ISSN: 1307-279X

Yıl: 2016 Cilt: 37-38 Sayfa: 989-992

doi: 10.15659/yeditepemj.17.01.386

ABERNETHY SYNDROME; REPORT OF A CASE

FATMA TUBA COŞKUN, MELTEM UĞRAŞ, ENDİ ROMANO, MERT LEVENT BARUT, ERCAN KOCAKOÇ

Congenital extrahepatic portosystemic shunt known as Abernethy malformation is a rarely seen disorder. It is classified into two types based on the pattern of anastomosis between the portal vein(PV) and inferior vena cava(IVC), and the presence or absence of an intrahepatic portal venous supply.  Patients with Abernethy malformations   have cardiac and hepatic abnormalities. It rarely can lead to hepatocellular carcinoma in the long term. Here we report a 16- years- old girl who is diagnosed as Abernethy malformation as a result of investigations of early puberty.

Anahtar Kelimeler (Keywords): ABERNETHY SYNDROME, portosystemic shunt, child

ABERNETHY SENDROMU; BİR OLGU SUNUMU

Konjenital ekstrahepatik portosistemik şant (Abernethy malformasyonu) çok nadir görülen bir bozukluktur.  It is classified into two types based on the pattern of anastomosis between the portal vein(PV) and inferior vena cava(IVC), and the presence or absence of an intrahepatic portal venous supply.  Patients with Abernethy malformations   have cardiac and hepatic abnormalities. It rarely can lead to hepatocellular carcinoma in the long term. Here we report a 16- years- old girl who is diagnosed as Abernethy malformation as a result of investigations of early puberty.

Anahtar Kelimeler (Keywords): Abernethy sendromu, portosistemik şant , çocuk

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