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- Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary AtresiaPublication . Guedes, Renata R.; Kieling, Carlos; Santos, Jorge Luiz dos; Rocha, Carolina da; Schwengber, Fernando; Adami, Marina R.; Chedid, Marcio F.; Vieira, Sandra M. G.Background Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis. Aims To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia. Methods All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninetyday mortality was also evaluated. Prognostic factors related to both endpoints also were studied. [...]
- Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomesPublication . Porta, Gilda; Carvalho, Elisa; Santos, Jorge Luiz Dos; Gama, Jorge; Borges, Cristian V.; Seixas, Renata; Ferreira, Alexandre; Miura, Irene; Silveira, Themis Reverbel; Silva, Luciana; Fagundes, Eleonora; Brandao, MA Bellomo; Sawamura, Regina; Vieira, Sandra; Melere, Melina; Marques, Cibele D F; Pugliese, Renata P.; Danesi, Vera; Porta, Adriana; Marsillac, Marise; Valladares, Marcia Angélica; Menezes, Daniela G.; Kieling, Carlos; Paula, Mariana N. de; Vasconcelos, Juliana R.; Ferreira, Cristina T.; Perin, Nilza; Resende, Leonardo R.; Maia, Jussara; Tommaso, Adriana M.A. De; Hessel, Gabrielsentation, laboratory findings, histological profile, treatments, and outcomes of children andadolescents with autoimmune hepatitis.Methods: The medical records of 828 children and adolescents with autoimmune hepatitiswere reviewed. A questionnaire was used to collect anonymous data on clinical presentation,biochemical and histological findings, and treatments.Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmunehepatitis-2. The female sex was predominant in both groups. The median age at symptomonset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher forautoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patientswith autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-foldhigher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were sig-nificantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels werelower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochem-ical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6%underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmunehepatitis-2: 2.4%).Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmunehepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higherdisease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.