The aim of this study is to describe the health status, health care received, and their impact on the quality of life in patients with hemophilia. Patients with severe factor VIII or IX deficiency without inhibitors or other chronic disease were enrolled. Turkish version of the Hemophilia-Specific Quality of Life Index (Haemo-QoL) questionnaire was administered to the pediatric patients aged 4 to 16 years and Haem-A-QoL to the adult patients. Joints were evaluated according to the World Federation of Hemophilia (WFH) orthopedic joint scores.Thirty-nine children/adolescents and 31 adult patients were enrolled. Mean Haemo-QoL scores were 39.6 +/- 15.0 for the children and mean Haem-A-QoL 47.4 +/- 14.1 for the adult patients, respectively. Internal consistency reliability was generally sufficient. Total Cronbach's alpha coefficient was >.70 (range .77-.96) in all the age groups. Mean total WFH orthopedic joint scores were 1.83 +/- 2.7, 4.9 +/- 4.96, and 6.94 +/- 6.15 in 4-7, 8-12, and 13-16-year-old groups, respectively. They were more impaired in the adult patients (16.23 +/-14.12). These results show that the Turkish version of the Haemo-QoL and Haem-A-QoL are reliable instruments to measure the quality of life in the pediatric and adult patients with severe hemophilia. When compared to the Haemo-QoL scores of an international multicenter West European study of children, quality of life in the Turkish patients were more impaired in the subscales of physical health, feeling, view, school and sport, and treatment as well as more impaired WFH joint scores. The authors recommend primary factor prophylaxis and encouraging the patients to learn home treatment to improve joint scores and quality of life.

The incidence of sevoflurane-induced emergence agitation was significantly lower in children premedicated with a midazolam and hydroxyzine combination compared to those premedicated with midazolam only. Furthermore, the midazolam and hydroxyzine combination provided better premedication quality than midazolam alone.

The diameter of the IJV in pediatric patients, especially infants, is often smaller than the diameter of the J-tip guidewire curve. We speculate that this may lead to impeded guidewires and failed cannulation. It must also be kept in mind that the Trendelenberg position might not facilitate IJV cannulation in children <2 years of age.

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