Valizadeh N, Farrokhi F, Alinejad V, Said Mardani Sm, Valizadeh N, Hejazi S, Noroozi M
Assistant professor of Hematology/Medical Oncology, Urmia University of Medical Sciences, Urmia, Iran.
Medical Student, Urmia University of Medical Sciences, Urmia, Iran.
Iran J Ped Hematol Oncol. 2014;4(2):68-71. Epub 2014 Apr 20.
Patients with thalassemia major and intermedia are susceptible to osteopenia and osteoporosis. The mechanism of osteoporosis in these patients is multifactorial. Transfusion related iron overload in endocrine organs leads to impaired growth hormone secretion, diabetes mellitus, hypothyroidism, hypoparathyroidism, lack of sex steroids and vitamin D deficiency that contribute to impairment in achieving an adequate bone mass .The aim of this study was assessment of frequency of bone loss in patients with thalassemia major and intermedia in Urmia City of West Azerbaijan, Iran.
In this cross sectional descriptive study,10 patients (lower than 18 y/o)with transfusion dependent thalassemia attending to Motahari and Emam Khomeini hospitals in Urmia city of Iran were enrolled and scanned for Bone Mineral Density (BMD) starting at around 10 years old.
Tenatients (6 male and 4 female) with transfusion dependent thalassemia (β-thalassemia major and intermedia) aged 13to 17 years in Urmia city of Iran were enrolled. Mean age of patients was 15.1±.37year old. Among them, 8 patients (80%)had low BMD and2 of them (20%) had normal BMD in lumbar spine. Only 30% of patients had low BMD in the neck of femur.
We should perform annual BMD in patients with thalassemia major and intermedia and hemoglobin H disease in age of higher than 8 year old and treat low BMD with administration of bisphosphonate, calcium and vitamin D supplements. Medical consultation with a rheumatologist and /or an endocrinologist should be performed in these patients. Changing lifestyle with mild daily exercise, adequate calcium containing foods, avoiding heavy activities, stop smoking, iron chelation therapy in adequate dosage, early diagnosis and treatment of endocrine insufficiency and regular blood transfusions can help to achieve an optimal bone density in these patients.
重型和中间型地中海贫血患者易患骨质减少和骨质疏松症。这些患者骨质疏松的机制是多因素的。内分泌器官中与输血相关的铁过载会导致生长激素分泌受损、糖尿病、甲状腺功能减退、甲状旁腺功能减退、性类固醇缺乏和维生素D缺乏，这些都会导致骨量不足。本研究的目的是评估伊朗东阿塞拜疆省乌尔米耶市重型和中间型地中海贫血患者的骨质流失频率。
在这项横断面描述性研究中，纳入了10例（年龄小于18岁）依赖输血的地中海贫血患者，这些患者在伊朗乌尔米耶市的莫塔哈里医院和伊玛目霍梅尼医院就诊，并从10岁左右开始进行骨密度（BMD）扫描。
纳入了伊朗乌尔米耶市10例年龄在13至17岁之间依赖输血的地中海贫血（重型β地中海贫血和中间型β地中海贫血）患者（6例男性和4例女性）。患者的平均年龄为15.1±0.37岁。其中，8例（80%）患者腰椎骨密度低，2例（20%）患者腰椎骨密度正常。仅30%的患者股骨颈骨密度低。
我们应对年龄大于8岁的重型和中间型地中海贫血患者以及血红蛋白H病患者每年进行骨密度检查，并用双膦酸盐、钙和维生素D补充剂治疗骨密度低的患者。这些患者应咨询风湿病学家和/或内分泌学家。通过适度的日常锻炼、摄入富含钙的食物、避免剧烈活动、戒烟、适当剂量的铁螯合治疗、早期诊断和治疗内分泌功能不全以及定期输血来改变生活方式，有助于这些患者达到最佳骨密度。
