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PHOTO 1 - Case: 4-year-old with 1 month of generalized swelling starting from face, frothy urine, pitting edema, ascites, no anemia, no jaundice, BP 90/60, urine protein 4+, no RBCs in urine
Q.1 - What is the most probable cause of swelling? (2 marks)
Nephrotic Syndrome - specifically Minimal Change Nephrotic Syndrome (MCNS), which accounts for 85% of idiopathic nephrotic syndrome in children.
Mechanism of swelling: Massive proteinuria (4+ protein, no hematuria) → hypoalbuminemia → reduced plasma oncotic pressure → fluid shifts from intravascular to interstitial space → edema. The characteristic onset from the face (periorbital area) progressing to dependent parts and whole body, combined with frothy urine (proteinuria), confirms nephrotic pathophysiology.
Q.2 - What are the differential diagnoses of anasarca? (2 marks)
| Category | Condition |
|---|---|
| Renal | Nephrotic syndrome (most likely in this child), Nephritic syndrome, Chronic kidney disease |
| Cardiac | Congestive heart failure (right-sided/biventricular) |
| Hepatic | Liver cirrhosis / hepatic failure → hypoalbuminemia |
| Nutritional | Kwashiorkor / protein-energy malnutrition |
| Endocrine | Hypothyroidism (myxedema) |
| Inflammatory | Sepsis, capillary leak syndrome |
| Lymphatic | Lymphedema |
Key differentiating point: In this child, the absence of hematuria, normal CVS, normal BP (90/60 is low - consistent with NS), 4+ proteinuria, and no RBCs in urine strongly points to nephrotic syndrome over nephritic or cardiac causes.
Q.3 - What are the investigations and expected findings? (4 marks)
Urine Investigations:
| Test | Expected Finding |
|---|---|
| Urine dipstick / urinalysis | Protein 3-4+; no blood |
| Urine microscopy | Oval fat bodies, fatty casts, lipiduria; no RBC casts |
| 24-hr urine protein (or spot urine protein:creatinine ratio) | >40 mg/m²/hr or >3.5 g/day (nephrotic range) in adults; >40 mg/m²/hr in children |
Blood Investigations:
| Test | Expected Finding |
|---|---|
| Serum albumin | Low (<2.5 g/dL, often <1.5 g/dL) |
| Serum total protein | Low |
| Serum cholesterol / lipid profile | High (hyperlipidemia - compensatory hepatic lipoprotein synthesis) |
| Serum creatinine & BUN | Usually normal in MCNS |
| Serum electrolytes | May show hyponatremia (dilutional) |
| Complement levels (C3, C4) | Normal (low complement suggests nephritic syndrome / MPGN) |
| ANA, Anti-dsDNA | Negative (rule out SLE) |
| Hepatitis B, C; HIV | Negative (rule out secondary NS) |
| FBC / CBC | No anemia expected in MCNS |
| Thyroid function | Normal |
Imaging:
- Renal ultrasound: Mildly enlarged, echogenic kidneys
Renal Biopsy: Not routinely done in a 4-year-old child with classic nephrotic syndrome unless atypical features are present.
Q.4 - How do you treat this condition? (4 marks)
Specific Treatment - Corticosteroids (First Line)
Initial episode (standard ISKDC regimen):
- Prednisolone 60 mg/m²/day (or 2 mg/kg/day, max 60 mg/day) as a single daily dose for 4-6 weeks
- Followed by prednisolone 40 mg/m² (or 1.5 mg/kg) on alternate days for 4-6 weeks, then taper and stop
Monitoring: Daily first-morning urine dipstick for protein. Remission = urine protein negative or trace for 3 consecutive days.
Supportive Treatment
-
Diet:
- Normal or slightly restricted protein (0.8-1 g/kg/day once in remission)
- Low sodium diet (to reduce edema)
- High caloric diet
-
Diuretics (if massive edema):
- Furosemide (loop diuretic) ± spironolactone
- Use cautiously; risk of hypovolemia
-
Albumin infusion: 20% albumin in severe hypoalbuminemia with circulatory compromise, followed by furosemide
-
Infection prophylaxis:
- Pneumococcal vaccine (before steroids if possible)
- Penicillin prophylaxis against pneumococcal peritonitis
- Treat any infection promptly
-
Anticoagulation: If thrombotic risk is high (severe hypoalbuminemia <2 g/dL), consider prophylactic anticoagulation.
