[Prognostic factors related to mortality of children with atresia of bile ducts]. Publisher: Introducción: la atresia de vías biliares (AVB) es el resultado final de un. Atresia de vías biliares vs. Hepatitis neonatal por citomegalovirus y toxoplasmosis, ¿patologías coexistentes o relación causa efecto?. Biliary Atresia – Clinical Series. Atresia das Vias Biliares – Revisão Casuística. Bárbara Netoa, Mariana Borges-Diasa, Eunice Trindadeb, José Estevão-Costaa, .

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Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children.

Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, and December 31, We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present bi,iares.

Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. There was a significant association between recurrent cholangitis and survival.

[Prognostic factors related to mortality of children with atresia of bile ducts].

Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery biliaree recurrence of cholangitis were the only factors significantly related to prognosis. Estudo descritivo, observacional e retrospetivo, incluindo-se doentes com atresia das vias biliares diagnosticados e tratados entre 1 de Janeiro de e 31 de Dezembro de Foram avaliados 18 doentes.

Preoperative evaluation may include: However, the definitive diagnosis is confirmed only by exploratory surgery [1] Fig. Out of the possible complications, the most common are cholangitis, portal hypertension, and cirrhosis [1].

Two cases were excluded due to loss of follow-up and misdiagnosis. The study population was characterized viae to the following parameters: Referring to preoperative evaluation, it was analyzed: Short- and long-term therapeutics were studied, in particular surgical antibiotic prophylaxis, corticotherapy, and ursodeoxycholic acid UDCA administration in the immediate postoperative period, as well as long-term antibiotherapy in order to prevent cholangitis episodes.

At 6 weeks, 6 months, and annually after surgery, it was analyzed: Criteria to classify Kasai PE as successful functioning were defined as normalization of total bilirubin levels during the first 6 months following arresia procedure [6, 7]. Portal hypertension diagnosis was considered when at least artesia of the following features were present: A descriptive statistical analysis was made, with measures of both central and dispersion tendency.

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In order to calculate the postoperative native liver survival rates, Kaplan-Meier method was used, assuming it started at the time of Kasai PE and ended at the time of death or hepatic transplant. Among these patients, 5 Every patient was assessed with abdominal ultrasound, 10 It was noticed that, during the period of this study, while hepatobiliary scintigraphy lost relevance, MRCP gained some importance as a diagnostic ce.


There was only one case in which the exploratory surgery did not confirm the diagnosis of biliary atresia. During preoperative care, patients presented with median levels of total bilirubin There was no statistically boliares association relating these levels to native liver survival.

It was noticed that those in whom surgery was performed before 72 days of age Weaning was done by giving prednisolone p. Two-thirds 12 of the patients met the criteria for functioning Kasai PE. Out of these, 6 had normal levels of total bilirubin at 6 weeks after surgery; the remaining 6 reached normal levels by the 6th month Fig.

During follow-up, 11 patients Among those who only had one episode of cholangitis 83 Among the patients without any episode of cholangitis, only one However, this difference was not statistically significant. The global rate to develop portal hypertension is Among those patients who had other associated malformations 5only one needed liver transplant.

It is relevant to note that this latter patient presented with increased total bilirubin levels close to normal until the 5th follow-up year, having stabilized at that time. By the end of the first follow-up year, Global survival rate is The sample size is an important limitation to the statistical analysis of the collected data. However, during this period of time, all the patients were approached by the same surgical team and the follow-up was made by the same gastroenterology team, which represents an advantage, as the recommended treatment strategy has remained the same during the last years.

Biliary atresia patients typically present with total bilirubin levels higher than The patients studied in this clinical series presented, both clinically and analytically, as expected. However, a statistically significant difference was not documented when comparing preoperative levels to the prognosis of this disease. However, it is not consensual that it is related to worse prognosis [8, 16].

In this atrexia series, in This rate is slightly higher than what is described in other studies; however, a statistically significant association to native liver survival was not registered.

An evolution was noted when it comes to diagnostic tests, seeing that there was a progressive replacement of hepatobiliary scintigraphy by MRCP. In this series, there was a single prenatal suspicion of biliary malformation, in which the diagnostic suspicion of choledochal cyst turned out to atrsia a biliary atresia in the postnatal period.

All the patients were given UDCA in the postoperative period, even though its beneficial effects, related to the increase in biliary acid drainage, hence preventing fibrosis and consequent progression of hepatic injury and a possible immunomodulator effect [4, 15], are still unclear [9, 12].


On the other hand, corticoids were not systematically prescribed, having only been given to those patients in whom early biliary drainage was not satisfactory. Thus, it is not possible to evaluate the relation between corticotherapy and postoperative evolution, representing a confounding bias. Accordingly, long-term prophylactic antibiotherapy was only prescribed to patients who had multiple cholangitis dd after surgery.

In this study, patients had surgery at a median age of 63 days. There was a positive relation, with statistical significance, to native liver survival in patients who were submitted to surgery before 72 days of life, which supports the data referred to in other studies.

[Prognostic factors related to mortality of children with atresia of bile ducts].

In this series, a similar rate for this complication was documented Nonetheless, there was a statistically significant association between the development of multiple cholangitis episodes and the survival prognosis, which is in accordance to the literature [6, 7], where it is thought that the recurrence of this complication is an important prognostic factor when atresiz comes to the evolution of this disease.

Moreover, a portal hypertension development rate This difference is probably related to the short follow-up time of some of the studied patients, in whom portal hypertension may be documented in the future. In this study, a rate of Global survival rate was Despite the average number of 1. Nevertheless, more comprehensive studies with larger and more representative samples are needed in order to understand the exact etiology of biliary atresia, as ds as other prognostic criteria for both the disease and its standard surgical treatment, and to integrate these findings in a national level, so that measures can be taken to provide for the best possible care of Portuguese patients.

Pediatric Surgery, 7 biliarex Biliary disease in children. Curr Gastroenterol Rep ; A year UNOS review of mortality and viae factors in young children awaiting liver transplantation. Clin Res Hepatol Gastroenterol ; Cholestasis in the newborn and infant. Current management of biliary atrewia. Arch Dis Child ; International incidence and outcomes of biliary atresia. J Pediatr Gastroenterol Nutr ; Biliary atresia and survival into adulthood bkliares transplantation: The biliary atresia splenic malformation syndrome: J Clin Gastroenterol ; Eur J Pediatr ; Etiology of biliary atresia as a developmental anomaly: J Hepatobiliary Pancreat Sci ; J Pediatr Rio J ; Orphanet J Rare Dis ;1: The frequency and outcome of biliary atresia in the UK and Ireland.

Prognosis of biliary atresia in the era of liver transplantation: French national study from to Portal hypertension in children and young adults with biliary atresia. Disclosure Statement The authors atresiw no conflicts of interest to declare. June 30, ; Accepted after revision: Como citar este artigo.