Background: Thalassemia major is a hemoglobin disorder characterized by regular transfusion requirement. Despite the regular transfusions and advanced iron chelation protocols, endocrine complications have been reported as the frequent morbidities of the disease.
Objective and hypotheses: The aim of the study was to i) investigate the prevalence of endocrine complications, ii) to examine the relationship between endocrine complications and metabolic parameters, iii) to investigate vitamin D status and bone mineral density in these patients.
Method: Clinical data of 85 thalassemia major patients (46 females, 38 males) with a mean age 19.35±9.5 years (3.4853.90 years) were evaluated from the thalassemia clinic in a single centre, in Turkey. Height and weight measurement, pre-transfusion haemoglobin, serum ferritin, calcium, phosphorus, alkaline phosphatase, free thyroxine, TSH and vitamin D concentration were examined. Age, gender, disease history, drug usage, chelation protocol, bone mineral density data were recorded from case files.
Results: Mean ferritin was 1991.14±1789 ng/ml, mean haemoglobin was 8.84±0.71. 73 (86.9%) of the patients were on chelation therapy with deferasirox, remaining were on deferiprone and/or deferoxamine treatment. Mean serum vitamin D concentration was 16.45 ng/dl, BMD was 0.85±0.14 g/cm2. Lumbar spine corrected z-score was 0.44±1.08 in children, T-score was −2.05±1.11 in adults. There was a negative correlation between BMD and vitamin D, ALP levels (r=−0.261, P=0.04, r=−0.48, P<0.01 respectively). Three patients had diabetes and were on insulin therapy, three had impaired glucose tolerance, seven had hypogonadism, three had hypothyroidism. Among 84 patients, 45 (53%) were in pediatric age group (≤18 years old), 16 out of 45 (35%) patients have short stature (height SDS ≤−2).One patient was on somatropine therapy.
Conclusion: Most frequent complication was vitamin D deficiency and osteopenia/osteoporosis in our cohort. We want to highlight the importance of vitamin D replacement and early prevention of osteoporosis in thalassemia patients.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology