Abernethy malformation associated with Caroli’s syndrome in a patient with a PKHD1 mutation: a case report

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Abernethy malformation associated with Caroli’s syndrome in a patient with a PKHD1 mutation: a case report

BACKGROUND Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal ...

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Abernethy malformation: a case report

BACKGROUND Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. CASE PRESENTATION A 5-year-old female child presented with short history of jaundice. A provisional ...

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ژورنال

عنوان ژورنال: Diagnostic Pathology

سال: 2017

ISSN: 1746-1596

DOI: 10.1186/s13000-017-0647-y