Endocrine Abnormalities in Thalassemia

Vullo, C; Sanctis, Vincenzo De; Katz, Maurice; Wonke, B; Hoffbrand, A. Victor; Bagni, B; Torresani, T.; Tolis, G.; Masiero, Massimo; Palma, A. Di; Borgatti, L

doi:10.1111/j.1749-6632.1990.tb24317.x

Cited by 54 publications

(36 citation statements)

References 38 publications

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“…A French study from 1993 showed the prevalence to be as high as 22.5% [84]. Shamshirsaz et al [1] in their multicentre study in Tehran have shown a prevalence of 7.6%, which was higher than the 3.6% -7%, reported by other workers [8,35,85] and the male: female ratio was 4:1, which was higher than several other reports [35,86].…”

Section: Hypoparathyroidismcontrasting

confidence: 48%

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: Hypoparathyroidismcontrasting

confidence: 48%

A Review of Endocrine Disorders in Thalassaemia

De¹,

Mistry²,

Wright³

et al. 2014

OJEMD

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“…17 Thus, established diabetic thalassemic patients seldom recover normal glucose tolerance. 18 On the other hand, in normoglycemic patients, pancreatic hemosiderosis may affect ␤ cell reserve and insulin resistance. The latter was notably unrelated to liver hemosiderosis, and it is unclear whether insulin resistance in thalassemics is related to iron in the fat or skeletal muscles.…”

Section: Resultsmentioning

confidence: 99%

A T2* magnetic resonance imaging study of pancreatic iron overload in thalassemia major

Au¹,

Lam²,

Chu³

et al. 2008

Haematologica

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We studied the utility of pancreatic magnetic resonance imaging (MRI) in 72 thalassemia major patients (21 diabetic, 51 normoglycemic). Diabetic patients were significantly older (p<0.0001) and had smaller pancreas volume (p<0.0001). The two groups were comparable for ferritin and MRI-T2* heart, liver and pancreas. Pancreatic T2* signals were abnormal in 80% of both groups, and correlated with heart T2*. In normoglycemic patients, cardiac T2* and log-pancreatic T2* values correlated with homeostatic model assessments HOMA-B (␤ cell reserve), HOMA-IR (insulin resistance) and fasting insulin/C-peptide levels. This suggested that improved chelation may improve ␤ cell reserve and prevent pancreatic atrophy.

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“…However, aging leads to the development of additional clinical problems in these patients, such as osteopenia and osteoporosis, which are frequently the cause of pathologic fractures and limb deformities [1,2]. So far, little information is available concerning the prevalence of bone mass alterations and the factors responsible for the development of osteopenia and osteoporosis in beta-thalassemia [3,4]. In a previous study, we found that 48% of female thalassemic patients have osteopenia and 38% have osteoporosis, while the corresponding male data are 76% and 18% (Lasco et al, unpublished data).…”

Section: Introductionmentioning

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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Endocrine Abnormalities in Thalassemia

Cited by 54 publications

References 38 publications

A Review of Endocrine Disorders in Thalassaemia

A Review of Endocrine Disorders in Thalassaemia

A T2* magnetic resonance imaging study of pancreatic iron overload in thalassemia major

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

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