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.

Icterícia como causa de internação neonatal: a experiência em um serviço terciário de Porto Alegre, RS / Jaundice in healthy term newborns treated at a tertiary center in Porto Alegre, RS

Introdução: Cerca de 60-80% dos recém-nascidos (RN) tornam-se ictéricos durante os primeiros dias de vida. Apesar de geralmente representar um fenômeno transitório, alguns pacientes necessitam de tratamento hospitalar. O objetivo deste estudo foi determinar a causa principal de icterícia neonatal em recém-nascidos saudáveis internados no Hospital Luterano e possíveis associações com diversas variáveis clínicas. Metodologia: Estudo retrospectivo em que foram estudados todos os casos de RN com icterícia neonatal internados para tratamento de hiperbilirrubinemia na UTI Neonatal do Hospital Luterano da ULBRA, no período de abril de 2007 a dezembro de 2008. Os resultados foram expressos em estatística descritiva e foi utilizado o teste exato de Fischer e o teste Qui-quadrado. O limite alfa considerado foi de 5%, com nível de significância de 0,05. Resultados: Dentre os RNs estudados (74), 52,7% eram do sexo masculino e 45,9% eram do sexo feminino. 14,8% dos pacientes nasceram de parto vaginal, enquanto que 85,1% nasceram de cesárea. A maioria dos recém-nascidos estudados (74,3%) foi considerada a termo. O diagnóstico mais frequente (37,8%) de icterícia dos pacientes internados para tratamento no serviço foi o de baixo aporte. Os pacientes do sexo masculino necessitaram de maior tempo de fototerapia do que as pacientes do sexo feminino (p=0,056). Conclusão: O diagnóstico de baixo aporte recebido pelos pacientes foi a causa mais frequente de icterícia. Os meninos necessitaram de um tempo significativamente maior de fototerapia para o tratamento da icterícia do que as meninas; também houve associação positiva da hiperbilirrubinemia com a baixa idade.

Introduction: About 60-80% of the newborns (NB) experience jaundice in the first days of life. Although jaundice is often a transitory phenomenon, some infants require hospital care. The aim of this study was to determine the main cause of neonatal jaundice among healthy newborns admitted to the Hospital Luterano and the possible associations with a number of clinical variables. Methods: A retrospective study in which all cases of NB with neonatal jaundice admitted for treatment of hyperbilirubinemia at the Neonatal ICU of the Hospital Luterano of ULBRA were studied, from Apr 2007 to Dec 2008. The results were expressed as descriptive statistics, and Fisher’s exact test and the Chi-square test were applied. The alpha limit considered was 5%, with level of significance at 0.05. Results: Among the 74 NB studied, 52.7% were males and 45.9% were females. 14.8% of the infants had a vaginal birth, while 85.1% had a cesarean delivery. Most of the studied infants (74.3%) were born full term. The most frequent cause for (37.8%) jaundice among these patients was inadequate intake. The male patients needed to stay longer on phototherapy than female patients (p=0.056). Conclusion: Low intake by the patient was the most frequent cause of jaundice in this series. The boys needed significantly more time on phototherapy than females, and there was a positive association of hyperbilirubinemia with low age.

Histopathological diagnosis of intra-and extrahepatic neonatal cholestasis

The histopathology of the liver is fundamental for the differential diagnosis between intra- and extrahepatic causes of neonatal cholestasis. However, histopathological findings may overlap and there is disagreement among authors concerning those which could discriminate between intra- and extrahepatic cholestasis. Forty-six liver biopsies (35 wedge biopsies and 11 percutaneous biopsies) and one specimen from a postmortem examination, all from patients hospitalized for neonatal cholestasis in the Pediatrics Service of Hospital de Clínicas de Porto Alegre, were prospectively studied using a specially designed histopathological protocol. At least 4 of 5 different stains were used, and 46 hepatic histopathological variables related to the differential diagnosis of neonatal cholestasis were studied. The findings were scored for severity on a scale from 0 to 4. Sections which showed less than 3 spaces were excluded form the study. Sections were examined by a pathologist who was unaware of the final diagnosis of each case. Bile tract permeability was defined by scintigraphy of the bile ducts and operative cholangiography. The F test and discriminant analysis were used as statistical methods for the study of the hepatic histopathological variables. The chi-square method with Yates correction was used to relate the age of the patients on the date of the histopathological study to the discriminatory variables between intra- and extrahepatic cholestaasis selected by the discriminant function test. The most valuable hepatic histopathological variables for the discrimination between intra- and extrahepatic cholestasis, in decreasing order of importance, were periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules, foci of myeloid metaplasia, and portal-portal bridges. The only variable which pointed to the diagnosis of intrahepatic cholestasis was myeloid metaplasia. Due to the small number of patients who were younger than 60 days on the date of the histopathological study (N=6), no variable discriminated between intra- and extrahepatic cholestasis before the age of 2 months and all of them, except for the portal expansion, were discriminatory after this age. In infants with cholestasis, foci of myeloid metaplasia, whenever present in the liver biopsy, suggested intrahepatic cholstasis. Periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules...

Jaundice in the newborn

Jaundice in the newborn is a relatively common symptom. It may be physiologic or due to a pathologic cause. Determination of the serum bilirubin is followed by serial determinations to document the rate of increase. Unconjugated hyperbilirubinemia, if left untreated, may reach toxic levels and cause bilirubin encephalopathy. Acceptable methods of treatment include phototherapy and exchange transfusion

Síndrome de alagille: dos casos en hermanos / Alagille syndrome: two cases in brothers

Se presentan dos casos de Síndrome de Alagille. Se describen las principales manifestaciones del cuadro, haciendo hincapié sobre signos y síntomas dermatológicos, los cuales, a menudo son mencionados someramente en la mayoría de las publicaciones