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.

Aplasia medular após transplante hepático em pediatria / Aplastic anemia after pediatric liver transplantation

A aplasia de medula é uma das mais raras (<1 por cento) e sérias complicações após o transplante hepático por insuficiência hepática aguda grave viral não A, não B e não C. Esta condição clínica, que acomete simultaneamente o tecido hepático e o hematopoético, foi descrita pela primeira vez em 1987, por Stock, e a fisiopatologia relacionada é uma condição imunomediada, provavelmente secundária à infecção viral desconhecida, e associada a grave prognóstico. A recuperação espontânea da aplasia medular adquirida habitualmente é muito rara e 50 por cento-70 por cento dos pacientes respondem ao tratamento imunossupressor com ciclosporina A (CsA) e glubulina antitimocítica (ATG), mesmo após o transplante hepático. Além do tratamento imunossupressor, outra opção é o transplante de medula óssea (TMO). Apresentamos o caso de uma criança com aplasia medular grave após transplante hepático, por insuficiência hepática aguda grave, que recebeu tratamento imunossupressor com CsA e ATG e evoluiu com recuperação completa das três séries do hemograma.

Aplastic anemia (AA) is one of the rarest (<1 percent) and most serious complications of liver transplantation for fulminant non-A, non-B and non-C hepatitis. It was first described in 1987 by Stock; the mechanism involved is an immunologically mediated condition secondary to an unknown viral infection. The disease is associated with a dismal prognosis. Spontaneous recovery from acquired AA is very rare however some patients (50-70 percent) recover after immunosuppressive therapy, such as Cyclosporin A (CsA) and Antithymocyte globulin (ATG), even after liver transplantation. Another treatment option is bone marrow transplantation. We report on a child who developed AA following liver transplantation for fulminant viral hepatitis that was treated with intensive immunosuppression including CsA and ATG and achieved complete recovery.

Immunohistochemical studies of stellate cells in experimental cholestasis in newborn and adult rats

BACKGROUND AND AIMS: Although there is much known about liver diseases, some aspects remain unclear, such as the nature of the differences between the diseases observed in newborn infants and those in adults. For example, how do newborns respond to duct epithelial cell injury? Do the stellate cells in newborns respond similarly to those in adults during biliary obstruction? METHODS: Ninety newborn Wistar rats aged six days, weighing 8.0 - 13.9 g each, and 90 adult rats weighing 199.7 - 357.0 g each, were submitted to bile duct ligation. After surgery, they were randomly divided and sacrificed on the 3rd, 5th, 7th, 14th, 21st or 28th day post-bile duct ligation. Hepatic biopsies were obtained and immunohistochemical semi-quantification of desmin and á-SMA expression was performed in hepatic stellate cells and in myofibroblasts in the portal space, and between the portal space and the liver lobule. RESULTS: Desmin expression in the myofibroblast cells post-bile duct ligation was higher in young rats, reaching its peak level in a shorter time when compared to the adult animals. The differences between the groups for á-SMA expression were less significant than for desmin. CONCLUSIONS: These findings indicate that there is an increase in the number of collagen-producing myofibroblast cells in young animals, suggesting that there is more intense fibrosis in this population. This finding may explain why young animals with bile duct obstruction experience more intense portal fibrosis that is similar to the pathology observed in the livers of newborns with biliary atresia.

Fibrose portal e periportal na obstrução extra-hepática experimental em ratos jovens e adultos: contribuição para o estudo da atresia das vias biliares / Portal and periportal fibrosis in experimental extra-hepatic biliary obstruction in young and adult rats

A atresia das vias biliares é afecção hepática da infância. A etiologia é desconhecida, e o diagnóstico baseia-se na biópsia hepática, cujo achado é a proliferação ductular. A ligadura do ducto biliar comum em ratos é modelo utilizado para estudo das doenças colestáticas. A proposta do trabalho foi estudar, em modelo experimental de obstrução biliar, as alterações histológicas hepáticas em ratos jovens e compará-las com o animal adulto / Biliary atresia is an hepatic disease of infancy. Etiology is unknown, and diagnosis is made by liver biopsy, with ductular proliferation being the main histological feature. Bile duct ligation in rats is an useful experimental model of biliary obstruction. The aim of this study of extra-hepatic cholestasis was analyse hepatic histological alterations in young rats compared to adult animals...

Hemangioma hepático / Hepatic hemagioma

Objetivos: Descrever um caso de hamangioma hepático em recém-nascido tratado com cirurgia. Métodos: Relata-se o caso de recém-nascidos do sexo masculino, de 6 dias de vida, com extensa lesão hepática, e sua evolução. Resultados: A criança evoluiu com quadro de descompensação hemodinâmica devido a coagulopatia de consumo e insuficiência respiratória. Os exames de imagem foram inconclusivos, sendo indicado procedimento cirúrgico. Foi realizada ressecção total da massa hepática e confirmado o diagnóstico de hamangioma por estudo anatomopatológico. A criança evoluiu satisfatoriamente, recebeu alta após 15 dias e encontra-se assintomática. Conclusões: O hemangioma hepático deve ter tratamento conservador, estando a cirurgia reservada para os casos de insuficiência cardíaca intratável e/ou coagulopatia consumptiva refratária