Failure of puberty and linear growth in beta-thalassemia major.

Abstract

Thalassemia major is a severe progressive hemolytic anemia and a serious medical problem worldwide. Endocrine dysfunctions are well described in patients with thalassemia major. Data for endocrine complications from developing countries are scant. Endocrine complications in developing countries may be frequent due to suboptimal iron chelation. The aim of this study was to evaluate the prevalence of delayed puberty and growth failure in patients with beta-thalassemia major. We evaluated the growth and sexual development of 146 patients with thalassemia major aged 10-22 years. The following data were recorded in questionnaire, age, sex, height, weight, serum ferritin levels and pubertal staging. Failure of puberty was present in 75.6% of boys and 68.4% of girls aged 12-22 years. Gonadotropin insufficiency was found in most of the patients with lack of puberty. There was a significant difference between the height of patients with pubertal development (153 ± 9.1 cm) and those with delayed puberty (140 ± 9.1), (p< 0.001). Short stature was present in 65.7% of patients. Sixty-nine percent of boys and 62.9% of girls were found to be less than 2 SD below the mean for normal height; after the age of 12, the percentage was 78.9% in girls and 83.8% in boys after the age of 14. We conclude that failure of puberty and impaired growth are very common in our thalassemic patients which necessitates newer protocols of treatment, correct blood transfusion and chelation therapy.