nephrotic syndrome, type 4
MONDO:0009733Mondo
Findings
No curated finding names nephrotic syndrome, type 4 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Nephrotic syndrome within the first three motnhs of life, characterized initially by increased mesangial matrix, with or without hypertrophy and hyperplasia of podocytes, and eventual glomerular sclerosis.
Definition from the Mondo Disease Ontology (MONDO:0009733), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Progressive · Childhood onset
HPO, annotations 2026-09-02
Features
7 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Diffuse mesangial sclerosisHPOHP:0001967
- 20 of 20 reported patients
- Nephrotic syndromeHPOHP:0000100
- 24 of 24 reported patients
- Stage 5 chronic kidney diseaseHPOHP:0003774
- 18 of 19 reported patients
- Ambiguous genitaliaHPOHP:0000062
- 7 of 24 reported patients
- GonadoblastomaHPOHP:0000150
- 2 of 24 reported patients
- Ectopic testisHPOHP:6000460
- 1 of 14 reported patients
- Renal insufficiencyHPO
Where it sits
- A kind of
Other names
3 names
Resolves to: nephrotic syndrome, type 4
- Also called
- congenital nephrotic syndrome - diffuse mesangial sclerosisnephrotic syndrome caused by mutation in WT1WT1 nephrotic syndrome