ad myoglobinuria ardiac valves) on (DIC) Table 9.1 Causes of ‘red’ or dark-colored urine Macroscopic hematuria Myoglobinuria, Hemoglobinuria Biologic pigments: Bilirubin = Urates = Melanin Inborn errors of metsbolism: * Alkaptonuria = Tyrosinosis © Porphyrinuria Drugs and food colors: © Chloroquine © Desterrioxamine ‘ Diphenylhydantoin Levadopa Methyldopa Metronidazole Nitrofurantoin Phenolphthalein Pyridium Rifampin Sulfa drugs. Food/dyes Beets BlackberriesTable 9.2 Etiology of hematuria Glomerular hematuria Acute glomerulonephritis. Chronic glomerulonephritis: © IgA nephropathy Mesangali proliferative glomerulonephritis, Membranoproliferative glomerulonephritis Focal segmental glomerulosclerosis Membranous nephropathy Systemic lupus erythematosus ‘Shunt’ nephritis Goodpasture's syndrome Inherited nephropathies: Alport's syndrome Benign familial hematuria Nail-patella syndrome Fabry disease Polycystic kidney diseases. Medullary cystic disease Congenital nephrotic syndrome Hemolytic uremic syndrome Henoch-Schénlein purpura Minimal change disease ‘Systemic vasculitis Diabetes mellitus Amvloidosis, Non-glomerular hematuria Urinary tract disorders: © Urinary tract infection (bacterial, viral, parasitic) Urinary calculi Hypercalciuria Chemical cystitis Urethral/bladder foreign body Hydronephrosis Obstructive uropathy Urinary tract trauma Renal and urinary tract tumors Specific parasitic infections (schistosomiasis) Tubulointerstitial diseases: ‘© Acute pyelonephritis ‘+ Nephrocalcinosis interstitial nephritis ‘© Nephrotoxins (including heavy metals, radiocontrast medium analgesics, antiretrovirals) Tuberous sclerosis © Acute tubular necrosis Vascular disorders: Renal veinfartery thrombosis Malignant hypertension ‘+ Nutcracker syndrome * Loin pain hematuria # Congestive heart failure Hematologic disorders: © Sickle cell trait/disease = CoagulopathiesTable 123 Characteristics of Finish-type congenital nephrotic syndrome (CNS) and diffuse mesangial sersis isttype CS Dias mesangial sclerosis mst of proteins mera Abit, mor conan in the fist ear of ie Ap-etopotin in amsiote Mid Ataysintesed suy normal Penta 259 bith weight Vasey noma Proteinuria ‘Massive (704) with hypestbuminemia <8) sully ss sere eal function rma ing the st yer Feral alae within few months ter decoy stoingy Dilatation of proximal utes Mesangial sess Genetics Nt gene mutations WI gene mutations in Dryers syndrome and soe case of ale ize mesangial sere"No proteirura ap normal 1 No tami 110 dots | Normal prysical examination Urine cure negative | {No tami 110 chron renal aisease Hypartensin | 1 aBnormal renal tuncbon + Eamly HO errone anal atsoaso {+ Famiyipaiont HO desinass heck pci a abr Negatively 1D Gurecete soe eet an Poste famy evaluation for erature Elevated urine calcunieeatnine|| | —_Apnormal renal urasound Furr imestgate + Detated heating est CConsidor vorign tami Hypercalria \rooge nemstur 1 Investigate for glomeruonsphiis hematuria Strucual onal disoaso |) | + anal ions igere 0.7 Agotithm for evasion of proteinuria,Table 9.6 Causes of proteinuria Artifactual Mulvovaginitis Infections © Non-sexually transmitted ‘+ Sexually transmitted diseases © Fungal Infection Menstrual blood contamination Urethritis Prostatitis Glomerular diseases (sce also Table 9.2) © Nephrotic syndrome Glomerulonephritis Hypertension Diabetes mellitus HIV nephropathy ‘Tubular diseases — inherited ‘= Cystinosis = Wilsons disease + Lowe syndrome Tubular diseases — acquired © Interstitial nephritis = Reflux nephropathy © Acute tubular necrosis © Heavy metal poisoning Non-pathologic proteinuria = Orthostatic proteinuria = Fever = Exercise Table 17.3 Presenting complaints in s with autosomal dominant polycystic kidn Presenting complaint Neonatal disease” Hematuria ‘Abdominal pain Hypertension Renal mass Frequency Urinary tract infection ProteinuriaTable 10.1 Conditions that present as acute nephritic syndrome 1. Acute