منابع مشابه
Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.
UNLABELLED One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope sc...
متن کاملBiliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines
Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inv...
متن کاملBiliary Atresia Associated with Polysplenia Syndrome, Situs Inversus Abdominus, and Reverse Rotation of Intestine
In 25% of cases of biliary atresia associated malformations are present; polysplenia constitute 10-50% of these associated anomalies. Biliary atresia found associated with polysplenia syndrome, heterotaxy, and reverse rotation of intestine, in isolation or in various combinations; however its association with polysplenia syndrome, situs inversus abdominus, and reverse rotation of gut in the sam...
متن کاملExtra-Hepatic Biliary Atresia in Association with Polysplenia and Intestinal Malrotation
The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai's portoenterostomy at 52nd day of life, the child ...
متن کاملBiliary atresia
Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coin...
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ژورنال
عنوان ژورنال: Annals of Surgery
سال: 1998
ISSN: 0003-4932
DOI: 10.1097/00000658-199804000-00022