Biliary atresia in children. A clinical case

Abstract

Biliary atresia (BA) is an intra- and/or extrahepatic inflammatory cholangiopathy that is characterized by progressive obliteration of the bile ducts within the first weeks of life, leading to liver fibrosis development. BA is one of the causes of terminal stage chronic liver diseases formation in children. The pathogenesis is based on the obliteration of the bile ducts, which causes impaired bile outflow, intrahepatic cholestasis, accumulation of toxic bile acids, and progressive damage to hepatocytes. Clinical manifestations of the disease usually occur within the first weeks of life and include prolonged jaundice, acholic stool, and as, a result, the development of hepatosplenomegaly and signs of portal hypertension appearance. In the absence of timely diagnosis and early surgical intervention, many children may have a rapid progression of liver fibrosis with the formation of biliary cirrhosis. In case of late BA diagnosis or impossibility of performing portoenterostomy by Kasai, liver transplantation remains the only radical treatment method.

Aim - to inform medical practitioners of the main clinical signs, volume of necessary additional examination methods, course of BA in children, and the directions of differential diagnosis.

Clinical case. The authors described a clinical case of BA. The main diagnostic characteristics of the clinical picture were highlighted: complaints, anamnesis features, data from laboratory and instrumental research methods, as well as treatment methods with mandatory early surgical intervention.

Conclusion. Timely verification of BA diagnosis depends on the correct diagnostic algorithm, which ensures early detection of pathology. This allows the patient to be referred for surgical treatment in a timely manner, which contributes to the liver functional capacity preservation, prevents the progression of the disease, and the development of complications. In addition, early diagnosis improves the prognosis and quality of life.

The study was carried out according to the Declaration of Helsinki. The agreement of parents was obtained for the study.

Authors declare no conflict of interest.