Bone disease in β thalassemia patients: past, present and future perspectives

Sanctis, Vincenzo De; Soliman, Ashraf T; Elsefdy, Heba; Soliman, Nada; Bedair, Elsaid; Fiscina, Bernadette; Kattamis, Christos

doi:10.1016/j.metabol.2017.09.012

Cited by 49 publications

(47 citation statements)

References 83 publications

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“…Bone disease is also becoming a major concern for thalassaemia patients, with the accompanying pain and fractures seriously affecting the quality of patients. 44,45 . The lifetime fracture rate escalates to 55% for adults with TM and to 71% for adults with TI.…”

Section: The Evolving Spectrum Of Comorbiditiesmentioning

confidence: 99%

The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation

Farmakis

Giakoumis

Angastiniotis³

et al. 2020

European J of Haematology

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Therapeutic advances in β‐thalassaemia have gradually lead to a significant improvement in prognosis over the past few decades. As a result, patients living in areas where disease‐specific programmes offering access to modern therapy are in place experience a new era of prolonged survival that tends to reach that of the normal population. This ageing thalassaemia population, however, faces a new spectrum of comorbidities resulting from increasing age that may jeopardise the advances in prognosis provided by current therapy and thus poses new challenges in diagnosis, monitoring and treatment. In this position paper of the Thalassaemia International Federation, we review the changing epidemiology and clinical spectrum of patients with β‐thalassaemia and propose actions to be undertaken in order to address the emerging spectrum of comorbidities resulting from ageing.

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Section: The Evolving Spectrum Of Comorbiditiesmentioning

confidence: 99%

The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation

Farmakis

Giakoumis

Angastiniotis³

et al. 2020

European J of Haematology

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“…Liver cirrhosis, hepatocellular carcinoma, cardiomyopathies, and hypogonadism are commonly described in patients with iron‐overload disorders . Clinical data have drawn considerable attention to the strong correlation between iron overload and the development of osteoporosis in disorders such as congenital and acquired anemias (eg, thalassemias, sickle cell disease) . Moreover, osteoporosis was initially described as a frequent complication in HFE ‐HH patients; however, most patients had evidence of gonadal hormone deficiency, which is likely the primary effect of iron on reported bone loss .…”

Section: Introductionmentioning

confidence: 99%

Despite Genetic Iron Overload, Hfe‐Hemochromatosis Mice Do Not Show Bone Loss

et al. 2019

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One of the most prevalent genetic iron overload disorders in Caucasians is caused by mutations in the HFE gene. Both HFE patients and Hfe‐mouse models develop a progressive accumulation of iron in the parenchymal cells of various tissues, eventually resulting in liver cirrhosis, hepatocellular carcinoma, cardiomyopathies, hypogonadism, and other pathologies. Clinical data and preclinical models have brought considerable attention to the correlation between iron overload and the development of osteoporosis in HFE/Hfe hemochromatosis. Our study critically challenges this concept. We show that systemic iron overload, at the degree present in Hfe −/− mice, does not associate with the microarchitecture impairment of long bones, thus excluding a negative effect of iron overload on bone integrity. We further reveal that Hfe actions in osteoblasts and osteoclasts are dispensable for the maintenance of bone and iron homeostasis in mice under steady‐state conditions. We conclude that, despite systemic iron overload, Hfe −/− mice present normal physiological bone homeostasis. © 2019 The Authors. JBMR Plus in published by Wiley Periodicals, Inc. on behalf of the American Society for Bone and Mineral Research.

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“…This system provides a balance between bone formation and resorption and through which a wide variety of biological mediators such as hormones, cytokines, and growth factors affect bone homeostasis. RANKL enhances osteoclastic function that elevated in β-TM patients, while OPG and OPG/RANKL ratio reduced, associated with low bone mineral density (BMD), this gives evidence that OPG/RANKL system plays a key role in the pathogenesis of osteoporosis in β-TM [10,11].…”

Section: Introductionmentioning

confidence: 87%

“…Ineffective erythropoiesis results in erythroid hyperplasia and marrow expansion secondary to extramedullary hematopoiesis [6], resulting in expansion of the medulla, thinning of cortical bone and resorption of cancellous bone causing a generalized loss of BMD [11]. Deposition of iron in the bones impairs osteoid maturation and inhibits mineralization locally; this occurs by a mechanism that includes the incorporation of iron into calcium hydroxyapatite crystals which consequently affects their growth [12].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Osteopathy in Patients With Beta-Thalassemia Major Using Different Iron Chelation Therapies

Shawkat

Jwaid

Awad

et al. 2018

Asian J Pharm Clin Res

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Objective: We aim to assess the bone mineral density (BMD) and bone biochemical parameters in Iraqi patients with β-thalassemia major (β-TM).Methods: Dual-energy X-ray absorptiometry scan was used to evaluate bone density and interpreted about Z-score which compares to the BMD of age-, sex-, and ethnicity-matched reference population. Biochemical parameters such as calcium, 25-OH Vitamin D, parathyroid hormone, and serum ferritin (SF) evaluated.Results: No statistical difference in SF between pediatrics and adults was determined; however, 66 patients were having their SF between 1000 and 2500 ng/ml and 122 patients with SF ˃2500 ng/ml. Calcium and Vitamin D levels are low in both adults and pediatrics. The bone status shows high percentages of osteoporosis 62% and 54.5% for pediatrics and adults, respectively, as well as osteopenia 27% and 34.3% for both pediatric and adults and to a lesser extent normal bone status 11% for each. Conclusion: Osteopathy has a high prevalence in Iraqi patients with β-TM and should receive an optimal transfusion and chelation therapy to prevent bone expansion. Calcium and Vitamin D should be routinely determined to prevent deficiency.

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Bone disease in β thalassemia patients: past, present and future perspectives

Cited by 49 publications

References 83 publications

The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation

The changing epidemiology of the ageing thalassaemia populations: A position statement of the Thalassaemia International Federation

Despite Genetic Iron Overload, Hfe‐Hemochromatosis Mice Do Not Show Bone Loss

Evaluation of Osteopathy in Patients With Beta-Thalassemia Major Using Different Iron Chelation Therapies

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