Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

Abstract

Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation. Optimal pre-transplant management of these potentially life threatening complications and maximizing nutrition and growth require the expertise of a multi-disciplinary team with experience caring for biliary atresia. The timing of transplant for biliary atresia requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with biliary atresia often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical.

Biliary Atresia: Clinical and Research Challenges for the Twenty‐First Century

“…Even with surgical treatment, BA remains the most common cause of end‐stage liver disease and the most frequent reason for liver transplantation in children. Yet, despite its frequency and serious consequences, the cause(s) and pathogenesis of BA remain unknown …”

“…At present, however, at least half of children still require liver transplantation before 2 years of age. In addition, those with a successful HPE who survive infancy with a native liver often have progressive hepatic dysfunction, and ultimately >75% of them will require liver transplantation before 20 years of life …”

Living donor liver transplantation for biliary atresia: An analysis of 2085 cases in the registry of the Japanese Liver Transplantation Society

“…37,38 Although the PELD/model for end-stage liver disease (MELD) score were not available in the present study, the JLTS has attempted to assess the influence of certain pre-LDLT variables, including the PELD/MELD score, on the outcome after LDLT for BA, and a study is now underway along with a nationwide live donor long-term survey. 37,38 Although the PELD/model for end-stage liver disease (MELD) score were not available in the present study, the JLTS has attempted to assess the influence of certain pre-LDLT variables, including the PELD/MELD score, on the outcome after LDLT for BA, and a study is now underway along with a nationwide live donor long-term survey.…”

Large-scale proteomics identifies MMP-7 as a sentinel of epithelial injury and of biliary atresia

“…This rare disease results from a rapidly progressive inflammatory and fibrosing injury to extrahepatic bile ducts that interrupts the flow of bile and produces severe liver injury in otherwise healthy infants. With an onset of pathological jaundice limited to a highly reproducible window in the first 3–4 months of life of affected infants, the early diagnosis and surgical intervention are critical for the restoration of bile flow and improvement in short-term outcome (7, 9, 10). However, neonatal jaundice is a common sign for several clinical syndromes.…”