SciELO - Scientific Electronic Library Online

 
vol.92 issue4Acute appendicitis in children. Clinical Pratice GuideClinical practice guidelines in congenital dilation of biliary tract in pediatric ages author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

  • Have no cited articlesCited by SciELO

Related links

  • Have no similar articlesSimilars in SciELO

Share


Revista Cubana de Pediatría

Print version ISSN 0034-7531On-line version ISSN 1561-3119

Abstract

VILLAMIL MARTINEZ, Ramón et al. Clinical practice guidelines in biliary atresia. Rev Cubana Pediatr [online]. 2020, vol.92, n.4, e1168.  Epub Dec 01, 2020. ISSN 0034-7531.

Based on the best scientific evidence available, it is presented the clinical practice guidelines on biliary atresia. This disease is defined as a progressive obstruction of the intra and/or extrahepatic bile ducts in newborns and young infants, causing severe cholestatic jaundice and cirrhosis of the liver. It is a rare disease of unknown etiology, with a higher incidence in Asian countries. It is clinically expressed by obstructive jaundice, acholia, choluria and hepatosplenomegaly. Laboratory tests show a direct hyperbilirubin and elevated liver enzymes, and in our hospital, the diagnosis is confirmed by a cholangiography, usually during a laparoscopy procedure. It has surgical treatment and it involves a Kasai portoenterostomy, with better results regarding biliary drainage if it is performed before 60 days of life, as well as liver transplant. This condition has a progressive course towards liver cirrhosis in early stages of life, mainly if the diagnosis and surgical treatment are not made timely, with implications for the survival and quality of life of these patients. Therefore, early referral of the patient with suspected biliary atresia to a specialized center is the cornerstone of the medical attitude. This clinical practice guidelines aims to offer standardized technical tools to improve the outcome for patients with this disease, as well as to contribute to teaching and research.

Keywords : Biliary atresia; cholestasis of the infant; Kasai portoenterostomy.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )