Om Sai Ram!!!

Day1: 27th Jan

Uworld 1: Respiratory
1. Surfactant: DPPC, L/S>2 (Fetal lungs are fully matured and will not suffer
from NRDS). Rx: corticosteroids to induce surfactant formation in premature
deliveries.
a. AFP: elevated Neural tube defect. (spina bifida)
b. Erythroblastosis fetalis: assess fetal hemolysis by measuring amniotic
fluid bilirubin levels.
c. Congenital Adrenal hyperplasia: aminocentesis check 17hydroxyprogesterone, HLA region of CS 6/abnormal genes by molecular
probes.
2. Cystic Fibrosis: mutation of CFTR gene defective post translational
folding and glycosylation Degradation of CFTR integral membrane protein
before it reaches cell surface. 508 mutation :: deletes 3nt that code for
Phenylalanine at 508.
a. Phelyketonuria: mutation in hepatocyte intracellular enzyme
phenylalanine hydroxylase. protein misfolding.
b. Sickle cell anemia: mutation in beta globin gene of HbS.
c. Marfan Syndrome: defect in extracellular glycoprotein, fibrillin 1.
Abnormal fibrillin 1 monomer disrupts fibrillin 1 polymerization and
prevent normal formation of microfibrils in interstitial connective
tissue.
d. Freidrichs Ataxia: Frataxin gene. Codes for mitochondrial protein
important in RESPIRATORY function and iron homeostasis, is mutated.
GAA repeat disrupts transcription.
3. Gravity: Supine patients aspirate into Posterior segments of upper lobes and
superior segments of lower lobes. Upright aspirate into BASILAR SEGMENTS
OF LOWER LOBE. Aspirated material goes down right main bronchus.

4. Fetal circulation: Most oxygenated blood in fetus carried by umbilical vein,

empties into IVC via Ductus venosus.

Om Sai Ram!!!

5. Cystic fibrosis: Autosomal Recessive

6. CGD: Nitroblue tetrazlolium, X linked mutation affecting NADPH oxidase.
The enzyme usually functions within activated phagocytes to produce
reactive oxygen species and activate granule proteases (eg elastase,
cathepsin G) in phagosomes. Mutation in NADPH Oxidase: impaired
intracellular killing by Neutrophils and macrophages Recurrant bacterial &
fungal infections involves lungs, skin and lymph nodes. Infections are
Catalase Positive organisms that destroy hydrogen peroxide produced by
their own metabolic activity.
Beta Lactamase: enzyme produced by bacteria confers Resistance to Beta
lactam antibiotics (eg penicillin)
Peniclllinase resistant penicillins (eg methicillin, oxacillin, nafcillin)
Beta lactamase inhibitors: (eg clavulinic acid, tazobactam, sulbactam)
Coagulase: produced by S aureus (+) prothrombin (fibrinogen to fibrin)..
Fibrin is coated on the organism and resistance to phagocytosis.
Lecithinase (Alpha toxin) w/ phosopholipase C : major virulence factor
of C. Perfringes. Toxin increases Platelet aggreagation & adherence molecule
is expressed on leukocytes and endothelial cells Vasooclusion and
ischemic necrosis of affected tissues.
7. Cystic fibrosis: high chloride content in sweat.
abnormal posttranslational processing of trans-membrane protein.

Om Sai Ram!!!
shunting of CFTR toward proteasome with complete absence of the
protein in apical membrane of affected epithelial cells.

7.

Om Sai Ram!!!
8. Macrophage mediated immunity to Mycobacterium tuberculosis is by TH1
cells. Without Th1 cells granuloma formation. Host is unable to mount
effective response & tuberculous infection would progress freely. IFNGAMMA, IL-12, TNF ALPHA : critical in formation & maintenance of
granulomas.

Thromboxane A2: powerful platelet aggregator and vasoconstriction.

Platelet activating factor: platelet aggregation, vasoconstriction,
bronchoconstriction, and increase leukocyte adhesion to endothelium. At low
conc. vasodilation and increases permeability of venules.
C3a: anaphylatoxin derived from C3. Simulates mast cell histamine release
inc vascular permeability and vasodilation.
IL-4: a. Thelpher cell differentiation into Th2 subset.
b. stimulates B cell growth and isotype switching to IgE.
IL-5: Promotes growth and differentiation of B cells and eosinophils and
stimulates isotype switching to IgA.

Om Sai Ram!!!
LTC4, D4,E4: vasoconstriction, increased vascular permeability and
bronchospasm.
9. Parietal pleura: somatic innervation (sensory afferent) nervessharp &
localized pain. Phrenic nerve (C3-C5) : motor innervation to diaphragm and
carries pain fibers from pleura.
Spinal accessory muscle: SCM and Trapezius muscle.
Vagus nerve: 10th cranial nerve: major source of Parasympathetic to viscera
of chest wall and foregut.
10.
Contact dermatitis, Granlumatous inflammation, tuberculin skin test
and candida extract skin reaction: delayed type hypersensitivity reactions
(DTH). Cells that mediate DTH reactions are TH1 lymphocytes that release
IFN-GAMMA recruitment and stimulation of macrophages.
11.
Elastins plasticity and ability to recoil upon release of tension
unique of desmosine crosslinking between four different lysine residues on
four different elastin fibers. This crosslinking is accomplished by action of
extracellular lysyl hydroxylase.
12.