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107 Cards in this Set
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DDx false negative proteinuria by dipstick?
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- LMW compounds may be missed d/t specificity for albumin
- Dilute urine |
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DDx false positive proteinuria by dipstick?
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- Alkaline urine
- Contaminants: chlorhexidine, radiocontrast |
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What level of proteinuria by dip is abnormal?
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1+ may be due to concentrated urine (>1.015)
2+ is always abnormal |
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Usual presenting sx of Alport syndrome?
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Young boy with isolated hematuria (never proteinuria without hematuria)
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Timeframe for occurrence of PSGN?
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7-21d after clinical sx of pharyngitis
Sx include hematuria/proteinuria and variable azotemia, HTN, edema |
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Sx of minimal change disease?
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Nephrotic syndrome
- 3-4+ proteinuria - Edema |
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Typical age range of orthostatic proteinuria?
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Rare <6 yrs old, common in adolescence
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DDx transient proteinuria?
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Stress, dehydration, exercise, fever
Proteinuria a/w exercise is mild (2+) and resolves after 48h rest Fever-associated proteinuria occurs >38.6 and resolves when fever resolves (repeat UA in 2-4 weeks when asx to document resolution) |
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DDx gross hematuria?
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Upper (kidney) - cola-colored urine without clots
Lower (ureter, bladder, urethra) - BRB +/- clots, sometimes with flank pain, frequency, dysuria |
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Renal complications of sickle cell trait?
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Painless, gross hematuria with clots, no trauma, +Fhx SCD, normal renal function, normal CBC
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Hematuria - how to r/o nephrolithiasis?
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Urine calcium, Urine Cr (isolated hypercalciuria without stones is rare)
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DDx hematuria:
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1) Glomerulonephritis - screen w/C3
2) Nephrolithiasis - screen with UCa, UCr 3) Hemorrhagic cystitis - a/w urgency, frequency and dysuria |
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Sx of HUS?
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1) Diarrheal prodrome (+/-)
2) Hemolytic anemia 3) Thrombocytopenia 4) Acute renal failure (oliguria) |
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Rx HUS?
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1) Supportive - careful fluid, electrolyte mgmt (avoid complications of hypoNa, hyperK, hyperPhos, fluid overload) - Make sure to consider insensibles (500mL/m2/day, plus urine/stool replacement ml/ml)
2) PRBCs if needed for anemia (plt transfusions may exacerbate microthrombi) |
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D/o's a/w schistocytes on peripheral smear?
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HUS - generally after illness
TTP - defects in ADAMTS13, older than HUS patients, CNS involvement common SLE |
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Lab tests suggestive of HUS?
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- Peripheral smear with schistocytes, burr cells, thrombocytopenia
- UA: hematuria, proteinuria - Elevated LDH - Elevated retic - Decreased haptoglobin/Hb/plts |
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Best test for HUS?
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CBC with peripheral smear - should see schistocytes, burr cells, thrombocytopenia
(Coombs test will be negative, not a/i mediated) |
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Sx HSP?
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1) Purpuric rash, generally extensor surfaces (esp buttocks, LE's)
2) Arthralgias/arthritis 3) GI sx: abd pain, hematochezia, intussusception (d/t submucosal bleeding) 4) Immune-complex-mediated GN - may have asx hematuria +/- proteinuria or nephrotic syndrome/acute GN (severe) |
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Sx acute GN?
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Range: cola-colored urine with normal renal fcn, versus severe GN with marked azotemia
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Worst px HSP?
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Initial presentation w/severe nephrotic syndrome, nephritis - although ESRD is uncommon in children
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Mgmt HSP?
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Recognize renal abnormalities early, reduce long-term morbidity
- If normal UA on presentation, check UA weekly until sx abate (monitor for development of proteinuria) - If >/= 2+ proteinuria (or Upr:UCr >.2) refer to Neph for renal bx Corticosteroids not indicated Use NSAIDS with great caution |
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DDx seizure in patient undergoing chronic dialysis?
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1) Electrolyte abnormalities (sodium rarely, hyperMg not seen, hypoglycemia but not a/w dialysis) most-likely hypoCa (due to hyperPhos, low 1,25-OH vitD)
2) Severe HTN (often d/t volume overload) |
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Sx hypertensive encephalopathy/PRESS?
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Severe HTN
- Often d/t inability to autoregulate and vasoconstrict, resulting in hyperperfusion, cerebral edema (generally occurs acutely in pts with no hx HTN) Seizure HA/vomiting AMS Vision changes |
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Rx hypertensive encephalopathy/PRESS?
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- Gradual reduction of BP (normalization over a few days) - if pt has chronic HTN, too-quick correction can result in cerebral infarction, transverse ischemic myelopathy, blindness
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Sx Williams syndrome?
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Hypercalcemia (infancy) - can cause hypercalciuria/nephrocalcinosis, resolves by 1-2 yrs
Intellectual disability with fluent speech, good social skills Supravalvular aortic stenosis (+/- coarct, PA stenosis, aortic hypoplasia) Renal artery stenosis (HTN) *Due to elastin gene mutations |
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Sx acute GN?
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Cola-colored urine
+/- associated sx (ex: sore throat) Trace edema Mild HTN Hematuria (+rbcs) Proteinuria (>2+) Azotemia (elevated Cr) |
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Defining lab test for nephritic syndrome?
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Elevated serum Cr
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Defining test for hypocomplementemic nephritis (e.g., post-infectious GN)?
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Low C' component 3 (C3)
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Concurrent pharyngitis, nephritis - dx?
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Synpharyngitic hematuria, often d/t IgA nephritis
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IgA nephritis - sx?
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Painless hematuria
Often concurrent pharyngitis Normal C3 |
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What is the best initial workup for children with gross hematuria?
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Renal/bladder u/s to evaluate for structural abn
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Workup for hematuria with clots?
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Check for structural causes:
- Ruptured cyst (PCKD types) - Renal mass (e.g., Wilms) - Stone - Venous thromobosis - Papillary necrosis - Hypercalciuria Bleeding d/o's: - SCD, von-Willebrand's Lower UG tract: - Hemorrhagic cystitis - Bladder calculi - Tumor |
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What to monitor if patient has cola/tea-colored urine?
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(Dx: GN)
- Close BP monitoring - Renal fcn monitoring - Check C3 - single best dx test in evaluation of acute GN (narrows dx to hypo/normocomplementemic) - If Cr rising, normal C3, or suspected SLE, refer for renal bx |
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Alport syndrome sx:
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1) Microscopic hematuria in young males
2) Progression to ESRD by age 30 (50-90%) 3) High-frequency sensorineural hearing loss in 50% 4) Ophtho findings: anterior lenticonus most common **Females may have mild, asx hematuria |
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Alport syndrome - heritance, defect, rx?
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X-linked (85% of cases)
COL4A5 gene - mutation encodes alpha-5 chain of type IV collagen 50% male children from female carriers affected - Rx is focused on BP control, reduction of proteinuria (ACEI's); if ESRD, consider renal transplant |
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Male with asx microscopic hematuria, +Fhx - ddx?
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Alport (X-linked, hearing loss, anterior lenticonus)
Thin GBM disease (males=females, familial, favorable px) |
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AR d/o
Chronic tubulointerstitial disease Sx include polyuria, polydipsia, anemia, azotemia ABSENT hematuria, proteinuria |
Juvenile nephronophthisis
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X-linked d/o
Congenital cataracts Fanconi syndrome (metabolic acidosis, glycosuria, hypophosphatemia, hypokalemia, amino aciduria) Intellectual disability |
Lowe syndrome
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Microscopic hematuria in patient on ketogenic diet - best guess dx?
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Hypercalciuria/nephrolithiasis
- Patients on ketogenic diet should receive empiric potassium citrate irrespective of presence/absence of hypercalciuria |
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Dx of hypercalciuria - how to screen?
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1) Random UCa/UCr - easier but less reliable b/c Ca can be influenced by diet
2) 24 Urine Ca, Cr, Na, K, Citrate collection |
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Nocturnal enuresis without daytime sx - best rx?
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Enuresis alarm
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Nocturnal enuresis with daytime sx of urgency, frequency - best rx?
