Open AccessBILATERAL RENAL DYSPLASIA ACCOMPANIED BY PANCREATIC FIBROSIS, MECONIUM ILEUS, AND SITUS INVERSUS TOTALIS
Author(s) -
Yoshikawa Yasuji,
Watanabe Teruo,
Kato Nobuyoshi
Publication year - 1981
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1981.tb02809.x
Subject(s) - medicine , meconium ileus , situs inversus , cystic fibrosis , dysplasia , autopsy , pathology , fibrosis , congenital hepatic fibrosis , renal dysplasia , meconium , gastroenterology , kidney , pregnancy , fetus , portal hypertension , genetics , cirrhosis , biology
An autopsy case of bilateral renal dysplasia with other congenital malformations is reported. Malformations included severe pancreatic fibrosis with meconium ileus, situs inversus totalis, cardiovascular anomalies, and others. The only syndrome of multiple congenital malformations involving renal dysplasia which is similar to the present case is Ivemark's syndrome, though the present case lacked hepatic lesions. Apart from the pancreatic lesion, there were no abnormalities compatible with cystic fibrosis (mucoviscidosis). It was considered that meconium ileus in the present case was caused by pancreatic achylia secondary to severe pancreatic fibrosis, unrelated to cystic fibrosis, but closely allied to renal dysplasia.