Nephrotic syndrome type 1 (NPHS1), also called congenital nephrotic syndrome of the Finnish, is an inherited disorder that causes progressive kidney disease. Affected children are usually born prematurely and kidney damage is usually present at birth or develops within the first few months of life. Additional symptoms include fluid accumulation inside the body, hypertension, high levels of protein in urine and low levels of protein in the blood. This syndrome is resistant to steroid therapy. Progressive kidney disease usually leads to end-stage renal failure and death within the first decade of life. Kidney transplant is curative, although there is a risk of recurrence of kidney disease after transplantation. Intellect is not affected by NPHS1. NPHS1 is due to pathogenic variants in the NPHS1 gene.