View clinical trials related to Biliary Atresia.
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The purpose of this study is to determine whether pentoxifylline reduces liver damage in infants with biliary atresia.
The investigators plan to invite all children in the UK with biliary atresia, treated at the three national centres (Birmingham, Kings College and Leeds), over a three year period to take part in a randomised control study. The investigators aim to determine the effectiveness of CoSeal® Surgical Sealant (an anti-adhesive agent) in reducing intra-abdominal adhesions (scar tissue) and the morbidity caused by these adhesions in children treated with a Kasai hepatoportoenterostomy. Adhesions are common, if not invariable, after any abdominal surgery. They cause intra-abdominal organs to become stuck to each other and the abdominal wall. This means they are no longer completely free to slide over each other. In particular patients have a lifetime risk that the bowel can become kinked or twisted leading to complications such as bowel obstruction. Adhesions also make repeat abdominal operations more difficult. The adhesions have to be divided in order to separate the organs from each other and the abdominal wall. This can lead to blood loss and increases the risk of damage to these organs. Anti-adhesive agents have been created to reduce the severity of these adhesions, but there is little in the medical literature to evaluate their effectiveness, particularly in children. Biliary atresia is an obliterative obstruction of the bile ducts that occurs in infants. Initially they are treated by an abdominal operation called a Kasai portoenterostomy to restore bile flow from the liver to the intestines. However approximately 40% of these children will go on to require a liver transplant operation in the first two years of life. If CoSeal® Surgical Sealant is effective this could reduce the patients lifetime risk of complications from abdominal adhesions and also facilitate repeat abdominal operations for these children, in particular for those who go on to require a liver transplant.
Biliary atresia (BA) is a perinatal disease of unclear etiology, characterized by inflammation and obliteration of intrahepatic and extrahepatic bile ducts, leading to cholestasis and cirrhosis. Kasai operation remains as the first line operative treatment in BA. In the previous studies, inhalation anesthetics might induce liver damage. The choice of inhalation anesthetic that minimally affects hepatic function is important. Therefore, the purpose of this study is to compare the postoperative recovery and hepatic function between desflurane and sevoflurane, two most frequently used inhalational anesthetics in patients undergoing Kasai operation.
The goals of the proposed work are two fold: Firstly, to see if the antibiotic vancomycin may be used for the early treatment of Biliary Atresia (BA) and Primary Sclerosing Cholangitis (PSC). The investigators hope to learn what effect Vancomycin has on the bacteria that are present in stool, body fluid or intestinal tissue on someone who has BA and PSC and if so by what mechanism. Secondly, the investigators hope to learn to characterize human intestinal microbial communities (microbiome: the collection or collectivity of microorganisms) using molecular methods, examine the mechanisms of interaction between host and microbiome using genomic approaches, and determine how the microbiome both preserves local health and promotes pathology. The investigators will focus on primary sclerosing cholangitis, biliary atresia, as well as states of health. The composition of the associated microbiome will be assessed based on ribosomal DNA and RNA sequences, and attention will be given to richness (diversity), evenness (relative abundance), and variation with respect to time, person, and anatomic niche. Host response at the adjacent mucosal surface will be assessed based on genome-wide gene expression patterns.
This study evaluated laparoscopic (videosurgery) versus conventional (open surgery) Kasai portoenterostomy (anastomosis of small intestine to the liver hilus) in children with biliary atresia. The study was stopped due to lower survival with native liver 6 months after the laparoscopic operation. Follow-up after 24 months confirmed superior results after conventional operation.
Biliary atresia is a congenital disorder of bile duct development or destruction of established but immature bile ducts. The study tests the hypothesis that post-operative steroids improve outcome following the Kasai procedure - the commonest surgical treatment.
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.
The Children Liver Disease Research and Education Network (ChiLDREN) is conducting a clinical trial to evaluate whether long-term treatment with corticosteroids improves the outcome of the Kasai or gall-bladder Kasai in infants with biliary atresia. In this clinical trial, ChiLDREN is testing whether corticosteroid therapy following the Kasai will improve bile drainage and long term outcome in infants with biliary atresia. Subjects in this trial must start treatment within 72 hours of the Kasai procedure and be part of a prospective study of the natural history of biliary atresia also being conducted by ChiLDREN (http://www.clinicaltrials.gov/ct/show/NCT00061828?order=3).
Abstract Biliary atresia (BA) is an idiopathic, progressive, and fatal disease if untreated. Since Kasai first introduced the operation for BA in 1959, there have been encouraging results in treating this disease. Ascending cholangitis is a frequent and often recurrent complication. It may worsen the prognosis, with an increase in mortality, secondary failure of restoration of bile flow, and possible exacerbation of portal hypertension. For patients who have had restoration of bile flow with a timely portoenterostomy, the recurrence of ascending cholangitis is the single most significant variable pertaining to long-term prognosis. Patients with multiple episodes of ascending cholangitis are more likely to require liver transplantation than those without multiple recurrences. Therefore, the prevention of cholangitis is crucial in the management of patients who have had a Kasai portoenterostomy. Some oral antibiotics, like trimethoprim-sulfamethoxazole (TMP/SMZ) and neomycin have showed the effect to prevent against ascending cholangitis. But, we should consider the problem of drug resistance after long-term use of antibiotics. Is there any better and safer treatment? Probiotics are live microorganisms, which have beneficial effects on human health. Many studies have showed that probiotics have effects to treat or prevent intestinal infection or inflammation even for patient after liver transplantation. The aim of this study is to investigate the possibility of use of probiotics in prophylaxis of ascending cholangitis. We want to enroll 20 BA patients aged 0 to 3 years, who had a Kasai operation. Ten patients are treated with neomycin (25 mg/kg/d, qid, 4 days a week). Another 10 patients receive Lactobacillus casei rhamnosus, Lcr 35 (8x108 CFU/day, bid) The duration of treatment is 6 months. Bacterial cultures of stool are performed before treatment and 1 month, 3 months and 6 months after treatment to evaluate the change of intestinal flora. Another 10 BA patients, from 1991 to 1996, aged 0 to 3 years, without prophylaxis after portoenterostomy, were served as the historical control group. Comparisons of the episodes of cholangitis, time to the first episode, and body weight change are made among the three groups.