1. Portal and periportal fibrosis in experimental extra-hepatic biliary obstruction in young and adult rats: contribution to biliary atresia study / "Fibrose portal e periportal na obstrução extra-hepática experimental em ratos jovens e adultos: contribuição para o estudo da atresia das vias biliares"

Biliary atresia is an hepatic disease of infancy. Etiology is unknown, and diagnosis is made by liver biopsy, with ductular proliferation being the main histological feature. Bile duct ligation in rats is an useful experimental model of biliary obstruction. The aim of this study of extra-hepatic cholestasis was analyse hepatic histological alterations in you

Publicado em: 2003

2. Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.

One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was conf

3. MULTIVARIATE ANALYSIS OF BILIARY FLOW-RELATED FACTORS AND POST-KASAI SURVIVAL IN BILIARY ATRESIA PATIENTS

RESUMO CONTEXTO: A atresia biliar representa a principal causa de colestase tratada cirurgicamente durante o período neonatal. Se a criança não for operada, ela evolui invariavelmente para cirrose biliar secundária. OBJETIVO: Avaliar, através de análise multivariada, os fatores prognósticos associados à presença de fluxo biliar e à sobrevida co

Arq. Gastroenterol.. Publicado em: 20/05/2019

4. Association of the reovirus S1 gene with serotype 3-induced biliary atresia in mice.

A panel of serotype 3 (T3) reovirus strains was screened to determine their relative capacities to cause lethal infection and hepatobiliary disease following peroral inoculation in newborn mice. A wide range of 50% lethal doses (LD50s) was apparent after peroral inoculation of the different virus strains. Two of the strains, T3 Abney and T3 clone 31, caused

5. Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of pat

Clinics. Publicado em: 10/07/2018

6. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers.

OBJECTIVE: The authors investigated risk factors for failure after portoenterostomy for biliary atresia using univariate and multivariable methods. SUMMARY BACKGROUND DATA: Kasai's portoenterostomy has gained worldwide acceptance as the initial surgical therapy for infants with biliary atresia. Although extended survival has been achieved for many patients,

7. Obstruction of extrahepatic bile ducts by lymphocytes is regulated by IFN-γ in experimental biliary atresia

The etiology and pathogenesis of bile duct obstruction in children with biliary atresia are largely unknown. We have previously reported that, despite phenotypic heterogeneity, genomic signatures of livers from patients display a proinflammatory phenotype. Here, we address the hypothesis that production of IFN-γ is a key pathogenic mechanism of disease usin

American Society for Clinical Investigation.

8. Glutathione S-transferases in neonatal liver disease.

AIMS: To investigate the distribution of alpha and pi class glutathione S-transferases (GST) in normal fetal, neonatal, and adult liver; and to examine changes in GST expression in neonatal liver disease. METHODS: alpha and pi class GST were immunolocalised in sections of formalin fixed liver tissue obtained from human fetuses (n = 21), neonates (n = 8), you

9. Orthotopic liver transplantation for congenital biliary atresia. An 11-year, single-center experience.

OBJECTIVE: The authors analyze a single center's 11-year experience with 190 orthotopic liver transplants for congenital biliary atresia. SUMMARY BACKGROUND DATA: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patie

10. Utilization of sialic acid as a coreceptor is required for reovirus-induced biliary disease

Infection of neonatal mice with some reovirus strains produces a disease similar to infantile biliary atresia, but previous attempts to correlate reovirus infection with this disease have yielded conflicting results. We used isogenic reovirus strains T3SA– and T3SA+, which differ solely in the capacity to bind sialic acid as a coreceptor, to define the rol

American Society for Clinical Investigation.

11. Extrahepatic Biliary Atresia

12. Advances in biliary atresia : from patient care to research

Biliary atresia, the most common cause of liver transplantation in children, remains a challenge for clinicians and investigators. The development of new therapeutic options, besides the typical hepatoportoenterostomy, depends on a greater understanding of its pathogenesis and how it relates to the clinical phenotypes at diagnosis and the rate of disease pro

Publicado em: 2011