Objective: To evaluate the endocrine function among Egyptian adolescent thalassemic patients.
Patients and methods: 54 patients and 28 age-matched normal controls was included. Anthropometric measurements, clinical pubertal assessment were done for all patients. Oral glucose tolerance test (OGTT) was done to all patients and controls with measuring serum insulin level at 0.120 minutes. Insulin sensitivity and release index were calculated. 32 patients had short stature and arrested or failure of puberty. Thyroid profile, insulin-like growth factor-1 (IGF1) and GH provocation by two tests and GnRH stimulation test was carried out.
Results: Among the 32 patients, 12 (40%) patients had sub-clinical hypothyroidism and 10 (33.3%) had growth hormone deficiency (GHD). Failure of puberty was confirmed in 71.4% of boys and 33.3% of girls, while arrested puberty was observed in 28.6% of boys and 66.7% of girls. All girls had amenorrhea, primary amenorrhea in 88.9% and secondary amenorrhea in 11.1%. Among the 54 patients, thirteen patients (24.1%) were diagnosed to have abnormal glucose tolerance (AGT); either diabetes in 6 (11.1%) cases or impaired GT(IGT) in 7 (13%) cases. Patients with AGT had significant higher mean postprandial insulin, fasting insulin resistance index (FIRI) and HOMA insulin resistance (IR) and significant lower mean HOMA if compared to the cases with normal GT (NGT).
Conclusion: GHD is an etiological factor in short stature thalassaemic. Delayed puberty is either due to failure of gonads or failure of the whole hypothalamic pituitary gonadal axis. Abnormal glucose tolerance is common which could be attributed to early impaired beta-cell function, along with increasing insulin resistance.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology