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.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Databases, Factual ; Factor IX/therapeutic use ; Factor VIII/therapeutic use ; Hemophilia A/*drug therapy/*epidemiology/ethnology ; Hemophilia B/*drug therapy/*epidemiology/ethnology ; Humans ; Incidence ; Infant ; Male ; Middle Aged ; Prevalence ; Registries ; Retrospective Studies ; Taiwan/epidemiology ; Young Adult

Background/Aims: Laryngeal symptoms are largely treated with empiric proton pump inhibitor (PPI) therapy if no apparent pathology shown on ear, nose, and throat evaluation and reflux-related etiologies are suspected. However, treatment response remains unsatisfactory. This study aimed to investigate the clinical and physiological characteristics of patients with PPI-refractory laryngeal symptoms. Methods: Patients with persistent laryngeal symptoms despite PPI treatment for ≥ 8 weeks were recruited. A multidisciplinary evaluationcomprising validated questionnaires for laryngeal symptoms (reflux symptom index [RSI]), gastroesophageal reflux disease symptoms, psychological comorbidity (5-item brief symptom rating scale [BSRS-5]) and sleep disturbance (Pittsburgh sleep quality index [PSQI]), esophagogastroduodenoscopy, ambulatory impedance-pH monitoring, and high-resolution impedance manometry were performed.Healthy asymptomatic individuals were also recruited for comparison of psychological morbidity and sleep disturbances. Results: Ninety-seven adult patients and 48 healthy volunteers were analyzed. The patients had markedly higher prevalence of psychological distress (52.6% vs 2.1%, P < 0.001) and sleep disturbance (82.5% vs 37.5%, P < 0.001) than the healthy volunteers. There were significant correlations between RSI and BSRS-5 scores, and between RSI and PSQI scores (r = 0.26, P = 0.010, and r = 0.29, P = 0.004, respectively). Fifty-eight patients had concurrent gastroesophageal reflux disease symptoms. They had more prominent sleep disturbances (89.7% vs 71.8%, P < 0.001) than those with laryngeal symptoms alone but similar reflux profiles and esophageal motility. Conclusions PPI-refractory laryngeal symptoms are mostly associated with psychological comorbidities and sleep disturbances. Recognition of these psychosocial comorbidities may help optimize management in these patients.

BACKGROUNDThe change of anaerobic exercise abilities during and after a high-altitude expedition or hypoxic exposure is not well studied. To evaluate the effects of an extreme-altitude expedition on anaerobic performance, the 10-second supramaximal test and endocrine hormones were evaluated before and after an expedition to Peak Lenin.

METHODSFour subjects (3 male and 1 female, age (30.5 +/- 16.5) years) were recruited into the study. Three sets of tests were performed, including a basic test at sea level and 20 days before first arrival at the base camp (3600 m), a middle test done at day after returning from the summit to the base camp and the post test at the 10th day after return to the sea level. Both the supramaximal test, performed by a cycle ergometer, and body composition, performed by bioelectrical impedance analysis, were completed before the basic test and post test. The endocrine hormones including cortisol, growth hormone, testosterone, noradrenaline, adrenaline, dopamine, glucagon and beta-endorphin were measured at all tests.

RESULTSComparing the conditions before and after the expedition, the body measurement parameters were decreased after the expedition, i.e., body weight (-4.22%, P < 0.05), fat-free mass (-2.09%, P < 0.01) and body fat (-8.95%, P = 0.172). The peak power relative/body weight ratio (PP/BW) was similar ((9.70 +/- 1.97) vs (9.11 +/- 1.80) W/kg, P = 0.093), while mean power/body weight ratio (MP/BW) was reduced significantly after the expedition ((9.14 +/- 1.77) vs (8.33 +/- 1.74) W/kg, P < 0.05). Peak power/fat-free mass (PP/FFM), mean power/fat-free mass (MP/FFM) and fatigue index (FI) were significantly lower after the expedition (PP/FFM: (11.95 +/- 1.71) vs (10.99 +/- 1.59) W/kg, P < 0.05; MP/FFM: (11.26 +/- 1.50) vs (10.04 +/- 1.55) W/kg, P < 0.005; FI (85.55 +/- 4.17)% vs (77.25 +/- 4.40)%, P < 0.05). Hormone assays showed a significant increase of noradrenaline (basic vs middle, P < 0.05) as well as decrease of adrenaline (P < 0.05). Meanwhile, a trend towards an increase in dopamine (basic vs middle) and a decrease of beta-endorphin (basic vs post) were also noted.

CONCLUSIONSThese results suggested that an expedition to an extreme altitude may have negative effects on anaerobic performance. It showed that a significant increase of noradrenaline (basic vs middle) as well as decrease of adrenaline after the expedition to Peak Lenin had occurred. The real physiological significance needs to be further investigated.

Adaptation, Physiological ; physiology ; Adolescent ; Adult ; Altitude ; Anaerobic Threshold ; physiology ; Dopamine ; blood ; Epinephrine ; blood ; Exercise Test ; Female ; Glucagon ; blood ; Growth Hormone ; blood ; Humans ; Hydrocortisone ; blood ; Male ; Middle Aged ; Norepinephrine ; blood ; Testosterone ; blood ; Young Adult ; beta-Endorphin ; blood