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

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.

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.

Factors predictive of an obstructive pathology among Filipino infants with neonatal cholestasis

@#<p style="text-align: justify;"><strong>Objective.</strong> To determine factors predictive of obstructive neonatal cholestasis among Filipino infants and to describe their outcome.</p><p style="text-align: justify;"><strong>Methods.</strong> Jaundiced infants within the first eight weeks of life with liver biopsy were included. Excluded were cholestasis secondary to metabolic or infective causes. Retrospective chart review (2009-2012) and prospective recruitment of patients (2013) were done. A final diagnosis of non-obstructive or obstructive neonatal cholestasis was made on clinical, biochemical, ultrasonographic, and histologic findings, using histology and/or operative cholangiogram as the gold standard. The outcome was assessed on the 6th and 12th months from diagnosis. The crude odds ratio for obstructive jaundice was computed. Multiple logistic regression on significant variables (p-value <0.05) was done.</p><p style="text-align: justify;"><strong>Results.</strong> Two hundred sixty-three (263) patients were included: 161 with non-obstructive and 102 with obstructive cause. Mean age at first consult was higher in those with obstruction. On logistic regression, females (OR:2.3), absence of a family history of idiopathic neonatal hepatitis (OR:4), and persistently pale/acholic stools (OR:13) were predictive of obstruction. 85% of patients with a non-obstructive cause are alive and well, while 80% of patients with obstruction have died.</p><p style="text-align: justify;"><strong>Conclusion.</strong> Among jaundiced infants females, the absence of a family history of idiopathic neonatal hepatitis and persistently pale yellow/acholic stools were predictive of obstruction. The outcome was poor in patients with obstructive jaundice.</p>

Guía de práctica clínica en atresia de las vías biliares / Clinical practice guidelines in biliary atresia

Basada en la mejor evidencia científica disponible, se presenta la guía de práctica clínica en atresia de vías biliares, la cual se define como una obstrucción progresiva de las vías biliares intra- o extrahepáticas en recién nacidos y lactantes pequeños y causa ictericia colestásica grave y cirrosis hepática. Es una enfermedad poco frecuente, de etiología desconocida, con mayor incidencia en países asiáticos. Clínicamente se expresa por ictericia obstructiva, acolia, coluria y hepatoesplenomegalia. Los complementarios expresan una hiperbilirrubinemia directa con aumento de las enzimas hepáticas, y el diagnóstico se confirma en nuestro hospital con la colangiografía, generalmente en el curso de una laparoscopía. El tratamiento es quirúrgico y consiste en la portoenterostomía de Kasai, con mejores resultados en cuanto al drenaje biliar si se realiza antes de los 60 días de vida, así como el trasplante hepático. La enfermedad tiene un curso progresivo hacia la cirrosis hepática en etapas tempranas de la vida, sobre todo si no se realiza el diagnóstico y tratamiento quirúrgico precozmente, con implicaciones en la supervivencia y calidad de vida de estos pacientes. Por tanto, referir precozmente al paciente con sospecha de atresia de vías biliares a un centro especializado es la piedra angular de la actitud médica. La presente guía de práctica clínica pretende ofrecer las herramientas técnicas estandarizadas para mejorar los resultados a los pacientes con esta enfermedad, así como contribuir con la docencia y las investigaciones(AU)

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(AU)

Atresia de vías biliares extrahepaticas en pediatria y citomegalovirus como causante / Extrahepatic biliary atresia in pediatrics and cytomegalovirus as a cause

La atresia de vías biliares es una alteración con gran importancia clínica, más frecuente en la primera infancia, se caracteriza por la obliteración proliferativa de los conductos biliares que progresa a fibrosis hepática, cirrosis e insuficiencia hepática en etapa terminal. El objetivo es presentar un caso clínico inusual y conocer la forma de actuar ante un caso similar. Se trata de paciente femenino de 4 meses de edad, con piel y mucosas ictéricas - verdosas, con hígado palpable debajo del reborde costal derecho, bazo palpable debajo del reborde costal izquierdo. Serología para Citomegalovirus positivo. La ecografía abdominal reporto: Atresia de vías biliares. Se indicó una portoenterostomia de Kasai, sin embargo por la edad de la paciente, el procedimiento quirúrgico no se realizó. La atresia de vías biliares es una patología poco frecuente, su etiología es desconocida, pero es importante saber reconocerla durante los primeros 2 meses de vida, para evitar complicaciones.

Biliary Atresia is a disorder with great clinical importance more frequent in early childhood, characterized by proliferative obliteration of the bile ducts that progresses to liver fibrosis, cirrhosis and end stage liver failure. The objective is to present an unusual clinical case and to know how to act in such a case. It is a female patient of 4 months of age, with icteric skin and mucosa - greenish, with palpable liver below the right costal ridge, palpable spleen below the left costal ridge. Serology for Cytomegalovirus positive.Abdominal ultrasound reported: Biliary Atresia A portoenterostomy of Kasai was indicated, however due to the age of the patient, the surgical procedure was not performed. Biliary Atresia is a rare disease, its etiology is unknown, but it is important to recognize it during the first 2 months of life, to avoid complications.

Trasplante hepático infantil: necesidad del país, normativa y legislación para priorizar a los pacientes pediátricos / Pediatric liver transplant, need of country, normative and legislation to prioritize pediatric patient

El Ecuador no contaba con un programa de trasplante hepático infantil y fue un problema para las autoridades de salud. Como alternativa de tratamiento se implementó un sistema de deriva-ción internacional para que los pacientes hayan accedido al trasplante en centros calificados, con la modalidad del donante vivo relacionado. Se logró acreditar en el 2019 en la ciudad de Cuenca, el primer programa de trasplante infantil para generar un cambio importante en el sis-tema de atenciones, pero fue necesario dejar clara todas las normas y regulaciones que involu-cren la prioridad de éstos pacientes y los aspectos técnicos quirúrgicos que han implicado la uti-lización de éste tipo de procedimientos como: split, hígado reducido y donante vivo relacionado.

Ecuador did not have a child liver transplant program and was a problem for health authorities. As an alternative treatment, an international referral system was implemented so that the patients had access to the transplant in qualified centers, with the modality of the living donor related. It was possible to accredit in 2019 in the city of Cuenca, the first child transplant program to generate a major change in the care system, but it was necessary to make clear all the rules and regulations that involve the priority of these patients and the technical aspects Surgical that have involved the use of these types of procedures such as: split, reduced liver and related living donor.

Correlación de los hallazgos de anatomía patológica y la sobrevida en los pacientes con atresia de vias biliares en los últimos diez años / Correlation of pathology, anatomy findings and survival in patients with biliary atresia during the past ten years

La atresia de vías biliares es una colangiopatía obstructiva neonatal1 caracterizada por cambios obstructivos progresivos tanto de la vía intra como extra hepática. El objetivo de este estudio fue correlacionar los hallazgos de la biopsia hepática y la sobrevida de los pacientes de atresia de vías biliares desde el 2004 hasta el 2014 en los Hospitales Pediátrico Baca Ortiz y Metropolitano de Quito. Es un estudio transversal analítico, en el que se incluyeron a 11 pacientes pediátricos. Los resultados obtenidos revelan que el sexo masculino fue el más afectado con histopatología no favorable. El grupo de edad fue de 1 mes hasta 4 meses, predominó el tipo III de atresia de vías biliares. Se encontró que hay un patrón de atresia de vías biliares como fibrosis, concreciones biliares, y ductulitis que tiene una evolución favorable. Sin embargo sí se encuentra además de lo citado células gigantes, degeneración plumosa esto revelaría signos de patrón no favorable. El patrón de biopsia favorable fue más frecuente en el sexo femenino, con nivel de albumina de 3,6 g/dl, bilirrubinas de 8,2 mg/dl. En el patrón de biopsia no favorable el sexo masculino fue más afectado, con niveles promedio de albúmina de 2,8 g/dl y bilirrubinas de 10,2 mg/dl. Las pruebas de función hepática (ALT, AST, GGT) tuvieron más de 2 DS y no hubo relación con los patrones estudiados. Los pacientes sometidos al procedimiento de Kasai tuvieron complicaciones como la colangitis en el 63,8% de los casos, no relacionándose con el patrón de la biopsia hepática. La sobrevida fue del 63,6%

