Bone Mineral Density of Patients with Thalassemia Major: Four-Year Follow-Up

Molyvda-Athanasopoulou, E.; Sioundas, Anastasios; Karatzas, Nicolas; Aggellaki, M.; Pazaitou, K.; Vainas, Iraklis

doi:10.1007/s002239900637

Cited by 38 publications

(31 citation statements)

References 8 publications

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“…A multi centre study group in Tehran [1] demonstrated that the prevalence of osteoporosis and osteopaenia in the lumbar region (L1 -L4) region were 50.7% and 39.4% respectively; the prevalence was 10.8% and 36.9% respectively in the femoral neck region. Similar results from other studies have also been reported [99,100]. Jensen et al [96] showed that the overall prevalence of "severely low" bone mass was 51%; the prevalence of "low" bone mass to be 45%.…”

Section: Osteoporosissupporting

confidence: 84%

A Review of Endocrine Disorders in Thalassaemia

De¹,

Mistry²,

Wright³

et al. 2014

OJEMD

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Endocrine dysfunction in thalassaemia is amongst the most common complication and is principally attributed to excessive iron overload and suboptimal chelation. The prevalence is quite high particularly in multiethnic populations but determining the prevalence is often difficult due to the widespread heterogeneity of the population and timing of exposure to chelation therapy. Disturbances in growth, pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes and disorderly bone growth are commonly encountered. Early detection and institution of appropriate transfusion regimen and chelation therapy and treatment of complications are the keys to managing this population including regular follow. In this article, we review the literature in relation to the various endocrine complications encountered in thalassaemia.

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Section: Osteoporosissupporting

confidence: 84%

A Review of Endocrine Disorders in Thalassaemia

De¹,

Mistry²,

Wright³

et al. 2014

OJEMD

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“…Hypogonadism and hypoparathyroidism are commonly seen in these patients besides elevated iron load, cortical thinning resulting from bone marrow expansion, negative effects of chelator agents on calcium, phosphorus absorption compose osteoporosis in thalassemias. 6 Osteoporosis was detected in 20.9% of our patients. Osteopenia/osteoporosis was found in 56-96% of thalassemia patients in studies conducted with large patient series.…”

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confidence: 66%

Common Complications in Beta-Thalassemia Patients.

Yaman¹,

Işık²,

Yaralı³

et al. 2013

UHOD

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Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 ß-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with diagnosis of thalassemia major and 11 with thalassemia intermedia. Totally, 56.7% of patients were male and 43.3% were female. Ages varied between 2-20 years with the mean age of 10.3±4.8 years. Mean ferritin level was 2212±1370 ng/mL (41-6263 ng/mL) for 4.5 years. Complications were increased with increasing age. Complication rates were significantly higher among thalassemia major patients compared to thalassemia intermedia patients. There was no statistically significant relationship between complications and mean ferritin levels. The most common complications were endocrine complications (38.8%). Cardiac complications developed in 22.4% of the patients; gastroenterological complications in 19.4%; allergic complications in 9%; infectious complications in 1.5%; and thrombosis was detected in 1.5%. The endocrine complications were osteoporosis, growth retardation, developmental delay, short stature, hypothyroidism, delayed puberty, hypogonadism, and diabetes mellitus. The cardiac complications were left ventricular wall hypertrophy, diastolic dysfunction, systolic dysfunction, heart failure, pericardial effusion, dilated cardiomyopathy, left ventricular dilatation, left atrial dilatation, and fatal arrhythmias. Mortality occurred in one (1.5%) out of 67 ß-thalassemia patient due to dilated cardiomyopathy and fatal arrhythmia. Keywords: Thalassemia major, Thalassemia intermedia, Complications ÖZET Beta Talasemili Hastalarda S›k Görülen KomplikasyonlarTalasemi hastalar›n›n son 40 y›l içerisinde yaflam sürelerinde uzama olmakla birlikte yafl artt›kça görülen komplikasyonlar hastalar›n yaflam kalitelerini olumsuz yönde etkilemektedir. Çal›flmam›zda 1 Ocak 2004-31 May›s 2009 tarihleri aras›nda hastanemizde takipli 67 ß-talasemi hastas›nda görülen komplikasyonlar retrospektif olarak incelenmifltir. Çal›flmaya dahil edilen hastalar›n 56's› talasemi majör, 11'i talasemi intermedia tan›s›yla takip edilmektedir. Olgular›n %56.7'si erkek, %43.3'ü k›z idi. Yafllar› 2-20 y›l aras›nda olup, yafl ortalamalar› 10.3±4.8 y›l olarak hesapland›. Hastalar›n 4.5 y›l boyunca bak›lan ortalama ferritin düzeyleri 2212±1370 ng/mL (41-6263 ng/mL) olarak bulundu. Hastalar›n komplikasyonlar›nda yaflla birlikte art›fl oldu¤u saptand›. Komplikasyonlar talasemi majörlü hastalarda talasemi intermedial› hastalara göre istatistiksel olarak anlaml› derecede daha s›k görüldü. Ortalama ferritin de¤eri ile komplikasyon geliflme s›kl›¤› aras›nda istatistiksel olarak anlaml› bir iliflki bulunmad›. Hastalar›m›zda en s›k rastlanan komplikasyonlar %38.8 oran› ile endokrin komplikasyonlard›. Olgular›n %22.4'ünde kardiyak, %19.4'ünde gastroenterolojik, %9'unda all...

