Abstract. Biliary Atresia is the most common cause of persistently direct (conjugated) hyperbilirubinemia in the first three months of life. It is a progressive inflammatory obliteration of the extra- and intrahepatic bile ducts whose pathogenesis remains speculative. This review updates the etio-pathological considerations, clinical manifestations, methods of diagnosis, surgical management, and results of this terrible disease in young infants. We will emphasize that persistent jaundice in the newborn period must be managed urgently, a diagnosis should be established early in life, and Kasai portoenterostomy offered to those infants with Biliary Atresia before their eighth week of life. This will allow more than one-third of children to survive.
Keywords: biliary atresia, hepatobiliary system, cholestatic diseases, ultrasound, liver parenchyma, echogenicity, gallbladder, hyperechoic cord, triangular cord sign, acholia, hepatomegaly, liver transplantation, cholestatic diseases.
References:
1. Акилов Х.А., Матякубов Х.Н .УСОВЕРШЕНСТВОВАНИЕ ДИАГНОСТИКИ И ХИРУРГИЧЕСКОЙ ТАКТИКИ ЛЕЧЕНИЯ МЕХАНИЧЕСКОЙ ЖЕЛТУХИ У ДЕТЕЙ Вестник экстренной медицины, 2017, X (4) ст.44.
2. Акилов Х.А., Урманов Н.Т., Акилов Б.Б., Примов Ф.Ш., Асадав Ш.Я., Ходжаяров Н.Р. БИЛИАРНАЯ АТРЕЗИЯ КАК ПРИЧИНА МЕХАНИЧЕСКОЙ ЖЕЛТУХИ Вестник экстренной медицины, 2017, X (4), ст.81-82.
3. Ортиқбоева Н.Т., Абдухалик-заде Г.А. ҲОМИЛА ИЧИ ИНФЕКЦИЯСИНИНГ ҲОМИЛА ИММУНОЛОГИК ҲИМОЯСИГА ТАЪСИРИ Журнал репродуктивного здоровья и уро-нефрологических исследований 2021 СТ. 311.
4. Акилов Х.А., Матякубов Х.Н , Диагностика и хирургическая коррекция кистозной трансформации внепеченночных желчных протоков у детей, Актуальные вопросы детской хирургии, анестезиологии и реаниматологии 2017 ст. 7-8.
5. Ramachandran P, Safwan M, Srinivas S, Shanmugam N, Vij M, Rela M. The extended Kasai portoenterostomy for biliary atresia: A preliminary report. J Indian Assoc Pediatr Surg. 2016 Apr-Jun;21(2):66-71. [PubMed]
6. Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, Sokol RJ. Biliary Atresia: Clinical and Research Challenges for the Twenty-First Century. Hepatology. 2018 Sep;68(3):1163-1173. [PMC free article] [PubMed]
7. Schooler GR, Mavis A. Cystic biliary atresia: A distinct clinical entity that may mimic choledochal cyst. Radiol Case Rep. 2018 Apr;13(2):415-418. [PMC free article] [PubMed]
8. Wang L, Yang Y, Chen Y, Zhan J. Early differential diagnosis methods of biliary atresia: a meta-analysis. Pediatr Surg Int. 2018 Apr;34(4):363-380. [PubMed]
9. Chung PHY , Zheng S ,Tam PKH.Biliary atresia: East versus west. Seminars in Pediatric Surgery. 2020; 29150950
10. Bezerra JA , Wells RG ,Mack CL ,Karpen SJ ,Hoofnagle JH ,Doo E et al. Biliary Atresia: Clinical and Research Challenges for the Twenty-First Century.Hepatology. 2018; 68: 1163-1173
11. Tam PKH ,Chung PHY ,St Peter SD ,Gayer CP ,Ford HR ,Tam GCH et al.Advances in paediatric gastroenterology.The Lancet. 2017; 390: 1072-1082