Biliary atresia: the Brazilian experience

• DOI: 10.2223/jped.2054
• Publication Date: 2010-12-22T07:29:56Z
• Authors: Elisa de Carvalho; Jorge Luiz dos Sa...; Themis Reverbel d...; Carlos Oscar Kieling; Luciana Rodrigues...; Gilda Porta; Irene Kazue Miura; Adriana Maria Alv...; Maria Ângela Bell...; Alexandre Rodrigu...; José Roberto de D...; José Tenário de A...; Grupo de Estudos ...
• Keywords: Male; 0301 basic medicine; Prognóstico; Portoenterostomia hepática; Infant; Portoenterostomy, Hepatic; Diagnóstico diferencial; Hepatic portoenterostomy; Atresia biliar; Prognosis; Liver Transplantation; 3. Good health; Survival Rate; 03 medical and health sciences; Biliary Atresia; Child, Preschool; Humans; Surgery; Differential diagnosis; Female; Biliary atresia; Epidemiologic Methods; Brazil

To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.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 (1982-2008) and compared regarding decades of surgery.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 post-portoenterostomy 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).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.