The investigation of gingival iron accumulation in thalassemia major patients.

BACKGROUND: Thalassemia major (TM) is an autosomal-recessive genetic blood disorder. Regular blood transfusions to improve chronic anemia caused by ineffective erythropoiesis and hemolysis lead to iron overload in many organs in TM patients. The aim of this study was to investigate the periodontal status and the iron accumulation in gingival tissues of TM patients and assess whether iron deposition in gingival biopsies could be an alternative method for the diagnosis of body iron overload in TM patients. MATERIALS AND METHODS: This study was conducted on 22 TM patients and 20 healthy matched controls. Plaque index, gingival index, and probing pocket depth were measured and gingival biopsies were obtained in all subjects. Venous blood samplings and liver biopsies were carried out only in patients with thalassemia. Gingiva and liver tissue samples were evaluated histopathologically for inflammation, iron accumulation, and fibrosis. RESULTS: There was no difference between the groups regarding periodontal health, and all patients had mild gingivitis. Gingival iron accumulation was observed only in the TM group. The iron accumulation was detected in the liver of all the patients with thalassemia. The gingival iron accumulation was correlated with neither serum ferritin levels nor hepatic iron accumulations. CONCLUSIONS: The periodontal tissues are affected by iron accumulation as well as hepatic, cardiac, and endocrine tissues in TM patients. Further studies investigating the usage of the gingival biopsy for prediagnosis of body iron overload in TM patients are needed.

Evaluation of angiogenesis with vascular endothelial growth factor in patients with thalassemia major.

BACKGROUND: Thalassemia major (TM) is an important cause of severe anemia that necessitates regular blood transfusion to prevent the profound weakness and cardiac decompensation caused by the anemia. However, iron overloading is an inevitable consequence of prolonged transfusion therapy. In addition, extramedullary hematopoiesis and hemosiderosis cause spleen, liver and marrow enlargement. In recent years the role of angiogenesis has been investigated in physiological and pathological conditions. However, it is known that angiogenetic factors, especially the vascular endothelial growth factor (VEGF), cause differentiation of the hemangioblast. METHODS: The effect of angiogenesis hasn't been investigated in TM patients yet, and in this study, angiogenesis was researched in 43 thalassemic patients by serum VEGF measurement. RESULTS: VEGF levels were not affected by hemoglobin levels, ferritin levels, or chelation type (P > 0.05). However, VEGF was positively affected by chelation starting age and negatively affected by yearly transfusion requirement of TM patients (P < 0.05). In addition, VEGF of patients who underwent splenectomy were higher than those who didn't undergo splenectomy (P < 0.05). CONCLUSION: Early chelating age will negatively influence the VEGF level, which increases angiogenesis, however, early starting transfusion age and regular blood transfusion will positively influence the VEGF level, which decreases angiogenesis in thalassemic patients.

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.

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.