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Anticholinergic - ex: oxybutynin
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Risk of affected child with enuresis if present in parents?
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40-45% if 1 parent affected; 75% if both parents affected
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6y F with daytime/nocturnal enuresis, +UTI on exam - next rx?
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Renal/bladder u/s
- Screen for hydronephrosis, stones DMSA scan useful as f/u after pyelo to eval for scarring MRI lumbar spine useful to look for tethered cord in child with acquired neurogenic bladder (ex: new-onset constipation, gait disturbance) Spiral CT abdomen can eval for renal stone if u/s negative and stone suspected |
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DDx enuresis (daytime/nocturnal)?
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UTI (hydronephrosis, stone)
Neurogenic bladder OSA - adenoidectomy a/w significant improvement of enuresis |
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DDx normocomplementemic GN?
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IgA nephritis
MPGN (1/3 normoC' at presentation) Alport syndrome HSP ANCA+ dz (Wegener's, microscopic polyangiitis) |
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DDx hypocomplementemic GN?
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Post-infectious (PSGN)
MPGN (2/3 hypoC' at presentation, +/- low C4) SLE nephritis (+ ANA, dsDNA) |
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Initial w/u of acute GN?
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UA + micro
Renal function tests (BUN/Cr) Electrolytes C3 ANA dsDNA |
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Classic sx of nephritic syndrome?
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Painless hematuria (cola-colored urine w/o clots)
H/o decreased urine output HTN Facial/pretibial edema |
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Name the disease: FT infant, respiratory distress, butterfly vertebrae, hypoplastic thumbs, large VSD, feeding/stooling ok
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VATER/VACTERL association is a good guess!
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VATER/VACTERL association: name the components...
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Vertebral anomalies
Anal atresia TE fistula Renal/Radial defects (or) Vertebral anomalies Anal atresia Cardiac defects TE fistula Renal anomalies Limb (radial) anomalies |
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DDx hypoplastic thumbs:
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VATER/VACTERL association
Thrombocytopenia Absent Radii (TAR) Fanconi anemia Rothmund Thompson syndrome |
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Common UA finding in acute tubular necrosis?
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Isosthenuria (cannot concentrate urine - SpGr generally 1.010 regardless of context) due to tubular injury
May also see hematuria, proteinuria |
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What rx for patient with bilateral renal artery stenosis?
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Beta-blocker is best choice - decrease HR (increase diastolic filling, stroke volume), minimize renin-angiotensin effects
**Avoid ACEIs, ARBs due to risk of drop in renal function, risk of renal failure -Diuretics may help as 2nd line rx, as may CCBs, but pick beta-blockers first (ex: atenolol) |
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Creatinine values in adolescents - what is the pattern?
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Males=females until early adolescence, when increased muscle mass develops in males
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When does the pediatric GFR reach adult GFR values?
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~18 months of age
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How is the serum creatinine in neonates?
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Elevated; this is reflective of maternal values - this generally returns to infant values (0.2-0.4mg/dL) after 10 days of age
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What are the levels of serum creatinine in preterm infants (<36wks gestation)?
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Higher than term infants (term infants are generally 0.2-0.4mg/dL)
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Diagnostic criteria for ADPKD in patients <30:
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>1 cyst in either kidney, or 1 cyst in both kidneys
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6y F toilet trained, with daytime, nocturnal enuresis despite negative exam, no urgency/frequency, negative UA, no stressors - dx?
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Ectopic ureter - 6x more common in females than males (often seen w/duplicate collecting system)
- Generally manifests as constant wetness in an otherwise healthy female who is toilet trained - Dx eval: u/s may miss, pursue further dx studies if highly suspected |
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Diagnostic evaluation of ectopic ureter?
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Magnetic resonance urography (MRU) to demonstrate small, dysplastic renal unit with ectopic ureter/location of ureteral orifice
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Dysuria, frequency, urgency and pyuria on UA with negative bacterial cultures - dx?
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Viral cystitis
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UTIs in first postnatal year due to urinary stasis - dx?
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Ureterocele - often seen with duplicate collecting system; seen on u/s as "mass in the bladder"
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Polyuria, polydipsia, short stature, azotemia, anemia, proteinuria - dx?
