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Nephrotic syndrome in children
In a child aged 1–12 years with oedema, heavy proteinuria and low albumin, the cause is almost always minimal change disease, so prednisone is started without a biopsy — and the response to steroids is what sets the prognosis.
Why it matters in the exam
The exam shows a child with periorbital oedema that has progressed to generalised oedema, 3+ protein on the dipstick and low albumin. The questions ask for the diagnosis, whether a biopsy is needed, the treatment and the complications: infection (primary pneumococcal peritonitis), thrombosis (including renal vein) and hypovolaemia. A companion question asks to distinguish it from a nephritic syndrome — haematuria, hypertension and a low C3 after a throat or skin infection.
The terms to remember: remission (urine free of protein for three days in a row), steroid resistance (no remission after 4 weeks of full-dose prednisone) and steroid dependence or frequent relapses. In steroid resistance, a biopsy and genetic testing are done, and FSGS is usually found. For frequent relapses, steroid-sparing drugs are used, such as levamisole, MMF, calcineurin inhibitors and rituximab.
In supportive care, it is important to restrict salt, to treat severe oedema cautiously (albumin with a diuretic only for hypovolaemia or disabling oedema) and to make sure the child is vaccinated against pneumococcus, and to avoid live vaccines during high-dose steroids.
Key points
- At age 1–12, nephrotic syndrome is usually minimal change disease, which responds to steroids.
- Definition: heavy proteinuria, albumin below 30 g/L and oedema.
- Prednisone 60 mg/m² per day for 4 weeks, then 40 mg/m² on alternate days for 4 weeks.
- No remission after 4 weeks = steroid resistance → biopsy (FSGS).
- Complications: pneumococcal peritonitis, thrombosis, hypovolaemia.
Summary table
| Issue | Nephrotic syndrome (minimal change) | Nephritic syndrome (post-streptococcal) |
|---|---|---|
| Definition | Proteinuria of 40 mg/m² per hour or more (or UPCR of 200 mg/mmol or more), albumin below 30 g/L, oedema | Haematuria with red-cell casts, hypertension, oedema, oliguria |
| Age and background | 1–12 years, sometimes after a viral infection | 1–2 weeks after a throat infection or 3–6 weeks after a skin infection |
| Laboratory | High cholesterol, normal complement | Low C3 that returns to normal within 8 weeks; ASO or anti-DNase B |
| Treatment | Prednisone 60 mg/m² per day for 4 weeks, then 40 mg/m² on alternate days for 4 weeks | Supportive: salt and fluid restriction, diuretics, treatment of hypertension |
| Complications | Infection (pneumococcus), thrombosis, hypovolaemia | Hypertensive encephalopathy, kidney failure |
Example question from the licensing exam (IMLE)
A 3-year-old boy presents with facial and limb edema. Urinalysis reveals significant proteinuria and blood work results are presented in the table. Test Result Normal values Serum albumin 1.5 mg/dl [g/dL in the Hebrew version] 3-5 Serum total cholesterol 300 mg/dl 100-150 Which of the following is a possible complicate his most likely disease?
- A. Intussusception
- B. Hemolytic anemia
- C. Gastrointestinal bleeding
- D. Renal vein thrombosis
- E. Mitral valve prolapse
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Sources
- IPNA Clinical Practice Recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome, 2022 — Definitions and initial steroid treatment
- KDIGO Clinical Practice Guideline for the Management of Glomerular Diseases, 2021 — Nephrotic syndrome in children
- Nelson Textbook of Pediatrics, 22nd edition (2024) — Nephrotic Syndrome; Acute Poststreptococcal Glomerulonephritis
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.