postinfectious glomerulonephritis Bacterial infections: Poststreptococcal glomerulonephritis ‘Staphylococci Pneumacocci Yersinia ‘Mycoplasma pneumoniae Viral infections: Influenza virus ‘Adenovirus Coxsackie virus Cytomegalovirus Epstein-Barr virus Varicella virus Mumps virus Measles virus Parvovirus B19 Membranoproliferative glomerulonephritis IgA nephropathy Henoch-Schénlein purpura nephritis Systemic lupus erythematosus nephritis Vasculitis ‘+ Microscopic polyangitis * Wegener's granulomatosis © Churg-Strauss syndrome /. Rapidly progressive glomerulonephritis, Table 18.1. Glomerular diseases associated with viral infections Infection Disease HW Collapsing glomerulopathy HBV Glomerulonephvts: lpus-k, membranoproliferatie, I nephropathy Other: memtranous nephropathy itil and immunctactoid nephropathy, and thrombotic micreangiogthy ‘Membranous nephropathy - mast common in len Membranoproiterative glomeruonephrogathy 1g nephropathy Foartrts nodosa ‘Membranoprolferative glomerulonephropathy (with or without cryaglobulineria)Table 11.1 syndrome in children Primary causes ‘Minimal change nephrotic syndrome (MCNS) Focal segmental glomerulosclerosis (FSGS) ‘Mesangial proliferative glomerulonephritis Mcmbranoprolifcrative glomerulonephritis (MPGN)/ ‘mesangiacapillary glomerulonephritis: ‘+ MPGN type ‘© MPGN type I! (dense deposit disease) ‘= MPON type Il ‘Membranous nephropathy (MN) ‘Secondary causes Systemic diseases associated with nephrotic syndrome: Henoch-Schénlein purpura ‘= Systemic lupus erythematosus © Diabetes mellitus = Sarcoidosis Infectious diseases associated with NS: Primary and secondary causes of nept ‘© Hepatitis B (usually associated with MN, rarely MPGN) ‘+ Hepatitis C (usually associated with MPGN) ‘¢ HIV (often with collapsing variant of FSGS) Hematolagy/oncolagy Leukemia ‘© Lymphoma (Hodgkin disease usually MCNS) Sickle cell anemia Drugs: ‘* Non-steroidal anti-inflammatory drugs (MCNS) = Gold ‘= Penicillarnine + Captopril Other: ‘Bee stings (MCNS) ‘© Food allergies ‘= Obesity (usually with FSGS) © Pregnancy-toxemia Authorsreerence Maker and Heymann” ive td 1,020 «1,012 Urine/plasma creatinine >40 <20 Urine Na (mEq/L) <20 >40 FENa < 1% > 2%Table 24.7 Clinical and investigative tools differentiating acute renal failure from chronic renal failure Acute renal failure Chronic renal failure Progressive rise in Stable elevated BUN and Cr BUN and Cr. History of ARF etiology History of chronic hypertension Normal growth Stunted growth Normal bones Renal osteodystrophy No broad urinary casts Broad waxy urinary casts Anemia usually mild Anemia usually severe Normal or enlarged kidneys Small shrunken kidneys Table 36.1 Classification of vesicoureteral reflux (VUR) Primary Congenital VUR resulting from malimplantation of the ureter in the bladder ~ associated with urinary tract infection Secondary Bladder outlet obstruction: = Posterior urethral valves = Bladder neck obstruction © Severe urethral stricture Neurogenic bladder: © Spina bifida—-meningomyelocele ‘Chronic bladder inflammation Urinary tract infection Traumatic: © Following bladder surgery © Following ureteral calculus extraction Table 36.2. Clinical conditions associated with a high risk for vesicoureteral reflux (VUR) Prenatal detection of hydronephrosis Febrile urinary tract infection Siblings of index patients with VUR Children of parents or close relatives with history of VUR Children with voiding dysfunction Children with high-grade urinary obstruction - including Hinman syndrome Maticystic dysplastic kidney-contralateral ureter pene ~Figure 36.5 Algorithm for diagnostic evaluation of urinary tract infection in children 2 months to 2 years. Table 36.4 Microbiologic criteria for positive urine culture Method of collection Threshold for diagnosis of UTI Suprapubic aspiration 1000 CFU/mi Catheterization of bladder 50 000 CFU/ml Midstream clean catch 100 000 CFU/ml Bagged urine culture