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Juvenile nephronophthisis (NPH)
AR d/o characterized by chronic tubulointerstitial disease, progresses to azotemia, and ultimately ESRD Dx based on clinical sx |
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Disorders a/w juvenile nephronophthisis (NPH)?
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- Senior-Loken syndrome: tapetoretinal degeneration, retinitis pigmentosa, blindness, nystagmus, coloboma, cataracts
- Joubert syndrome: NPH with aplasia of the cerebellar vermis (ataxia), retinal coloboma/retinitis pigmentosa, hepatic fibrosis, skeletal defects (cone-shaped epiphyses) |
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Retinitis pigmentosa with renal disease - best guess?
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Juvenile nephronophthisis-related d/o's (Senior-Loken, Joubert's)
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Fanconi syndrome:
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Glycosuria, metabolic acidosis, aminoaciduria, hypoPhos
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Lowe syndrome:
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Fanconi syndrome, mental retardation, congenital cataracts,
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Fanconi syndrome, FTT, cystine crystal deposition in the cornea resulting in severe photophobia
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Nephropathic cystinosis (lysosomal storage disorder)
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Nephropathic cystinosis:
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Fanconi syndrome, FTT, cystine crystal deposition in the cornea resulting in severe photophobia
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4y F UTI 1m ago, now VCUG shows smooth-walled bladder, absence of VUR, mildly narrowed urethra - rx?
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None; no need for repeat VCUG, mildly narrowed urethra is normal variant
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DDx red urine?
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Hematuria
Hemoglobinuria Myoglobinuria Porphyrinuria UTI pathogens (e.g., Serratia) Foods - beets, blackberries, food dyes Medications - deferoxamine, rifampin, phenolphthalein |
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Next step for patients with primary glomerulopathy (nephrotic syndrome) who are steroid-unresponsive?
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Renal bx - establish histo of disease process
Rx include cyclosporine, maybe tacrolimus (2nd/3rd-line rx at present) |
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Rx for primary glomerulopathy resulting in nephrotic syndrome?
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Corticosteroids - 90% response rate after 4-6 week trial
(50-70% may experience at least 1 relapse during disease course) |
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Secondary complications of NF1?
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- HTN (idiopathic) although renal artery stenosis or pheochromocytoma may be present
- Optic pathway tumors - Skeletal deformations - Speech impediments - Learning disabilities - Short stature - Constipation - Chronic HA |
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Features of nephrotic syndrome that suggest secondary (other dz related) glomerulopathy?
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Moderate/severe HTN
Gross hematuria Azotemia Hypocomplementemia +ANA +Hep B, Hep C or HIV |
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Causes of nephrotic syndrome?
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Primary glomerulopathies (minimal change disease, focal segmental glomerulosclerosis, mesangial proliferative GN)
Secondary glomerulopathies (SLE nephritis, MPGN due to Hep C, HIV nephropathy) |
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Vitamin D status in chronic renal failure?
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Low nephron mass leads to decreased activity of 1-alpha hydroxylase activity, and decreased conversion of 25-OH vitamin D to 1,25-OH vitamin D
- Low 1, 25-OH vitamin D is associated with phosphorus retention and secondary hyperparathyroidism, causing renal osteodysrophy (renal rickets) |
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Best test to differentiate between acute and chronic renal failure?
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Kidney size: acute renal failure is a/w inflamed, enlarged kidneys, while chronic renal failure is associated w/ small, shrunken kidneys on u/s
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Fatigue, growth failure, elevated BUN/Cr, HTN, anemia: dx?
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Chronic renal failure - characterized by marked loss of excretory function
- IGF-1 concentrations are actually increased, but high IGF-binding protein concentrations prohibits binding/function |
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If hypercalciuria is present, what other labs to check?
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Serum Ca/Phos, iCa, PTH, 25-OH vit D and 1,25-OH vit D; also consider eval for RTA
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UTI pathogens a/w urolithiasis?
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Proteus - struvite stone formation
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Risk factors for renal stones:
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Hypercalciuria, hyperoxaluria, decreased urine Mg, decreased urine citrate
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How to differentiate central vs nephrogenic DI?
