Nephrotic Syndrome in Children โ
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๐ง Nephrotic syndrome (children) occurs when podocyte injury leads to heavy protein loss in urine ๐ฉธโก๏ธ๐ง.
Loss of albumin lowers plasma oncotic pressure โ oedema ๐ง, while effective circulating volume may be low.
The liver responds with hyperlipidaemia ๐ง, and loss of anticoagulants (e.g., antithrombin) increases thrombosis risk ๐ฉธ.
โ
Definition (typical paediatric nephrotic syndrome)
- Generalised oedema ๐ง
- Nephrotic-range proteinuria: urine PCR >200 mg/mmol ๐งช
- Hypoalbuminaemia: albumin <25 g/L โฌ๏ธ
- Hyperlipidaemia ๐ง (supportive, not required for diagnosis)
โ ๏ธ Causes (childhood)
- Idiopathic nephrotic syndrome (mostly Minimal Change Disease) โ
- Secondary: infection, SLE/HSP, drugs, malignancy (less common)
- Other glomerular diseases with nephrotic-range proteinuria ยฑ nephritic features
๐งพ Clinical pattern categories
- โ
Steroid-sensitive (SSNS) โ remission with standard prednisolone course (1โ4 weeks)
- โป๏ธ Steroid-dependent (SDNS) โ relapse during taper or soon after stopping steroids
- โ Steroid-resistant (SRNS) โ failure to remit with initial steroid course; specialist pathway/biopsy required
๐ค Symptoms & signs
- Oedema: periorbital ๐๏ธ โ generalised (legs, ascites, scrotal/vulval)
- Weight gain โ๏ธ, lethargy, reduced urine output ๐ฑ
- Frothy urine โ๏ธ (protein)
- Red flags: abdominal pain, fever, breathlessness, severe headache, cool peripheries ๐จ
๐ฌ Investigations (first presentation)
- Urine: PCR/ACR, dipstick (blood), culture if febrile; microscopy as indicated
- Bloods: U&E/creatinine, albumin, cholesterol, FBC, CRP; LFTs if needed
- Atypical feature screen: complement (C3/C4), ANA, infection screens as indicated
- Renal biopsy: atypical features, steroid resistance, age extremes, systemic features (specialist decision)
๐ฉบ General management (key points)
- Early paediatric nephrology involvement ๐ฉโโ๏ธ๐จโโ๏ธ
- Salt restriction ๐งโฌ๏ธ (effective for oedema)
- Fluid management ๐ง โ assess intravascular volume carefully
- Diuretics ๐ โ specialist-guided only (risk of hypovolaemia/AKI)
- Albumin infusion ๐ โ severe/refractory oedema under specialist protocol
- Daily weight โ๏ธ and oedema charting
- Avoid NSAIDs ๐ซ (renal perfusion risk)
๐ Prednisolone for first presentation (common UK regimen)
- 60 mg/mยฒ/day (max 60โ80 mg) for 4 weeks
- 40 mg/mยฒ alternate days for 4 weeks
- Taper: 5โ10 mg/mยฒ alternate days weekly over ~4 weeks ๐
- Most SSNS children achieve remission early โ
๐งฏ Relapse & escalation
- Relapses are common; management guided by relapse frequency & steroid toxicity
- Frequent relapses / SDNS: steroid-sparing agents (levamisole, cyclophosphamide, calcineurin inhibitors) via nephrology
- SRNS: specialist pathway + biopsy; consider ACEi/ARB for proteinuria reduction
โ ๏ธ Complications to monitor
- Infections ๐ฆ (pneumococcal, cellulitis, peritonitis)
- Hypovolaemia / shock ๐จ (tachycardia, cool peripheries, abdominal pain, oliguria)
- AKI ๐ฉบ (from hypovolaemia, sepsis, nephrotoxins)
- Thrombosis / VTE ๐ฉธ (abdominal pain, haematuria, chest symptoms, limb swelling)
๐ Vaccines & infection prevention (UK)
- Routine immunisations up to date โ
- Annual influenza vaccine ๐ฆ โก๏ธ๐
- Pneumococcal vaccination/boosters per local pathway ๐
- Check varicella status; manage exposure carefully if immunosuppressed
- Avoid live vaccines during high-dose steroids & shortly after stopping
๐ References
-
NHS Greater Glasgow & Clyde (NHS Scotland). Idiopathic nephrotic syndrome: management in children.
View guideline
-
infoKID (UK paediatric kidney information). Nephrotic syndrome.
View infoKID page