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Bone Abstracts (2017) 6 LB11 | DOI: 10.1530/boneabs.6.LB11

1Hirabai Cowasji Jehangir Medical Research Institute, Pune, Maharashtra, India; 2Royal Manchester Children’s Hospital, Manchester, UK; 3Yashoda Hematology Clinic, Pune, Maharashtra, India.

Objective: Expansion of bone marrow, accumulation of iron, growth failure and delayed puberty affect bone health in thalassemia; data on bone density and geometry are scarce. Our objective was to assess bone density (by Dual Energy X-ray Absorptiometry, DXA) and geometry (by peripheral quantitative computed tomography, pQCT) in children with thalassemia major.

Methods: Children with thalassemia were recruited from a hematology clinic in Pune (India) (Mean age 9.8±3.0y). History, anthropometry, haemoglobin concentrations, serum ferritin, and lumbar spine and total body bone density (GE Lunar iDXA, Madison, WI) were performed in 71 patients (boys: 38, age 5–18 years). Puberty (tanner staging) was classified as pre-pubertal (tanner stage=1) and entered puberty (tanner stage≥2). pQCT (STRATEC XCT-2000) of the radius of non-dominant hand at 4% and 66% were performed (n=21), z-scores were computed (manufacturers data).

Results: Mean height and weight Z-scores were −1.9±1.1, −1.6±1.0 respectively. Mean haemoglobin was 7.0±1.6g/dl and ferritin was 3005.3±2183.4 ng/ml (range: 14–17.5g/dl, 11–307 ng/ml respectively) suggesting very poor chelation. Of the 71 patients, 18(26%) had history of low-impact fractures. LS BMAD and TBLH BMD Z-scores (Crabtree,2016) were −1.6±2.3 (40% < −2) and −1.6±1.4 (34% < −2) respectively. Patients with fractures had significantly (p<0.05) lower TBLH BMD than patients without fractures (−2.3±1.4, −1.3±1.4 respectively).

At 4% site the mean distal radial trabecular density for age Z-scores were 1.8±1.5 (none below −2), total density for age Z-score was 0.3±1.4 (10% below −2). The cortical density for age and strength strain index (SSIPol3) for age Z-scores at 66% radial site were −0.8±1.4 (21% below −2) and −1.7±0.7 (37% below −2) respectively. Mean cortical thickness at 66% radius was 1.3±0.3 mm, the mean periosteal and endosteal thickness was 34.6±3.0 mm (pre-pubertal: 33.8±2.7 mm, entered puberty: 35.7±3.3 mm, NS) and 26.6±3.3 mm (pre-pubertal: 26.4±3.8 mm, entered puberty: 26.8±2.8 mm, NS) respectively.

Conclusion: In poorly controlled short Indian thalassemia major patients, bone health was affected as judged by history of fractures, low lumbar spine and total body bone density, cortical density, strength strain index and poor bone accrual during puberty.

Disclosure: The authors declared no competing interests.

Volume 6

8th International Conference on Children's Bone Health


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