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Abstract(s)
A atresia biliar é a causa mais comum de icterícia obstrutiva nos primeiros três meses de
vida, levando à fibrose progressiva e destruição dos ductos biliares intra e extra-hepáticos.
Clinicamente, caracteriza-se por icterícia prolongada, acolia das fezes e colúria. Quando não
tratada, a fibrose progride e culmina na morte até aos 2 anos de vida. A atresia biliar requer
uma intervenção para restaurar o fluxo biliar e preservar o fígado – a portoenterostomia de
Kasai. Se não tratada precocemente e adequadamente, a doença evolui para insuficiência
hepática crónica e cirrose biliar com necessidade de transplante hepático, sendo a atresia
biliar a indicação mais comum deste procedimento na infância. O diagnóstico atempado de
atresia biliar é crucial para melhorar os resultados clínicos destes doentes, uma vez que a
cirurgia precoce está associada a sobrevida mais longa com fígado nativo. No entanto, o
diagnóstico precoce e preciso permanece complexo porque existe sobreposição de
características da atresia biliar com outras causas de colestase neonatal.
Através de uma revisão bibliográfica, o principal objetivo desta dissertação é analisar os
artigos dos últimos 10 anos relacionados com o diagnóstico da atresia biliar, debruçandose sobretudo sobre os métodos de diagnóstico atuais e promissores para a atresia biliar.
Nesta revisão constatou-se um grande avanço nos métodos de diagnóstico e têm sido
propostos novos métodos promissores como é o caso do biomarcador sérico MMP7, da
elastografia e da via laparoscópica na colangiografia intraoperatória. Contudo, são métodos
que precisam de ser mais estudados, sendo que alguns necessitam ainda de validação
diagnóstica com estudos prospetivos multicêntricos.
Biliary atresia is the most common cause of obstructive jaundice in the first three months of life, leading to progressive fibrosis and destruction of intrahepatic and extrahepatic bile ducts. Clinically, it is characterized by prolonged jaundice, acholic stools and choluria. When untreated, fibrosis progresses and ultimately results in death by the age of 2 years old. Biliary atresia requires intervention to restore bile flow and preserve the native liver – the Kasai portoenterostomy. If not treated early and properly, the disease progresses to chronic liver failure and cirrhosis, requiring liver transplant, making biliary atresia the most common indication for this procedure in childhood. Appropriate diagnosis is crucial to improve the clinical outcomes of these patients, as early surgery is associated with longer survival with the native liver. However, early and accurate diagnosis remains complex because there is an overlap of features of biliary atresia with other causes of neonatal cholestasis. Through a literature review, the main objective of this thesis is to analyze articles from the last 10 years related to the diagnosis of biliary atresia, focusing mainly on current and promising diagnostic methods for this disease. This review revealed significant developments in diagnostic methods, with new promising methods proposed, such as the serum biomarker MMP7, elastography, and laparoscopic intraoperative cholangiography. However, these methods still require further study, and some need diagnostic validation through prospective multicentred studies.
Biliary atresia is the most common cause of obstructive jaundice in the first three months of life, leading to progressive fibrosis and destruction of intrahepatic and extrahepatic bile ducts. Clinically, it is characterized by prolonged jaundice, acholic stools and choluria. When untreated, fibrosis progresses and ultimately results in death by the age of 2 years old. Biliary atresia requires intervention to restore bile flow and preserve the native liver – the Kasai portoenterostomy. If not treated early and properly, the disease progresses to chronic liver failure and cirrhosis, requiring liver transplant, making biliary atresia the most common indication for this procedure in childhood. Appropriate diagnosis is crucial to improve the clinical outcomes of these patients, as early surgery is associated with longer survival with the native liver. However, early and accurate diagnosis remains complex because there is an overlap of features of biliary atresia with other causes of neonatal cholestasis. Through a literature review, the main objective of this thesis is to analyze articles from the last 10 years related to the diagnosis of biliary atresia, focusing mainly on current and promising diagnostic methods for this disease. This review revealed significant developments in diagnostic methods, with new promising methods proposed, such as the serum biomarker MMP7, elastography, and laparoscopic intraoperative cholangiography. However, these methods still require further study, and some need diagnostic validation through prospective multicentred studies.
Description
Keywords
Atresia Biliar Colestase Neonatal Diagnóstico Icterícia Obstrutiva