Korean J Pathol.  1997 Apr;31(4):361-365.

Potter's Syndrome with Adult Polycystic Renal Disease: An autopsy case report

Affiliations
  • 1Department of Pathology, College of Medicine, Chungbuk National University, Chungju 360-240, Korea.
  • 2Department of Pediatrics, College of Medicine, Chungbuk National University, Chungju 360-240, Korea.

Abstract

Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.

Keyword

Potter's syndrome; Adult polycystic kidney disease(Potter type III)

MeSH Terms

Adult*
Autopsy*
Epithelial Cells
Fibrosis
Humans
Keratins
Mucin-1
Pancreatic Cyst
Polycystic Kidney Diseases*
Keratins
Mucin-1
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