Active clinical trials for "Biliary Atresia"

Biliary atresia (BA) is the most frequent cause of chronic cholestasis in neonates, accounting for at least 50% of pediatric liver transplantation. BA incidence is estimated to range from 1:5000 to 1:19000 live births. All patients will die due to complications of liver cirrhosis if the operation is not performed. Recently, mesenchymal stem cell (MSC) transplantation has been found as a promising therapy for liver cirrhosis in adults. Bone marrow-derived stem cell transplantation was also performed successfully for children with BA. Compared to MSC isolation from bone marrow, isolating MSCs from umbilical cord (UC) tissue is a less invasive procedure. Furthermore, UC-derived MSCs (UC-MSCs) have been demonstrated to be safe and effective for liver cirrhosis in adults and different pediatric diseases, including liver cirrhosis due to primary biliary cirrhosis. The investigators will compare the outcomes of 17 Kasai operated BA patients who receive UC-MSC transplantation to 17 BA patients who only undergo Kasai operation. Two transplantations of UC - MSCs will be performed via the hepatic artery: the first transplant will be performed at baseline, and the second one will be performed 6 months later with a dosage of 1 million MSCs per kg of body weight. The frequency and severity of the adverse events or serious adverse events associated with UC-MSC injection at 72 hours post-injection will be used to assess the safety. The efficacy of the therapy will be measured using Pediatric End-Stage Liver Disease (PELD) score, liver function, and liver biopsy. This study would open a novel cell therapy to improve outcomes of patients with BA.

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.

To evaluate the effects of sevoflurane on hepatic blood flow (HBF) and hepatic arterial buffer response (HABR) in infants with obstructive jaundice by Doppler ultrasound.Twenty-five infants with biliary atresia (1-3 months-of-age) scheduled for a Kasai procedure were enrolled. portal vein blood flow (PBF), hepatic arterial blood flow (HABF) and hepatic blood flow (HBF) were measured by Doppler ultrasound before induction, and after inhalation of 2 and 3% sevoflurane.

Maternal microchimerism has been discussed as an etiological mechanism in infantile (perinatal) biliary atresia (BA). In Kasai's operation (resection of the liver hilum plaque followed by hepato-portoenterostomy) surgeons frequently encounter swollen portal and mesenteric lymph nodes. Lymph nodes were sampled during Kasai' s operation and examined for maternal DNA.

Our study aims to develop a screening test for biliary atresia (BA) using dry blood spot to improve patient survival by early diagnosis. Newborn screening dry blood spot will be examined for the direct bilirubin (DB), γ-GT or matrix metalloproteinase-7 (MMP-7) levels. These findings will promote early diagnosis for BA and hence improve the survival.

Biliary atresia is the commonest etiology of neonatal cholestasis and is the most common indication for pediatric liver transplantation world-wide. Kasai-portoenterostomy (KPE) is the operative procedure of choice which helps in restoration of biliary flow and preventing rapid progression of fibrosis. Only 50-60% of infants have a successful surgery in terms of normalization of bilirubin (<2 mg/dL) after 3 months. Remaining 40-50% have rapid progression of PHT and eventual decompensation. Additionally, around 50-70% of infants with successful KPE have 1 or more episodes of cholangitis, and the severe ones if left untreated lead to progressive portal hypertension. Moreover in Indian setting a significant number of infants with biliary atresia reach late when the KPE is not feasible, and this group develops very rapid PHT and decompensation. Hence, overall around 70-80% of infants and children develop PHT within 5 years of age. Variceal bleed has been shown to be an important determinant of survival in infants with high bilirubin. Usage of beta-blockers in adult cirrhotics has been shown to reduce the progression of varices and incidence of variceal bleed. Although many pediatric hepatology centers worldwide use beta-blockers, there has been no controlled trial specifically to address this issue in children with biliary atresia. So, we planned this study to evaluate the efficacy of beta-blockers as primary prophylaxis for prevention of variceal bleed in biliary atresia children.

Background: It is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. Case reports: A two-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy). Discussion: It is important to carefully look into the relationship between preduodenal portal vein and biliary atresia because the patient at a risk of injury from this aberrant vein at operative intervention.

Kasai portoenterostomy is the key standard operation for biliary atresia. The aim of the study is to evaluate the short-term outcome of Kasai portoenterostomy for biliary atresia infants in Upper Egypt.

we propose this study and try to find out possible clinical applicable non-invasive imaging indices or its combination with the laboratory indices to predict the status of hepatic fibrosis in BA patients.

The investigators plan to investigate the use of US shear wave elastography (SWE), a newly available imaging technology, in children with suspected/known BA.