Biliary atresia is a rare disease of extrahepatic gall duct obstruction that leads to close - stage liver disease in child . The obstruction of bile duct leads to the accumulation of bile in the liver itself giving rise to inflammatory process in the liver that finally leave toliver hurt . It is the most common cause of cholestasis in newborn and also the most usual cause of liver transplantation in tyke . bilious atresia is more common in people of Asiatic or African - American origin with a slightly more distaff taste .
It can be congenital known as fetal ( embryonic ) bilious atresia that is present at the clip of birth and may or may not be related to other congenital anomaly . The other type is acquired , also known as perinatal biliary atresia , which is mostly assure 2 to 4 weeks after the birthing of the neonate with the first sign ofjaundice .
Causes and Pathogenesis of Biliary Atresia
The exact reason of biliary atresia is still unnamed , but there are several hypotheses regarding its onset . In some babe , there has been some evidence regarding change and disfigurement in the early development of gall duct intrauterine that could be catch in antenatalultrasound . Congenital biliary atresia has been associated with other inborn anomalies such as polysplenia , asplenia , situs inversus , choledochal cyst and hepatic vascular inflow anomalies , which hold strong grounds that all these anomalies are a result of problems associate in the first trimester when organogenesis takes topographic point . genetic science have also been studied regarding pathogenesis of biliary atresia that might predispose to viral infections . However , there is no strong grounds of any genetic predisposition , as it does not run in family and there have been cases where one twin is stomach with biliary atresia and the other born healthy .
Perinatal biliary atresia has been affiliate with GI viral infections let in cytomegalovirus , reovirus type 3 , Epstein Barr virus , human papillomavirus and rotavirus case A. These computer virus have been eff to induce proinflammatory factors responsible for the wrong of bile duct . resistant arrangement dysfunction has also been postulated as a cause for biliary atresia . All these studies necessitate further validation to demonstrate the right pathogenesis for bilious atresia .
Can Biliary Atresia Be Prevented?
Biliary atresia is a fatal disease if left untreated . Since the causa of it is largely unknown , so nothing can be done to preclude the onset of the disease . If there were any definite causal agency for the developing of biliary atresia , then one could work on prevent the cause , but there is no definitive cause , just hypothesis , so nothing can be done to prevent the disease once if affects a child . However , one can always forbid the progression of the disease and ensue complication by early diagnosing and discourse . Biliary atresia and fibrosis related to it is a apace progressing status , so it is necessary to take prompt treatment for it before it is too late and the complications consociate with it can not be prevented . The treatment should be done as early as potential , usually before the babe is 3 calendar month older to get effective postoperative results . Kasai procedure helps children to turn and remain in expert health for several years .
The only in force direction of biliary atresia is Kasai procedure that involves removal of the obliterated extrahepatic gall duct and re - establish the connection between liver and small gut by connecting a segment of minor intestine directly with the liver . This subprogram helps regain bile drain in about 80 % cases . There is normalization of bilirubin story within 3 months postoperatively , whereas the stool colour also comes back to normal within first postoperative workweek . The most vernacular complications of OR include cholangitis and portal high blood pressure .
There are high number of cases around 20 to 25 % where Kasai procedure is not successful , the gall flow is not reconstruct after which life threatening complicatedness of fibrosis and cirrhosis develops that mandate liver transplant . The five - yr natural selection rate with native liver is 40 to 65 % , whereas , three - year survival rate with liver transplantation is about 85 to 90 % .
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