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Biliary atresia

Last reviewed: 13 Sep 2025
Last updated: 12 Sep 2023

Summary

Definição

História e exame físico

Principais fatores diagnósticos

  • neonatal jaundice
  • acholic stool
Detalhes completos

Outros fatores diagnósticos

  • dark urine
  • bruising
  • hepatomegaly
  • ascites
Detalhes completos

Fatores de risco

  • genetic predisposition
  • viral infection
  • environmental or behavioral exposures
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • serum total and direct or conjugated bilirubin
  • newborn screen (includes tests for galactosemia, thyroid dysfunction, cystic fibrosis, and a variety of metabolic diseases)
  • prothrombin time (PT), INR
  • CBC with differential
  • serum AST, ALT, ALP, and gamma-GT
  • abdominal ultrasound
Detalhes completos

Investigações a serem consideradas

  • hepatobiliary scintigraphy (technetium Tc 99m-di-isopropyl-acetanilido-imino-diacetic acid scan)
  • liver biopsy
  • cholangiogram
  • CXR
  • infection screen: blood and urine cultures
  • urine PCR for cytomegalovirus
  • plasma or serum amino acids
  • alpha-1 level and protease inhibitor (Pi) type
  • random serum cortisol
  • urinary organic acids
  • urinary succinylacetone
  • urinary bile acids
  • serum lactate/pyruvate ratio
Detalhes completos

Algoritmo de tratamento

AGUDA

infants with biliary obstruction without end-stage liver disease

infants with biliary obstruction with end-stage liver disease

CONTÍNUA

post hepatoportoenterostomy

Colaboradores

Autores

Jessi Erlichman, MPH

Senior Director

Research Administration

The Children's Hospital of Philadelphia

Philadelphia

PA

Declarações

JE receives payment for contributions to UpToDate.

Kathleen Loomes, MD

Professor of Pediatrics

Department of Pediatrics

The Children's Hospital of Philadelphia

Philadelphia

PA

Declarações

KL receives payment for contributions to UpToDate. KL is on an advisory panel for Albireo Pharmaceuticals and Mirum Pharmaceuticals and is a consultant for Travere Therapeutics.

Agradecimentos

Jessi Erlichman and Dr Kathleen Loomes would like to gratefully acknowledge Dr Jonathan A. Flick and Dr Barbara A. Haber, previous contributors to this topic.

Declarações

JAF and BAH declare that they have no competing interests.

Revisores

Benjamin L. Shneider, MD

Professor of Pediatrics

Service Chief in Pediatric Gastroenterology, Hepatology and Nutrition

Baylor College of Medicine

Texas Children’s Hospital

Houston

TX

Declarações

BLS has received research funding from, and been a consultant to, several pharmaceutical companies that make antibiotic agents that might be used for treating bacterial prostatitis.

Mark D. Stringer, MD

Former Professor of Paediatric Surgery

University of Otago

Dunedin

New Zealand

Declarações

MDS declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017;64:154-68.Texto completo  Resumo

Squires RH, Ng V, Romero R, et al. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology. 2014;60:362-98.Texto completo  Resumo

Martin P, DiMartini A, Feng S, et al; AASLD. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014;59:1144-65.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Biliary atresia images
  • Diagnósticos diferenciais

    • Extrahepatic biliary obstruction (e.g., choledochal cyst, spontaneous perforation of common bile duct, bile duct stricture or tumor, neonatal sclerosing cholangitis)
    • Hepatic viral infections (e.g., CMV, enterovirus, HSV, echovirus, adenovirus, hepatitis B virus, HIV, rubella, reovirus type 3, parvovirus B19, EBV)
    • Alagille syndrome
    Mais Diagnósticos diferenciais
  • Guidelines

    • Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of NASPGHAN and ESPGHAN​
    • Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline
    Mais Guidelines
  • Patient information

    Jaundice in newborn babies

    More Patient information
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