ACTA ENDOCRINOLOGICA (BUC)

The International Journal of Romanian Society of Endocrinology / Registered in 1938

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April - June 2005, Volume 1, Issue 2
Endocrine Care


Fica SV, Albu A, Vadareanu F, Barbu C, Bunghez R, Nitu L, Marinescu D

Endocrine disorders in ?-thalassemia major: cross-sectional data

Acta Endo (Buc) 2005, 1 (2): 201-212
doi: 10.4183/aeb.2005.201

Chronic transfusion regimen and chelating therapy has dramatically improved the life expectancy of thalassemic patients. The aim of this study was to assess the prevalence of endocrine disturbances in patients with beta-thalassemia major. Subjects were 64 patients with a mean age of 19.45 ? 6.82 years found in haematological care at the National Institute of Transfusional Haematology. All the patients were evaluated clinically and biologically. LH, FSH, estradiol, testosterone, TSH, free T4, insulin were measured by chemiluminescence; mean ferritin value was used to assess iron overload. Fifty one patients (79.68%) -27 male and 24 female in our group were at pubertal or adult age. Eleven boys (40.74%) had delayed puberty, 10 (37%) arrested puberty and 4 (14.8%) had reached complete sexual maturation. In the female group, 6 (25%) had delayed puberty, 4 (16.66%) arrested puberty and 14 (58.32%) reached full sexual development. Half of both the male and female patients with complete sexual maturation had hypogonadotropic hypogonadism at the evaluation moment. Moreover, 34 (53.12%) of our patients had pathological short stature, 11 (17.18%) primary hypothyroidism, 5 (7.8%) hypoparathyroidism, 3 (4.68%) diabetes mellitus and 6 (9.37%) insulin resistance. We found a significantly higher mean ferritin value in patients with endocrine disturbances of any type compared to subjects without endocrinopathies. In conclusion, our data showed that hypogonadism and short stature were the most frequently found endocrine disturbances. Early form of hypogonadism had a major clinical impact on sexual development and final height. These results suggest that early endocrine evaluation and treatment are necessary in order to improve the quality of life of these patients.

Keywords: thalassemia major, endocrine disturbances, hypogonadism, short stature

Correspondence: Carmen Barbu, MD, PhD, ?Elias Hospital?, Bd Marasti 17, sector 1, Bucharest, fax:+40213161614, email: carmen-barbu@home.ro

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