Nephrology Flashcards
(200 cards)
1
Q
- How is neonatal AKI classified and staged?
A
Classification: Modified KDIGO staging
- Based on serum creatinine rise and urine output reduction
2
Q
- What are the key differences between prerenal, intrinsic, and postrenal AKI?
A
Prerenal: Decreased perfusion (FeNa <1%)
- Intrinsic: Tubular or glomerular damage (FeNa >2%)
- Postrenal: Obstruction (hydronephrosis on imaging)
3
Q
- What are the key laboratory findings in prerenal AKI?
A
Findings: BUN:Cr ratio >20:1, low urine sodium (<20), high urine osmolality, low FeNa (<1%)
4
Q
- What is the management of neonatal AKI?
A
Management: Fluid and electrolyte balance, avoid nephrotoxins, diuretics if fluid overload, dialysis if refractory
5
Q
- What are the stages of AKI based on KDIGO criteria?
A
KDIGO stages:
Stage 1:
Serum Creatinine: ↑ by ≥0.3 mg/dL within 48 hrs OR 1.5–1.9× baseline
Urine Output: <0.5 mL/kg/h for 6–12 hrs
Stage 2:
Serum Creatinine: 2.0–2.9× baseline
Urine Output: <0.5 mL/kg/h for ≥12 hrs
Stage 3:
Serum Creatinine: ≥3.0× baseline OR ≥4.0 mg/dL OR initiation of renal replacement therapy
Pediatrics: eGFR <35 mL/min/1.73 m²
Urine Output: <0.3 mL/kg/h for ≥24 hrs OR anuria ≥12 hrs
6
Q
- What is the role of fractional excretion of sodium (FeNa) in AKI evaluation?
A
FeNa: Helps distinguish types of AKI
- <1% in prerenal, >2% in ATN
- Not reliable if on diuretics
7
Q
- How is neurogenic bladder managed in pediatric patients?
A
Management: Clean intermittent catheterization (CIC), anticholinergics, monitor renal function and bladder pressures
8
Q
- What conditions are associated with abnormal flow curves in uroflowmetry?
A
Abnormal curves: Plateau (obstruction), staccato (intermittent contraction), interrupted (underactive bladder)
9
Q
- What is the initial treatment for overactive bladder in children?
A
Initial treatment: Timed voiding, behavioral therapy, fluid optimization, constipation management
10
Q
- What pharmacologic agents are used for refractory overactive bladder?
A
Pharmacologic agents: Oxybutynin, solifenacin, tolterodine (anticholinergics)
- Used after behavioral failure
11
Q
- What is the role of a bladder and bowel dysfunction (BBD) program in pediatric nephrology?
A
BBD program: Integrated approach for constipation, voiding dysfunction, and recurrent UTIs
- Improves urinary continence and prevents renal damage
12
Q
- What are red flags in a child presenting with enuresis?
A
Red flags: Daytime incontinence, abnormal stream, recurrent UTIs, poor growth, back pain, spinal stigmata
13
Q
- What is monosymptomatic nocturnal enuresis and how is it treated?
A
Monosymptomatic: Enuresis without daytime symptoms
- Treat with reassurance, motivational therapy, enuresis alarm, desmopressin if >6 years
14
Q
- What is the initial evaluation of a child with enuresis?
A
Evaluation: History, voiding diary, urinalysis, screen for constipation and UTI
- Ultrasound if abnormal exam or daytime symptoms
15
Q
- How is CKD staged in pediatrics?
A
Staging (KDIGO):
- G1: ≥90, G2: 60–89, G3a: 45–59, G3b: 30–44, G4: 15–29, G5: <15 mL/min/1.73m²
16
Q
- How is anemia of CKD managed?
A
Management: Iron supplementation, erythropoiesis-stimulating agents (ESAs)
- Target Hgb ~11–12 g/dL
17
Q
- What is the management of mineral bone disease in CKD?
A
Management: Phosphate binders, active vitamin D analogs (calcitriol), monitor calcium/phosphate/PTH
18
Q
- What is the role of dietary phosphate restriction in CKD?
A
Dietary phosphate restriction: Reduces hyperparathyroidism and bone disease
- Limit processed foods and dairy
19
Q
- What is the impact of growth hormone therapy on renal function in CKD?
A
GH therapy: Stimulates linear growth in CKD
- No major effect on GFR
- Improves final adult height if started early
20
Q
- What is the first-line antihypertensive agent in children with proteinuric CKD?
A
First-line: ACE inhibitors or ARBs
- Reduce proteinuria and delay CKD progression
21
Q
- What is the definition of chronic kidney disease (CKD) in children?
A
CKD: Kidney damage or GFR <60 mL/min/1.73m² for ≥3 months
- Includes structural or functional abnormalities
22
Q
- What vaccinations are recommended in children with CKD?