-
Treat hyperlipidemia: Statins if persistent after remission.
Q.5 - What are the common complications of this condition? (3 marks)
Complications of Nephrotic Syndrome:
-
Infections (most common and life-threatening)
- Spontaneous bacterial peritonitis (Streptococcus pneumoniae most common)
- Septicemia, cellulitis
- Mechanism: urinary loss of immunoglobulins + immunosuppressive steroids
-
Thromboembolic disease
- Renal vein thrombosis, pulmonary embolism, cerebral thrombosis
- Mechanism: loss of protein C, protein S, antithrombin III + increased fibrinogen
-
Acute kidney injury (AKI)
- From hypovolemia / reduced effective circulating volume
-
Hyperlipidemia / dyslipidemia
- Increased cardiovascular risk
-
Malnutrition and growth retardation
- Prolonged hypoalbuminemia and steroid use
-
Steroid side effects (from treatment)
- Cushingoid features, hypertension, osteoporosis, cataracts, growth retardation, immunosuppression
-
Vitamin D deficiency and iron deficiency anemia (loss of vitamin D-binding protein and transferrin in urine)
-
Hyponatremia (dilutional)
PHOTO 2 - Case: 4-year-old with anasarca, frothy urine, BP 100/60, facial onset swelling
Q.15 - What is your diagnosis? (1 mark)
Nephrotic Syndrome - most likely Minimal Change Nephrotic Syndrome (MCNS)
Basis: age 4 years, facial onset anasarca, frothy urine (proteinuria), no hematuria implied, low-normal BP - classic presentation.
Q.16 - What are the investigations you would like to order? (4 marks)
(Same as Q.3 above - see detailed table)
Summary:
- Urine: dipstick (protein 4+), microscopy (no RBC casts, fatty casts), 24-hr urine protein or spot P:Cr ratio
- Blood: serum albumin (low), cholesterol (high), creatinine (normal), electrolytes, CBC, C3/C4 (normal)
- Serology: ANA, Anti-dsDNA (negative), Hepatitis B/C, HIV
- Renal ultrasound
Q.17 - What are the causes of proteinuria in children? (3 marks)
A. Transient (Non-pathologic) Proteinuria:
- Fever, exercise, dehydration, seizures, stress, orthostatic proteinuria (most common in adolescents)
B. Pathologic Proteinuria:
| Type | Causes |
|---|---|
| Glomerular | Minimal change NS, FSGS, Membranous nephropathy, Post-streptococcal GN, IgA nephropathy, Lupus nephritis, HSP nephritis, Alport syndrome |
| Tubular | Fanconi syndrome, renal tubular acidosis, heavy metal toxicity |
| Overflow | Myoglobinuria, hemoglobinuria, multiple myeloma (Bence-Jones proteins) |
| Secondary | Diabetes mellitus, SLE, infections (HIV, Hep B, Hep C), drugs (NSAIDs, gold, penicillamine), malignancy (Hodgkin lymphoma) |
(Harriet Lane Handbook; National Kidney Foundation Primer on Kidney Diseases, 8e)
Q.18 - How do you treat this child? (5 marks)
Diagnosis: Nephrotic Syndrome, first presentation
Corticosteroid Therapy (First Line - ISKDC Protocol):
- Prednisolone 2 mg/kg/day (max 60 mg/day) for 4 weeks (daily dose)
- Then 1.5 mg/kg on alternate days for 4 weeks, then taper
Supportive Measures:
- Low-sodium diet (reduce edema)
- Normal protein intake (avoid low protein which worsens hypoalbuminemia)
- Diuretics (furosemide) for severe symptomatic edema
- Albumin infusion (20% human albumin) if albumin <1.5 g/dL with circulatory compromise
- Antibiotics - treat infections promptly; pneumococcal prophylaxis
- Vaccinations - Pneumococcal vaccine, Varicella vaccine (before steroids if possible)
- Anticoagulation if hypercoagulable state is severe
- Monitoring: Daily urine protein dipstick at home
- Restrict activity during active edema; no need to restrict when in remission
Q.19 - On 5th day of treatment - altered sensorium, headache, monoparesis of right upper limb. What is the likely cause? (2 marks)
Most likely cause: Cerebral venous sinus thrombosis (CVST) or arterial thromboembolic stroke
Explanation:
- Nephrotic syndrome creates a hypercoagulable state due to urinary loss of:
- Antithrombin III
- Protein C and Protein S
- Plasminogen
- Combined with increased hepatic synthesis of fibrinogen, factor V, and VIII
- Thrombosis can occur in cerebral veins/sinuses or arteries
- Altered sensorium + headache + focal neurological deficit (monoparesis) = classic stroke/CVST presentation
