Iron Deposition in the Anterior Pituitary in Homozygous Beta-Thalassemia: MRI Evaluation and Correlation with Gonadal Function

Berkovitch, Matitiahu; Bistritzer, Tzvy; Milone, Stephen D.; Perlman, Kato L.; Kucharczyk, Walter; Olivieri, Nancy F.

doi:10.1515/jpem.2000.13.2.179

Cited by 60 publications

(39 citation statements)

References 11 publications

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“…Moreover, a pituitary secretory defect in b-thal was previously suggested by demonstrating iron deposition in the anterior pituitary in correlation with gonadal function [18]. Our findings suggest that up to one quarter of adult b-thal patients are GH deficient.…”

Section: Discussionsupporting

confidence: 68%

Growth hormone reserve in adult beta thalassemia patients

Vidergor¹,

Goldfarb

Gläser

et al. 2007

Endocr

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Reduced serum insulin-like growth factor-1 (IGF-1) and hypogonadotrophic hypogonadism are common features of adult beta-thalassemia, and warrant evaluation of the growth hormone (GH)-IGF-1 axis. The aim of this study was to determine GH reserve in beta-thalassemia patients (9 females, 7 males, 15 major, 1 intermedia), age 29.3 +/- 6.9 years, BMI 21.3 +/- 1.9 kg/m2, and in 20 age, sex and BMI-matched healthy controls, using the GH-releasing hormone (GHRH)-arginine test. The associations between peak GH response and hormonal and biochemical indices were evaluated. Using BMI-related cut-off limits for peak GH response in the GHRH-arginine test, 4/16 beta-thalassemia patients had peak GH lower than 11.5 microg/l, the cut-off limit suggested for lean subjects, and were diagnosed as GH deficient (GHD). Using 9 microg/l as the cut-off limit 2/16 patients were GHD. Reduced serum IGF-1 and IGFBP-3 were present in 69% and 19% of the patients, respectively. Peak GH did not correlate with serum IGF-1, TSH, and fT4 levels or gonadal status. Neither peak GH nor IGF-1 correlated with serum ferritin. Our findings suggest that GHD is present in up to a quarter of adult beta-thalassemia patients. The clinical benefits of GH therapy need to be determined. GHD alone does not account for the high prevalence of reduced IGF-1 in adult beta-thalassemia.

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Section: Discussionsupporting

confidence: 68%

Growth hormone reserve in adult beta thalassemia patients

Vidergor¹,

Goldfarb

Gläser

et al. 2007

Endocr

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“…Hypothyroidism, hypoparathyroidism, diabetes mellitus, and mainly hypogonadism (as delayed puberty and/or secondary hypogonadism) are considered as major causes of osteopenia/osteoporosis in TM (2-4, 6). Hemosiderosis of the pituitary gonadotrophic cells and iron deposition in the testes and ovaries are involved in the pathogenesis of endocrine complications in TM (27). Hypogonadism is a well-recognized cause of osteoporosis and osteopenia not only in patients with TM but also in the general population and is characterized by high bone turnover with enhanced resorptive phase (28).…”

Section: Acquired Factorsmentioning

confidence: 99%

Bone Disease in Haemoglobin Disorders

Voskaridou

Terpos

2013

Thalassemia Reports

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Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

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“…7,10 Furthermore, increased R2 and decreased pituitary height have been shown to predict clinical and biochemical HH in adults with thalassemia major. 6,7,11 There has also been evidence that increased R2 precedes pituitary volume loss and hypogonadism. 12 We have demonstrated that R2 deposition can be seen in children as young as 4 years of age, with volume loss beginning in the second decade of life.…”

mentioning

confidence: 99%