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“…It is known that sex steroid replacement therapy stimulates bone formation and breakdown, the effects of which are particularly pronounced during the last years of puberty and in states of gonadal steroid deficiency [17,19]. HRT has been shown to be associated in normal young men and women, and in thalassemic patients, with increases in bone turnover [29] and BMD [30].…”

Section: Discussionmentioning

confidence: 99%

Effects of Hormonal Replacement Therapy on Bone Metabolism in Young Adults with Beta-thalassemia Major

Lasco

Morabito

Gaudio

et al. 2001

Osteoporosis International

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The aim of our cross-sectional study was to evaluate the effects of hormonal replacement therapy (HRT) on a population of young thalassemics in order to understand better the role of hypogonadism in the balance of bone metabolism. Markers of bone turnover and bone mineral density (BMD) were measured in 40 young patients (mean age 19.8 +/- 4.5 years) with beta-thalassemia major: 20 subjects were biochemically eugonadal. since they were undergoing HRT (group A, treated patients), and 20 were hypogonadic, having suspended HRT (group B, untreated patients). We also examined 20 healthy control subjects (group C) matched for age, anthropometric features and sex to the study groups. Our study shows that young thalassemic patients exhibit a significant loss of cortical and trabecular bone [aBMD L2-L4: 0.886 +/- 0.052 g/cm2 (group A), 0.726 +/- 0.040 g/cm2 (group B), 1.083 +/- 0.090 g/cm (group C); aBMD femoral neck: 0.890 +/- 0.071 g/cm2 (group A), 0.700 +/- 0.065 g/cm2 (group B), 0.934 +/- 0.076 g/ cm2 (group C)]. Osteoporosis is only observed at the lumbar level in treated thalassemic patients, while in untreated patients it involves the femoral neck also. Bone turnover in thalassemic patients is higher in the resorptive phase, than in the neoformation phase and this is more marked in hypogonadic untreated patients. In conclusion, our data demonstrate the important role played by hypogonadism in the development and deterioration of osteopenia/osteoporosis in thalassemia major. Consequently, sex hormone replacement therapy represents an appropriate tool in the prevention and treatment of osteoporosis in thalassemics, probably together with bisphosphonates in cases with severely increased bone resorption.

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Bone Mineral Density of Patients with Thalassemia Major: Four-Year Follow-Up

Cited by 38 publications

References 8 publications

A Review of Endocrine Disorders in Thalassaemia

A Review of Endocrine Disorders in Thalassaemia

Common Complications in Beta-Thalassemia Patients.

Effects of Hormonal Replacement Therapy on Bone Metabolism in Young Adults with Beta-thalassemia Major

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