Reproductive hormones and hypothalamic-pituitary-ovarian axis in female patients with homozygous beta-thalassemia major.
BACKGROUND
Homozygous beta-thalassemia major (hbeta-TM) is an autosomal recessive hemoglobinopathy with prominent endocrine complications. Our aims were to evaluate reproductive hormones, hypothalamic-pituitary-ovarian axis, and fertility in female patients with hbeta-TM.


METHODS
One hundred forty-four female patients with hbeta-TM, aged 18 years or above were enrolled in the study. Seventy-four age-matched healthy female volunteers served as controls. Two blood samples were drawn from each subject at 20-minute intervals for the determination of the resting levels of the following hormones: luteinizing hormone, follicle-stimulating hormone, estradiol, insulin-like growth factor-1, and insulin-like growth factor binding protein-3. The hypothalamic-pituitary-ovarian axis was also assessed using the gonadotropin-releasing hormone test. Menstruation status and growth rate were also noted. Puberty was assessed in both groups by Tanner classification.


RESULTS
The mean age of puberty in the control group and hbeta-TM patients was 10.8+/-1.2 and 16.4+/-2.2 years, respectively (P=0.01). The mean age of menarche was 11.4+/-1.3 and 16.8+/-2.1 years in control subjects and patients with thalassemia, respectively (P=0.01). Of studied subjects with hbeta-TM, 61 (42.4%) were short in stature (height below the third percentile and below -2 SD scores). The baseline and peak levels of luteinizing hormone, follicle-stimulating hormone, and estradiol after gonadotropin-releasing hormone test in thalassemic group were significantly lower than in the control group.


CONCLUSIONS
Further interventional studies are warranted to better clarify the treatment options to attain normal puberty and sexual maturation in patients with thalassemia.
Safarinejad
Mohammad Reza
MR
Department of Urology, Urology and Nephrology Research Centre, Shahid Beheshti University MC, Tehran, Iran. safarinejad@urologist.md
D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004958:Estradiol / Q000097:blood; D005260:Female; D005298:Fertility; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000097:blood; D006720:Homozygote; D006728:Hormones / Q000097:blood*; D006801:Humans; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology*; D018972:Insulin-Like Growth Factor Binding Protein 3; D018969:Insulin-Like Growth Factor Binding Proteins / Q000097:blood; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D007986:Luteinizing Hormone / Q000097:blood; D008875:Middle Aged; D010053:Ovary / Q000503:physiopathology*; D011627:Puberty; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood*
D006728:Hormones; C515497:IGFBP3 protein, human; D018972:Insulin-Like Growth Factor Binding Protein 3; D018969:Insulin-Like Growth Factor Binding Proteins; D007987:Gonadotropin-Releasing Hormone; D004958:Estradiol; D007334:Insulin-Like Growth Factor I; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone