Abstract
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m2/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
Abbreviations:
- NS: nephrotic syndrome
- MCD: minimal change disease
- FSGS: focal segmental glomerulosclerosis
- SSNS: steroid-sensitive nephrotic syndrome
- SRNS: steroid-resistant nephrotic syndrome
- FRNS: frequently relapsing nephrotic syndrome
- KDIGO: Kidney Disease Improving Global Outcomes
- ESRD: end-stage renal disease
- VPF: vascular permeability factor
- CLC-1: cardiotrophin-like cytokine 1
- suPAR: soluble urokinase-type plasminogen activator receptor
- AKI: acute kidney injury
- DVT: deep vein thrombosis
- LCAT: lecithin-cholesteryl acyltransferase
- RAAS: renin-angiotensin-aldosterone system
- ENaC: epithelial sodium channel
- ANA: antinuclear antigen
- anti-dsDNA: anti-double stranded DNA
- ANCAs: anti-neutrophil cytoplasmic antibodies
- ASOT: anti-streptolysin titres
- CNIs: calcineurin inhibitors
- MMF: mycophenolate mofetil
- CNS: congenital nephrotic syndrome
- CMV: cytomegalovirus
- uPCR: urine protein:creatinine ratio