Bile duct atresia is a neonatal obstructive cholangiopathy1 characterize by progressive obstructive changes of both intra and extra hepatic pathways. The objective of this study was to correlate pathology, anatomy and survival in patients with biliary atresia during the period from 2004 to 2014 in the Pediatric Hospital Baca Ortiz and Metropolitano Hospital of Quito. This is a cohort longitudinal study. That includes 11 pediatrics patients whose cases met the inclusion criterion. Results show that males were more affected and they have an unfavorable histopalothogy. The age group was from 1 to 4 months and type III biliary atresia was predominant. It was found that there is a pattern of biliary atresia as fibrosis, concretions and ductulitis that have favorable evolution but if there are gigant cells, this indicates a poor prognosis. The favorable histopathological pattern was more frequent in females, with albumin level of 3.6g/dl, and bilirubin of 8.2mg/dl. Males were more frequently affected by an unfavorable patern. They presented an average albumin level of 2.8 g/dl, whith avarege level of albúmina 2,8g/dl and bilirubin of 10.2 mg/dl. Liver function tests (ALT, AST, GGT) had more than 2 SD and there was no relation to the patterns studied. The patients who were exposed to the Kasai procedure had complications such as cholangitis in 63.8%, not being related to the pattern of liver biopsy. Survival was 63.6%.

Análise multivariada de variáveis relacionadas ao fluxo biliar e sobrevida pós-Kasai em pacientes com atresia biliar / Multivariate analysis of biliary flow-related factors and post-kasai survival in biliary atresia patients

ABSTRACT BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

RESUMO CONTEXTO: A atresia biliar representa a principal causa de colestase tratada cirurgicamente durante o período neonatal. Se a criança não for operada, ela evolui invariavelmente para cirrose biliar secundária. OBJETIVO: Avaliar, através de análise multivariada, os fatores prognósticos associados à presença de fluxo biliar e à sobrevida com fígado nativo após a realização da portoenterostomia de Kasai. MÉTODOS: O estudo analisou 117 pacientes com atresia biliar submetidos à portoenterostomia e com material histológico adequado para avaliação. O modelo de regressão logística foi utilizado para avaliar a presença de fluxo biliar. Sobrevida foi estudada através das curvas Kaplan-Meier e ajuste do modelo de Cox. RESULTADOS: Um terço dos pacientes obteve fluxo biliar e a mediana de idade à cirurgia foi de 81 dias. Idade à cirurgia, albumina, complicação pós-operatória, BASM (do inglês, biliary atresia structural malformation), arquitetura hepática, diâmetro do maior canalículo no porta hepatis e cirrose, segundo o escore de Ishak, foram as variáveis iniciais da análise multivariada. Idade à cirurgia maior que 90 dias de vida foi a única variável associada à ausência de drenagem biliar. A análise de sobrevida mostrou que as variáveis: ausência de fluxo biliar (P<0,0001), idade à cirurgia maior que 90 dias (P=0,035) e presença de BASM (P<0,0001), isoladamente, predizem morte ou necessidade de transplante hepático. Na análise multivariada, ausência de fluxo biliar (P<0,0001 HR:6,25 [IC95% 3,19; 12,22]) e presença de BASM (P=0,014 HR:2,16 [IC95% 1,17; 3,99]) mostraram-se associadas, com significância estatística, a menor sobrevida com fígado nativo. CONCLUSÃO: Idade à cirurgia maior que 90 dias foi identificada como fator de risco independente para ausência de fluxo biliar. Além disso, a presença de drenagem biliar e a ausência de malformações estruturais da atresia biliar são variáveis fundamentais para a maior sobrevida com fígado nativo.

