Management of beta‐thalassemia–associated osteoporosis

Giusti, Andrea; Pinto, Valeria; Forni, Gian Luca

doi:10.1111/nyas.13041

Cited by 35 publications

(34 citation statements)

References 61 publications

(121 reference statements)

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“…Subclinical microfractures, particularly throughout the vertebral column, are now recognized as an increasing problem in this population . Recently, atypical femoral fracture as a result of long‐term bisphosphonate use was observed for the first time in thalassemia …”

Section: Low Bone Massmentioning

confidence: 99%

The importance of nutrition for health in patients with transfusion‐dependent thalassemia

Fung

2016

Annals of the New York Academy of Sciences

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Nutrition is increasingly being seen by care providers as important for patients with thalassemia. However, the definition of optimal nutritional support and the means to provide that support remain elusive. This review focuses on the relationships between nutritional status and three common comorbidities in patients with thalassemia: low bone mass, growth deficiency, and diabetes. The association between low bone mass and specific nutrient deficiencies (e.g., vitamin D, zinc) is clear, while the role of nutritional adequacy for young patients with growth deficiencies requires careful analysis to distinguish it from chronic anemia and endocrine insufficiency. Correction of isolated nutritional deficiencies in small cohorts of patients with thalassemia has shown some promise at improving both bone health and linear growth in the short term. However, the long-term safety and acceptability of these practices need to be evaluated. On the whole, there are few well-designed, adequately powered studies of the role of general dietary or specific micronutrients in the cause, treatment, or prevention of these commonly observed morbidities in thalassemia. Until these data are gathered, it is suggested that patients with thalassemia be monitored frequently and that their nutritional deficiencies be corrected when observed in order to advance their overall health and quality of life.

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Section: Low Bone Massmentioning

confidence: 99%

The importance of nutrition for health in patients with transfusion‐dependent thalassemia

Fung

2016

Annals of the New York Academy of Sciences

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“…The pathogenesis of thalassemia-related osteoporosis is multifactorial and dependent on genetic variants, bone marrow expansion, iron burden, iron chelators, renal involvement, and endocrine condition; therefore, osteoporosis management is difficult in these patients ( 11 ). This study showed that single-dose zoledronic acid therapy over a year (4-mg, single dose once yearly) was effective in improving lumbar spine BMD in the treatment of osteoporosis in patients with thalassemia.…”

Section: Discussionmentioning

confidence: 99%

“…Zoledronic acid is a potent inhibitor of bone resorption, and likely improves bone density through direct inhibition of osteoclasts by interfering with protein prenylation ( 10 ). Zoledronic acid has been tested with different dosing regimens (4 mg IV every 3 months or 4 mg IV every 6 months) for osteoporosis management in thalassemia patients ( 11 ). Zoledronic acid 4 mg IV every 3 months is one of the most promising protocols ( 8 - 9 , 12 ) but evidence is lacking with respect to other potential doses (such as IV zoledronic acid administered annually) for the treatment of low BMD associated with thalassemia ( 13 - 15 ).…”

Section: Introductionmentioning

confidence: 99%

“…Regarding the complex etiology of thalassemia-associated osteoporosis, all bisphosphonates must be administered in higher doses (sum of dose during a year) in patients with thalassemia-associated osteoporosis than in women with post-menopausal osteoporosis to ensure similar effectiveness ( 17 ). A 3- to 5-year treatment period should be considered for management of osteoporosis with bisphosphonates ( 18 , 19 ); longer treatment periods or doses >5 mg annually are not advisable considering the short-term exposure and the unknown safety (and potential side effects) of other potential regimens ( 11 ).…”

Section: Introductionmentioning

confidence: 99%

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Administration of Intravenous Zoledronic Acid Every 3 Months vs. Annually in beta-thalassemia Patients with Low Bone Mineral Density: a Retrospective Comparison of Efficacy

Darvishi‐Khezri¹,

Kosaryan²,

Akbarzadeh³

et al. 2018

Med Arch

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Introduction:The benefit of annual administration of zoledronic acid in the management of thalassemia-associated osteoporosis is unknown.Aim:The aims of this study were to evaluate the efficacy of treatment with two different dosing regimens of IV zoledronic acid (annually versus every 3 months) for increasing low bone mineral density (BMD) in patients with osteoporosis associated with β-thalassemia as annually and 3-monthly on bone density in patients.Materials and Methods:This retrospective, single-center study analyzed patients’ clinical records and bone density measurements. Those enrolled in the study were 14 to 53 years of age, had documented β-thalassemia, and were treated with IV zoledronic acid on either an annual or every 3 months dosing regimen. Dual-energy X-ray absorptiometry was used to obtain the z-score for BMD in the lumbar spine and femoral neck.Results:Thirty-four patients were enrolled in the study; 15 (44.1%) had been treated annually, and 19 (55.9%) had been treated every month. In patients receiving treatment with the once-yearly dose of zoledronic acid, significant increases were observed in the lumbar spine BMD z-score, from -2.45 ± 0.69 to -1.97 ± 0.82 (P=0.02). When comparing BMD across the two treatment regimens, the mean lumbar spine BMD was 0.82 greater (95% CI 0.31, 1.33, P=0.003) and the mean femoral neck BMD 0.37 greater (95% CI -0.15, 0.87, P=0.1) in the group receiving annual zoledronic acid treatment.Conclusions:In patients with thalassemia-associated osteopenia, annual treatment with zoledronic acid increases lumbar spine bone density while being more effective, less expensive, and associated with fewer adverse events than dosing every 3 months.

