Pediatric and Developmental Pathology

, Volume 2, Issue 1, pp 94-100

First online:

Ivemark's Renal-Hepatic-Pancreatic Dysplasia: Analytic Approach to a Perinatal Autopsy

  • Robert W.  BendonAffiliated withDepartment of Pathology, Kosair Children's Hospital, 231 E. Chestnut Street, Louisville, KY 40202, USA

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A case of Ivemark's renal-hepatic dysplasia syndrome is presented. The findings are suggestive of a ductal obstructive process anatomically located at the junction of ducts with the parenchyma. The differential diagnosis includes Meckel syndrome, short rib polydactyly syndromes, and glutaric aciduria type 2. The molecular basis for this syndrome remains unknown.

Key words: kidney, liver, pancreas, dysplasia, glomerulocystic, Ivemark's syndrome