- Additionally, corticosteroid treatment may worsen hypertension contributing to risk
Other causes to consider:
- Hypertensive encephalopathy
- Hyponatremia (dilutional) causing cerebral edema
- Infection/meningitis (due to immunosuppression)
(National Kidney Foundation Primer on Kidney Diseases, 8e, p.190)
Q.20 - After recovery and discharge, child presents again one month later with facial and trunk swelling. What are the possible causes? (2 marks)
Possible causes of new swelling:
- Relapse of nephrotic syndrome (most likely) - relapse is very common in MCNS; occurs in ~60-70% of children. Defined as urine protein 3+ or 4+ for 3 consecutive days after remission.
- Steroid-induced side effects masking partial remission
- Steroid non-compliance leading to undertreated disease
- Frequently relapsing nephrotic syndrome (2+ relapses within 6 months, or 4+ in 12 months)
- Intercurrent infection triggering relapse (e.g., upper respiratory infection is the most common trigger)
Q.21 - Child is now hypertensive with trace urine protein. A student says persistent hypertension is indication for renal biopsy. Would you proceed? Explain with reason. (3 marks)
Answer: Yes, this is an indication to proceed with renal biopsy.
Reasoning:
- The clinical picture has changed: previously nephrotic (heavy proteinuria, hypoalbuminemia), now hypertensive with only trace proteinuria
- Hypertension is not a typical feature of minimal change disease (MCNS) - it suggests a different or evolving pathology
- Indications for biopsy in this context include:
- Age >12 years or <12 months
- Persistent hypertension
- Hematuria (macroscopic)
- Persistent elevated creatinine
- Low complement (C3, C4)
- Steroid resistance (no remission after 4-8 weeks of adequate steroids)
- Steroid dependence
- Systemic features suggesting secondary cause (lupus, vasculitis)
- The combination of hypertension + changing proteinuria pattern raises suspicion for FSGS, IgA nephropathy, or secondary glomerular disease
- Renal biopsy will guide diagnosis and direct second-line therapy
(Goldman-Cecil Medicine; Harriet Lane Handbook)
Q.22 - What are the other indications for renal biopsy in nephrotic syndrome? (5 marks)
| Indication | Rationale |
|---|---|
| Age <12 months or >12 years | MCNS less likely; other pathologies more common |
| Steroid resistance | No remission after 4-8 weeks of full-dose steroids; need histology to guide treatment |
| Steroid dependence | Two consecutive relapses during taper or within 14 days of stopping |
| Frequently relapsing | To guide long-term immunosuppressive therapy |
| Macroscopic hematuria | Suggests nephritic rather than pure nephrotic pathology |
| Persistent microscopic hematuria | Possible IgA nephropathy, Alport syndrome |
| Persistent hypertension | Not typical of MCNS; suggests FSGS, IgAN, or secondary cause |
| Low complement levels (C3, C4) | Suggests MPGN, SLE nephritis, post-infectious GN |
| Suspected secondary cause | SLE (positive ANA), Hepatitis B/C, HIV, diabetes, malignancy |
| Persistent renal insufficiency (elevated creatinine) | Not expected in pure MCNS; warrants histological diagnosis |
| Systemic features | Rash, arthritis, multisystem involvement |
(Harriet Lane Handbook, 23rd ed., p.704; Goldman-Cecil Medicine)
PHOTO 3 - Case: 4-year-old male with anasarca for 2 weeks; swelling started from face, worse in morning, resolves by evening; BP 90/60, pitting pedal edema, ascites (fluid thrill), weight 15 kg
Q.1 - Enlist the FIVE most common causes of anasarca in children (5 marks)
- Nephrotic Syndrome (most common in children - MCNS accounts for 85% of primary cases)
- Congestive Cardiac Failure / Congenital Heart Disease (increased venous pressure)
- Liver disease / Cirrhosis (reduced albumin synthesis → hypoalbuminemia)
- Protein-Energy Malnutrition / Kwashiorkor (nutritional hypoalbuminemia)
- Acute Nephritic Syndrome / Glomerulonephritis (Na and water retention)
Other causes include: severe anemia, hypothyroidism (myxedema), capillary leak syndrome, sepsis.
