VAHIDI A, TORABINEJAD M, AHMADI A, FARAHMANDINIA Z, KIANI MOGHADDAM G, MEGHDADI B. A CROSS-SECTIONAL CONTROLLED STUDY OF GONADAL FUNCTION AND PUBERTAL DEVELOPMENT IN THALASSEMIA MAJOR. Med J Islam Repub Iran 2003; 17 (1) :5-10
URL:
http://mjiri.iums.ac.ir/article-1-711-en.html
From the Department of Pediatrics, Kerman University of Medical Sciences, Kerman, I.R. Iran.
Abstract: (5619 Views)
Early and regular blood transfusion therapy in patients with homozygous
beta-thalassemia decreases the complications of severe anemia and prolongs survival.
In the long term, however, the beneficial effects of transfusions are limited
by the organ damage resulting from iron overload. Endocrine complications in
patients with thalassemia major in developing countries may be frequent due t o
suboptimal iron chelation.
T he goal of this study was to investigate the gonadal function and secondary
sex characteristics in thalassemic patients. We studied 71 randomly selected adolescent
thalassemic patients and 30 age- and sex-matched controls. Sexual maturity
rating (SMR), height and weight, and gonadotropin, sex steroid, and ferritin
levels were evaluated.
Cases had significantly lower mean height and weight. Also, serum levels of
gonadotropins and sex hormones were significantly lower in cases with thalassemia
than in controls. Gonadotropin and sex steroid levels were lower in cases
with thalassemia who had not used deferoxarnine regularly compared to those
with a regular chelation therapy regimen. All of the control subjects had sexual
maturity ratings of II or above, while 36.6% of thalassemic cases were in Tanner
stage I. About 53.3% of controls had surpassed all levels of sexual maturity, while
only 2.8% of cases were in the stage SMR V. Distribution of SMR ratings was
significantly different in cases and controls.
These findings clearly show that a high percentage of thalassemic patients in
this part of the country suffer from various endocrine abnormalities, especially
impairment in height growth and sexual maturity. It is wise to consider more
sophisticated treatment modalities in these patients, including the administration
of sex hormones for the compensation of hormonal abnormalities resulting from
hemosiderosis.
Type of Study:
Original Research |
Subject:
Pediatric