Biliary atresia: we still operate too late

Kieling, Carlos Oscar; Santos, Jorge Luiz dos; Vieira, S; Ferreira, Cristina Helena Targa; Linhares, Ana Raniele; Lorentz, Andrea Longoni; Silveira, Themis Reverbel da

doi:10.2223/jped.1825

Cited by 17 publications

(16 citation statements)

References 24 publications

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“…A idade observada, no momento da portoenterostomia, foi superior à desejável, revelando o encaminhamento tardio, um problema já descrito anteriormente em nosso país 18,19 . Desse modo, no Brasil, o encaminhamento tardio dos pacientes com AB para portoenterostomia continua sendo um problema a ser resolvido em nível nacional, independentemente da região ou da categoria da cidade, seja capital ou cidade do interior.…”

Section: Discussionunclassified

“…Para aqueles que apresentam complicações como hipertensão portal e cirrose, o TxH proporciona uma boa qualidade de vida 11 . Vários países têm descrito suas experiências em relação à AB 5,[12][13][14][15][16][17] , porém há poucos relatos isolados sobre a experiência com AB no Brasil 18,19 . O presente estudo teve como objetivo avaliar as características clínicas, epidemiológicas e prognósticas de crianças brasileiras portadoras de AB.…”

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: Discussionunclassified

Section: Introductionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

J Pediatr (Rio J)

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“…8,14,19,22,23 The median age of infants at the time of referral is a cause for concern. In the EHC group, age at referral was over 60 days (77 days) of life and higher than that in the IHC group.…”

Section: Discussionmentioning

confidence: 99%

Diagnóstico diferencial de colestase neonatal: parâmetros clínicos e laboratoriais

Bellomo-Brandão¹,

Arnaut²,

Tommaso³

et al. 2010

J. Pediatr. (Rio J.)

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Objective: To evaluate if clinical and laboratory parameters could assist in the differential diagnosis of intra and extra-hepatic neonatal cholestasis (NC). Methods:Retrospective study of NC patients admitted at the Pediatric Hepatology Outpatient Clinic of the teaching hospital of Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil, between December 1980 and March 2005. The approach to the diagnosis of NC was standardized. According to diagnosis, patients were classified into two groups: I (intra-hepatic neonatal cholestasis) and II (extra-hepatic neonatal cholestasis). In order to verify if there was association with the categorical variable, the chi-square and Mann-Whitney tests were used, with corrections for age for the covariance analysis (ANCOVA). The determination of accuracy of the clinical and laboratory variables for differentiation of the groups was made using the analysis of the ROC curve. Results:One hundred and sixty-eight patients were evaluated (group I = 54.8% and group II = 45.2%). In the patients with less than 60 days of life there was predominance of intra-hepatic causes, whereas, in those older than 60 days, there was predominance of extra-hepatic etiology (p < 0.001). Median birth weight was lower in group I (p = 0.003), as well as length at birth (p = 0.007). Median values of direct bilirubin were higher in group II (p = 0.006). Values of gamma-glutamyltransferase (GGT) (10 times higher than the limit of normality) presented sensitivity of 56.3%, specificity of 91.5%, and accuracy of 75.7% for the diagnosis of extra-hepatic cholestasis. Conclusion:In the present study, extra-hepatic NC presented greater weight and length at birth, fecal hypocholia/ acholia, choluria, hepatomegaly, increase in GGT (10.8 times higher than the limit of normality), and a delay for investigation in the tertiary center.J Pediatr (Rio J). 2010;86(1):40-44: Neonatal cholestasis, children, hepatitis.

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“…The authors estimated that if portoenterostomy were performed before 46 days of life, up to 5.7% of the liver transplantations performed in France in patients younger than 16 years of age could be avoided. A delay in referral and surgery remains a problem worldwide because of difficulties in differentiating BA from other causes of prolonged jaundice in the young infant (31). In Taiwan, a universal screening system was established using an infant stool color card previously tested in other countries.…”

Section: Early Diagnosis and Clinical Outcomementioning

confidence: 99%