A
Recommended: Hepatitis B, pneumococcal, influenza, varicella (live vaccines if not immunosuppressed)
23
Q
- What are the effects of chronic metabolic acidosis on pediatric bone and growth?
A
Effects: Impaired growth, rickets, osteopenia
- Due to bone buffering and resistance to GH and IGF-1
24
Q
- What are the signs of chronic metabolic acidosis in children?
A
Signs: Poor growth, rickets, bone pain, muscle weakness
- Often seen in chronic RTA or CKD
25
37. What is thin basement membrane disease and how is it differentiated from Alport syndrome?
Thin basement membrane disease: Benign familial hematuria
- EM: Uniformly thinned GBM, no splitting
- Normal renal function, no hearing loss
26
128. What are the renal findings in Lowe syndrome?
Lowe syndrome: X-linked disorder with cataracts, hypotonia, and Fanconi syndrome
- Caused by OCRL1 mutation
27
62. What genetic mutations are associated with nephronophthisis?
Genes: NPHP1 most common
- Other: NPHP2–20
- Ciliopathy disorders
28
106. What are the features of Gitelman syndrome?
Gitelman: Hypokalemia, metabolic alkalosis, low magnesium, low urinary calcium, normal BP
29
105. What are the features of Bartter syndrome?
Bartter: Hypokalemia, metabolic alkalosis, normal BP, high renin and aldosterone, hypercalciuria
30
166. What is hepatorenal syndrome in pediatrics?
Hepatorenal syndrome: Functional renal failure in severe liver disease
- Due to splanchnic vasodilation and renal vasoconstriction
31
167. How is hepatorenal syndrome diagnosed in children?
Diagnosis: Exclusion of other causes, history of liver failure, low urine sodium, oliguria, high serum creatinine
32
168. What is the pathophysiology of hepatorenal syndrome?
Pathophysiology: Portal hypertension → NO-mediated vasodilation → renal hypoperfusion and vasoconstriction
33
108. What is the treatment for Bartter and Gitelman syndromes?
Treatment: Potassium and magnesium supplementation, NSAIDs for Bartter, thiazide avoidance in Gitelman
34
36. What is the role of electron microscopy in diagnosing Alport syndrome?
Role: Shows GBM thickening, splitting, and lamellation (basket-weave appearance)
- Diagnostic of Alport syndrome
35
127. What is cystinosis and how does it present renally?
Cystinosis: Lysosomal storage disorder
- Presents with Fanconi syndrome, photophobia, hypothyroidism, and progressive CKD
36
34. What are the features of Alport syndrome?
Features: Hematuria, progressive renal failure, hearing loss, anterior lenticonus
- X-linked or autosomal inheritance
37
191. What are the renal manifestations of mitochondrial disorders in children?
Mitochondrial disorders: Proximal tubulopathy, renal tubular acidosis, nephrotic syndrome
- May present with Fanconi syndrome
38
35. What is the genetic defect in Alport syndrome?
Genetic defect: COL4A5 (X-linked), COL4A3/4 (AR or AD)
- Affects type IV collagen in GBM
39
12. What genetic mutations are associated with SRNS?
Mutations: NPHS1 (nephrin), NPHS2 (podocin), WT1, LAMB2
- Often familial and early onset
40
138. What imaging modalities are used to evaluate secondary hypertension?
Imaging: Renal Doppler US (initial), CT angiography, MR angiography
- Sometimes nuclear renogram with ACE inhibition
41
70. How is hypertension managed in children with PKD?
Management: ACE inhibitors or ARBs
- Monitor BP and kidney function closely
42
137. What is renovascular hypertension and how is it diagnosed?
Renovascular HTN: Due to narrowing of renal arteries (fibromuscular dysplasia or stenosis)
- Diagnosed with Doppler, CTA, or MRA
43
140. What is white coat hypertension and how is it diagnosed?
White coat HTN: Elevated BP in clinic but normal at home/ABPM
- Diagnosed by ABPM showing normal 24h average BP
44
164. What investigations are essential in neonatal AKI?
Investigations: Serum creatinine, electrolytes, urine output monitoring, urinalysis, renal ultrasound
45
13. What is the most common cause of acute glomerulonephritis in children?
Most common: Post-streptococcal glomerulonephritis (PSGN)
- Follows streptococcal pharyngitis or skin infection
46
4. What is the gold standard investigation for vesicoureteral reflux (VUR)?
Gold standard: Voiding cystourethrogram (VCUG)