Q.2 - Differentiate Nephritic Syndrome from Nephrotic Syndrome (5 marks)
| Feature | Nephrotic Syndrome | Nephritic Syndrome |
|---|---|---|
| Proteinuria | Massive (>3.5 g/day adult; >40 mg/m²/hr children) | Mild to moderate (<3.5 g/day) |
| Hematuria | Absent (or microscopic in 30%) | Present - HALLMARK (dysmorphic RBCs, RBC casts) |
| Hypertension | Usually absent / low BP (due to low oncotic pressure) | Present (Na/water retention) |
| Edema | Severe (anasarca), peri-orbital onset | Mild to moderate |
| Urine color | Normal or frothy | Cola/tea colored (hematuria) |
| Serum albumin | Low (<2.5 g/dL) | Usually normal |
| Serum cholesterol | High (hyperlipidemia) | Usually normal |
| Lipiduria | Present (oval fat bodies) | Absent |
| Complement (C3/C4) | Normal (in MCNS/FSGS) | Often low (MPGN, PSGN, SLE) |
| GFR / Creatinine | Usually normal (in MCNS) | Reduced (oliguria, azotemia) |
| Urine output | Normal | Oliguria |
| Mechanism | Loss of podocyte charge/structure → proteinuria | Inflammatory glomerular injury → hematuria + Na retention |
| Common cause in children | MCNS (85%) | Post-streptococcal GN (most common) |
| Renal biopsy | Not initially needed (empirical steroids) | Often needed for diagnosis |
(National Kidney Foundation Primer on Kidney Diseases, 8e; Harriet Lane Handbook)
Q.3 - What is Nephrotic Range Proteinuria and how is the diagnosis of Nephrotic Syndrome made? (2+3 = 5 marks)
Part 1: Nephrotic Range Proteinuria (2 marks)
Nephrotic range proteinuria is defined as:
- Adults: >3.5 g/1.73 m² per 24 hours (or spot urine protein:creatinine ratio >3.5 mg/mg)
- Children: >40 mg/m²/hr (on 24-hr collection) or spot urine protein:creatinine ratio >2 mg/mg (>200 mg/mmol)
- On urine dipstick: typically 3+ or 4+ protein
Part 2: Diagnosis of Nephrotic Syndrome (3 marks)
Nephrotic syndrome is diagnosed by the presence of ALL of the following:
| Criterion | Value |
|---|---|
| 1. Proteinuria (nephrotic range) | >3.5 g/day (adult); >40 mg/m²/hr (child); dipstick 3-4+ |
| 2. Hypoalbuminemia | Serum albumin <3.5 g/dL (typically <2.5 g/dL in children) |
| 3. Edema | Periorbital/facial onset, anasarca |
| 4. Hyperlipidemia | Elevated total cholesterol, LDL, triglycerides |
- Lipiduria (fatty casts, oval fat bodies) is also a typical finding
- In children: A 4-8 year old with classic features (facial edema, heavy proteinuria, no hematuria, normal complement, normal BP) is treated empirically with steroids as MCNS without biopsy
(National Kidney Foundation Primer on Kidney Diseases, 8e, p.190)
Q.4 - How is Nephrotic Syndrome classified as per the response to steroid therapy? (3 marks)
| Classification | Definition |
|---|---|
| Steroid-Sensitive (Steroid-Responsive) | Complete remission (protein negative/trace) within 4-8 weeks of full-dose prednisolone. ~95% of MCNS respond. |
| Frequently Relapsing | Two or more relapses within 6 months of initial response, OR four or more relapses in any 12-month period |
| Steroid-Dependent | Two consecutive relapses occurring during steroid taper OR within 14 days of stopping steroids |
| Steroid-Resistant | Failure to achieve remission (persistent 3-4+ proteinuria) after 4-8 weeks of full-dose corticosteroid therapy |
