Acquired hemophilia A is a rare but severe autoimmune bleeding disorder. It is more frequent in the elderly and results from the presence of autoantibodies directed against clotting factor VIII. In this review, we briefly report on the present state of knowledge regarding acquired hemophilia A, analyzing its epidemiology, pathogenesis, diagnostic, and clinical features. We also describe the main characteristics of this disorder according to its association with different conditions and the most important advances in the treatment of bleeding episodes and the eradication of the autoantibody. Am.
This recombinant concentrate is efficacious in FVII-deficient patients. It is safe since any risk of transmission of blood-borne viruses is eliminated.
Background and Objectives: Factor VII (FVII) deficiency is a rare coagulation disorder, historically treated with prothrombin complex concentrates or plasma–derived FVII concentrates. We treated such patients (n = 17) with a recombinant, activated FVII preparation. Materials and Methods: Twenty–seven spontaneous bleeding episodes were treated and 7 major and 13 minor surgical interventions were carried out. The dosages employed ranged from 8.08 to 70.5 Ìg/kg body weight. Results: A mean dose between 22 and 26 Ìg/kg was sufficient to normalise the prothrombin time. Fifteen haemarthroses were treated with single doses and results were excellent in 13 cases. In 5/6 bleeding episodes of other types, the treatment gave either excellent or at least effective results. Haemostasis was secured in the 7 major and 13 minor surgical interventions. One patient developed antibodies 4–5 weeks after an extremely high dose. Otherwise, there were no side effects and no evidence of a thrombotic tendency. Conclusion: This recombinant concentrate is efficacious in FVII–deficient patients. It is safe since any risk of transmission of blood–borne viruses is eliminated.
This cross-sectional study included postmenopausal women, aged 45-75 years, with the aim to assess the presence of vulvovaginal atrophy (VVA) confirmed by a clinical assessment in the Italian population attending menopausal/gynecological centers. Apart from baseline variables, women scored vaginal, vulvar and urinary VVA symptoms. Impact of VVA on sexual function and quality of life (QoL) was assessed thorough EuroQoL questionnaire (EQ5D3L), Day-to-Day Impact of Vaginal Aging (DIVA), Female Sexual Function Index (FSFI) and Female Sexual Distress Scale-revised (FSDS-R). A physical examination was carried out in accordance with routine gynecological practice. VVA was confirmed in 90% of the 1226 evaluable patients (aged 59.0 ± 7.3 years). The prevalence of postmenopausal women with VVA confirmed by gynecological clinical assessment was 75.3%. The patients with VVA confirmed (n ¼ 926) had more severe symptoms (p < .0005), lower QoL (EQ-visual analog scale, p ¼ .008 and DIVA, p < .0005) and worsened sexual function (FSFI and FSDS-R, p < .0005 for both) when compared with the patients having nonconfirmed VVA (n ¼ 140). VVA is highly prevalent among postmenopausal Italian women. The objective of VVA confirmation is associated with severe symptoms and impaired QoL and sexual function. A proactive approach of Italian clinicians to promote regular and early gynecological evaluation should be performed in order to delay the advancing of the disorder.
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