- Detects reflux and grades severity
- Done after first febrile UTI if risk factors present
47
15. What are the typical lab findings in PSGN?
Labs: Low C3, normal C4, elevated ASO or anti-DNase B titers, hematuria, RBC casts, mild proteinuria
48
16. What is the role of complement levels in glomerulonephritis?
Complement levels help differentiate types of GN
- Persistent low C3 suggests MPGN or lupus
- Transient low C3 in PSGN
49
17. What conditions present with low complement glomerulonephritis?
Low C3 GN: PSGN, lupus nephritis, membranoproliferative GN, endocarditis-related GN
50
19. What are the clinical features of IgA nephropathy?
Features: Recurrent gross hematuria (often post-URTI), normal C3, mild proteinuria
- Biopsy: Mesangial IgA deposits
51
20. How is Henoch-Schönlein purpura nephritis differentiated from IgA nephropathy?
Both show IgA deposition
- HSP: Also has systemic features (purpura, arthralgia, abdominal pain)
- More common in younger children
52
21. What are the clinical features of membranoproliferative glomerulonephritis (MPGN)?
Features: Hematuria, proteinuria, hypertension, low complement, nephritic-nephrotic presentation
- Often chronic course
53
10. What is the typical urine finding in nephritic syndrome?
Typical finding: Hematuria with red cell casts
- May also have proteinuria and leukocyturia
54
14. What are the key features of post-streptococcal glomerulonephritis (PSGN)?
Features: Hematuria, edema, hypertension, low C3
- Often occurs 1–3 weeks after strep infection
55
18. What conditions present with normal complement glomerulonephritis?
Normal C3 GN: IgA nephropathy, HSP nephritis, Alport syndrome, thin basement membrane disease
56
155. What is C3 glomerulopathy and how is it diagnosed?
C3 glomerulopathy: GN due to alternative complement dysregulation
- Biopsy shows C3 dominance without immunoglobulins
57
156. How is C3 glomerulopathy differentiated from immune complex GN?
Differentiation: C3 glomerulopathy has isolated C3 deposition
- Immune complex GN shows IgG, IgM, or C1q on biopsy
58
9. What are the indications for kidney biopsy in nephrotic syndrome?
Indications: Steroid resistance, age >10 years, hematuria, hypertension, impaired renal function
59
151. What are the causes of congenital nephrotic syndrome?
Causes: Genetic mutations (NPHS1, NPHS2), infections (syphilis, CMV), autoimmune disorders, alloimmune (maternal antibodies)
60
7. What complications are associated with nephrotic syndrome?
Complications: Infections (SBP), thrombosis, hypovolemia, hyperlipidemia, acute kidney injury
61
6. What are the hallmark features of nephrotic syndrome?
Hallmarks: Proteinuria >40 mg/m²/hr or >3.5 g/day, hypoalbuminemia, edema, hyperlipidemia
- May show frothy urine
62
152. What is the typical presentation of Finnish-type congenital nephrotic syndrome?
Presentation: Heavy proteinuria at birth or within 3 months, edema, large placenta
- Mutation in NPHS1 (nephrin gene)
63
154. What is the management strategy for congenital nephrotic syndrome?
Management: Nutritional support, albumin infusion, ACE inhibitors, bilateral nephrectomy, dialysis, and renal transplant
64
153. What are the complications of congenital nephrotic syndrome?
Complications: Hypoalbuminemia, infections, thromboembolism, hypothyroidism, growth failure
65
5. What is the most common cause of nephrotic syndrome in children?
Most common cause: Minimal change disease
- >80% of nephrotic syndrome cases in children
- Excellent response to steroids
66
50. What are the causes of nephritic-nephrotic syndrome overlap in children?
Causes: Lupus nephritis, MPGN, IgA nephropathy, infections
- Features: Both hematuria and heavy proteinuria
67
11. What are the features of steroid-resistant nephrotic syndrome (SRNS)?
SRNS: No remission after 4 weeks of high-dose steroids
- Requires biopsy and genetic testing
- High risk of progression to CKD
68
92. What is the impact of constipation on urinary symptoms in children?
Constipation: Causes bladder outlet obstruction, incomplete emptying, and increases UTI risk
- Treatment improves urinary symptoms
69
93. How is daytime urinary incontinence evaluated?
Evaluation: History, bladder diary, urinalysis, post-void residual, ultrasound, rule out constipation
70
130. What is the management of primary hyperoxaluria?
Management: High fluid intake, citrate, vitamin B6 (pyridoxine), low-oxalate diet
