The Identification of osteopenia and osteoporotic changes in pediatric thalassemia patients at thalassemia center, Misan city, Iraq; An intervention study

Abstract

Background: Thalassemia syndrome is a heterogeneous group of hereditary disorders affecting the synthesis of globin chains of adult hemoglobin's, leading to ineffective erythropoiesis, hemolysis, and moderate to severe anemia. The study aimed to identify the incidence of osteopenia and osteoporosis and its relation to some of the probable risk factors as age, gender, genetic and family history, diet and environmental that could be influencing bone mineral density among thalassemia patients and to show the results of interventional chelating therapy on bone mineral density (BMD) before and after administer at thalassemia center in Misan city, Iraq.

Methods: A cross-section interventional study, applied through a period from September 2020 to April 2021, enrolled 50 thalassemia patients attended Thalassemia Center, Misan, Iraq. Twenty-four patients were males and 26 were females. The information regarding patient characteristics (Age, gender, age at diagnosis, family members and\or relative with thalassemia, and patients treated with chelating therapy or not) were collected. They were scanned for BMD at lumbar spine with dual-energy X-ray absorptiometry (DEXA). Blood samples collected for the estimation of serum ferritin, vitamin D3 and Hb levels. Deferoxamine (Desferal), Deferasirox (Exjade), and Deferiprone (L1) are used as iron chelating therapy.

Results: Ninety-percent of cases were B-thalassemia major type (B-THM), while 5 patients (10%) had B-thalassemia intermedia (THI). Fifty-two percent were female, and (48%) were male. About 38(76%) patients were aged between (10-14 years), and 12(24%) were between (>14-18 years). Majority of cases had age at first diagnosis  were below five-year age. Most patients 23(46%) had Hb level of (<7 g/dl). Most patients 32(64%) had ferritin level between (1000-5000) ng/dl. Low vitamin D3 level found in 40(80%) of patients. Osteopenia was higher in number and severity among THM patients than THI patients 35(70%) vs. 4(8%) respectively, as well as osteoporosis found in 9(18%) in THM patients, while none in THI patients with no significant difference  (P=0.1). Insignificantly differences had found between all age groups among types of TH. Osteopenia among female was insignificantly more than male 21(42%), and 18(36%), respectively, as well as osteoporosis 5(10%), and 4(8%). In relation to the age at first diagnosis of thalassemia, serum vitamin D3 level, Hb level, serum ferritin level, family history of thalassemia, relative members with history of thalassemia had insignificant difference on BMD. Before therapy and after therapy relation, osteopenia was 39(78%) decreased to 29(58%), while osteoporosis before was 9(18%) increased to 16(32%). In relation to Z-score change (before and after therapy) among age groups, in both groups the number of osteopenia cases reduced, while increased of osteoporosis, besides, increased severity with significant difference (P=0.02). Among gender, and age of diagnosis, all categories showed insignificantly difference in BMD and severity.

Conclusions: A predominance of osteopenia over osteoporosis in spite of therapy, mainly in thalassemia major. The severity of bone density reduction correlates insignificantly with  younger age at diagnosis, deficiency of vitamin D3, lower Hb level, high S. ferritin and significantly correlates with increasing age.