Active clinical trials for "Biliary Atresia"

Cholangitis is the most common postoperative complication of biliary atresia, with a reported incidence of 40-90%, which seriously affects the surgical effect, survival rate and the quality of life and prognosis of patients. Without of direct evidence, the diagnosis of cholangitis sometimes is difficult to make, thus most of them are diagnosed based on the symptoms of children. According to literature reports, different centers and regions have different diagnostic criteria for postoperative cholangitis after hepatic portoenterostomy, which has a great influence on the accuracy of the incidence rate and appropriate treatment of cholangitis, and also brings differences in the analysis of the causes and prognostic factors of cholangitis. Based on the above reasons, we used the Delphi method，in which worldwidely 48 experts participated in, to establish the diagnostic scoring system for postoperative cholangitis after biliary atresia. Now we aimed to verify the specificity and sensitivity of the new scoring system through clinical cases, in order to unify and standardize the diagnostic criteria and provide help for the diagnosis and treatment of cholangitis after biliary atresia.

Little is known about the factors that cause biliary atresia nor the factors that influence disease progression. The purpose of this study is to collect the pertinent clinical information, genetic material and body fluid samples to enable investigators to address the following aims: To identify the gene or genes implicated in the etiology of BA; To identify polymorphisms that may be important in disease progression such as HLA polymorphisms; To characterize the natural history of the older, non-transplanted child with BA.

Biliary atresia, idiopathic neonatal hepatitis, and specific genetic cholestatic conditions are the most common causes of jaundice and hyperbilirubinemia that continue beyond the newborn period. The long term goal of the Childhood Liver Disease Research Network (ChiLDReN) is to establish a database of clinical information and plasma, serum, and tissue samples from cholestatic children to facilitate research and to perform clinical, epidemiological and therapeutic trials in these important pediatric liver diseases.

Many factors have been proven that may affect NLS in BA patients after KP;however,the early predictors for NLS were not be finally detected.This study was designed to evaluate and identify the preoperative and early postoperative factors associated with NLS for early prediction in BA patients after KP.

Biliary atresia is a serious congenital anomaly characterized by persistent and progressive cholestatic jaundice. The incidence of biliary atresia is more common in East Asia, especially China, with an incidence of 2 per 10,000 live births. Liver transplantation is the only effective way to treat end-stage liver disease. However, distant organ damage, affecting the heart, brain, kidneys, lungs, and intestines, is still an important factor affecting the long-term survival of children after surgery. Desflurane is a volatile anesthetic commonly used in surgery. In order to observe the effect of desflurane on the incidence of early postoperative pediatric acute respiratory distress syndrome (PARDS) with biliary atresia who underwent living donor liver transplantation, and explore the related mechanism, a total of 165 infant patients underwent living liver transplantation due to biliary atresia from March 2023 to October 2023 are included in our single-center prospective study. They are randomly divided into propofol group (n=55), propofol and desflurane group (n=55) and desflurane group (n=55) according to the difference of intraoperative anesthesia maintenance. Gender, age, height, weight, PELD scores and other preoperative basic data are recorded. Operation time, anhepatic time and intraoperative blood loss volume are recorded. The basic information of liver donors are also recorded. Postoperative mechanical ventilation time, ICU stay time, tacrolimus concentration, total length of hospital stay and mortality during hospitalization are recorded. According to the the definition of PARDS recommended by the 2015 Pediatric Acute Lung Injury Consensus Conference is used as the diagnostic and grading criteria for postoperative PARDS, and the incidence and grading of PARDS within the first seven days after surgery are evaluated in the three groups. Peripheral blood is collected immediately after anesthesia induction, 30min after reperfusion and at the end of surgery to detect serum levels of HMGB1, IL-6 and TNF-α by enzyme linked immunosorbent assay (ELISA).

This study is non-inferiority trial design. This study aimed to investigate the effect of prophylactic oral antibiotics on preventing cholangitis in biliary atresia (BA) patients after Kasai portoenterostomy (KP) by comparing the cholangitis rate in BA patients who received prophylactic oral antibiotics and those who did not. The patients were followed up for 2 years after KP.

The goal of this clinical trial is to compare the safeness and effectiveness of traditional esophagogastroduodenoscope (EGD) and wired magnetically assisted capsule endoscopy (MACE) in the diagnosis of esophageal varices in biliary atresia (BA) patients. The main questions it aims to answer are: Subjects who do wired magnetically assisted capsule endoscopy do not need to open the mouths during the process, this study also want to know whether wired magnetically assisted capsule endoscopy can reduce the generation of droplets. Diagnostic accuracy between traditional esophagogastroduodenoscope and wired magnetically assisted capsule endoscopy in biliary atresia patients with esophageal varices. Participants will do either traditional esophagogastroduodenoscope or wired magnetically assisted capsule endoscopy.

Biliary atresia is the most severe form of cholestatic liver disease. The children have high morbidity and mortality and get devastating pruritus and fatigue, failure to thrive, progressive hepatic failure and impaired neurodevelopment. The etiology is mostly unknown. More than half need a new liver from a living or deceased donor during childhood. However, correct timing of the transplantation is extremely difficult because of lack of consensus based on clinical assessment tools. All though the incidence is low, the cost of this disease is tremendous from both a clinical and human perspective. So far, protocolized neurodevelopment tests, genetic profiling, precise malnutrition evaluation based on clinical appearance, biochemical markers and brain MRI-scans, body composition, immunological function, level of physical activity and optimal time of transplantation in cholestatic children are unknown. The aim is to determine risk factors for neurocognitive impairment in children suffering from severe cholestasis in order to determine optimal time for liver transplantation from a brain perspective. In a prospective study, the investigators will investigate risk factors related to brain-, heart-, gut- and immunological function in the Danish cohort. This cohort consists of 75 children aged 0-18 years. In addition, 30 aged and gender matched healthy and 20 tetra fallot children will serve as control groups. The children will undergo extensive and advanced liver function evaluation, genetic profiling, nutrition and immunological status, neuro-imaging and neurocognitive evaluation at time of diagnose, 2 years of age, pre-school, pre-teenage, and teenage. In case of a liver transplantation, additional neuro-cognitive tests will be performed

The aim of this study was to developed and validated models to predict hepatic decompensation and survivals in pediatric patients with cirrhosis and compared these models with currently available models.

Better survival and prognosis of biliary atresia (BA) depend on early diagnosis and timely Kasai portoenterostomy. Identifying BA from other causes of infantile cholestasis at early stage of the disease still remains a major challenge. In this study, the investigators aim to develop and validate a scoring system to screen BA in infants with cholestasis.