Major causes of congenital nephrotic syndrome

Primary causes

Genetic disorders

Finnish-type congenital nephrotic syndrome due to variants of NPHS1, which encodes nephrin
Familial focal segmental glomerulosclerosis due to variants of NPHS2, which encodes podocin
Denys-Drash syndrome due to variants of WT1, which encodes the transcription tumor suppressor
Pierson syndrome due to variants of LAMB2, which encodes laminin beta 2
Familial steroid-resistant nephrotic syndrome due to variants of NPHS3 (PLCE1), which encodes phospholipase C epsilon
Other genetic disorders that present with congenital nephrotic syndrome include Galloway-Mowat syndrome, mitochondrial disorders, Herlitz junctional epidermolysis bullosa, and some cases of nail-patella syndrome

Idiopathic nephrotic syndrome*

Secondary causes

Infection

Congenital syphilis
Congenital toxoplasmosis

Immune disorders

* Idiopathic nephrosis rarely occurs at birth, more commonly presenting during the first year of life. All of the morphologic variants of idiopathic nephrotic syndrome seen in older children can occur at this time including minimal change disease, diffuse mesangial proliferation, and focal segmental glomerulosclerosis.

Graphic 80781 Version 4.0

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Major causes of congenital nephrotic syndrome