Loading...
2 results
Search Results
Now showing 1 - 2 of 2
- 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.
- Environmental risk factors associated with biliary atresia in Rio Grande do sul, BrazilPublication . Longo, Larisse; Ferrari, Jéssica Tonin; Hoss, Giovana Regina Weber; Fratta, Leila Xavier Sinigaglia; Vieira, Sandra Maria Gonçalves ; Silveira, Themis Reverbel da; Silva, Fernando Antônio de Abreu e; Sanseverino, Maria Teresa; Santos, Jorge Luiz dosBiliary atresia (BA) seems to be a multifactorial disorder in which environmental factors interact with the patient’s genetic constitution. This study aimed to analyze information concerning environmental risk factors associated with BA in southern Brazil. A case-control study with mothers of patients with BA and mothers of patients with cystic fibrosis (CF) was conducted. Inquiry included questions related to exposition to environmental risk factors during the periconceptional and gestational (second and third trimesters) periods. Mothers of BA patients had smoked during pregnancy more frequently in comparison with the mothers of CF patients, but no significant difference was found in a multivariate analysis. There was no between group difference in terms of seasonality, but the multivariate analysis showed a significant difference within the BA group between date of conception in winter compared to other seasons. In conclusion, smoking during pregnancy seemed to increase the risk of BA while date of conception in winter decreased it.