Extrahepatic biliary atresia: current concepts and future directions

Carvalho, Elisa de; Ivantes, Claudia Alexandra Pontes; Bezerra, Jorge A.

doi:10.2223/jped.1608

Cited by 54 publications

(78 citation statements)

References 101 publications

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“…Em relação à terapêutica, foram avaliados os dados relativos a realização, ou não, dos procedimentos cirúrgicos (portoenterostomia e TxH), a data destes e a evolução pós-operatória, até a data do último seguimento. O diagnóstico da AB baseou-se em critérios clínicos, bioquímicos, histológicos, de imagem e cirúrgicos, conforme já estabelecidos 6,11 .…”

Section: Pacientes E Métodosunclassified

“…A AB se manifesta nas primeiras semanas de vida e apresenta uma distribuição universal, com incidência variável nas diferentes regiões do mundo [2][3][4][5] . A etiologia da AB ainda não foi completamente elucidada e vários mecanismos têm sido propostos para explicar a colangiopatia progressiva observada nesta entidade 6,7 . O diagnóstico precoce da AB e o tratamento cirúrgico (portoenterostomia), que restabelece o fluxo biliar, realizados preferencialmente antes dos 60 dias de vida, são fundamentais, uma vez que o diagnóstico tardio relaciona-se à necessidade de TxH ou morte nos primeiros 3 anos de vida [8][9][10] .…”

Section: Introductionunclassified

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Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

J Pediatr (Rio J)

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Objective: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.Methods: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers and compared regarding decades of surgery.Results: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year postportoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). Conclusions:This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life. J Pediatr (Rio J). 2010;86(6):473-479:Biliary atresia, portoenterostomy, hepatic, surgery, diagnosis, differential, prognosis. ResumoObjetivo: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008) e Sudeste (p = 0,0012), embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6%. A sobrevida aumentou nas últimas décadas (anos 1980 versus 90, p = 0,002; anos 1980 versus 2000, p < 0,001; anos 1990 versus 2000, p < 0,001). A sobrevida de 4 anos pós-portoenterostomia, com ou sem TxH, foi de 73,4%, inversamente correlacionada à idade no momento da portoenterostomia (80, 77,7, 60,5% para ≤ 60, 61-90, > 90 dias, respectivamente). Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3%). A sobrevida de 4 anos com fígado nativo foi de 36,8%, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6% para ≤ 60, 61-90, > 90 dias, respectivamente). Método Conclusões:Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portad...

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Section: Pacientes E Métodosunclassified

Section: Introductionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

J Pediatr (Rio J)

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“…7,22 Nevertheless, less invasive preliminary tests capable of refining the indications for laparotomy are necessary to avoid unnecessarily subjecting patients with cholestasis due to intrahepatic causes to the procedure.…”

Section: Discussionmentioning

confidence: 99%

Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar

Roquete¹,

Ferreira²,

Fagundes³

et al. 2008

J. Pediatr. (Rio J.)

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Objectives: To define the sensitivity, specificity and accuracy of the ultrasound triangular cord sign and hepatic histopathology, in isolation or in combination, for diagnostic differentiation between biliary atresia and intrahepatic cholestasis. Methods:This was a retrospective study carried out between January 1990 and December 2004. Fifty-one cases of biliary atresia and 45 of intrahepatic cholestasis were analyzed. Histopathology was performed blind by a pathologist.The triangular cord sign was identified in ultrasound reports as the only diagnostic sign of biliary atresia. Sensitivity, specificity and accuracy were calculated for the triangular cord sign and histology both in isolation and in combination.The gold standard for diagnosis of biliary atresia was the appearance of the extrahepatic biliary tree via laparotomy. Results:The triangular cord sign alone had sensitivity of 49%, specificity of 100% and accuracy of 72.5%.Histopathology compatible with extrahepatic biliary obstruction alone had 90.2% sensitivity, 84.6% specificity and 87.8% accuracy. The triangular cord sign and histopathology in isolation or combination resulted in sensitivity of 93.2%, specificity of 85.7% and accuracy of 90.3%. Conclusions:Finding the triangular cord sign on ultrasound is an indication for laparotomy. If the triangular cord sign is negative, liver biopsy is indicated; if histopathology reveals signs of biliary atresia, explorative laparotomy is indicated. In cases where the triangular cord sign is absent and histopathology indicates neonatal hepatitis or other intrahepatic cholestasis, clinical treatment or observation are recommended in accordance with the diagnosis.J Pediatr (Rio J). 2008;84(4):331-336: Neonatal cholestasis, biliary atresia, ultrasound, liver biopsy.

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“…1 Liver fibrosis is a complex process that involves extensive extracellular matrix (ECM) remodeling and proteolysis. 2 Using microarray analysis, Chen et al 3 identified many molecular markers associated with BA of which at least one-third are related to ECM remodeling.…”

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Persistent elevation of hepatocyte growth factor activator inhibitors in cholangiopathies affects liver fibrosis and differentiation

et al. 2011

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Alteration of cell surface proteolysis has been proposed to play a role in liver fibrosis, a grave complication of biliary atresia (BA). In this study we investigated the roles of hepatocyte growth factor activator inhibitor (HAI)-1 and -2 in the progression of BA. The expression levels of HAI-1 and -2 were significantly increased in BA livers compared with those in neonatal hepatitis and correlated with disease progression. In BA livers, HAI-1 and -2 were coexpressed in cells involved in ductular reactions. In other selective cholangiopathies, ductular cells positive for HAI-1 or HAI-2 also increased in number. Inflammatory cytokines, growth factors, and bile acids differentially up-regulated expression of HAI-1 and -2 transcripts in fetal liver cells and this induction could be antagonized by a cyclooxygenase-2 inhibitor. Conditioned media from cell lines stably overexpressing HAI-1 or HAI-2 enhanced the fibrogenic activity of portal fibroblasts and stellate cells, suggesting that both proteins might be involved in liver fibrosis. Because HAI-1 and -2 colocalized in ductular reactions sharing similar features to those observed during normal liver development, we sought to investigate the role of HAI-1 and -2 in cholangiopathies by exploring their functions in fetal liver cells. Knockdown of HAI-1 or HAI-2 promoted bidirectional differentiation of hepatoblast-derived cells. In addition, we showed that the hepatocyte growth factor activator, mitogen-activated protein kinase kinase 1, and phosphatidylinositol 3-kinase signaling pathways were involved in hepatic differentiation enhanced by HAI-2 knockdown. Conclusion: HAI-1 and -2 are overexpressed in the liver in cholangiopathies with ductular reactions and are possibly involved in liver fibrosis and hepatic differentiation; they could be investigated as disease markers and potential therapeutic targets. (HEPATOLOGY 2012;55:161-172)

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Extrahepatic biliary atresia: current concepts and future directions

Cited by 54 publications

References 101 publications

Biliary atresia: the Brazilian experience

Biliary atresia: the Brazilian experience

Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar

Persistent elevation of hepatocyte growth factor activator inhibitors in cholangiopathies affects liver fibrosis and differentiation

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