Table 2.

Renal phenotype as a function of underlying genetic defect

Age at Onset (days)	Mutated Gene			No Mutation
	NPHS1a,b	NPHS2	Otherc
Proteinuria
0 to 7d	47	8	1	11
8 to 30	12	5	2	6
31 to 60	4	4	1	4
61 to 90	0	3	2	1
NS
0 to 7d	46	7	1	9
8 to 30	14	6	2	7
31 to 60	3	2	0	4
61 to 90	0	5	3	2

^aOnly patients with two mutated NPHS1 alleles were considered for genotype–phenotype associations (n = 63).

^bThree patients bearing one NPHS1 mutation and one single-nucleotide polymorphism in the compound heterozygous state presented proteinuria and NS during the first week of life (n = 2) and at 45 days after birth (n = 1). In addition, three patients with single heterozygous mutations in NPHS1 presented NS within the first week of life.

^cIncludes patients with mutations in WT1, LAMB2, and PLCE1 and inherited mitochondrial disorders.

^dPatients with NS detected in utero or stillbirths with CNS were considered to have a disease onset at day 0.