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Toronto Notes 2019 Nephrology Pediatrics P71 Nephrology
Common Pediatric Renal Diseases
Table 33. Common Manifestations of Renal Disease
Neonate
Flank Mass
Hematuria Anuria/Oliguria
Child and Adolescent Cola/Red-Coloured Urine
Gross Hematuria Edema
HTN
Polyuria
Proteinuria Oliguria Urgency
Common Causes
Hydronephrosis, polycystic disease (autosomal dominant or recessive subtypes), tumour Renal vein thrombosis, asphyxia, malformation, trauma
Bilateral renal agenesis, obstruction, asphyxia
Acute GN (post-streptococcal, HSP, IgA nephropathy, etc.), hemoglobinuria (hemolysis), myoglobinuria (rhabdomyolysis)
Urologic disease (nephrolithiasis, trauma, etc.), UTI, acute GN
Nephrotic syndrome, nephritis, acute/chronic renal failure, consider cardiac or liver disease
GN, renal failure, dysplasia (consider coarctation, drugs, endocrine causes)
DM, central and nephrogenic DI, renal Fanconi’s syndrome (genetic/metabolic/acquired causes), hypercalcemia, polyuric renal failure (renal dysplasia)
Orthostatic, nephrotic syndrome (MCD, etc.), GN
Dehydration, ATN, interstitial nephritis, acute or chronic kidney disease (i.e. renal failure) UTI, vulvovaginitis
Hemolytic Uremic Syndrome
Definition
• simultaneousoccurrenceofthetriadof1)non-immunemicroangiopathichemolyticanemia, 2) thrombocytopenia, and 3) acute renal injury
Epidemiology
• annualincidenceof1-2per100,000inCanada
• mostcommoncauseofacuterenalfailureinchildren
Etiology
• diarrheapositiveHUS:90%ofpediatricHUSfromE.coliO157:H7,shigatoxin,orverotoxin • diarrheanegativeHUS:otherbacteria,viruses,drugs,familial/genetic
Pathophysiology
• toxinbinds,invades,anddestroyscolonicepithelialcells,causingbloodydiarrhea
• toxinentersthesystemiccirculation,attachesto,andinjuresendothelialcells(especiallyinkidney),
causing a release of endothelial products (e.g. von Willebrand factor, platelet aggregating factor)
• platelet/fibrinthrombiforminmultipleorgansystems(e.g.kidney,pancreas,brain,etc.)resultingin
thrombocytopenia
• RBCsareforcedthroughoccludedvessels,resultinginfragmentedRBCs(schistocytes)thatare
removed by the reticuloendothelial system (hemolytic anemia)
Clinical Presentation
• initialpresentationofabdominalpainanddiarrhea,followedbybloodydiarrhea;within5-7dbeginsto show signs of anemia, thrombocytopenia, and renal insufficiency
• pallor,jaundice(hemolysis),edema,petechiae,HTN
Investigations
• CBC(anemia,thrombocytopenia),bloodsmear(schistocytes),electrolytes,renalfunction,urinalysis (microscopic hematuria), stool cultures, and verotoxin/shigella toxin assay
Management
• mainlysupportive:nutrition,hydration,ventilation(ifnecessary),bloodtransfusionforsymptomatic anemia
• monitorelectrolytesandrenalfunction:dialysisifelectrolyteabnormality(hyperkalemia)cannotbe corrected, fluid overload, or uremia
• steroids are not helpful
• antibioticsarecontraindicatedasdeathofbacterialeadstoincreasedtoxinreleaseandworseclinical
course
Prognosis
• deathin<5%ofcases,5-25%long-termrenaldamage(HTN,proteinuria,decreasedrenalfunction)