Value of serum gamma-glutamyl transpeptidase combined with direct bilirubin in the diagnosis of biliary atresia in infants / 中国当代儿科杂志

OBJECTIVE@#To study the value of serum gamma-glutamyl transpeptidase (GGT) combined with direct bilirubin (DB) in the diagnosis of biliary atresia.@*METHODS@#A total of 667 infants with cholestasis who were hospitalized and treated from July 2010 to December 2018 were enrolled as subjects. According to the results of intraoperative cholangiography and follow-up, they were divided into biliary atresia group with 234 infants and cholestasis group with 433 infants. The two groups were compared in terms of age of onset, sex, and serum levels of total bilirubin (TB), DB, alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bile acid (TBA), and GGT. A receiver operating characteristic (ROC) curve analysis was performed for indices with statistical significance, and the area under the ROC curve (AUC) and the optimal cut-off value for diagnosis were calculated.@*RESULTS@#The biliary atresia group had a significantly younger age of onset than the cholestasis group (P0.05), while the biliary atresia group had significantly higher serum levels of TB, DB, TBA, and GGT than the cholestasis group (P<0.05). GGT combined with DB had the highest AUC of 0.892 (95% confidence interval: 0.868-0.916) in the diagnosis of biliary atresia. At the optimal cut-off values of 324.0 U/L for GGT and 115.1 μmmol/L for DB, GGT combined with DB had a sensitivity of 79.8% and a specificity of 83.2% in the diagnosis of biliary atresia.@*CONCLUSIONS@#GGT combined with DB has high sensitivity and specificity in the diagnosis of biliary atresia and can be used as an effective indicator for diagnosis of biliary atresia in infants.

False-negative Hepatobiliary Scintigraphy for Biliary Atresia / 대한핵의학회잡지

We present the case of a patient with biliary and duodenal atresia who showed false-negative hepatobiliary scintigraphy results. The patient was born at 37 weeks and 2 days of gestation. Her mother had undergone amnioreduction after detection of a double-bubble ultrasound sign in the fetal abdomen. At 2 days of age, total serum bilirubin level was elevated. On hepatobiliary scintigraphy 4 days later, the gallbladder was visualized from 30 min and it showed duodeno-gastric reflux at 240 min. After 24 h, the radiotracer was almost washed out in the hepatic parenchyma, but there was retention in the gastroduodenal junction. Because the biliary to duodenal transit was visible, biliary atresia seemed unlikely. Abdominal ultrasonography at 7 days of age showed a small dysmorphic gallbladder, but triangular cord sign was not definite. Magnetic resonance cholangiography revealed atretic gallbladder. Although cystic and common bile ducts were visible, the proximal common hepatic bile duct was not visible. The next day, serum total bilirubin levels remained elevated (17.1 mg/dl) with direct bilirubin level of 1.2 mg/dl. Kasai portoenterostomy with duodeno-duodenostomy was performed at 10 days of age. Histopathological evaluation showed a fibrous obliteration of the common bile duct, consistent with that of biliary atresia.

Feasibility of Spin-Echo Echo-Planar Imaging MR Elastography in Livers of Children and Young Adults

PURPOSE: To assess the feasibility of the use of spin-echo echo-planar imaging (SE-EPI) magnetic resonance elastography (MRE) in livers of children and young adults. MATERIALS AND METHODS: Patients (≤ 20 years old) who underwent 3T SE-EPI MRE were included retrospectively. Subjects were divided into three groups according to the purpose of the liver MRI: suspicion of fatty liver or focal fat deposition in the liver (FAT group), liver fibrosis after receiving a Kasai operation from biliary atresia (BA group), and hepatic iron deposition after receiving chemotherapy or transfusions (IRON group). Technical failure of MRE was defined when a stiffness map showed no pixel value with a confidence index higher than 95%, and the patients were divided as success and failure groups accordingly. Clinical findings including age, gender, weight, height, and body mass index and magnetic resonance imaging results including proton density fat fraction (PDFF), T2*, and MRE values were assessed. Factors affecting failure of MRE were evaluated and the image quality in wave propagation image and stiffness map was evaluated using the appropriate scores. RESULTS: Among total 240 patients (median 15 years, 211 patients in the FAT, 21 patients in the BA, and 8 patients in the IRON groups), technical failure was noted in six patients in the IRON group (6/8 patients, 75%), while there were no failures noted in the FAT and BA groups. These six patients had T2* values ranging from 0.9 to 3.8 ms. The image quality scores were not significantly different between the FAT and BA groups (P > 0.999), while the scores were significantly lower in the IRON group (P < 0.001). CONCLUSION: The 3T SE-EPI MRE in children and young adults had a high technical success rate. The technical failure was occurred in children with decreased T2* value (≤ 3.8 ms) from iron deposition.

