No abstract available.
Biliary Atresia*

No abstract available.
Biliary Atresia*

The liver is an important organ of the human body, playing a major role in the metabolism and storage of nutrients, synthesis of protein and other nutrients, as well as detoxifying many metabolic by-products. The response of the foetal and newborn liver to external insult and injury is limited. This is because the ability of the closely interdependent structures of a developing liver of expressing in the face of a variety of insults is limited as well. Thus most infants with insults to the liver present as cholestatic jaundice with variable degree of pale stools, enlarged liver and conjugated hyperbilirubinaemia. Biliary atresia, an idiopathic condition characterized by progressive fibrosing obliteration of both intra- and extrahepatic bile ducts, is the most important cause of neonatal cholestasis worldwide, including Malaysia. It is also the most important indication for childhood liver transplantation the world over. Challenges facing infants with biliary atresia include a delay in the diagnosis and late surgery, leading to a poor outcome. This often results from a failure to recognise the potential serious nature of an infant with prolonged cholestatic jaundice and pale stools among health care professionals. (JUMMEC 2010; 13(2): 72-79)
Biliary Atresia

Objective. To determine factors predictive of obstructive neonatal cholestasis among Filipino infants and to describe their outcome.

Methods. Jaundiced infants within the first eight weeks of life with liver biopsy were included. Excluded were cholestasis secondary to metabolic or infective causes. Retrospective chart review (2009-2012) and prospective recruitment of patients (2013) were done. A final diagnosis of non-obstructive or obstructive neonatal cholestasis was made on clinical, biochemical, ultrasonographic, and histologic findings, using histology and/or operative cholangiogram as the gold standard. The outcome was assessed on the 6th and 12th months from diagnosis. The crude odds ratio for obstructive jaundice was computed. Multiple logistic regression on significant variables (p-value <0.05) was done.

Results. Two hundred sixty-three (263) patients were included: 161 with non-obstructive and 102 with obstructive cause. Mean age at first consult was higher in those with obstruction. On logistic regression, females (OR:2.3), absence of a family history of idiopathic neonatal hepatitis (OR:4), and persistently pale/acholic stools (OR:13) were predictive of obstruction. 85% of patients with a non-obstructive cause are alive and well, while 80% of patients with obstruction have died.

Conclusion. Among jaundiced infants females, the absence of a family history of idiopathic neonatal hepatitis and persistently pale yellow/acholic stools were predictive of obstruction. The outcome was poor in patients with obstructive jaundice.

Biliary atresia is a progressive obliterative cholangiopathy, but its actual causes are still unknown. However, a number of factors, such as developmental malformation, viral infection, toxicity of bile constituents and an anatomical abnormality in the hepatobiliary system, have been considered. Herein, two very unusual cases of biliary atresia, associated with ileal atresia, are reported. The pathogenesis of this particular type of biliary atresia is still a matter for debate.
Bile ; Biliary Atresia* ; Intestinal Atresia

No abstract available.
Biliary Atresia* ; Hepatitis*

Biliary Atresia ; Cholestasis* ; Prognosis

During 5 years (1994-1998), 147 children suffering from congenital biliary atresia were admitted to NIP. Clinical manifestations include icterus, discolored stool and hepatomegaly. The gallbladder is absent; ultrasonography shows that its diameter doesn't change before, during and after meal in all patients. 136 patients among those children require a liver's transplantation. It means that, every years, there were 27 children suffering from biliary atresia who require a liver's transplantation.
Liver Transplantation ; Biliary Atresia

No abstract available.
Biliary Atresia* ; Hepatitis*

No abstract available.
Biliary Atresia* ; Fractures, Spontaneous*