Nephrotic syndrome type 2 (NPHS2) is an inherited disorder characterized by progressive kidney disease. Symptoms typically start to appear in infancy or childhood, though later onset has been reported. Children with NPHS2 have high levels of protein in their urine, low protein levels in their blood, high fat levels in their blood and excessive fluid accumulation throughout the body. Progression to end-stage renal disease is usually rapid, occurring within the first or second decade. This syndrome is resistant to steroid therapy. Intellect is not affected by NPHS2. NPHS2 can be treated with kidney transplant, which is typically curative; however, recurrence of kidney disease following transplant has been reported. NPSH2 is caused by pathogenic variants in the NPHS2 gene.