- Liver/kidney transplant in severe cases
71
129. What are the renal effects of hyperoxaluria in children?
Hyperoxaluria: Leads to calcium oxalate nephrolithiasis and nephrocalcinosis
- May progress to ESRD
72
150. What is the long-term follow-up for a child with a single functioning kidney?
Follow-up: Monitor BP, renal function, urine protein
- Avoid contact sports; screen for CKD progression
73
159. What are common nephrotoxic drugs in children?
Nephrotoxic drugs: Aminoglycosides, NSAIDs, cisplatin, amphotericin B, vancomycin, calcineurin inhibitors
74
133. What is the definition of hypertensive emergency in children?
Definition: Severe elevation in BP with acute end-organ damage (neurologic, cardiac, renal)
75
136. What drugs are used in pediatric hypertensive emergencies?
Drugs: IV labetalol, nicardipine, esmolol, hydralazine
- Avoid rapid-acting nifedipine
76
87. How does dysfunctional voiding contribute to urinary tract infections?
Dysfunctional voiding: Incomplete bladder emptying → residual urine → infection risk
- Often coexists with constipation
77
103. What is the approach to evaluating a child with hypokalemia?
Approach: History, labs (electrolytes, ABG, renin, aldosterone), urine potassium, ECG
- Rule out acid-base imbalance
78
126. What are the renal manifestations of Wilson disease?
Renal effects: Proximal tubular dysfunction (Fanconi-like syndrome), nephrocalcinosis, hematuria, proteinuria
79
110. What is the emergency management of hyperkalemia?
Management: Calcium gluconate (cardioprotection), insulin + glucose, beta-agonists, sodium bicarbonate, dialysis if refractory
80
160. What are the mechanisms of nephrotoxicity with aminoglycosides?
Mechanism: Accumulates in proximal tubule, causes oxidative stress and necrosis
- Presents as non-oliguric AKI
81
157. What are the renal manifestations of systemic vasculitis in children?
Vasculitis: Hematuria, proteinuria, hypertension, elevated creatinine
- Seen in HSP, polyarteritis nodosa, ANCA vasculitis
82
135. What is the initial management of hypertensive emergency in children?
Initial management: Admit to ICU, control BP gradually to avoid cerebral hypoperfusion
83
158. What are the renal findings in juvenile systemic lupus erythematosus (SLE)?
SLE nephritis: Proteinuria, hematuria, hypertension, nephrotic or nephritic picture
- Biopsy classifies severity (Class I–VI)
84
132. What is the evaluation protocol for a hypertensive child?
Evaluation: BP measurement, urinalysis, renal function, electrolytes, renal ultrasound, ABPM if needed
85
169. How is glomerular filtration barrier structure related to proteinuria?
Proteinuria occurs when barrier fails
- Damage to podocytes, GBM, or endothelial fenestrations increases permeability
86
149. How is multicystic dysplastic kidney (MCDK) diagnosed and managed?
Diagnosis: Renal US shows multiple noncommunicating cysts, no normal parenchyma
- Management: Observation; contralateral kidney must be healthy
87
61. What is nephronophthisis and how does it present in children?
Nephronophthisis: AR tubulointerstitial disease
- Presents with polyuria, polydipsia, anemia, growth failure, and progressive CKD
88
195. What renal lesions are associated with neurofibromatosis type 1?
NF1: Can develop renal artery stenosis → secondary hypertension
- Rarely Wilms tumor or other masses
89
197. What is HIV-associated nephropathy (HIVAN)?
HIVAN: FSGS variant seen in advanced HIV
- Presents with nephrotic-range proteinuria and rapid GFR decline
90
170. What are the key components of the glomerular filtration barrier?
Components: Fenestrated endothelium, glomerular basement membrane (GBM), podocyte slit diaphragm
91
185. How is orthostatic proteinuria differentiated from persistent proteinuria?
Diagnosis: Elevated protein/Cr in daytime sample, normal in first morning void
- No other signs of kidney disease
92
49. What is the definition of proteinuria in children based on spot urine protein/creatinine ratio?
Definition: Spot urine protein/creatinine ratio >0.2 mg/mg
- >2 mg/mg suggests nephrotic range proteinuria
93
48. What is orthostatic (postural) proteinuria and how is it diagnosed?
Diagnosis: Compare first morning and daytime urine protein/Cr ratio
- Elevated in daytime, normal in morning
94
47. What are red flag signs in a child with hematuria?
Red flags: HTN, edema, proteinuria, family history of renal disease, abnormal renal function, sensorineural hearing loss
95
63. What is the most common type of polycystic kidney disease (PKD) in children?
Most common: Autosomal recessive polycystic kidney disease (ARPKD) in infancy
- ADPKD typically presents later
96
46. What is the initial evaluation of a child with persistent hematuria?
Evaluation: Urinalysis, urine microscopy, urine protein/Cr ratio, renal US, family history
