitalized (not acquired) pt: fluroquinolone alone or ceftriaxone plus azithromycin
-Hospital-acquired PNA: tailor towards gram-neg rods
-ceftazidime or cefepime
-imipenem
-pipercillin/tazobactam
-Ventilator-associated PNA: Combo of 3 drugs
1. Ceftazidime or cefepime OR pip/tazo OR imipenam
2. Aminoglycoside OR fluoroquinolone
3. Vancomycin OR linezolid
-Legionella PNA is common in organ transplant recipients, people with renal failure or chronic lung
disease, and smokers. Presents with GI sx and hyponatremia

Lung Abscess
-Dependent zones for aspiration leading to abscess: posterior segments of the upper lobes and
superior segments of the lower lobes (more likely R side)
-lower cavitation = aspiration; upper cavitation = TB
-Culprits
oral anaerobes: Prevotella, peptrostreptoccus, fusobacterium, bacteroides
other: s aureus, s pneumo, aerobic gram-neg bacilli
-foul-smelling sputum is consistent with anaerobic infxn and clues into the dx.
-Tx: until cavity is gone or CXR is improved
-gram + = ampicillin or amoxicillin/clavulanic acid, amp/sulbactam, or vanco for aureus
-anaerobes = clindamycin or MTZ
-gram - = fluoroquinolone or cetazidime
Tuberculosis
-Xray findings in healed primary TB (so not active infxn):
-Ghon complex: calcified primary focus with an associated LN
-Ranke complex: Ghon complex undergoes fibrosis and calcification
-Miliary TB: may present with organomegaly, reticulonodular infiltrates on CXR, and choroidal
tubercles in the eye
-HIV pts: normal dx clues go out the window – PPD negative, atypical CXR findings, sputum more
likely to be negative, and no granulomas
Dx/Tx:
-If PPD positive, next step is CXR to r/o active disease.
-If there’s no active disease, give isoniazid (plus B6 aka pyridoxine) for 9mos to
decrease risk of active infection. Even if the pt had a BCG vaccine
-PPD positivity
-normal pt, no risk factors: >15mm is positive
-Risk factors: >10mm is positive
-Immunocompromised or abnormal CXR: >5mm
-If symptomatic or has abnml CXR, next step is sputum acid-fast test
-active TB = RIPE(S) tx and isolation until sputum is negative for acid-fast bacilli
-only discontinue tx if LFTs rise to 3-5x the upper limit

Meningitis
-acute triad: fever, nuchal rigidity, AMS
-MC orgs and empiric treatment
-Neonates: GBS, E coli, Listeria
-Empiric tx: cefotaxime + ampicillin + vancomycin (AG if <4weeks)
-Kids >3mos: Neisseria meningitidis, S pneumo, H flu
-ceftiaxone or cefotaxime + vancomycin
-Adults 18-50: S pneumo, N meningitidis, H flu
- ceftiaxone or cefotaxime + vancomycin
-Elderly >50: S pneumo, N mening, Listeria
- cefotaxime or ceftriaxone + ampicillin + vancomycin
-IC’d: Listeria, gram-neg bacilli, S pneumo
-ceftazidime (for psuedo) + amp + vanc
-Aseptic: enterovirus and HSV usually, can also be certain bacteria, parasites, or fungi
-self-limiting. Supportive tx
-CT is rec’d before LP to look for elevated ICP or lesions.

Encephalitis
-Organisms (usually viruses)
-HSV
-Arbovirus: EEE, west nile
-Entero: polio for ex
-less common: measles, mumps, EBV, CMV, VZV, rabies, prion disease
-nonviral infectious causes: Toxoplasmosis, cerebral aspergillosis
-noninfectious: metabolic, T-cell lymphoma
-Clinical
-prodrome: HA, malaise, and myalgias
-acutely ill with signs of meningitis, delirium, and behavioral abnormalities, FND
-Dx:
-CSF will have lymphocytosis with nml glucose similar to viral meningitis (asceptic)
-CSF PCR is a better test
-MRI is imaging modality of chocie.
-Tx: only have treatment for HSV (acyclovir) and CMV (ganciclovir or foscarnet)

Viral Hepatitis
-HBV is a/w polyarteritis nodosa (PAN); HCV is a/w with cryoglobulinemia
-HCV does not cause significant acute illness
-HAV serology: anti-HAV persists for life, so IgM is acute and IgM is previous infection.
-RNA is more useful for HCV
-Chronic HBV tx: IFN-alpha
-HCV treatment
-IFN-alpha and ribavirin
-newer cures: all end in –vir

Botulism
-results from preformed toxins (usually improperly stored food). Can be killed by cooking at high
temps.
-wound contamination is another source
-symmetric, descending flaccid paralysis (starts from face). Usually a/w GI issues.
-Dx: identifying toxin from serum, stool, or gastric contents
-Tx: antitoxin (toxoid) and supportive respiratory care. If from wound, then add cleaning and
penicillin.