The comparison of desflurane and sevoflurane on postoperative hepatic function of infant with biliary atresia undergoing Kasai operation

BACKGROUND: Biliary atresia is an extrahepatic progressive obliterate cholangiopathy that occurs in infants. Kasai procedure, a surgical method that can help re-establish bile flow from the liver into the intestine, is its first line treatment. Since infants with biliary atresia already have advanced hepatic dysfunction, all kinds of schemes should be considered to minimize further liver damage during surgery. The objective of this study was to compare the postoperative hepatic functions between the two commonly used inhalational anesthetics in infants undergoing the Kasai procedure (i.e., desflurane and sevoflurane). METHODS: This prospective, randomized, double-blind, single-center, and parallel group study included 40 children undergoing Kasai procedure. They were randomly allocated to Group S (sevoflurane) or Group D (desflurane). All the patients were anesthetized with designated anesthetic agent with the end-tidal concentration of about 0.8–1 minimum alveolar concentration. Postoperative hepatic functions were assessed by aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, prothrombin time, and total bilirubin. RESULTS: A total of 38 patients were selected for the study. In both groups, AST, ALT were increased in magnitude to the peak on postoperative day 0 and decreased to preoperative value at postoperative day 3. There were no significant differences between the groups in any laboratory results related to liver function. CONCLUSIONS: Sevoflurane and desflurane, inhalation anesthetics for maintaining anesthesia used in infants undergoing the Kasai procedure, did not show any difference in preserving postoperative hepatic function.

Quantitative Imaging in Pediatric Hepatobiliary Disease

Pediatric hepatobiliary imaging is important for evaluation of not only congenital or structural disease but also metabolic or diffuse parenchymal disease and tumors. A variety of ultrasonography and magnetic resonance imaging (MRI) techniques can be used for these assessments. In ultrasonography, conventional ultrasound imaging as well as vascular imaging, elastography, and contrast-enhanced ultrasonography can be used, while in MRI, fat quantification, T2/T2* mapping, diffusion-weighted imaging, magnetic resonance elastography, and dynamic contrast-enhanced MRI can be performed. These techniques may be helpful for evaluation of biliary atresia, hepatic fibrosis, nonalcoholic fatty liver disease, sinusoidal obstruction syndrome, and hepatic masses in children. In this review, we discuss each tool in the context of management of hepatobiliary disease in children, and cover various imaging techniques in the context of the relevant physics and their clinical applications for patient care.

Patrón clínico y epidemiológico de la atresia de vías biliares en Cuba / Clinical and epidemiological pattern of biliary atresia in Cuba

Introducción: La atresia de vías biliares es una colangiopatía infrecuente que se presenta en recién nacidos entre la segunda y cuarta semana de vida. Objetivo: Determinar el patrón clínico-epidemiológico de la atresia de vías biliares en Cuba. Método: Estudio descriptivo en la población con atresia de vías biliares(n= 30) atendida en el Hospital William Soler (enero 2011-diciembre 2015). Se midieron los rasgos clínicos, humorales y variables epidemiológicas con análisis de incidencia (por 1 000 nacidos vivos) y pruebas estadísticas con significación para plt;0,05). Resultados: La incidencia en Cuba es de 0,47x 10 000 nacidos vivos (1: 21 078 nacidos vivos), en Mayabeque, la más alta con 1: 6 784. Todos tuvieron ictericia y 96,7 por ciento coluria. Se presentaron concentraciones elevadas de bilirrubina total (media= 184,9 µmol/L), ligera elevación de alaninoaminotransferasa (media= 201,8 U/L) y aspartatoaminotransferasa (media= 279,5 U/L), mayor aumento en la concentración de gammaglutamiltransferasa (media= 588 U/L) que de fosfatasa alcalina (media=1 557,1 u/L) e incremento del colesterol (6,8 mmol/L) con triglicéridos normales. El 70 por ciento de los sometidos a intervención quirúrgica antes de los 60 días de nacido restablecieron el flujo biliar contra 35,5 por ciento que no lo lograron cuando se intervinieron posteriormente. Conclusiones: La incidencia en la enfermedad en Cuba asciende, sin preferencia de género y es superior en Mayabeque. Son típicas las manifestaciones de ictericia, coluria, hiperbilirrubinemia, hipertransaminasemia ligera, hipercolesterolemia con alteración de gammaglutamiltransferasa más que de la fosfatasa alcalina y restablecimiento del flujo biliar en operados antes de los 60 días de nacido(AU)