97
22. What is the pathophysiology of MPGN?
Pathophysiology: Immune complex or complement-mediated injury to glomeruli
- Leads to mesangial and endocapillary proliferation
98
192. How does glycogen storage disease affect the kidneys?
Glycogen storage disease: Can cause nephromegaly, proteinuria, glomerulosclerosis (especially type I and III)
99
30. How is HUS managed in pediatric patients?
Management: Supportive care (fluids, electrolytes)
- Avoid antibiotics and anti-motility agents in STEC
- Dialysis if needed
100
193. What is the renal phenotype of Dent disease?
Dent disease: X-linked proximal tubulopathy with low molecular weight proteinuria, hypercalciuria, nephrocalcinosis, CKD
101
194. What are the renal findings in tuberous sclerosis complex?
Tuberous sclerosis: Renal angiomyolipomas (AMLs), cysts, rarely RCC
- Monitor for bleeding or mass effect
102
27. What is the recommended initial workup in a child with suspected lupus nephritis?
Initial workup: Urinalysis, renal function, C3/C4, ANA, anti-dsDNA, renal biopsy for classification
103
26. What is the most common form of lupus nephritis in children?
Most common: Class IV (Diffuse Proliferative GN)
- Severe, requires aggressive immunosuppression
104
8. How is minimal change disease diagnosed and treated?
Diagnosis: Clinical in typical cases (no biopsy needed)
- Treatment: Corticosteroids (prednisolone)
- Relapse common
105
64. How is autosomal recessive polycystic kidney disease (ARPKD) diagnosed?
Diagnosis: Bilateral enlarged echogenic kidneys on ultrasound, history of oligohydramnios, family history
106
23. What are the different types of MPGN based on pathogenesis?
Types:
- Type I: Immune complex mediated
- Type II (dense deposit disease): Complement mediated
- Type III: Mixed features
107
69. What are common ultrasound findings in ARPKD?
Findings: Bilaterally enlarged, echogenic kidneys with poor corticomedullary differentiation
- Cysts usually microscopic
108
66. What is the gene mutation responsible for ARPKD?
Gene: PKHD1 on chromosome 6
- Encodes fibrocystin/polyductin
109
77. What are the long-term renal outcomes in children with PUV?
Outcomes: Risk of CKD and ESRD
- Lifelong urologic and nephrologic follow-up needed
110
182. How is microalbuminuria detected and monitored?
Detection: Spot urine albumin-to-creatinine ratio (ACR)
- Morning sample preferred
- Monitor progression or response to ACEi
111
68. What are the extrarenal manifestations of autosomal dominant PKD?
Extrarenal: Hepatic cysts, pancreatic cysts, intracranial aneurysms (Berry), cardiac valve abnormalities
112
65. What are the key features of ARPKD?
Features: Enlarged kidneys, pulmonary hypoplasia, Potter facies, liver fibrosis, systemic hypertension
113
73. What are the prenatal findings suggestive of PUV?
Prenatal signs: Bilateral hydronephrosis, thick-walled bladder, oligohydramnios, keyhole sign on ultrasound
114
175. What are the signs of sickle cell nephropathy?
Signs: Nocturia, polyuria, isosthenuria, microalbuminuria, decreased GFR
- Common in older children
115
75. How is PUV diagnosed postnatally?
Diagnosis: Voiding cystourethrogram (VCUG)
- Shows dilated posterior urethra and bladder trabeculation
116
176. How is sickle cell nephropathy managed?
Management: ACE inhibitors, hydration, BP control, avoid nephrotoxins
- Monitor urine protein and GFR
117
180. What is the impact of chronic hypokalemia on renal function?
Chronic hypokalemia: Impairs urine concentration → nephrogenic DI, tubulointerstitial fibrosis, cyst formation
118
199. What are the modalities of dialysis used in pediatric patients?
Modalities: Peritoneal dialysis (common in infants), hemodialysis, continuous renal replacement therapy (CRRT)
119
142. What is the most common renal tumor in childhood?
Most common tumor: Wilms tumor (nephroblastoma)
- Peak age: 3–4 years
- Good prognosis with treatment
120
144. What is the initial imaging study for suspected Wilms tumor?
Initial study: Abdominal ultrasound
- Confirms solid renal mass and assesses contralateral kidney
121
145. What is the role of CT in Wilms tumor diagnosis?
CT abdomen/pelvis: Used for staging, detecting metastases, involvement of IVC or renal vein
122
146. What are the treatment modalities for Wilms tumor?
Treatment: Nephrectomy, chemotherapy (vincristine, actinomycin D), radiotherapy if advanced
- Based on stage and histology
123
177. What is tumor lysis syndrome and how does it affect the kidneys?
TLS: Oncologic emergency due to massive tumor cell lysis
- Releases uric acid, phosphate, potassium → AKI
124
178. What are the renal findings in tumor lysis syndrome?
Findings: Hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, oliguric AKI
125
179. How is tumor lysis syndrome prevented and managed?
Prevention: IV hydration, allopurinol or rasburicase, close electrolyte monitoring
- Dialysis if severe
126
147. What are poor prognostic factors in Wilms tumor?
Poor prognosis: Anaplasia, tumor rupture, bilateral disease, unfavorable histology, age >4 years
127
143. What syndromes are associated with Wilms tumor?
Associated syndromes: WAGR (Wilms, Aniridia, Genitourinary anomalies, Retardation), Beckwith-Wiedemann, Denys-Drash
128
124. How is Fanconi syndrome diagnosed?
Diagnosis: Urinalysis (glucosuria, proteinuria, aminoaciduria), blood tests (acidosis, hypophosphatemia, low uric acid), urinary losses
129
187. What is the role of β2-microglobulin and N-acetyl-β-D-glucosaminidase (NAG) in renal tubular assessment?
Markers: Indicate proximal tubular injury
- Elevated in Fanconi syndrome, drug toxicity, tubular proteinuria
130
116. How is renal tubular acidosis managed in children?
Management: Alkali therapy (bicarbonate or citrate), potassium supplementation if needed
- Treat underlying cause
131
114. How is renal tubular acidosis diagnosed?
Diagnosis: Blood gas (low HCO3−), serum electrolytes, urine pH, urine anion gap
- Type 1: Urine pH >5.5, Type 2: pH <5.5 after acidosis
132
113. What are the features of proximal (type 2) renal tubular acidosis?
Proximal RTA (type 2): Bicarbonate wasting
- Features: Hypokalemia, low urine pH, associated with Fanconi syndrome
133
112. What are the features of distal (type 1) renal tubular acidosis?
Distal RTA (type 1): Impaired H+ secretion
- Features: Hypokalemia, nephrocalcinosis, high urine pH, growth failure
134
125. What is the treatment of Fanconi syndrome?
Treatment: Replace losses (alkali, phosphate, potassium, calcitriol)
- Treat underlying cause if reversible
135
121. What is the pathophysiology of Fanconi syndrome?
Fanconi syndrome: Generalized dysfunction of the proximal tubule
- Leads to loss of glucose, phosphate, bicarbonate, amino acids, and uric acid
136
198. What are the indications for renal replacement therapy in children with AKI?
Indications: Severe electrolyte imbalance, acidosis, fluid overload, uremia, persistent oliguria/anuria despite management
137
171. What are the causes of hemoglobinuria and how is it differentiated from hematuria?
Hemoglobinuria: Free hemoglobin in urine due to intravascular hemolysis
- No RBCs on microscopy, +blood on dipstick
138
188. What are the renal implications of hypothyroidism in children?
Effects: Reduced GFR, hyponatremia, increased serum creatinine
- May cause growth delay via impaired renal perfusion
139
186. What is the clinical relevance of serum cystatin C in pediatric nephrology?
Cystatin C: Marker of GFR not influenced by muscle mass
- Useful in children with low muscle mass or malnutrition
140
183. What are the causes of transient proteinuria in children?
Causes: Fever, exercise, stress, dehydration, seizure
- Usually resolves spontaneously
141
161. What are the renal causes of neonatal acute kidney injury (AKI)?
Causes: Perinatal asphyxia, sepsis, dehydration, nephrotoxic medications, renal vein thrombosis, congenital anomalies
142
162. What are the clinical signs of AKI in a neonate?
Signs: Oliguria/anuria, fluid overload, rising creatinine, electrolyte disturbances, poor feeding, lethargy
143
181. What is the definition and clinical importance of microalbuminuria in children?
Microalbuminuria: Urine albumin excretion between 30–300 mg/day
- Early marker of glomerular damage, especially in diabetes or SCD
144
174. What are the renal complications of sickle cell disease in children?
Complications: Hyposthenuria, hematuria, proteinuria, papillary necrosis, CKD, FSGS
145
173. What foods or substances can cause false red urine (pseudohematuria)?
Pseudohematuria: Beetroot, blackberries, rifampicin, porphyria, food dyes
- No RBCs on microscopy
146
172. What are the causes of myoglobinuria and how is it recognized?
Myoglobinuria: From rhabdomyolysis (trauma, seizures, toxins)
- Dark urine, no RBCs, ↑CK, +blood on dipstick
147
196. What are the renal complications of HIV in pediatric patients?
Complications: HIVAN, AKI from infections or drugs, electrolyte abnormalities, proteinuria, nephrotic-range disease