Lower UTI
-no fever
-presence of epithelial cells or bacteria without WBCs on UA indicates contamination
-thus WBCs aka pyuria (greater than 10 leukocytes) is best indicator
-don’t need a urine Cx unless elderly, diabetes, recurrent UTIs, sx lasting over a week, or use of a
diaphragm.
-Tx
-Acute, uncomplicated UTI (any of these options)
-Oral TMP/SMX for 3 days
-Nitrofurantoin for 5-7 days – do not give if early pyelo is suspected
-Fosfomycin 1 dose – don’t give if early pyelo is suspected
-Cipro for 3 days
-Phenazopyridine can be given for dysuria
-Pregnant women
-amp, amox, or oral cephalosporins for 7-10d
-Do NOT give Fluoroquinolones (can cause fetal arthropathy)
-Men: same as women but for 7 days
-Recurrent: PPX – single dose of TMP/SMX after intercourse or at 1st sx. Or give low-dose
TMP-SMX for 6mos.

Pyelonephritis
-add less common organisms to the differential: Enterococcus faecalis and S aureus
-complications
-sepsis
-emphysematous pyelonephritis: gas-producing bacteria in diabetics
-chronic pyelo and scarring - rare unless there’s underlying renal dz
-UA: look for pyuria, bacteriuria, and leukocyte casts
-obtain urine Cx in ALL pts with suspected pyelo
-imaging if complicated or fails tx (renal US, CT, IVP or retrograde urogram)
-Tx: based on gram-stain
-uncomplicated pyelo
-TMP-SMX or fluoroquinolone for 10-14days for most gram negs
-amoxicillin for gram-pos
-a single dose of ceftriaxone or gentamicin if often given before PO tx
-repeat cx after 48hrs. if fails to respond  imaging
-if patient is very ill, elderly, pregnant, unable to tolerate PO, or uroseptic
-hospitalize, IVF, IV amp plus gent until afebrile. Then switch to PO for 2 weeks
Prostatitis
-acute is less common than chronic. Acute is MC in younger men, gram-negs predominate and is more
serious and requires tx
-recurrent exacerbations and UTIs are common in chronic prostatitis
-Dx:
-avoid DRE because it my cause bacteremia (but its described as a boggy, exquisitely tender
prostate in acute disease)
-UA and Urine Cx shows numerous sheets of WBCs and positive cx in acute
-Chronic: presence of WBCs in expressed prostatic secretions
-Tx:
-acute: TMP/SMX or a fluoroquinolone and doxycycline. IV if serious. PO for 4-6weeks if mild
-Chronic: fluoroquinolone for a long time (difficult to tx)

Figure 18

Random infectious stuff


-HSV-1 is a/w Bell’s palsy
-Culture of HSV is the gold standard
-syphillis: if allergic to PCN, give doxy or tetracycline
-chancroid: painful genital ulcer(s) that can be deep with ragged borders and with a purulent base.
Unilateral tender inguinal LAD (buboes) that appears 1-2 weeks after ulcer. No constitutional
findings.
-dx: clinical only and r/o other causes of ulcer
-tx: azithromycin or ceftriaxone
-Lymphogranuloma Venereum (caused by C. trachomatis)
-painless ulcer followed by tender inguinal LAD (unilateral usually) a few weeks later and
constitutional symptoms
-complication: proctocolitis may develop with perianal fissures and rectal strictures. May
even get lymphatic obstruction and elephantiasis
-tx is doxycycline
-Pediculosis Pubis (pubic lice aka crabs)
-org: Phthirus pubis. Transmitted via sex, clothes, or towels
-severe pruritis of pubes. Other hairy areas can be involved too
-Dx: visual inspection. Tx: permethrin of all hairy areas. Tx partners
-Cellulitis
-local trauma, breaks in skin = GAS
-wounds/abscesses = S aureus
-immersion in water = Pseudomonas, aeromonas hydrophilia, vibrio vulnificus
-acute sinusitis = H influenzae
-usually treat with a staphyloccal penicillin or a cephalosporin IV until signs of infection
improve
-Erysipelas is a cellulitis that is usually confined to the dermis and lymphatics
-usually caused by GAS
-*spectrum: cellulitis  erysipelas  necrotizing fasciitis
-tx: PCN or erythromyciin
-Necrotizing Fasciitis (deep soft tissue infection that goes to the fascial planes)
-strep pyogenes (GAS) and C perfringens
-pain out of proportion, crepitus, necrosis, can lead to cutaneous anesthesia
-complications: sepsis, toxic shock syndrome, and multiorgan failure
-rapid surgical exploration and excision of devitalized tissues is absolutely necessary
followed by broad-spec abx.
-Tetanus
-exotoxin in contaminated wounds that blocks inhibitory transmitters in the NMJ
-Tx
-ICU and respiratory support (if nec) and give diazepam for tetany
-passive immunization with single IM dose of tetanus IG followed by active
with Td
-Common bugs and osteomyelitis (OM)
-catheter septicemia = S aureus
-prothetic joint = coag-neg staph (s epidermis)
-diabetic foot ulcer = polymicrobial
-nosocomial = Pseudomonas
-IVDU, neutropenia = pseudo, fungal
-sickle cell = salmonella
-OM dx and tx
-ESR/CRP are very useful in monitoring response to tx
-needle aspiration or bone bx is the most direct and accurate way for dx
-MRI is the most effective imaging study
-Tx: IV abx (high bone penetration – cephalosporins, fluoroquinolones, vanc, linezolid,
daptomycin, and clindamycin) for 4-6wks and surgical debridement
-Gonococcal Arthritis
-acute monoarthritis (knee MC) or oligoarthritis, and often progresses within days in a
migratory or additive pattern
-tenosynovitis in hands and feet
-disseminated: fever, chills, rash
-tx: ceftriaxone
-Acute infectious arthritis (other than Gonococcal)
-S aureus is MCC
-Dx: joint aspiration for any infectious arthritis (including Gono) look for WBC over 50,000
-tx: staph drugs (MSSA or MRSA) for immunocompetent. Extend to gram-neg coverage if
immunocompromised.
-complications (both gono and staph): destruction of surrounding structures, avascular
necrosis, sepsis
-Lyme disease
-serologic studies are the most important to confirm dx. Tx is doxy
-amoxicillin is given to kids under 8 or pregnant women
-Rocky mountain spotted fever
- Rash start peripherally and spreads centrally. Beings 4-5 days after fever.
-tx is doxy. If CNS manifestations or pregnant, give chloarmphenicol
Figure 19
Figure 20
Figure 21

