This phenotype is rather comparable (possibly even milder) to the much less severe clinical phenotype described in patients with SRNS/FSGS caused by the in trans association of a pathogenic NPHS2 variant and the non-neutral polymorphism c.686G > A (p.Arg229Gln) that causes late-onset SRNS at a mean age of 13 years (range 0–39 years) and progresses to ESRD by the age of 26 years (ranging from 10 to 50 years) (10, 12, 16). Here, NPHS2 is linked to focal segmental glomerulosclerosis.