Histological evolution of fibrosis in patients with biliary atresia / Evolução histológica da fibrose em pacientes com atresia biliar

ABSTRACT Objective: To evaluate the evolution of histological findings of patients with biliary atresia (BA), emphasizing the progression of fibrosis by comparing the diagnostic liver biopsy to the surgical liver biopsy, performed during Kasai portoenterostomy. Methods: Retrospective study with 51 patients with BA submitted to portoenterostomy, and both diagnostic (DLB) and surgical liver biopsies (SLB). The samples were blindly reviewed by two pathologists. Results: Median age at DLB and at SLB was 69 and 77 days, respectively. The median time between biopsies was eight days. Cirrhosis was more frequent in SLB than in DLB, both according to the Metavir score (p = 0.006) and the Ishak score (p = 0.016). The Metavir score increased one or more points in 29/51 (56.9%), with evidence of progression to liver cirrhosis in 11/29 (37.9%) of those who had progression of fibrosis. Median age at surgery of those who had a progression of fibrosis was 77 days, while in those 11 who progressed to cirrhosis, this median was 92 days. Cirrhosis was seen in 12/51 (23.5%) SLB patients. The clinical variable age at surgery had a statistically significant difference regarding the presence or absence of cirrhosis in SLB (p = 0.024). Cirrhosis was not related to survival with native liver or biliary drainage. Conclusion: Most infants with BA have liver fibrosis at diagnosis and it progresses rapidly. The presence of cirrhosis is correlated with the age at surgery, which suggests the importance of this clinical variable in the evolution of fibrosis.

RESUMEN Objetivo: Analizar la evolución de los hallazgos histológicos de pacientes con atresia de vías biliares (AVB), resaltando la progresión de la fibrosis y comparando la biopsia hepática diagnóstica (BHD) con la biopsia hepática quirúrgica (BHQ) mediante la hepatoportoenterostomía de Kasai. Método: Estudio retrospectivo con 51 pacientes con AB sometidos a hepatoportoenterostomía, BHD y BHQ. Resultados: La edad mediana para BHD y BHQ fue 69 días y 77 días, respectivamente. El tiempo mediano entre las biopsias fue ocho días. La cirrosis fue más frecuente en la BHQ de lo que en la BHD, tanto de acuerdo con el escore de Metavir (p = 0,006) como con el escore de Ishak (p = 0,016). El escore de Metavir aumentó un punto o más en 29/51 (59,9%) pacientes, con evidencias de progresión hacia cirrosis hepática en 11/29 (37,9%), en los pacientes con progresión de la fibrosis. La edad mediana para cirugía de los que tuvieron progresión de la fibrosis fue 77 días; en los 11 que evolucionaron hacia cirrosis, esa mediana fue 92 días. La variable clínica de edad en el momento de la cirugía presentó diferencia estadísticamente significante con respecto a la presencia o ausencia de cirrosis (p = 0,024). La cirrosis no fue asociada a la sobrevida con hígado nativo o drenaje biliar. Conclusión: La mayor parte de los niños con AVB tiene fibrosis hepática en el momento del diagnóstico, y la enfermedad avanza rápidamente. La presencia de cirrosis está relacionada con la edad al momento de la cirugía, lo que sugiere la importancia de esa variable clínica en la evolución de la fibrosis.

RESUMO Objetivo: Avaliar a evolução dos achados histológicos de pacientes com atresia biliar (AB), enfatizando a progressão da fibrose e comparando a biópsia hepática diagnóstica (BHD) com a biópsia hepática cirúrgica (BHC), realizada durante a portoenterostomia de Kasai. Método: Estudo retrospectivo com 51 pacientes portadores de AB submetidos a portoenterostomia, BHD e BHC. Resultados: A idade mediana para BHD e BHC foi de 69 e 77 dias de idade, respectivamente. O tempo mediano entre as biópsias foi de oito dias. A cirrose foi mais frequente na BHC do que na BHD, tanto de acordo com o escore de Metavir (p = 0,006) quanto com o escore de Ishak (p = 0,016). O escore de Metavir aumentou um ou mais pontos em 29/51 (56,9%) pacientes, com evidências de progressão para cirrose hepática em 11/29 (37,9%), naqueles com progressão da fibrose. A idade mediana para cirurgia dos que tiveram progressão da fibrose foi de 77 dias; nos 11 que evoluíram para cirrose, essa mediana foi de 92 dias. A variável clínica da idade no momento da cirurgia apresentou diferença estatisticamente significativa em relação à presença ou à ausência de cirrose (p = 0,024). A cirrose não foi associada à sobrevida com fígado nativo ou drenagem biliar. Conclusão: A maioria das crianças com AB tem fibrose hepática no momento do diagnóstico, e a doença progride rapidamente. A presença de cirrose está correlacionada com a idade à cirurgia, o que sugere a importância dessa variável clínica na evolução da fibrose.