UnreliableTable 36.6 Factors associated with increased risk for renal parenchymal scarring following acute pyelonephritis Urinary tract abnormalities: Vesicoureteral reflux Urinary obstruction Duplicated collecting system N Delay in treatment of acute pyelonephritis >48 hours 3. Recurrent acute pyelonephritis Table 24.8 Complications of acute renal filure Metabolic Cardiovascular Gastoinestnal Neurologic Hematologic Infectious Hypertalemia Pulmonary edema Nausea, vomiting, anorexia Altered mental status Anemia Preumonia Metabolic acidosis Aninythmias Malnutrition Iretataity Bleeding Sensis Hyponatremia Petcaritis Gastits Seizures Infected Vstes Hypocaleeria Myocarial infarction Gi bleeding Somnelence Hyperohosphatemia Hypertension Gh ulcers oma Table 24.9 Non-dialytic treatment of hyperkalemia Agent ‘Mechanism Dose Onsct of action Sodium polystyrene sulfonate Exchanges Na" fork zeros colonic mucosa Tl PO or PR with sorbitol 1-2hours (Calcium gluconate ‘Stabiizes the myocardial membrane potential Tmlfig IV over 5-18 minutes Immediate lucene and insulin Stimulates cellular uptake of K Glucose OS gg, 0 minutes insulin 0.1 unitsfkg aver 30 minutes [Beagoniss (albutcrl) ‘Stimulates collar uptake of K* '5-100mg dose by nebulizer 0 minutes Seaium bicarbonate ‘Shits K* into celts ‘mmol/kg W over 10 minutes 15 minutes Table 22.1 K/DOQI criteria for definition of chronic kidney disease in children? A patient has CKD if either of the following criteria are present: 1. Kidney damage for 23 months, as defined by structural or functional abnormalities of the kidney, with or without decreased GFR, indicated by one or more of the following ‘Abnormalities by kidney biopsy ‘Abnormalities based on imaging tests ‘Abnormalities in the composition of the blood or urine. 2. GFR <60mi/min/1.73m? for 23 month, with or without the signs of kidney damage listed above.KEY LEARNING POINTS © Urinalysis and microscopy should be done to confirm that red urine is due to haematuria. ‘© UTTis the commonest cause of frank haematuria. © The finding of isolated microscopic haematuria in the majority of children is short lived and does not require extensive assessment. © The finding of hacmaturia cither frank or microscopic, in association with significant proteinuria, hypertension or impaired renal function warrants further specialist investigation. RO Sy ‘© Ultrasound is the most useful and least invasive form of urinary tract imaging available. ‘* Radioisotope imaging has replaced IVU as second-line imaging of the urinary tract, particularly in the assessment of patients with UTI. ‘* Computed tomography and MRI imaging have very restricted indications. McuG per tract’ ymptoms, recur- R/renal scarring) ‘if abnormality onKEY LEARNING POINTS. © Clinical features of UTI are often non-specific and every young child with an unexplained fever should have a urine culture. + Ancgative combination dipstick urinalysis result does not exclude UTI particularly in infants. © All children should have a urinary tract ultrasound afer a first proved UTI. Subsequent investigation should be dictated by age, presenting symptoms and family history. © VUR is found in up to 30 per cent of children * Causes of generalized oedema « * 1 Renal: nephrotic syndrome; acute glomerulonephritis; r acute renal failure; chronic renal failure 5 Cardiac: congestive cardiac failure \ Hepatic: chronic liver disease; hepatic venous r outflow obstruction Neonatal: extreme prematurity; perinatal asphyxia; hyaline membrane disease; haemolytic disease; congenital lymphoedema; Turner syndrome; cystic fibrosis Evaluation of ncphrolithiasis and/or nephrocalcinosis © Ultrasound and abdominal X-ray. biochemical stone analysis suggests a cystine stone, the key investigation is urinary amino acid chromatography. If analysis suggests a uric ine ratio, plasma urat HGPRT and APRT. If analysis suggests a struvite stone, metabolic evaluation is