Skin and Hypersensitivity Disorders

Acne Vulgaris
-Mild/moderate: 1st – topical benzoyl peroxide. 2nd topical retinoids. 3rd topical erythromycin or
clindamycin
-Moderate/severe nodular pustular acne: after trying above…1st - systemic abx (tetracyclines,
macrolides, bactrim). 2nd – oral retinoids (isoretinoin) as last resort. Do not give in pregnancy (must
have 2 negative preg tests before treatement and use 2 forms of birth control starting 1 month before
till 1 month after treatment.

Rosacea
-Tx: topical MTZ for acute. Systemic abx are used for maintenance
-isoretinoin if refractory
Tinea versicolor
-well demarcated hypo and hyperpigmented lesions that are well-demarcated. Usuallly affects the
trunk.
-KOH prep: spaghetti and meatballs
-tx: oral or topical* antifungals

Seborrheic dermatitis
-dandruff may progress to yellowish flakes with surrounding scaly erythematous patches if severe
-Tx:
-sunlight
-dandruff shampoo
-topical ketoconazole or corticosteroids if severe

Contact Dermatitis
-irritant
-not immune-mediated and doesn’t need sensitization
-rash appears right after exposure
-allergic
-type IV HSR – takes hours to days

Pityriasis Rosea
-hearld patches and christmas tree pattern
-NOT contagious
-spontaneously remits. Only tx is antihistamines supportively

Erythema Nodosum
-painful, red, subQ nodules, typically located over the anterior aspect of the tibia
-usually in young women and resolves in a few weeks
-causes: GAS, sarcoidosis, IBD, behcet dz, fungal infxns, pregnancy, meds (OCPs, sulfa, amiodarone,
abx), syphilis, Tb, idiopathic
-look for and treat underlying condition. Otherwise tx is supportive

Erythema multiforme
-erythematous macules/papules that resemble target lesions that can become bullous. May be
painful or pruritic
-may be caused by meds (sulfa or PCN or other), But think HSV
-acyclovir
-Spectrum for drug reactions: EM  SJS  TEN
-aggressive IVF and supportive care

Lichen Planus
-4 Ps: Pruritic, Polygonal, Purple, flat-topped Papules
-MC seen on wrists, shins, oral mucosa, and genitalia
-tx with glucocorticoids
Bullous Pemphigoid
-B for basement on IF (hemidesmosome)
-systemic or topical steroids

Pemphigus Vulgaris
-Pimp Dez (desmosomes (IgG ab against desmoglien))
-fish net on IF
-starts in oral mucosa (cuz dez is doing oral)
-systemic steroids or other immunosuppressant
Dermatophytes
-topical antifungals except for tina capitis (will have associated hair loss and maybe itching) or
oncyhmycosis (oral griseofulvin)

Psoriasis
-1st line tx is topical corticosteroids. New and effective treatment are the vitamin D derivatives
calcipotriene and calcitriol

Other
Figure 22