Failure of puberty in Egyptian beta thalassemic patients: experience in north east region - Dakahlia province.
BACKGROUND
Endocrine complications in thalassemia major (TM) are classically considered to be the result of iron deposition in the endocrine glands. Hypogonadotropic hypogonadism, which still remains the commonest endocrinopathy in patients with TM, has been proven to be the result of hemosiderosis of the gonadotroph cells of the pituitary gland.


OBJECTIVE
To evaluate the prevalence of delayed puberty and hypogonadotropic hypogonadism in transfusion-dependent patients with beta-TM.


METHODS
Growth and sexual development of 40 patients with TM (20 males, 20 females) aged 12-22 years were evaluated. Thirty healthy individuals aged 12-20 years served as a control group. The following parameters were measured in every patient: age, sex, height, weight, body mass index (BMI) and Tanner's pubertal staging. For all patients, the following investigations were done: ophthalmological evaluation, audiograms, skeletal survey, echocardiography, serum ferritin, liver function tests, hepatitis profile, serum calcium, phosphorus and blood sugar. Thyroid, parathyroid hormones, serum follicle stimulating hormone (FSH), luteinizing hormone (LH), testosterone (T) and estradiol (E2) hormone were also measured.


RESULTS
Failure of puberty was present in 80% of boys and 75% of girls aged 12-22 years. Gonadotropin insufficiency was found in most of the patients with lack of puberty. Arrested puberty was noted in five boys (25%) and six girls (30%). Ten girls (50%) did not menstruate, two (10%) had oligomenorrhea, one (5%) had irregular menstrual cycles and two (10%) developed secondary amenorrhea. Using univariate analyses and stepwise logistic regression analysis after adjustment for confounding factors, serum ferritin at the time of the study was identified as an independent risk factor for hypogonadotropic hypogonadism, with an odds ratio of 28.40 (95% confidence interval 3.25-245.15), P = 0.003 with a B value of 3.24 (standard error, 1.12).


CONCLUSIONS
We conclude that failure of puberty is common in our thalassemic patients which necessitates newer protocols of treatment, correct blood transfusion and chelation therapy.
Abdelrazik
Nabil
N
Department of Pediatrics, Mansoura Faculty of Medicine, Mansoura University Children's Hospital, Mansoura, Egypt. nabeelabdelrazik2003@yahoo.com
Ghanem
Hossam
H
D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002657:Child Development; D003430:Cross-Sectional Studies; D004534:Egypt / Q000208:ethnology; D005260:Female; D005293:Ferritins / Q000097:blood; D006062:Gonadotropins / Q000172:deficiency; D006128:Growth; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008297:Male; D011628:Puberty, Delayed / Q000453:epidemiology / Q000209:etiology*; D012307:Risk Factors; D017086:beta-Thalassemia / Q000150:complications* / Q000453:epidemiology
D006062:Gonadotropins; D005293:Ferritins