RESUMO
Background: Immune tolerance induction (ITI) aims to eradicate anti-factor VIII (FVIII) antibodies (inhibitors) in persons with hemophilia A. However, this burdensome treatment fails in 10% to 40%. To estimate the chance of ITI success in clinical decision making, it is important to identify the predictors of ITI success. Objectives: We performed a systematic review and meta-analysis to summarize the current evidence on determinants of ITI outcome in persons with hemophilia A. Methods: A literature search was conducted to identify randomized controlled trials, cohort, or case-control studies reporting on the predictors for ITI outcome in persons with hemophilia A. The main outcome was ITI success. Methodological quality was assessed using an adapted Joanna Briggs Institute checklist, rating as high if ≥11 of 13 criteria were met. Pooled odds ratios (ORs) for ITI success were calculated for each determinant. ITI success was defined as negative inhibitor titer (<0.6 BU/mL), FVIII recovery ≥66% of expected, and FVIII half-life ≥6 hours in 16 (59.3%) studies. Results: We included 27 studies, involving 1,734 participants. Methodological quality of 6 (22.2%) studies (418 participants) was rated as high. Twenty different determinants were assessed. Historical peak titer ≤100 BU/mL (compared with >100 BU/mL, OR, 1.7; 95% CI, 1.4-2.1), pre-ITI titer ≤10 BU/mL (compared with >10 BU/mL, OR, 1.8; 95% CI, 1.4-2.3), and peak titer during ITI ≤100 BU/mL (compared with >100 BU/mL, OR, 2.7; 95% CI, 1.9-3.8) were associated with a higher chance of ITI success. Conclusion: Our results suggest that determinants related to the inhibitor titer are associated with ITI success.
Assuntos
Hemofilia A , Hemofilia B , Humanos , Hemofilia A/epidemiologia , Prevalência , Hemofilia B/epidemiologiaAssuntos
Alelos , Inibidores dos Fatores de Coagulação Sanguínea/imunologia , Hemofilia A/genética , Hemofilia A/imunologia , Isoanticorpos/imunologia , Lactase/genética , Polimorfismo de Nucleotídeo Único , Brasil/epidemiologia , Frequência do Gene , Genótipo , Hemofilia A/tratamento farmacológico , Hemofilia A/epidemiologia , Humanos , Índice de Gravidade de DoençaRESUMO
The corneomandibular reflex was first described in 1902 by German doctor Von Sölder, who reported the presence of the reflex in patients and in the general population. For him, the corneomandibular reflex represented an exaggeration of the normal response. This was one of the more than 250 reflexes described in the beginning of the 20th century, during the so called "race of the reflexes".
O reflexo córneo-mandibular foi descrito pela primeira vez em 1902 pelo alemão Von Sölder, que relatou a presença de reflexos em pacientes e indivíduos normais. Segundo ele, o reflexo córneo-mandibular representa um exagero da resposta convencional. Este é um dos mais de 250 reflexos descritos no início do século XX, durante a chamada "corrida dos reflexos".
Assuntos
Humanos , Reflexo , Córnea , Mandíbula , HistóriaRESUMO
Fanconi anaemia (FA) is a rare genetic chromosomal instability syndrome caused by impairment of DNA repair and reactive oxygen species (ROS) imbalance. This disease is also related to bone marrow failure and cancer. Treatment of these complications with radiation and alkylating agents may enhance chromosomal breakage. We have evaluated the effect of amifostine (AMF) on basal and mitomycin C (MMC)-induced chromosomal breakage in FA blood cells using the micronucleus assay. The basal micronuclei count was higher among FA patients than healthy subjects. Pre-treatment with AMF significantly inhibited micronucleation induced by MMC in healthy subjects (23.4 +/- 4.0 - MMC vs 12.3 2.9 - AMF --> MMC) MN/1000CB, p < 0.01, one way ANOVA) as well as in FA patients (80.0 +/- 5.8 - MMC vs 40.1 +/- 5.8 - AMF --> MMC) MN/1000CB, p < 0.01, ANOVA). Release of ROS by peripheral blood mononuclear cells treated with AMF -> MMC and measured by chemoluminometry showed that AMF-protection was statistically higher among FA patients than in healthy individuals. Based on these results we suggest that AMF prevents chromosomal breakage induced by MMC, probably by its antioxidant effect.
Assuntos
Amifostina/farmacologia , Quebra Cromossômica/efeitos dos fármacos , Anemia de Fanconi/tratamento farmacológico , Linfócitos/efeitos dos fármacos , Mitomicina/efeitos adversos , Adolescente , Adulto , Células Sanguíneas , Estudos de Casos e Controles , Criança , Anemia de Fanconi/genética , Feminino , Humanos , Masculino , Micronúcleos com Defeito Cromossômico/efeitos dos fármacos , Espécies Reativas de Oxigênio/metabolismoRESUMO
Os ambientes frigoríficos expõem os trabalhadores a esforços repetitivos, posturas etereotipadas e desconforto térmico. Esses riscos estão associados a problemas musculoesqueléticos devido às respostas dos tecidos moles ao excesso de solicitação das estruturas dos membros superiores, e ainda, aos acidentes de trabalho causados pelas diminuição da destreza, que tem como origem, provavelmente, a isquemia provocada pela queda da temperatura do tecido musculoesquelético. O resfriamento de todo corpo ou de parte dele resulta em desconforto, distúrbio da sensibilidade e da função neuromuscular, por último, injúria por frio. A prevenção do resfriamento através de uso de roupas protetoras, sapatos, luvas e capacetes ou gorros interfere na mobilidade e na destreza do trabalhador. Há um" custo de proteção", de modo que os movimentos se tornam restritos e exaustivos. Deve-se enfatizar a tríade: proteção, formação e ergonomia