148
184. What are the causes of orthostatic proteinuria?
Causes: Tall adolescents, postural changes, long periods of standing
- Common benign condition in older children
149
1. What are the diagnostic criteria for urinary tract infection (UTI) in infants?
Criteria: >100,000 CFU/mL of a single organism on clean-catch or >50,000 CFU/mL on catheterized specimen
- Symptoms: fever, irritability, vomiting
150
141. What are the clinical signs of Wilms tumor in children?
Signs: Asymptomatic abdominal mass, hematuria, hypertension, abdominal pain, fever
- Rarely crosses midline
151
78. What is the role of renal ultrasound in evaluating antenatal hydronephrosis?
Role: First-line postnatal evaluation tool
- Assesses kidney size, structure, hydronephrosis, and bladder wall
152
76. What is the treatment of posterior urethral valves?
Treatment: Endoscopic valve ablation
- Initial bladder decompression with catheterization
153
74. What are the postnatal complications of posterior urethral valves?
Complications: Bladder dysfunction, recurrent UTIs, CKD, poor growth, pulmonary hypoplasia (if severe)
154
72. What is posterior urethral valves (PUV) and how does it present?
PUV: Congenital obstruction of posterior urethra in males
- Presents with weak stream, poor voiding, UTI, hydronephrosis
155
71. What is the most common congenital anomaly of the kidney and urinary tract (CAKUT)?
Most common CAKUT: Hydronephrosis
- Often due to pelvi-ureteric junction (PUJ) obstruction
- Detected prenatally
156
67. What is the typical presentation of autosomal dominant PKD in childhood?
Presentation: Incidental finding of renal cysts, early-onset hypertension, hematuria
- Usually in adolescence or adulthood
157
56. What is the cause of growth failure in pediatric CKD?
Cause: Nutritional deficiency, metabolic acidosis, GH resistance
- Treat with GH, nutrition, bicarbonate
158
54. What are the complications of CKD in children?
Complications: Anemia, growth retardation, electrolyte disturbances, acidosis, bone disease, hypertension
159
52. What are the most common causes of CKD in children?
Common causes: CAKUT (e.g., posterior urethral valves, dysplasia), glomerular diseases, hereditary nephropathies
160
45. How is hematuria classified and what are its common causes in children?
Classification:
- Microscopic vs gross
- Glomerular: RBC casts, proteinuria
- Nonglomerular: Isolated RBCs, no proteinuria
- Common causes: UTI, trauma, IgA nephropathy
161
79. What are the Society for Fetal Urology (SFU) grades of hydronephrosis?
SFU grading:
- Grade 1: Renal pelvis only
- Grade 2: Few calyces
- Grade 3: All calyces
- Grade 4: Thinning parenchyma
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43. What is the most common cause of postrenal AKI in neonates?
Most common cause: Posterior urethral valves (PUV)
- Can lead to bilateral hydronephrosis and renal damage
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38. What is the most common cause of acute kidney injury (AKI) in children?
Most common cause: Prerenal AKI due to dehydration or hypovolemia
- Reversible with fluids
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33. What is the treatment of atypical HUS in children?
Treatment: Eculizumab (anti-C5 monoclonal antibody)
- Supportive care, dialysis if needed
- Plasma exchange sometimes used
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32. What is the underlying cause of aHUS?
Cause: Mutations in complement regulatory genes (e.g., CFH, CFI, MCP)
- Leads to unregulated complement activation and endothelial injury
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31. What is atypical hemolytic uremic syndrome (aHUS) and how is it different from typical HUS?
aHUS: No diarrhea prodrome, associated with complement dysregulation
- More severe, often recurrent
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29. What is the most common cause of HUS in children?
Most common cause: Shiga toxin-producing E. coli (STEC), especially E. coli O157:H7
- Often follows gastroenteritis
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28. What are the clinical signs of hemolytic uremic syndrome (HUS)?
Signs: Triad of microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury
- Often preceded by diarrhea
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25. How is lupus nephritis classified histologically?
ISN/RPS Classification (I to VI)
- Based on activity and chronicity
- Guides treatment decisions
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24. What are the typical histologic findings in MPGN on biopsy?
Findings: Mesangial proliferation, double-contour (tram-track) appearance, subendothelial deposits
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3. What are the indications for renal imaging after a UTI in children?
Indications: Recurrent UTIs, atypical UTI, abnormal growth, poor response to treatment
- Common imaging: Renal US, VCUG
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2. What is the most common causative organism of UTI in children?
Most common organism: Escherichia coli
- Especially uropathogenic strains with P fimbriae
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42. What are common causes of intrinsic AKI in children?
Causes: Acute tubular necrosis (ATN), glomerulonephritis, HUS, interstitial nephritis
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148. What is the most common cause of bilateral renal enlargement in neonates?
Most common: ARPKD or bilateral hydronephrosis
- May also include congenital nephrotic syndrome
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80. When is a voiding cystourethrogram (VCUG) indicated in neonates?
