Causes of nephrotic syndrome in infants and children

Primary nephrotic syndrome refers to nephrotic syndrome in the absence of systemic disease.

Idiopathic nephrotic syndrome:

Minimal change disease
Primary focal segmental glomerulosclerosis (FSGS)

Primary membranous nephropathy

Membranoproliferative glomerulonephritis (MPGN)

IgA nephropathy

C3 glomerulopathy

Secondary nephrotic syndrome refers to nephrotic syndrome that is either associated with systemic disease or is secondary to another process that causes glomerular injury.

Membranous nephropathy:

Systemic lupus erythematosus
Chronic hepatitis B infection

Secondary FSGS due to nephron loss resulting from:

Kidney scarring
Renal hypodysplasia

Infections:

Group A streptococcus (ie, poststreptococcal glomerulonephritis)
Infective endocarditis
Hepatitis B or C
HIV
CMV
Parvovirus B19
Congenital syphilis
Malaria

Lupus nephritis

Vasculitides:

IgA vasculitis (Henoch-Schönlein purpura)
ANCA-associated vasculitis

Drugs and toxins:

NSAIDS
Heroin
Lithium
Gold
Mercury

Malignancy:

Hodgkin disease
Non-Hodgkin lymphoma

Others:

Sickle cell disease
Hemolytic uremic syndrome
Amyloidosis

Genetic causes (causative genetic variant)

Finnish type congenital nephrotic syndrome (NPHS1)
Autosomal recessive steroid-resistant nephrotic syndrome (NPHS2)
Diffuse mesangial sclerosis (WT1, PLCE1, LAMB2)
Alport syndrome (COL4A5, COL4A4, COL4A3)
Charcot-Marie-Tooth disease (INF2)
Denys-Drash syndrome (WT1)
Fabry disease (GLA)
Frasier syndrome (WT1)
Galloway-Mowat syndrome (WDR73, LAGE3)
Herlitz junctional epidermolysis bullosa (ITGB4, ITGB3)
Mitochondrial disorders
Nail-patella syndrome (LMX1B)
Pierson syndrome (LAMB2)
Schimke immuno-osseous dysplasia (SMARCAL1)
Alpha-1 antitrypsin deficiency (SERPINA1)

This table summarizes the causes of nephrotic syndrome in infants and children. For additional details, refer to separate UpToDate content on nephrotic syndrome in infants and children.

IgA: immunoglobulin A; HIV: human immunodeficiency virus; CMV: cytomegalovirus; ANCA: antineutrophil cytoplasmic antibodies; NSAIDS: nonsteroidal anti-inflammatory drugs.

Adapted from: Niaudet P. Nephrotic syndrome: Classification and evaluation. In: Pediatric Nephrology, 8th ed, Emma F, Goldstein SL, Bagga A, et al (Eds), Springer Cham 2022.

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Causes of nephrotic syndrome in infants and children