infundibulopelvic stenosis-multicystic kidney syndrome
Findings
No curated finding names infundibulopelvic stenosis-multicystic kidney syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Infundibulopelvic stenosis-multicystic kidney syndrome is a rare, genetic renal malformation syndrome characterized by variable degrees of malformation in the pelvicalyceal system (including unilateral or bilateral calyceal dilatation, infundibular stenosis, hypoplasia or stenosis of the renal pelvis) which lead to multicystic kidney. Clinically it exhibits abdominal, lumbar or flank pain, recurrent urinary tract infections, hypertension, proteinuria and often progresses to renal insufficiency. Calyceal dilatation and hydronephrosis are frequently seen on imaging.
Definition from the Mondo Disease Ontology (MONDO:0010971), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of