Introduction: Biliary atresia is an infrequent colangiopaty that it is present in newborns among the second and the forth weeks of life. Objective: To determine the clinical and epidemiological pattern of biliary atresia in Cuba. Method: Descriptive study in the population presenting biliary atresia (n= 30) attended in William Soler Hospital (from January, 2011 to December, 2015). Clinical and humoral features, and epidemiological variables were measured by an incidence analysis (per 1 000 live births) and statistical tests with significance of p<0,05. Results: Incidence in Cuba is of 0.47 x 10 000 live births (1: 21 078 live births); in Mayabeque province, it is registered the highest incidence 1: 6 784. All the patients presented icterus and 96.7 percent presented choluria. High concentrations of total bilirubine (mean= 184.9 µmol/L), slight increase of alaninoaminotransferasa (mean= 201.8 U/L) and aspartatoaminotransferasa (mean= 27.5 U/L) than in the alcaline fosfatase (mean= 1 557.1 U/L); and cholesterol increase (6.8 mmol/L) with normal triglycerides were present. 70 percent of the patients that underwent surgeries before reaching 60 days of life could reestablish the biliar flow. 35 percent did not achieve this while underwent a surgery after 60 days of life. Conclusions: The incidence of this disease is increasing in Cuba, not having gender preferences and it is higher in Mayabeque province. Manifestations of icterus, choluria, hyperbilirubinemia, light hypertransaminasemia, hypercholesterolemia with gammaglutamiltransferasa alteration higher than alcaline fosfatase, and the reestablishment of the biliary flow in patients being operated before the 60 days of life, are common(AU)

Uso de la tarjeta colorimétrica visual para la detección oportuna de atresia de vías biliares / Colorimetric card use for early detection visual biliary atresia

Resumen: Introducción: La atresia de vías biliares (AVB) es una condición que provoca obstrucción al flujo biliar, y de no corregirse quirúrgicamente, provoca cirrosis y la muerte antes de los 2 años de edad. En México, a partir del año 2013 se incorporó la tarjeta colorimétrica visual (TCV) para la detección oportuna de la AVB a la Cartilla Nacional de Salud (CNS). El objetivo de este estudio fue evaluar el impacto de la TCV para la detección de AVB antes y después de su incorporación a la CNS. Métodos: Estudio ambispectivo, observacional y analítico. Se incluyeron pacientes con AVB atendidos en dos hospitales pediátricos de tercer nivel de atención. Se compararon la edad de referencia, el diagnóstico y la cirugía antes y después de la incorporación de la TCV. Además, se realizó un cuestionario a los padres para conocer su percepción sobre la TCV. Resultados: En 59 niños no hubo diferencias en la edad al diagnóstico (75 vs 70 días) ni en la edad al momento de la cirugía (84 vs 90 días) entre antes y después de la implementación de la TCV. Solo el 30% de los padres recibieron información del uso de la TCV y solo el 38% identificaron las evacuaciones anormales. Conclusiones: Este estudio no mostró cambios en el tiempo para la detección oportuna de AVB mediante el uso de la TCV. Por lo tanto, es necesario reforzar el programa en los tres niveles de atención en nuestro país.

Abstract: Background: Bile duct atresia (BVA) is a condition that causes obstruction to biliary flow, not corrected surgically, causes cirrhosis and death before 2 years of age. In Mexico from 2013 the visual colorimetric card (VVC) was incorporated for the timely detection of BVA to the National Health Card (NHC). The aim of this study was to evaluate the impact of VCT for the detection of BVA before and after the use of NHC incorporation. Methods: Ambispective, analytical observational study. We included patients with AVB treated in two pediatric hospitals of third level care. We compared the age of reference, diagnosis and surgery before and after incorporation of the TCV. In addition, a questionnaire was made to the parents to know their perception about the TCV. Results: In 59 children, there were no differences in age at diagnosis (75 vs 70 days) and age at surgery (84 vs 90 days) between the pre and post-implementation period of the VVC. The questionnaire showed that 10 (30%) of the parents received information about the use of the VVC and 13 (38%) identified the abnormal evacuations. Conclusions: This study did not show changes in time for the timely detection of BVA by using VVC. Therefore, it is necessary to reinforce the program in the three levels of care in our country.

