Title: International Clinical Practice Guidelines for Diagnosing & Treating Biliary Atresia
Edition: Original
Classification: Standard guideline
Field: Diagnosis and Treatment
Countries and regions: China
Guidelines users: Pediatric surgeons, pediatric transplantation doctors, pediatric infection doctors, pediatric gastroenterologists, pediatric nutritionists, pathology doctors, imaging doctors, gynecologists, etc.
Evidence classification method: The GRADE ratings of very low-, low-, moderate-, or high-quality evidence reflect the extent of confidence that the diagnostic measures obtained are correct. A high level of evidence refers to consistent evidence from well-performed and high-quality studies or systematic reviews (low risk of bias, direct, consistent, and precise). Moderate/ low level of evidence refers to evidence from studies or systematic reviews with few important limitations. Very low level of evidence refers to evidence from studies with serious flaws, only expert opinion, or standards of care. Evidence from randomized controlled trials (RCT) starts at high quality, and evidence that includes observational data starts at low quality due to mixed residual evidence. The certainty of the evidence increases or decreases for several reasons. Very low-quality evidence: the real effect is likely to be significantly different from the estimated effect; low-quality evidence: the real effect may be significantly different from the estimated effect; moderate-quality evidence: the authors believe that the true effect may be close to the estimated effect; high-quality evidence: the authors are confident that the true effect is similar to the estimated effect.
Development unit: Jianghua Zhan
Registration time: 2023-06-07
Registration number: PREPARE-2023CN369
Purpose of the guideline: Biliary atresia (BA) progresses rapidly and early diagnosis is very important. If not treated in time, biliary cirrhosis, portal hypertension, and even liver failure may occur. Kasai portoenterostomy (KPE) is the first choice of treatment, the 5-year survival rate of autologous liver after the operation is about 80%. Around 75% of patients need to perform liver transplants finally, the 10-year survival rate is about 60%. Accordingly, the treatment of BA is necessary while the treatment efficacy is still unsatisfactory in some patients. Although KPE has been performed widely, the post-operative regulation strategy is still not unified, and some patients have liver transplants very early after KPE. In summary, the diagnosing and treatment strategy is not standardized and unified. BA has imposed a huge economic and medical resources burden on families and societies, and some families of patients chose to give up treatment. Therefore, it is necessary and of great significance to draw up an international guideline for diagnosing & treating BA. This guideline aims to help doctors diagnose and treat the disease, provide the latest insight for pediatric surgeons who are appropriate to perform KPE, prompt pediatric liver transplant surgeons what are appropriate surgical indications for liver transplantation in necessary situations, and guide how to manage after the operation.