Bone mineral density in thalassemia major patients from antalya, Turkey.

Aim. We assessed the bone mineral density and related parameters in nine adults, thirty-eight pubertal, prepubertal totally forty-seven patients with thalassemia major living in Antalya, Turkey. Materials and Methods. We measured height and pubertal staging in last five years by six-month intervals. Average ferritin and hemoglobin concentrations were calculated for last three years. The levels of hydroxyproline, calcium, phosphorus, and creatinine were measured in 24 h urine, and those of parathormone, IGF 1, osteocalcine, alkaline phosphatase, calcium, ionized calcium, magnesium, phosphorus, creatine, blood glucose, thyroid stimulating hormone, alanine transaminase, and aspartate transaminase were determined in serum, and also the bone mineral density was measured. Results. The average L1-L4 bone mass density was 27.1 ± 10.1 g cm(-2); the average bone mineral content was 0.65 ± 0.11 g. of the patients with a Z-score under 2.5. A moderate relationship was found between the bone mass density age and height. Subjects in low pubertal staging and short stature (<3% percentile) have significantly lower bone mass densities P < 0.001. Conclusion. he prevalence of osteoporosis is high in patients with thalassemia major, possibly related to delayed puberty.

Different desferrioxamine usage in the patients with thalassemia major: a cost-effect analysis.

Regular desferrioxamine (DFO) usage in patients with thalassemia major (TM) ameliorates hepatic, cardiac and endocrine dysfunction, improves growth and sexual maturation and prolongs survival. The difficulties of administering DFO with classic pumps are well known. The aim of this study was to compare the iron accumulation and cost effects between the continuous 48 hours infusion of DFO with infusor pump and the intermittent 40 hours infusion with classic pump in patients with TM. A total of 54 patients with TM were divided to two groups, first group includes 27 patients (18 female, 9 male) aged between 5.5 and 20.5 years, and were infused a total of 100 mg/kg DFO with infusor in 48 hours. Second group includes 27 patients (18 female, 9 male) aged between 6 and 22 years, were infused a total of 200 mg/kg DFO in 4 days with an intermittent infusions for about in 40 hours. After one year of treatment, the patients were compared from a clinical view point and cost of medical treatment. No statistical difference was found between infusor pump and classic pump in terms of cost-effectiveness.

Psychosocial burden of beta-thalassaemia major in Antalya, south Turkey.

beta-thalassaemia is a recessively inherited blood disorder characterised by chronic anaemia. It requires monthly blood transfusions and regular iron chelation. Thousands of affected children are born annually and the magnitude of the problem is most severe in developing countries. Ninety-nine children and 32 adults with thalassaemia major, and 112 parents of patients were interviewed in Antalya, south Turkey, using specifically designed questionnaires to evaluate psychosocial burden. The education of most of the thalassaemic children of school age (60%) was affected, mainly due to having to attend hospital for investigation and transfusions. A high level of parental anxiety (82%) was reported. Nearly half of the families (47%) had employment and financial problems as a result of thalassaemia, yet there was a low level of marital breakdown (1.8%). A substantial majority (93%) of the parental couples would have chosen to terminate an affected pregnancy if they had known that the foetus had thalassaemia major. The results reflect the need for a national policy for public education and screening of thalassaemia in Turkey in order to offer prenatal diagnosis for all families at risk of homozygous thalassaemia.