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“…Thalassemia-associated osteoporosis is among the most commonly observed complications of the disease seen as a result of various factors such as hypogonadism, delayed puberty, bone marrow expansion, iron overload, desferrioxamine (DFO) toxicity, genetic factors, calcium, Vitamin D and GH /IGF-1 deficiencies, hypoparathyroidism, hypothyroidism and decreased physical activity 1,2,3,4,5 . Severity of the bone disease in thalassemia is associated with the underlying type of thalassemia and its treatment.…”

Section: Introductionmentioning

confidence: 99%

Zoledronik asit osteoporozlu β-talasemi hastalarında kemik mineral yoğunluğunu arttırır: Bir vaka kontrol çalışması

Evran

Gürkan

Tuli

2019

Cukurova Medical Journal

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This study addresses the efficacy of zoledronic acid treatment in osteoporotic patients with β-thalassemia. Materials and Methods: Thirty-nine thalassemic patients were enrolled in this study. Bone mineral density was measured using Dual energy x-ray absorbtiometry (DEXA), and biochemical markers of osteoporosis in serum and urine were measured at 0, 6, and 12 months of treatment. Patients with thalassemia were randomly received either zoledronic acid 4 mg IV once every six months or calcitriol 0.25 mcg / day for 12 months. A subgroup analysis was also performed according to gonadal status. Results: There were 21 males and 18 females, mean age of 23.1 ± 1.3 and bone age of 16.3±0.35 years. At the end of 12 months, bone mineral densities of the lumbar vertebra and femoral neck significantly increased in the zoledronic acid group. In patients with hypogonadism who received hormone replacement therapy, t-scores significantly increased. Serum NTX levels indicated significant increase in both treatment groups compared with the control group at the end of the treatment. A significant increase was obtained in urinary NTX level in hypogonadal patients who received zoledronic acid, and in serum NTX and IGF-1 levels in normogonadal patients who received calcitriol. Conclusion: In our study, zoledronic acid resulted in significant improvement in bone mineral density and tscores. This improvement was in correlation with IGF-1 levels. Amaç: Bu çalışmada osteoporozu olan β-talasemi hastalarında zoledronik asit tedavisinin etkinliği incelenmiştir. Gereç ve Yöntem: Çalışmaya 33 β-talasemili hasta ile kontrol grubu olarak yaş ve cinsiyetleri uyumlu 25 sağlıklı gönüllü alındı. Hastalarda 0. 6. ve 12.aylarda DEXA ve serum ve idrarda osteoporozun bazı biyokimyasal belirteçleri ölçüldü. Talasemili hastalar 12 ay süreyle her altı ayda bir 4 mg İV zoledronik asit ve 0.25 mcg/gün kalsitriol alanlar olmak üzere rastgele iki ayrı gruba ayrıldı. Gonadal fonksiyonlarına göre de iki alt gruba ayrılarak takip edildiler. Bulgular: Hastalar yaş ortalaması 23.1±1.3 ve kemik yaşı ortalaması 16.3±0.35 yıl olan 21 erkek, 18 kadından oluşmaktaydı. On iki ay sonunda zoledronik asit grubunda lumbar vertebra ve femur boynu kemik mineral yoğunluğu anlamlı artış gösterdi. Hipogonadik olup hormon replasmanı yapılan hastalarda lumbar vertebra ve femur boynu t-skoru anlamlı artış gösterdi. Serum NTX düzeyleri tedavinin sonunda her iki tedavi grubunda da kontrol grubuna göre belirgin olarak yükselme gösterdi. Hipogonadik hastalar içinde zolendronik asit alanlarda idrar NTXdüzeylerinde, normogonadik hastalar içinde kalsitriol alanlarda idrar NTX ve serum IGF-1 düzeylerinde anlamlı artış saptandı. Sonuç: Çalışmamızda zoledronik asit kemik mineral yoğunluğu ve t-skorlarında belirgin düzelmeler sağlamıştır. Bu düzelme IGF-1 düzeyi ile korelasyon göstermiştir.

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Management of beta‐thalassemia–associated osteoporosis

Cited by 35 publications

References 61 publications

The importance of nutrition for health in patients with transfusion‐dependent thalassemia

The importance of nutrition for health in patients with transfusion‐dependent thalassemia

Administration of Intravenous Zoledronic Acid Every 3 Months vs. Annually in beta-thalassemia Patients with Low Bone Mineral Density: a Retrospective Comparison of Efficacy

Zoledronik asit osteoporozlu β-talasemi hastalarında kemik mineral yoğunluğunu arttırır: Bir vaka kontrol çalışması

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