Hallazgos por imagen en pacientes con atresia de vías biliares en el Hospital de Especialidades Carlos Andrade Marín en el período de enero 2016 - marzo 2017 / Imaging findings in patients with biliary atresia in Carlos Andrade Marín Specialties Hospital, in the period between Jan 2016-Mar 2017

Introducción. Revisión de los hallazgos clínicos y radiológicos de la atresia de la vía biliar, una anomalía del desarrollo biliar que tiene un riesgo alto de mortalidad por las complicaciones asociadas si no se la corrige oportunamente. Material y métodos. Serie de siete casos reportados en el Hospital de Especialidades Carlos Andrade Marín en el periodo enero 2016 a marzo 2017, seis mujeres y un varón, todos con cuadro clínico de ictericia prolongada, acolia, patrón de colestasis, hallazgos por imagen y biopsias sugestivas de atresia de vías biliares. Resultados. Todos los pacientes presentaron ictericia y coluria, en exámenes de laboratorio patrón colestásico en todos los pacientes, en los estudios de imagen el hallazgo predominante en sospecha de atresia de vías biliares fue la ausencia de la vesícula biliar y el signo del cordón triangular solamente en tres pacientes, en la biopsia hepática todos mostraron algún grado de fibrosis hepática. Discusión. La atresia de la vía biliar es una enfermedad compleja y grave, en nuestra serie de casos se confirma la utilidad del ultrasonido como método de diagnóstico de atresia de vías biliares ya que es un método mucho más accesible que la resonancia magnética que generalmente tiene menor disponibilidad y mayor costo. Conclusión. El ultrasonido abdominal es una herramienta extremadamente valiosa en sospecha clínica de atresia de vías biliares, debe realizarse un diagnóstico oportuno para mejorar el pronóstico de los pacientes.

Introduction. This is a review of clinical and radiological findings of biliary atresia, an anomaly of biliary tree development that carries a high risk of mortality due to complications unless they are corrected in a short time. Materials and methods. We reviewed a series of seven cases reported at Carlos Andrade Marín Specialties Hospital, from January 2016 to March 2017. Six women and one male were followed. All of them showed clinical signs such as long term jaundice, cholestasis, as well as, imaging findings and liver biopsies, suggesting biliary tract atresia. Results. All patients presented jaundice and choluria, in laboratory tests a cholestasic pattern in all of them, in the imaging studies the predominant finding in suspicion of biliary atresia was the absence of gallbladder and the triangular cord sign in only three patients, in the liver biopsy all of them showed some degree of liver fibrosis. Discussion. Bile duct atresia is a complex and serious disease. In our case serie we confirmed the utility of ultrasound as diagnosis method in biliary duct atresia, is more available than magnetic resonance which is more expensive and less available in different centers. Conclusion. Abdominal ultrasound is an extremely valuable tool in clinical suspicion of biliary atresia, a timely diagnosis must be made to improve patient´s prognosis.

The Prognostic Value of CD8⁺ and CD45RO⁺ T Cells Infiltration and Beclin1 Expression Levels for Early Postoperative Cholangitis of Biliary Atresia Patients after Kasai Operation

BACKGROUND: Postoperative cholangitis is a common but severe complication after Kasai portoenterostomy for biliary atresia (BA). This study aimed to identify its prognostic factors. METHODS: Two sets of liver paraffin-embedded tissue samples were collected from BA patients who received Kasai portoenterostomy (n = 25 and n = 31, respectively). Patients were divided into non-cholangitis and cholangitis groups. The infiltration of CD4+, CD8+, CD45RO+, CD68+ cells and expression of Beclin1 were quantitatively evaluated in immunohistochemical analysis. RESULTS: Cholangitis group had a significantly lower CD8+ T cell infiltration but a higher CD45RO+ cell infiltration, and a lower Beclin1 level than non-cholangitis group (all P < 0.01). Multivariate logistic regression analysis indicated that infiltration of CD8+ cells (odds ratio [OR], 0.112; 95% confidence interval [CI], 0.022–0.577) and CD45RO+ cells (OR, 3.88; 95% CI, 1.37–11.03), and Beclin1 level (OR, 0.088; 95% CI, 0.018–0.452) were independent influence factors for early postoperative cholangitis. Receiver operating characteristic (ROC) analysis showed that area under ROC curve (AUROC) values for CD8+ cells, CD45RO+ cells and Beclin1 were 0.857, 0.738 and 0.900, respectively. CONCLUSION: Our findings demonstrated the CD8+ cells, CD45RO+ cells and Beclin1 level possessed the prognostic value for early postoperative cholangitis following Kasai operation, which may be helpful to develop new prevention and treatment strategies for postoperative cholangitis.

Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia / IL-6, TNF-α, IL-10 e estado nutricional em pacientes pediátricos com atresia biliar

Abstract Objectives: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. Methods: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child-Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. Results: IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (p < 0.001), 0.21 vs. 0.14 (p = 0.007), and 0.65 vs. 0.36 (p = 0.004), respectively. IL-6 and IL-10 were positively correlated with disease severity (0.450 [p = 0.001] and 0.410; [p = 0.002], respectively). TNF-α did not show a significant correlation with disease severity (0.100; p = 0.478). Regarding nutritional evaluation, IL-6 was negatively correlated with the standard deviation score of height-for-age (−0.493; p < 0.001) and of triceps skinfold thickness-for-age (−0.503; p < 0.001), respectively. IL-10 exhibited a negative correlation with the standard deviation score of height-for-age (−0.476; p < 0.001) and the standard deviation score of triceps skinfold thickness-for-age (−0.388; p = 0.004). TNF-α did not show any significance in both anthropometric parameters (−0.083 (p = 0.555) and −0.161 (p = 0.253). Conclusion: The authors suggest that, in patients with cirrhosis secondary to biliary atresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status.

Resumo Objetivos: Avaliar se há associações entre a IL-6, o TNF-α, a IL-10 e a estado nutricional em pacientes com cirrose secundária a atresia biliar e comparar com controles saudáveis. Métodos: Os parâmetros usados na avaliação nutricional foram desvio padrão de estatura para a idade e espessura da prega cutânea do tríceps para a idade. A gravidade da cirrose foi avaliada por meio da classificação de Child-Pugh e do PELD/MELD. As citocinas no soro foram medidas por citometria de fluxo - técnica de Cytometric Bead Array. Resultados: A IL-6, o TNF-α e a IL-10 foram significativamente maiores no grupo de cirrose em comparação com o grupo de controle [2,4 em comparação com 0,24 (p < 0,001)], [0,21 em comparação com 0,14 (p = 0,007)] e [0,65 em comparação com 0,36 (p = 0,004)], respectivamente. A IL-6 e a IL-10 demonstraram correlação positiva com a gravidade da doença (0,450; p = 0,001) e (0,410; p = 0,002), respectivamente. O TNF-α não mostrou relevância na gravidade da doença (0,100; p = 0,478). Com relação à avaliação nutricional, a IL-6 demonstrou correlação negativa com o desvio padrão de estatura para a idade (−0,493; p < 0,001) e o desvio padrão de espessura da prega cutânea do tríceps para a idade (−0,503; p < 0,001), respectivamente. A IL-10 demonstrou correlação negativa com o desvio padrão de estatura para a idade (−0,476; p < 0,001) e o desvio padrão de espessura da prega cutânea do tríceps para a idade (−0,388; p = 0,004), respectivamente. O TNF-α não mostrou relevância em ambos os parâmetros antropométricos [(−0,083; p = 0,555); (−0,161; p = 0,253)]. Conclusão: Assim, sugerimos que, em pacientes com cirrose secundária a atresia biliar, IL-6 pode ser usado como um possível biomarcador de suporte do estado nutricional deficiente e níveis aumentados de IL-10 podem ser usados como um possível biomarcador de suporte, em fase inicial, de deterioração do estado nutricional.