Telemonitoring of Neonatal Intensive Care Units: Preliminary Experience in the State of Minas Gerais, Brazil.

OBJECTIVE: This article describes the implementation and functioning of a telemonitoring center of neonatal intensive care units (NICUs) organized by the Telehealth Center of the Medical School of the Federal University of Minas Gerais who signed an agreement with the Minas Gerais State Department of Health (SES/MG, in Portuguese), Brazil. STUDY DESIGN: This is a descriptive retrospective study from December 2012 to June 2014 of a NICU telemonitoring project. RESULTS: Twelve Web conferences have been performed. Six thousand, eight hundred and thirty nine (6,839) online teleconsultations of pediatricians and nurses with experience in neonatology and intensive care have been performed, of which 174 were with subspecialists, with an average response time of 6 hours. DISCUSSION: The project has demonstrated to be satisfactory, with fast response in relation to the demands of subspecialists, promoting care, and management support to the NICU. The qualification via Web conferences prevents the displacement of the professionals to big centers for continuous medical education. CONCLUSION: The telemonitoring of NICU can contribute in the process of management, regulation of beds, reduction of displacements and transferences, adhesion to best practices, care support, and qualification of the involved professionals.

Factors Associated With Bleeding Secondary to Rupture of Esophageal Varices in Children and Adolescents With Cirrhosis.

OBJECTIVE: Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. The present study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD: A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out. The esophageal and gastric varices were classified, and the portal hypertensive gastropathy, laboratory findings, and Child-Pugh classification were measured. RESULTS: Factors observed in univariate analysis to be associated with UGIB were the presence of esophageal varices of medium caliber or larger, portal hypertensive gastropathy, presence of red spots on esophageal varices, Child-Pugh class B or C, and hypoalbuminemia (P < 0.05). After multivariate logistic regression analysis, the significant factors were the presence of red spots on esophageal varices and the presence of gastric varices. When separated the autoimmune hepatitis, nonbiliary atresia patients (all patients except the patients with biliary atresia), and biliary atresia groups the findings in the univariate analysis were the presence of esophageal varices of medium or larger caliber, presence of red spots on varices, and presence of gastric varices in the autoimmune hepatitis patients and nonbiliary atresia patients and presence of red spots on esophageal varices, presence of gastric varices, and Child-Pugh classification B or C in biliary atresia group (P < 0.05). After multivariate logistic regression analysis, no statistical significance was found for any factor analyzed in any groups. CONCLUSIONS: The presence of gastric varices and red spots on esophageal varices were related to episodes of UGIB secondary to rupture of esophageal varices. When these findings are observed, indications for endoscopic primary prophylaxis should be evaluated. More studies are, however, necessary to better understand this problem.

Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome.

OBJECTIVES: This is a cohort study of 134 children and adolescents with a known diagnosis of autoimmune hepatitis (AIH). During follow-up, some of them developed autoimmune sclerosing cholangitis (ASC). This study describes the characteristics of the patients upon diagnosis, and their response to treatment and any complications, and compares the patients who developed ASC during follow-up (ASC group) with those who did not (AIH group). METHODS: A total of 73.1% of the patients were girls with a median age upon diagnosis of 10.41 (7.41-12.53) years. RESULTS: Of 134 patients, 28 (20.9%) developed cholestatic manifestations, with features of ASC. A few differences were observed between the AIH and ASC groups when they were analyzed by χ test, such as the smaller predominance of girls in ASC group (P = 0.04), and more common asymptomatic presentation in the ASC group (P = 0.01). Cirrhosis was observed in 68% of biopsies, with no significant difference between groups (P = 0.43). Of 16 deaths, 15 were in the AIH group and 1 in the ASC group (P = 0.22). Of 11 transplants, 10 were in the AIH group and one in the ASC group (P = 0.53). The presence of cirrhosis at baseline was associated with a smaller survival probability (P = 0.015). The survival rate by Kaplan-Meier method was 94% at 5 years and 80% at 10 years, and was similar in both the groups (P = 0.08). CONCLUSIONS: No statistically significant difference was observed between the groups in relation to prognosis and response to treatment.