Study of enzyme replacement therapy for Gaucher Disease: comparative analysis of clinical and laboratory parameters at diagnosis and after two, five and ten years of treatment.

OBJECTIVE: To evaluate the impact of enzyme replacement therapy for Gaucher Disease on clinical and laboratory parameters after two, five and ten years of treatment. METHODS: Data were collected from patient records and analyzed using BioEstat software (version 5.0). Student's t-test, Analysis of Variance (ANOVA), Wilcoxon test and Kruskal-Wallis test were used for statistical analysis. Hepatomegaly and splenomegaly were analyzed using the Kappa test. RESULTS: There was a significant increase in hemoglobin levels (p-value <0.01) and platelet counts (p-value=0.01) within two years of therapy. At the same time, the frequencies of splenomegaly (p-value <0.01) and hepatomegaly (p-value <0.05) reduced. These results were similar at five and ten years of enzyme replacement therapy. CONCLUSIONS: There are substantial and quick (within two years) laboratory and clinical responses to enzyme replacement therapy. These improvements continue as long as enzyme replacement therapy is administered every two weeks, as recommended by the literature.

Study of enzyme replacement therapy for Gaucher Disease: comparative analysis of clinical and laboratory parameters at diagnosis and after two, five and ten years of treatment

Objective: To evaluate the impact of enzyme replacement therapy for Gaucher Disease on clinical and laboratory parameters after two, five and ten years of treatment. Methods: Data were collected from patient records and analyzed using BioEstat software (version 5.0). Student's t-test, Analysis of Variance (ANOVA), Wilcoxon test and Kruskal–Wallis test were used for statistical analysis. Hepatomegaly and splenomegaly were analyzed using the Kappa test. Results: There was a significant increase in hemoglobin levels (p-value <0.01) and platelet counts (p-value = 0.01) within two years of therapy. At the same time, the frequencies of splenomegaly (p-value <0.01) and hepatomegaly (p-value <0.05) reduced. These results were similar at five and ten years of enzyme replacement therapy. Conclusions: There are substantial and quick (within two years) laboratory and clinical responses to enzyme replacement therapy. These improvements continue as long as enzyme replacement therapy is administered every two weeks, as recommended by the literature...

Autoanticorpos contra antígenos celulares e sua correlação com o genótipo viral em pacientes com infecção pelo vírus da hepatite C (HCV) / Autoantibodies against cellular antigens and its relationship to virus genotype in hepatitis C virus (HCV) infected subjects

Objetivo: estabelecer relação entre a presença de fator anti-núcleo em células HEp-2 (FAN HEp-2) e o genótipo viral empacientes portadores de infecção pelo vírus da hepatite C (HCV). Método: estudo transversal analítico de prontuários de51 pacientes, com anti-HCV positivos, atendidos no Núcleo de Medicina Tropical/Universidade Federal do Pará e grupocontrole constituído de doadores de sangue. Foi realizada sorologia para anti-HCV, Reação de Polimerase em Cadeiaem Tempo Real RPCTR, genotipagem e dosagens bioquímicas e pesquisa de FAN HEp-2. Os dados foram submetidos atestes estatísticos com auxílio do Programa BioEstat 5.0, sendo aceito como significante o valor de p < 0,05. Resultados:dos 51 pacientes portadores de HCV, 28.9% (13) apresentaram FAN positivo, 88.2.% (45) com genótipo tipo 1 e 11,8%(6) com genótipo tipo 3. Os pacientes que se apresentaram com anticorpos detectáveis não apresentaram níveis deAST, ALT, AST/ALT, ?-GT e fosfatase alcalina, significativamente, diferente daqueles com auto-anticorpos negativos.Conclusão: o estudo demonstra que os auto-anticorpos presentes na amostra possuem padrão citoplasmático e estãorelacionados ao genótipo tipo1

Objective: This paper aims to establish a relationship between anti-nuclear fator (HEp-2) and virus genotypes in HepatitisC virus infected sunjects. Methods: This is a single-center, transversal analytical study that enrolled 51 out-patients atthe Núcleo de Medicina Tropical (Tropical Medicine Center) of the Universidade Federal do Para (Federal Universityof Para) in Brazil, compared to a control group of blood donors. HCV serology, real-time polymerase chain reaction,genotyping, biochemistry and anti-nuclear fator (HEp-2) analysis were carried out for each patient. Statiscal analysiswere made in Bioestat 5.0 software, considering ? ? 0,05 and power of 80%. Results: Out of 51 HCV subjects, 28.9%(13) also had anti-nuclear factor (HEp-2), 88.2.% (45) HCV type 1 and 11,8% (6) HCV type 3. There was no significantdifference in aspartate transaminase (AST), alanine transaminase (ALT), AST/ALT ratio, gamma glutamyl transpeptidase(GGT) e alcaline phosphatase (ALP) serum levels between patients presenting with anti-nuclear factor (HEp-2) and theones without it. Conclusion: Autoantibodies present in this sample showed a cytoplasmic pattern and were related toHCV type 1.

Idade materna como fator de risco para o baixo peso ao nascer: estudo de meta-análise / Maternal age as a risk factor for low weight of children at birth: a meta-analysis study

Introdução: o nascimento de crianças com baixo peso é provavelmente o fator mais importante que afeta a morbidade na infância, a mortalidade neonatal e pós-natal, tendo a idade materna como um de seus fatores predisponentes. Objetivo: estudar a importância da idade materna como fator de risco sobre o nascimento de crianças com baixo peso. Método: com a utilização da meta-análise, foram selecionados três estudos retrospectivos e randomizados para associar, estatisticamente, os seus resultados. O risco relativo (RR), a odds ratio (OR) e a significância estatística (valor de p) foram calculados de acordo com o método de Mantel-Haenszel, utilizando-se o programa Epi Info, versão 6.04c. Resultados: os resultados mostraram a influência da idade materna no BPN, com RR de 1,29, OR 1,36 e p=0,00000004, para as mães na faixa etária <20 anos em relação aos filhos de mães de 20 a 34 anos; e com RR de 1,27; OR de 1,33 e p=0,0007 para as mães na faixa etária > 34 anos em relação as de 20 a 34 anos de idade. Conclusão: os resultados dessa meta-análise mostraram que a idade materna < 20 anos e > 34 anos de idade se constitui em fator de risco para o nascimento de crianças com baixo peso