ESPE Abstracts

Introduction: Thalassemia is an autosomal recessive inherited disease that requires frequent blood transfusion. The life expectancy of thalassaemic children improved with regular blood transfusion and chelating agents. Hemosiderin precipitation in endocrine glands of patient with Transfusion Dependant Thalassaemia (TDT) leads to progressive gland dysfunction. The adrenal insufficiency is one of the complications that results from iron deposition either in the pituitary or adrenal glands. It is defined as impaired synthesis and release of cortisol when it is required

Objectives: To detect the prevalence of adrenal insufficiency in Omani Children and young people live with TDT, cared by Sultan Qaboos University Hospital, a tertiary centre in Oman.

Methods: A prospective cross sectional study targeted all patients with TDT who are currently under age of 20 years old. They were evaluated clinically for symptoms and signs of adrenal insufficiency. They had basic laboratory workup including CBC, ferritin level, basal cortisol, and ACTH levels followed by low dose Synacthen test (LDSST) [(dose of 500ng/1.73m², equivalent to 0.5 mg/1.73m²) x Body surface area (m²)] to identify patients with secondary or partial adrenal insufficiency. Normal response of the LDSST is considered when the peak cortisol exceeds 550 nmol/L. Inadequate response if the peak cortisol is between 350 to 550nmol/l, and adrenal insufficiency if less than 350 nmol/l, for which patients require regular doses of hydrocortisone replacement therapy and strict follow up.

Results: 90 patients were recruited and 76 (38M: 38F) of them have completed the study. Their mean age was 9.30±4.9 years. The prevalence of adrenal insufficiency is 13.2%, secondary in nature. Majority of the studied patients 57.9% had inadequate response to LDSST (Peak cortisol between 350-550nmol/L), indicating partial adrenal insufficiency, for which they require steroid cover only during sick days. Mean serum ferritin level was high among all patients, but highest values were unexpectedly mainly in patients with normal adrenal response to LDSST. Saying that, there was no significant statistical difference for the ferritin level of patients with different adrenal responses (P<0.398). Interestingly, the serum phosphate level was significantly higher in adrenal insufficiency group compared to others (P<0.008), however it remained within the normal range.

Conclusion: Routine assessment of TDT patients for adrenal insufficiency is highly recommended, as early detection of adrenal insufficient patients and implementing an appropriate treatment plan may results into saving lives and reducing the possibility of unexpected adrenal crisis for TDT patients during intercurrent infection or illnesses.