Hereditary sclerosing glomerulopathy in the conorenal syndrome

Susan R. Mendley*, Andrew K Poznanski, Benjamin H. Spargo, Craig Langman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Kidney failure is recognized to occur in association with bone malformations, yet the renal disease often is incompletely characterized. In the syndrome of cone-shaped epiphyses of the phalanges and renal failure (conorenal syndrome), the kidney disease has been previously labeled "nephronophthisis" (now termed "medullary cystic disease"). We report two siblings with the conorenal syndrome in whom longitudinal clinical study has been possible and from whom kidney biopsy specimens were obtained prior to renal failure; their renal disease is incompatible with medullary cystic disease. The variable clinical course and nephropathology of this syndrome are characterized. These results call into question the association of medullary cystic disease of the kidney with other syndromes of bone dysplasia with renal failure.

Original languageEnglish (US)
Pages (from-to)792-797
Number of pages6
JournalAmerican Journal of Kidney Diseases
Volume25
Issue number5
DOIs
StatePublished - Jan 1 1995

Keywords

  • Conorenal syndrome
  • bone dysplasia
  • cone-shaped epiphyses
  • dysostosis
  • glomerulosclerosis
  • hereditary renal disease

ASJC Scopus subject areas

  • Nephrology

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