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In DI, Uosm remains constant during H20 deprivation while weight, Sosm decreases. After aVP given, failure to concentrate urine indicates nephrogenic (vs central) DI
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Nephrogenic DI - heritance, etiology?
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- X-linked in 90% of cases
- Arginine vasopressor type 2 receptor (V2R gene) |
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Electrolyte abnormalities in children with CKD?
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HypoCa, hypo 1,25-OH vit D - both result from Phos retention, impaired 1-hydroxylation (usually on rx for Ca, vit D)
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Stridor in child with CKD - best guess dx?
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Hypocalcemia - due to hyperPhos, bicarb therapy resulting in metabolic alkalosis
- Sx include perioral/extremity paresthesias, carpopedal spasm, laryngospasm, seizure |
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Sx hyperkalemia?
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Muscle weakness, paresthesias, cardiac conduction abnormalities
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Sx hyperMg?
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Generally asx
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Sx hypoPhos?
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Proximal muscle weakness, cardiac dysfunction, ataxia, seizures, coma
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Common renal sequelae of ifosfamide chemotherapy?
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Fanconi syndrome (glycosuria, phosphaturia, aminoaciduria, proteinuria and altered GFR)
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Common renal sequelae of cisplatin chemotherapy?
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HypoMg and hypoK - due to tubular damage; may also see polyuria, azotemia
Altered renal function, Mg wasting may persist after rx completed |
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Sx hyponatremia?
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Nausea, vomiting, hypothermia, lethargy, agitation, HA, seizure
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HypoK, hypoMg, hypoCl, metabolic alkalosis - ddx?
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Bartter's syndrome, Gitelman's syndrome - check urine Cl for dx
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Polydipsia, polyuria, PHOTOPHOBIA, hypokalemia, metabolic acidosis, hypoPhos: dx?
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Nephropathic cystinosis - see cystine accumulation on slit lamp eval; also a/w hypothyroidism
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Most common cause of Fanconi syndrome in pediatrics?
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Nephropathic cystinosis: lysosomal storage d/o resulting in cystine accumulation, cellular destruction
AR, CTNS gene affected |
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Decreased urinary Na, K, HCO3, Phos, AAs, protein, glucose... dx?
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Fanconi syndrome (proximal tubulopathy)
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When to admit for HTN?
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HA, vision changes, epistaxis, seizure, pulmonary edema with CHF, HTN d/t renal failure
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First line rx for inpatient HTN relief?
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Vasodilators (CCBs, nitroprusside, hydralazine)
Alternatively, short-acting beta-blocker can be used acutely (CONTRAINDICATED in ASTHMA) *These rx can be titrated quickly to therapeutic BPs |
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First line rx for outpatient (asx) HTN relief?
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Diuretics (thiazide), ACEIs, ARBs
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Hydronephrosis:
1) VCUG can r/o? 2) MAG-3 radioisotope can r/o? |
1) R/o VUR, bladder ureterocele
2) R/o UPJ obstruction - lasix may precipitate renal colic!! |
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Dx hydronephrosis within infancy?
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UPJ obstruction
UVJ obstruction Single-system ureterocele VUR Posterior urethral valves (usually causes bilateral hydronephrosis) |
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Plan for acute, isolated microscopic hematuria (w/o edema, HTN or proteinuria)?
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Repeat in 1-2 weeks
If persists, check BUN/Cr, CBC, ESR, C', ANA, anti-dsDNA |
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Discomfort in infant after furosemide given for MAG-3 radioisotope scan - dx?
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UPJ obstruction - lasix precipitates "renal colic"
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Best guess - palpable abdominal mass in newborn infant?
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Usually d/t kidney:
- Multicystic dysplastic kidney - Unilateral MCDK = 1 functional kidney (but 30% of functioning kidneys have VUR - impt to dx early!) - Hydronephrosis due to UPJ obstruction |
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Renal scintigraphy also =
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MAG-3 radioisotope scan, often done with diuretic (ex: furosemide)
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% of patients with nephrotic syndrome that may also have microscopic (NOT gross) hematuria?
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20% - also, mild azotemia is occasionally seen (as well as mild BP elevation) but not as significant as in nephritic syndrome
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