Notes:
- Steroid-resistant cases require renal biopsy and second-line agents (calcineurin inhibitors, MMF, rituximab)
- Frequently relapsing/steroid-dependent: second-line options include cyclophosphamide, levamisole, MMF, calcineurin inhibitors (cyclosporine, tacrolimus), rituximab
(Harriet Lane Handbook, 23rd ed., p.703-704)
Q.5 - Write the steroid prescription for a 15 kg child (2 marks)
Diagnosis: Nephrotic Syndrome, first presentation
Weight: 15 kg
Calculation:
-
Induction dose: Prednisolone 2 mg/kg/day
- 2 × 15 = 30 mg/day (well within max 60 mg/day)
- Given as a single morning dose (after breakfast to reduce GI side effects)
- Duration: 4-6 weeks (standard ISKDC: 4 weeks daily OR up to 6 weeks)
-
Maintenance/Alternate day dose: Prednisolone 1.5 mg/kg on alternate days
- 1.5 × 15 = 22.5 mg (round to 22.5 mg or 25 mg) on alternate days
- Duration: 4-6 weeks, then taper and stop
Prescription:
Rx: Tab. Prednisolone 30 mg orally once daily (morning, after food) For 4 weeks Then: Tab. Prednisolone 22.5 mg orally on alternate days (morning, after food) for 4 weeks Then review for taper
Monitoring:
- Daily first-morning urine dipstick for protein (parent records at home)
- Remission = protein negative/trace for 3 consecutive days
- Blood pressure monitoring (steroid can cause hypertension)
- Watch for steroid side effects: growth, BP, glucose, mood
Key References:
- Harriet Lane Handbook, The Johns Hopkins Hospital, 23rd ed. - Nephrology Chapter (Nephrotic Syndrome, p.703-705)
- National Kidney Foundation Primer on Kidney Diseases, 8e - Nephrotic Syndrome, p.190
- Goldman-Cecil Medicine - Renal Biopsy Indications, p.2743All three question sets have been answered comprehensively above. Here is a brief summary of coverage:
All 22 questions answered across the three exam papers:
Photo 1 (15 marks - Q1 to Q5):
- Q1: Nephrotic syndrome (MCNS) as cause of swelling - pathophysiology explained
- Q2: 7 differential diagnoses of anasarca with key differentiating points
- Q3: Full investigation panel with expected findings (urine, blood, imaging)
- Q4: Complete treatment - prednisolone regimen + 8 supportive measures
- Q5: 8 complications including infections, thromboembolism, AKI, steroid side effects
Photo 2 (25 marks - Q15 to Q22):
- Q15: Diagnosis - Nephrotic Syndrome (MCNS)
- Q16: Investigation panel
- Q17: Causes of proteinuria in children (transient + pathologic by type)
- Q18: Full treatment protocol with steroid dosing
- Q19: Cerebral venous sinus thrombosis (hypercoagulable complication)
- Q20: Relapse causes (relapse most likely, infection trigger)
- Q21: Renal biopsy justified - hypertension is atypical for MCNS
- Q22: 11 indications for renal biopsy in nephrotic syndrome
Photo 3 (20 marks - Q1 to Q5):
- Q1: 5 most common causes of anasarca in children
- Q2: Detailed comparison table - Nephritic vs. Nephrotic syndrome (12 features)
- Q3: Definition of nephrotic-range proteinuria + diagnostic criteria
- Q4: 4 steroid-response classifications with definitions
- Q5: Precise prescription - Prednisolone 30 mg/day × 4 weeks → 22.5 mg alternate days × 4 weeks
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