BILIARY ATRESIA AND THE POLYSPLENIA SYNDROME
نویسندگان
چکیده
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 کاملThe Role of Appendix in the Management of Biliary Atresia Associated with Bowel Atresia
Biliary atresia is characterized by the progressive obliteration of extra- and intrahepatic biliary duct system leading to the obstruction of bile flow in infancy. The cause(s) of biliary atresia remain unclear and many surgical options for bypassing the atretic segment have been described.1-3 Biliary atresia may be associated with small bowel atresia. The surgical interventions for biliary dr...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1987
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198704010-00610