Summary
Definição
História e exame físico
Principais fatores diagnósticos
- neonatal jaundice
- acholic stool
Outros fatores diagnósticos
- dark urine
- bruising
- hepatomegaly
- ascites
Fatores de risco
- genetic predisposition
- viral infection
- environmental or behavioral exposures
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
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
Algoritmo de tratamento
infants with biliary obstruction without end-stage liver disease
infants with biliary obstruction with end-stage liver disease
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
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.
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 diferenciaisGuidelines
- 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 GuidelinesPatient information
Jaundice in newborn babies
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