Prevalence, Incidence, and Factor Concentrate Usage Trends of Hemophiliacs in Taiwan

PURPOSE: Hemophilia A and B (HA, HB) are the most common X-linked inherited bleeding disorders. The introduction of factor concentrates has allowed for control of the lifelong chronic disease. However, no studies have been published regarding the epidemiology of hemophilia in Taiwan. Our aim was to determine the prevalence, incidence, and mortality rate, as well as trends in the use of factor concentrates, in individuals with hemophilia in Taiwan. MATERIALS AND METHODS: A retrospective study was conducted using the National Health Insurance Research Database between 1997 and 2007. RESULTS: We identified 988 males with hemophilia (HA : HB ratio=5.4 : 1). The mean prevalence per 100000 males was 6.7+/-0.1 for HA and 1.2+/-0.1 for HB. The estimated mean annual incidence per live male birth was 1 in 10752 for HA and 1 in 47619 for HB. Standardized mortality ratios for males with hemophilia (all severities) or severe hemophilia were 1.3- and 2.1-fold higher than that of the general male population, respectively. Mean factor VIII (FVIII) and factor IX (FIX) usage was 1.5003+/-0.4029 and 0.3126+/-0.0904 international units (IUs) per capita, respectively. Mean FVIII and FIX usage per patient with hemophilia (all severities) or severe hemophilia was 44027+/-11532 and 72341+/-17298, respectively, and 49407+/-13015 and 74369+/-18411 IUs per person with HA or HB, respectively. CONCLUSION: Our data revealed epidemiologic and factor concentrate usage trends in males with hemophilia in Taiwan, highlighting a need for improvements in the mandatory National Health Insurance registry. A better-designed, patient-centered registry system would enable more detailed patient information collection and analysis, improving subsequent care.

Ligature of external carotid artery as an optional technique in a patient with von Willebrand disease

The von Willebrand disease (vWD) is a hereditary coagulopathy. There is no gender predilection. Clinically characterized by mucocutaneous bleeding, especially nose bleeding, menorrhagia and bleeding after trauma. This article reports a case of a 52-year-old Caucasian male patient with vWD, who presented with extensive bleeding in the tongue after a lacerating injury caused by accidental biting, and describes some clinical, pathological and treatment aspects of vWD. After repeated attempts to suture the wound and replace clotting factors, a decision was made to perform the ligature of the external carotid artery ipsilateral to the injury. There was favorable resolution of the case, with a good aspect of the scar 2 months after ligation. This case reinforces that it is extremely important to make a thorough review of medical history of all patients, searching for possible bleeding disorders or previous family history.

A doença de von Willebrand (DvW) é uma coagulopatia hereditária. Não há predileção por sexo. Clinicamente caracteriza-se por hemorragias mucocutâneas, sobretudo nasais, menorragias e hemorragias pós-trauma. Este artigo relata um caso clínico de DvW em paciente de 52 anos de idade, leucoderma, do sexo masculino, que apresentou extensa hemorragia em bordo lateral de língua após ferimento lacerante, além de descrever alguns aspectos clínicos, patológicos e terapêuticos da DvW. Após repetidas tentativas de sutura do ferimento e reposição dos fatores de coagulação, optou-se pela ligadura da artéria carótida externa ipsilateral ao ferimento, com resolução favorável do caso, notando-se bom aspecto cicatricial 2 meses após a ligadura. Este caso reforça que é de extrema importância a realização de anamnese criteriosa, buscando-se identificar possíveis distúrbios hemorrágicos prévios ou antecedentes familiares.

Management of hemophilia in developing countries.

Coagulation disorder are common in India. In the absence of any epidemiological studies it is expected that there are at least 50,000 severe hemophilics in our country. The factor concentrates are not easily available in developing countries which poses a major problem while managing severe bleeding episodes in these patients. Various strategies which could be useful while managing these cases in developing countries have been reviewed.

Terapia transfusional com plasma e derivados / Transfusion therapy with plasma and derivatives

Nesta revisäo säo analisadas as principais indicaçöes para o uso do plasma e seus derivados. Particularmente, säo focalizadas a utilizaçäo dos fatores de coagulaçäo no tratamento das hemofilias e da doença de von Willebrand, e o uso terapêutico da albumina e das imunoglobulinas

Management and surveillance of the hemophiliacs at the National Institute of Hematology and Blood Transfusion of Vietnam.

Surveillance and management 295 times of 29 hemophiliacs was carried out from 1985 to 1990. Among them, 113 were hospitalized, 103 with bleeding and 5 for prevention. Joint bleeding was the most frequent (46.96%), thereafter nose bleeding and bleeding from the gum, the mouth and mucous membrane (15.53%). Hemophiliacs having inhibitors to factor VIII were 13.79%. Fresh frozen plasma, cryoprecipitates, traditional medicine were used in the treatment. The effects of cryoprecipitates were shown to be better. The traditional medicine gave hope for prevention.