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The Korean Journal of Pathology 1994;28(2): 200-202.
Meckel-Gruber Syndrome: An autopsy case report.
Kyung Ha Kang, Duck Hwan Kim, Hee Jin Chang, In Sook Kim, Jin Hee Sohn, Jung Il Suh
Department of pathology, National Medical Center, Seou, Korea.
ABSTRACT
Meckel-Gruber syndrome is a quite rare congenital disorder, characterized by posterior encepalocele, cleft palate and lip, polycystic kidneys, hepatic fibrosis with bile duct proliferation, and postaxial polydactyly and syndactyly. We experienced an autopsy case of M eckel-Gruber syndrome in a second baby of 28 year-old woman. At 26 weeks of gestation, congenital anomaly was detected on ultrasonographic examination and the pregnancy was terminated. Familial history was not noted.
Key Words: Meckel-Gruber syndrome; Polycystic kidney; Hepatic fibrosis with bile duct proliferation
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