the choice of treatment depends on the timing of diagnosis; however, in this study, surgical revision or correction produced worse outcomes than percutaneous angioplasty. The reduction of risk factors and early detection of vascular complications are key elements to a successful transplantation.

Children with portal hypertension with a CPR below 114 and a risk score greater than -1.2 are more likely to have present EV. Therefore, these two tests can be helpful in selecting children for endoscopy.

In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

This study suggests that hypoxia-ischemia is present in the livers of patients with BA, progresses over time and leads to a decreased cholangiocyte mass.

ResumoObjetivo: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. of cases, the age was below 60 days. There was no age difference at diagnosis for the three decades in the study. Patients from the countryside (median: 87.0; IQR25-75: 69.0-115.0 days) were referred significantly later (p = 0.007) than those living in Porto Alegre and the metropolitan area (median: 68.0; IQR25-75: 55.5-98.0 days). The ratio of patients younger than 60 days was significantly lower (p = 0.013) for those from the countryside. Survival periods with native liver for all patients was 46.2% in 2 years, falling continuously until reaching 15.3% in 20 years. Patients operated before reaching 60 days of age had longer survival periods with native livers (log rank < 0.0001). MétodosConclusions: Late performance of portoenterostomy was a constant in the past 25 years, and this delay led to shorter survival periods with native livers for biliary atresia patients.J Pediatr (Rio J). 2008;84(5):436-441: Biliary atresia, neonatal cholestasis, surgery, prognosis, survival. IntroduçãoA atresia biliar (AB) é a doença hepática crônica que mais freqüentemente afeta as crianças e é a causa mais comum de colestase neonatal. Sua etiologia permanece desconhecida e provavelmente resulta de um processo heterogêneo em que o insulto inicial, seja infeccioso, isquêmico ou tóxico, precipita a destruição inflamatória progressiva dos ductos biliares nos pacientes geneticamente suscetíveis 1 . A AB tem distribuição universal, e estima-se que afeta 1 em cada 5.000 a

High IL-6 levels were found in children with chronic liver disease at nutritional risk. Inflammatory activity may be related to nutritional status deterioration in these patients.

No abstract

We retrospectively analysed 33 children and adolescents who had been hospitalized in a liver transplant unit within the previous 10 years for acute liver failure (ALF). The patients' age varied between 2 months and 15 years of age (median 6.2 +/- 5.3), and 21 (63%) were male. Thirteen patients (39%) were immunoglobulin-M anti-hepatitis A virus (HAV) sero-positive. Eleven cases (33%) had an undetermined aetiology. The 13 children with HAV ALF were between 17 months and 15.6 years of age (median 5.8 +/- 4.6) and eight were male (61.5%). All were on a list for urgent liver transplant. Of these, five (38%) died while waiting for a liver. Only one patient recovered spontaneously. Seven patients received a liver transplant; three died in the immediate postoperative period and one died 45 days after transplant. Three children are alive 1, 2 and 5 years after transplant. We conclude that HAV was the most frequent cause of ALF, which had high mortality even when a liver transplant was possible. The results support universal HAV vaccination in this area.