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      Atresia biliar por citomegalovirus: un reporte de caso Translated title: Cytomegalovirus-associated biliary atresia: Case report

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          Abstract

          Resumen La atresia biliar asociada con inmunoglobulina M (IgM) positiva para citomegalovirus (CMV) es una entidad infrecuente que se caracteriza por la obliteración inflamatoria progresiva de los conductos intra- o extrahepáticos producida por una reacción autoinmune perinatal contra el CMV. El diagnóstico se realiza con IgM positiva para CMV y biopsia hepática con evidencia de atresia de las vías biliares. El conocimiento y la identificación temprana de esta patología conduce a un manejo quirúrgico temprano, mejorando considerablemente el pronóstico de estos pacientes. Se presenta un caso clínico de una paciente de 82 días de edad con un cuadro de acolia, coluria e ictericia de inicio tardío, asociado con hiperbilirrubinemia a expensas de la directa, elevación de perfil hepático e IgM positiva para CMV. La colangiorresonancia intraoperatoria confirmó el cuadro de atresia de las vías biliares. Se realizó una derivación biliodigestiva tipo Kasai y la toma de biopsia hepática que confirmó el cuadro clínico.

          Translated abstract

          Abstract Biliary atresia associated with positive cytomegalovirus IgM results is a rare condition characterized by progressive inflammatory obliteration of the intra- or extrahepatic ducts. It is caused by a perinatal autoimmune reaction against cytomegalovirus (CMV). Diagnosis is made based on positive IgM for CMV and liver biopsy with evidence of bile duct atresia. Knowledge and timely identification of this disease leads to early surgical management, considerably improving the prognosis of these patients. This is the clinical case of an 82-day-old female patient with late-onset acholia, choluria, and jaundice, associated with conjugated hyperbilirubinemia, elevated liver function tests and positive CMV IgM results. Intraoperative cholangioresonance confirmed bile duct atresia. The Kasai procedure was performed, and a liver biopsy was taken, confirming the diagnosis.

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          Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening.

          Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence. All patients with biliary atresia who were living in France and born between 1986 and 2002 were included. Median follow-up in survivors was 7 years. Included in the study were 743 patients with biliary atresia, 695 of whom underwent a Kasai operation; 2-, 5-, 10-, and 15-year survival rates with native liver were 57.1%, 37.9%, 32.4%, and 28.5%, respectively. Median age at Kasai operation was 60 days and was stable over the study period. Whatever the follow-up (2, 5, 10, or 15 years), survival rates with native liver decreased when age at surgery increased (< or =30, 31-45, 46-60, 61-75, and 76-90 days). Accordingly, we estimated that if every patient with biliary atresia underwent the Kasai operation before 46 days of age, 5.7% of all liver transplantations performed annually in France in patients younger than 16 years could be spared. Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. These findings indicate a rational basis for biliary atresia screening to reduce the need for liver transplantations in infancy and childhood.
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            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.

            Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.
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              Biliary atresia: A comprehensive review.

              Biliary atresia presents as an obliterative cholangiopathy with neonatal jaundice and pale stools. The disease exhibits aetiological heterogeneity with a multiplicity of potential causative factors, both developmental and environmental. A number of clinical variants making up a minority of all cases can be defined relatively precisely which match suggested aetiology better although in most it still remains speculative. These include the syndromic form (BASM), the cystic form and those associated with CMV IgM antibodies. We review not only the clinical evidence for a developmental or an immune-mediated aetiology perhaps triggered by perinatal viral exposure but also several other recently suggested concepts such as microchimerism, gene susceptibility and environmental toxins.
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                Author and article information

                Journal
                rcg
                Revista Colombiana de Gastroenterologia
                Rev Col Gastroenterol
                Asociación Colombiana de Gastroenterología (Bogotá, , Colombia )
                0120-9957
                April 2021
                : 36
                : suppl 1
                : 63-66
                Affiliations
                [2] Bogotá Bogotá orgnameUniversidad de los Andes Colombia
                [6] Bogotá Arauca orgnameUniversidad Nacional de Colombia Colombia
                [7] Bogotá Bogotá orgnameUniversidad de los Andes Colombia
                [1] Bogotá orgnameUniversidad Nacional de Colombia Colombia
                [4] Bogotá Bogotá orgnameUniversidad del Rosario Colombia
                [3] Funza orgnameHospital General de la Orinoquía Colombia
                [5] Bogotá Bogotá orgnameUniversidad del Rosario Colombia
                Article
                S0120-99572021000500063 S0120-9957(21)03600000063
                10.22516/25007440.576
                8b7f9d95-ef29-44b3-adb5-df57346666b5

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 24 May 2020
                : 31 July 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 17, Pages: 4
                Product

                SciELO Colombia

                Categories
                Casos clínicos

                citomegalovirus,Biliary atresia,Cytomegalovirus,Obstructive jaundice,Atresia biliar,ictericia obstructiva

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