unnecessary. * If the biochemical stone analysis suggests cal- jum oxalate/calcium phosphate or if there is no stone recovered, the following investiga should be carried out: — Urinalysis and pH = Urine culture— Urinary calcium, oxalate and urate creatinine ratios — Urinary citrate creatinine ratio — Urinary amino acid and organic acid screen _ If spot urines are abnormal, a second voided early morning urine sample should be taken and subsequently a 12-24 hour collection. - Plasma urea and electrolytes calcium, phos- phate, magnesium, parathyroid hormone, urate, 25 and 1,25 (OH), D, Table 12.4 Levels of management of steroid sensitive nephrotic syndrome, 1 Initial episode Prednisolone 60 mg/m? per day (max. 80 mg) for ‘one month Prednisolone 40 mg/m? on alternate days (max. 40 mg) for one month Progressive withdrawal by 10mg/m? each week over the next month 2. First two relapses Prednisolone 60 mg/m? per day (max. 80 mg) until remission followed by 40 mg/m? on alternate days. (max. 40 mg) for one month 3. Frequent relapser Prednisolone 0.1-0.5 mg/kg on allemate days for 3-6 months 4 Relapse on prednisolone —_Levamisola 2.5mg/kg > 0.5 mg/kg alternate alternate days for days 4-12 months 5 Continuing relapsing Cyclophosphamide 2 mg/kg course on prednisolone —_—per day for 12 weeks or >0.5 ma/kg altermate chlorambucil 0.2 mg/kg per days day for 8 weeks 6 Post-alkylating therapy ‘As 2-3 above relapse 7 Continuing relapsing Ciclosporin 5 mg/kg course on prednisolone —_—per day for 12-24 months > 0.5 me/kg alternate daysKEY LEARNING POINTS The majority of children with polydipsia do not have impaired urinary concentrating ability. Children with renal tubular disease present with non-specific symptoms in early life and may experience significant clinical dehydration associated with relatively trivial intercurrent illness. * Chronic constipation, growth retardation and rickets are classic features of 2 renal Fancon Bytes KEY LEARNING POINTS «The symptoms of CRF are very non-specific but growth failure, unexplained anaemia and polyuria are common. + Hypertensive encephalopathy and congestive cardiac failure may be the first presentation of advanced renal failure. © Management of the child with chronic and endstage renal failure is multidisciplinary. © Successful renal transplantation is the treatment of choice for children with endstage renal failure. « Living related renal transplant donation is commoner in childhood endstage renal failure. Table 12.41 Differential diagnosis of metabolic acidosis High plasma anion gap Normal plasma anion gap Ketoacidosis ‘Gastrointestinal HCO; loss eg. diabetic, (+ve uAG) starvation 2g. diantoea, bowel augmentation, cystoplasty Renal failure Renal HCO; loss (—ve UAG) @g. renal tubular acidosis, acetazolamide, Fanconi syndrome Other ‘Other e.g. intoxication eg. total parenteral nutrition, ‘with ethylene giycol, cholestyramine, adrenal methanol, salicylate, insufficiency lactic acidosis either primary or secondary: ‘to circulatory failureUUme OCT Lead ee a] Glomerulonephrtis Nephrotic Syndrome Postinfectious (both streptococcal as well as, ‘Minimal change nephrosis other bacteria viruses, and parasites) Henoch-Schénlein nephritis Focal segmental glomeruloscerosis Immunoglobulin A nephropathy ‘Membranoprotterative Membranoprolieratve glomerulonephritis ‘Membranous nephropathy Familial nepbitis Congenital nephrotic syndrome Systemic lupus erythematosus ‘Systemic lupus erythematosus Immune complex nephritis (infective endocarditis Henoch-Schanlein nephritis or *shunt nephritis”) Rapidly progressive glomerulonephritis (Wegener Immunoglobulin A nephropathy ‘granulomatosis or polyartertis nodosa) Familial nephritis aa Parameter Prerenal Renal Uyz—random mEq/L <0 >40 FEya* t% Utne osmolality (msm) 2800 a0Antenatal hydronephrosis (APD 25 mm) | Postnatal ultrasound 48-72 hours after birth a No hydronephrosis * Prophylactic antibiotics Hydronephrosis detected (APD <7 mm) —_ are not indicated (APD 27 mm) + Counsel all families onUTI signs and | ‘symptoms voua Repeat ultrasound 4-6 weeks of age awa. VUR present VUR absent No hydronephrosis Hydronephrosis detected (APD <7 mm) (APD 27 mm) Monitortor Diuretic | | UTI renography No turer vous testing required (isolated antenatal hydronephrosis) vr present VUR absent Monitor for Diuretic UTI renography aa STL) Type 1 (Classic, Type 2 Type 4 (Aldosterone Distal) (Proximal) Deficiency) Growth failure +t ++ 44+ Serum potassium Normal or low Normal or low High Nephrocalcinosis Frequent Rare Rare Low citrate excretion ++ + + Fractional excretion of <5% 5%-10% <10% filtered HCO at normal serum HCOs levels Daily alkali treatment 1-3 5-20 1-3 (mEq/kg) Daily potassium Decreases with Increases with requirement correction correction Urine pH 55 <55 <55 Presence of other Rare Common Rare tubular defects:Urine Anion Gap a ‘Negative (Adequato urine NH,-) Positive (Low urine NH,") Proximal RTA or Urine pH and Plasma K* Gl bicarbonate loss _ ~ Urine pH >5.5 and Utine pH <5.5 and Jow-nomal K high Distal RTA Type 4 RTA, Figure 13-4. Diagnosis of renal tubular acidosis in patients with typerchioremic metabolic acidosis and ‘normal serum anion cao. (Adaoted trom Lash JP. Arruda JA’ Laboratory evaluation of renal tubular Pa a CM 1. Escherichia coll causes 90% of cases. 2. Antibiotic sensitivity testing is important because of the increasing incidence of ampicillin- resistant E. cat 3. Infections may be caused by ascending bacteria from the urethral area, 4. Glean-bagged specimens are unreliable for diagnosis because of their high contamination rate, 5. Uncircumeised male infants have a 10-fold greater risk forinfection than circumcised male infants. KEY POINTS: VESICOURETERAL REFLUX 1. Spontaneous resolution more likely with grades | to II 2. Thirty percent of these patients will have siblings with vesicoureteral reflux, but sequelae are rare, 3. About 30% of white children with urinary tract infections will have vesicoureteral reflux (less in certain racial groups). 4, Prophylactic antibiotics are intended to prevent pyelonephritis and to limit renal scarring and hypertension, but their utility in this regard is being questioned.Febrile female infant aged 3 to 24 months with no known urinary tract abnormalities Difference between Nephritic syndrome « Nephrotic syndrome Nephritie Syndrome Nephrate Syndrome ‘Age of onset Usually oder children (School aged) | Usually younger children (Pre-achool children) Proceeding case | Preceeding URTT or Pyoderma may be Present > Clinical features | Oiguria, Edema, Hematusia,hyperten- | Anasarca, Oliguria (Hemataria and hyperts- sion sion sere) > Renal fanction | RFT-may beadversely afected RFT rarely affected in Minimal change Tests (RFT) 2 Hyponatremia and Hyperkatemia | nephrotic eyndrome may be present o BUN/Creatinine may be increased > cBc Hidecreased dus to hemodilution | HIb/WBC increace duo to hemoconcentation > ESR owally between 50-100 Voually > 100 UTI~10% 0 25% Probabilly of UTI ~3% 10 8% \ UTI <2% / Urine dipstick Urine dipstick Urine dipstick Urine dipstick nitrite and nitrite and nitrite and nitrite and leukocyte esterase | leukocyte esterase leukocyte esterase | loukocyte esterase: negative (LR =0.2) | positive (LR.= 28) negative (LR =0.2) | positive (LR = 28) Probability of UTI | Probability of UTI Probability of UTI_| Probability of UTI ~2% to 6%. ~75% to 90% <2% ~46% t0 71% Urine dipstick Urine lipstick nitrite or nitrite or leukocyte esterase leukocyte esterase positive (LR = 6) positive (LR = 6) Probability of UTI Probability of UTI ~40% 10 66% ~15% t0 34% Figure 13-5. Diagnostic algorithm for febrile female infants aged 8 to 24 months suspected of having a trnary tract infection, LR = likelihood ratio. (From Shaikh N, Morone NE, Lopez J: Does this child have a urinary tract infection? JAMA 298:2902, 2007)