Infundibulopelvic stenosis: A long-term followup

D. A. Husmann, S. A. Kramer, R. S. Malek, T. D. Allen

Research output: Contribution to journalArticle

11 Scopus citations

Abstract

We followed 21 patients with congenital infundibulopelvic stenosis, a rare obstructive disorder of the intrarenal collecting system, for a median of 11 years (range 2 to 28 years). Of these patients 19 (90%) had evidence of bilateral renal disease. In particular, 10 patients had bilateral infundibular pelvic stenosis, 6 a contralateral cystic dysplastic kidney and 3 a congenitally absent kidney. Extended observations of the patients with this disorder revealed that end stage renal disease or renal insufficiency developed in 8 patients (37%), all with bilateral renal anomalies. Renal biopsies in patients with end stage renal failure revealed widespread areas of renal dysplasia proximal to the stenotic infundibulum with focal or global glomerulosclerosis of the glomeruli, which was not involved in the dysplastic process. These pathological findings are consistent with the presence of a renal hyperfiltration injury. Our data suggest that the prognosis of infundibulopelvic stenosis is dependent upon the extent of total renal mass involved with the disease process and the duration of followup.

Original languageEnglish (US)
Pages (from-to)837-840
Number of pages4
JournalJournal of Urology
Volume152
Issue number3
DOIs
StatePublished - Jan 1 1994

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Keywords

  • abnormalities
  • inborn errors
  • kidney diseases
  • kidney pelvis
  • renal tubular transport

ASJC Scopus subject areas

  • Urology

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