Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996

Chardot, Christophe; Carton, Matthieu; Spire-Bendelac, Nathalie; Pommelet, C Le; Golmard, JL; Auvert, Bertran

doi:10.1002/hep.510300330

Cited by 249 publications

(236 citation statements)

References 22 publications

(31 reference statements)

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“…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] . O prognóstico dos pacientes com AB tem melhorado nas últimas décadas, atingindo sobrevida de 90% dos casos 10 , devido à realização da portoenterostomia em idade adequada e do TxH seguido de imunossupressão.…”

Section: Introductionunclassified

“…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] . O prognóstico dos pacientes com AB tem melhorado nas últimas décadas, atingindo sobrevida de 90% dos casos 10 , devido à realização da portoenterostomia em idade adequada e do TxH seguido de imunossupressão. Mais de 50% dos pacientes submetidos a portoenterostomia tornam-se anictéricos e, entre eles, muitos chegam à adolescência sem TxH.…”

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: Introductionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

J Pediatr (Rio J)

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“…Consequently, EHBA is the most common indication for liver transplantation in the pediatric population (16). Currently, the 10-year survival for infants who have EHBA is only 70% (1,5).…”

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confidence: 99%

Effect of Rotavirus Strain on the Murine Model of Biliary Atresia

Allen¹,

Jafri²,

Donnelly³

et al. 2007

J Virol

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Biliary atresia is a devastating disorder of the newborn in which afflicted infants develop inflammation and fibrosis of the extrahepatic biliary tract, resulting in cirrhosis and end-stage liver disease. Infection with a virus is thought to be a contributing factor in the etiology of biliary atresia. In the murine model of biliary atresia, perinatal exposure to rhesus rotavirus (RRV) results in biliary epithelial cell infection causing bile duct obstruction. The purpose of this study was to determine if tropism for the biliary epithelial cell was unique to RRV. Newborn mice underwent intraperitoneal injection with five strains of rotavirus: RRV (simian), SA11-FM (simian/bovine), SA11-SM (simian), EDIM (murine), and Wa (human). RRV and SA11-FM caused clinical manifestations of bile duct obstruction and high mortality. SA11-SM caused clinical signs of hepatobiliary injury but the mortality was markedly reduced. EDIM and Wa caused no sign of hepatobiliary disease. The systemic and temporal distribution of viral protein and live virus varied according to the injected strain. Immunohistochemistry revealed that RRV and SA11-FM targeted the biliary epithelial cells. In contrast, SA11-SM was found in the liver but in not in the biliary epithelium. These results indicate that strain-specific characteristics dictate tropism for cells of hepatobiliary origin which in turn impact the ability to induce the murine model of biliary atresia.

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“…It typically occurs in the first month of life as icterus and acholia with or without hepatomegaly in previously healthy neonates of average body weight for gestational age. The optimal treatment is to establish satisfactory bile flow from the liver to the gastrointestinal tract by Kasai portoenterostomy during the first 8 weeks of life (1,2). If this fails and/ or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia (BA) represents the most frequent pediatric indication.…”

Section: Introductionmentioning

confidence: 99%

Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants

Boskovic

Kitic²,

Prokić³

et al. 2014

Turk J Gastroenterol

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Background/Aims: Extrahepatic biliary atresia (EHBA) is the most important cause of neonatal cholestasis. The validity of different diagnostic methods in the diagnosis of EHBA in developed countries has been presented elsewhere, but data from developing countries with low national incomes are scarce. The aim of this study was to investigate the relative accuracy and roles of abdominal ultrasonography, duodenal tube test (DTT), and liver biopsy in the diagnosis of EHBA in Serbia. Materials and Methods: The study included 156 infants with cholestasis admitted at the Mother and Child Health Care Institute. Data were collected according to the medical records observation technique. Results: Extrahepatic biliary atresia was diagnosed in 72 of 156 infants with cholestasis. The frequency was insignificantly higher in females than in males (1.25:1). Most patients were diagnosed prior to 60 days of life (median 58, range 30-67). In a group of 156 infants with cholestasis, 109 had ultrasound, liver biopsy, duodenal tube test, and intraoperative cholangiography done. Liver biopsy confirmed surgical disease in 71/109 patients and denied it in 38/109 patients (sensitivity-Sn 98%, specificity-Sp 100%, diagnostic efficiency of test-DgEf 99.08%). Duodenal tube test had Sn 97%, Sp 72%, and DgEf 88.99%, and the ultrasound findings showed Sn 78%, Sp 81%, and DgEf 77.92%. Five-year survival rate after Kasai operation was 76%. Conclusion: A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Histology examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease.

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Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996

Cited by 249 publications

References 22 publications

Biliary atresia: the Brazilian experience

Biliary atresia: the Brazilian experience

Effect of Rotavirus Strain on the Murine Model of Biliary Atresia

Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants

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