VCUG indicated: Postnatal UTI with abnormal US, bilateral hydronephrosis, suspected PUV, family history of VUR
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82. What are the clinical consequences of untreated VUR?
Consequences: Recurrent pyelonephritis, renal scarring, hypertension, CKD
- More severe in bilateral or high-grade VUR
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139. What are the indications for ambulatory blood pressure monitoring (ABPM) in children?
Indications: Suspected white coat hypertension, CKD, solid organ transplant, evaluation of treatment response
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134. What are the clinical features of hypertensive emergency?
Features: Headache, vomiting, seizures, visual changes, encephalopathy, papilledema
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131. What are the renal causes of hypertension in children?
Renal causes: Glomerulonephritis, CKD, renal artery stenosis, polycystic kidney disease, reflux nephropathy, Wilms tumor
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123. What are the causes of Fanconi syndrome?
Causes: Cystinosis, galactosemia, Wilson disease, Lowe syndrome, drugs (ifosfamide), multiple myeloma (in adults)
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122. What are the clinical features of Fanconi syndrome in children?
Features: Polyuria, polydipsia, hypophosphatemic rickets, metabolic acidosis, growth failure, proteinuria, glucosuria
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120. What is the management of nephrocalcinosis?
Management: Correct underlying cause, control calcium and phosphate, citrate therapy, hydration, avoid nephrotoxic drugs
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119. How is nephrocalcinosis diagnosed and monitored?
Diagnosis: Renal ultrasound (echogenic pyramids), CT scan if unclear
- Monitor with imaging and calcium/phosphate levels
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118. What are the causes of nephrocalcinosis in children?
Causes: Distal RTA, hypercalcemia, hyperparathyroidism, vitamin D toxicity, loop diuretics, medullary sponge kidney
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115. What are the causes of high anion gap metabolic acidosis in pediatric nephrology?
Causes: Uremia, lactic acidosis, DKA, toxins (methanol, ethylene glycol), severe dehydration, renal failure
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111. What are the causes of metabolic acidosis with a normal anion gap in children?
Causes: Diarrhea, renal tubular acidosis (RTA), ureteral diversion, hypoaldosteronism
- Normal anion gap = hyperchloremic acidosis
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81. What is vesicoureteral reflux (VUR) and how is it graded?
VUR: Retrograde flow of urine from bladder to ureters/kidneys
- Graded I to V based on VCUG findings
- Grades IV–V = high grade
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109. What are the causes of hyperkalemia in pediatric patients?
Causes: Hemolysis, acidosis, renal failure, medications (ACEI, K-sparing diuretics), hypoaldosteronism
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104. What are the renal causes of hypokalemia?
Renal causes: Diuretics, renal tubular acidosis, Bartter syndrome, Gitelman syndrome, hyperaldosteronism
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102. What are the clinical features of hypokalemia?
Features: Weakness, constipation, arrhythmias, polyuria
- ECG: U waves, flattened T waves, arrhythmias
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101. What are the causes of hypokalemia in children?
Causes: GI losses (diarrhea, vomiting), renal losses (diuretics, RTA), intracellular shifts (insulin, alkalosis), poor intake
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99. What is the clinical presentation of a neurogenic bladder in children?
Presentation: Urinary retention, dribbling, recurrent UTI, incontinence, elevated PVR
- Often associated with spinal dysraphism
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95. What is the significance of a bell-shaped curve in uroflowmetry?
Bell-shaped curve: Indicates normal detrusor contraction and sphincter relaxation
- Suggests normal voiding physiology
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94. What is the diagnostic utility of uroflowmetry in pediatric voiding disorders?
Uroflowmetry: Measures voiding pattern
- Identifies flow abnormalities suggestive of dysfunctional voiding or obstruction
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86. What are the risk factors for recurrent UTI in children?
Risk factors: Female sex, uncircumcised males, constipation, VUR, bladder dysfunction, poor hygiene
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85. What is the most common type of surgical correction for VUR?
Most common surgery: Ureteral reimplantation (open or laparoscopic)
- Other: Endoscopic injection of bulking agents (Deflux)
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84. When is surgical correction indicated in VUR?
Surgical indications: High-grade VUR with recurrent pyelonephritis, breakthrough infections, renal scarring, non-compliance with medical therapy
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83. What is the management approach to low-grade VUR (grades I–III)?
Low-grade VUR: Observation + prophylactic antibiotics
- Many cases resolve spontaneously with age
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107. How do you differentiate Bartter from Gitelman syndrome biochemically?
Bartter: High urinary calcium, presents early
- Gitelman: Low urinary calcium, presents later, with hypomagnesemia
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200. What is the recommended approach to transition of care in pediatric CKD patients reaching adulthood?
Transition: Gradual process involving pediatric and adult nephrologists, education, independence in care, psychosocial support
