Comparison of Pubertal Development between Subjects with Sickle Cell Anaemia and Individuals with Normal Haemoglobin in South-western Nigeria

Background

Sickle cell disease (SCD) is a haemoglobinopathy which results from a point mutation in the β-globin gene of haemoglobin. It is a disease of global health concern, with significantly higher morbidities and mortalities in low-income countries. Although an increasing proportion of affected children now survive into adulthood in these countries, there remains a significant risk of long-term complications such as endocrine disorders. Previous studies have reported delayed pubertal development in SCD, there is, however, paucity of recent studies on pubertal development, and the factors that influence the attainment of puberty, in SCD patients in Nigeria.

Methods

The present study was a cross-sectional study involving 139 eligible subjects with sickle cell anaemia (SCA), aged 6 to 18 years, and 133 individuals with haemoglobin AA (HbAA). Pubertal staging of subjects and controls was done using the Tanner criteria, while blood samples were obtained from individuals who had attained puberty for testosterone, estradiol, follicle stimulating hormone (FSH) and luteinizing hormone (LH). Hormonal assays were performed using Rayto RT 6100R ELISA microplate reader for FSH and LH, and RapidR Labs ELISA kits for oestradiol and testosterone.

Results

Female SCA subjects attained thelarche at a mean age of 12.3±1.71years compared to 9.8±1.86 years in controls; adrenarche at 13.2±1.81years against 10.9±1.67years in controls, and menarche at a mean age of 14.1 ± 1.7years compared to 12.3±1.3years in controls. Males with normal haemoglobin also attained puberty earlier than SCA. Mean LH and FSH were higher in SCA subjects (8.29±33.75mIu/ml and 6.81±10.61mIU/ml respectively) compared to mean values of 3.17±5.0mIU/ml and 3.85±3.88mIU/ml respectively in controls. Estradiol and Testosterone levels were however higher in the control group.

Conclusion and recommendation

The present study confirms delayed onset of puberty in patients with SCA compared with HbAA controls. Screening for endocrine complications should therefore be incorporated into the comprehensive care model for SCA for early detection of impairments and prompt institution of appropriate interventions.