UNASSIGNED: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE.
UNASSIGNED: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey.
UNASSIGNED: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed.
UNASSIGNED: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6-12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for <3 months by nearly 50%. UDCA is prescribed for <3 months, ≤6 months, and 6 months-1 year by 47.1%, 23.5%, and 23.5% responders, respectively. Nearly 50% prescribe steroids (mostly prednisolone), and among them, two-thirds prescribe it for 6-12 weeks. Approximately 60% give antiviral drugs to children who are cytomegalovirus immunoglobulin M positive. In our survey, 50% of experts perform 5-10 KPE per year, and 25% each perform 10-15 and >15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus.
UNASSIGNED: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA.

The purpose of this study was to prepare clinical practice guidelines for biliary atresia according to the Medical Information Network Distribution Service (MINDS) Handbook for Clinical Practice Guideline Development 2014. The guideline drafting group determined 25 clinical questions (CQs) essential for daily clinical practice. These CQs were grouped into the following four major categories: diagnosis, treatment, complications, and prognosis. A systematic literature search was performed by the systematic review team, and medical evidence was extracted from articles published until July 2014 on PubMed and the Japan Medical Abstracts Society. To reach a consensus, majority voting (>70%) of the panel of the guideline drafting group was adopted according to the Delphi technique. Recommendations for 21 CQs were appropriately provided. However, for four CQs (CQ2, stool color; CQ9, steroid administration; and CQ24 and CQ25, liver transplantation), recommendations could not be determined due to a scarcity of evidence. The first clinical practice guidelines for biliary atresia were successfully created using procedures recommended by the MINDS. It is expected that these guidelines will be used worldwide, and that the standardization of biliary atresia treatment will improve treatment outcomes.

Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.

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Initial surgical therapy of biliary atresia (BA) consists of the radical excision of extrahepatic remnants and portoenterostomy. However, despite this procedure, which was introduced by Kasai, BA remains the commonest indication for paediatric liver transplantation. The goal of the work group on the technical aspects of Kasai portoenterostomy procedures during the European Biliary Atresia Registry Conference 2007 was to achieve consensus on various operative and perioperative aspects relevant for paediatric surgeons. Although there is still disagreement regarding some of the technical details of the Kasai operation, a remarkable consensus has emerged in most areas of the actual surgery. No consensus was achieved on the role of postoperative drainage, the routine application of steroids, the use of oral prophylactic antibiotics, and the treatment of postoperative cholangitis. Nevertheless, the wide variation in reported results, which was a feature of this conference, suggests that there are still areas where improvement in outcomes can be anticipated by changes in technique or practice.