Epidemiology of Hereditary Coagulation Bleeding Disorders: A 15-Year Experience From Southern Iran

Background: Data on the frequency of hereditary bleeding disorders (HBDs) and associated mortality and morbidities during a long-term follow-up from Iran are scarce. Objective: This study evaluated the epidemiologic features among patients with HBD in one of the largest referral centers in southern Iran. Methods: In this cross-sectional study, 619 patients with HBD were evaluated during the period 1996 to 2011. Aside from baseline characteristics and type of factor deficiency, associated morbidities including viral infections, neurological disorders, asthma, thalassemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency, diabetes, hypertension, cardiac and renal diseases were evaluated. Furthermore, among patients who died, the underlying disease and etiology of death were also evaluated. Results: Patients’ mean age was 24.4 ± 13.5 years. Factor VIII deficiency was the most prevalent type (50.4%) of HBD, and combined Von–Willebrand and factor XIII deficiency (2.3%) was the most prevalent type of combined factor deficiency. A total of 0.5% had hepatitis B and 11.5% had hepatitis C. Cardiac disease was seen in 1.5%, hypertension in 0.2%, renal disease in 0.2%, and diabetes in 1.3% of patients. Overall, 5.2% had intracranial hemorrhage, 2.1% had epilepsy, and 0.8% had mental retardation. During the 15-year follow-up, 22 patients died; car accident was the leading cause of death in this population. Conclusion: Associated morbidities were seen in 24.3% of patients with HBD. Most prevalent morbidities were HCV infections (11.5%) and neurological disease (7.3%). The mortality rate among patients with HBD was 3.4%, and the most common cause of death was accident, which is similar to that of normal Iranian populations

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire

CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (β-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with β-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with β-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with β-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state