Incidence Rates of Endocrine Complications in Polytransfused Beta-Thalassemia Patients

Abstract

Introduction:

β-thalassemia is a hereditary chronic hemolytic anemia characterized by a defect in the synthesis of beta-globin chains, which is particularly common in the Mediterranean region, southern Asia, and the Middle East. Transfusion programs and chelation treatment have considerably extended the life expectancy of patients. This has led to an increase in the prevalence of cardiac, hepatic and endocrine complications.

Objectives:

Describe the frequency of endocrine complications in patients with polytransfused β-thalassemia.

Study the relationship between endocrine complications and ferritin levels.

Method:

It's a transversal descriptive, analytical and monocentric study which was carried out in the Mustapha University Hospital pediatric department and which involved 87 patients with beta thalassemia major (46 Girls and 41 Boys), most of whom were regularly followed for several years, and treated with a transfusion regimen (more than 10 transfusions) combined with chelation therapy.

All patients were evaluated by a clinical history, a physical examination, measurement of height and weight, and evaluation of pubertal development stage. To assess glucose tolerance, fasting blood glucose and an oral glucose tolerance test (OGTT) were performed. Serum levels of free T4 (FT4), thyroid-stimulating hormone (TSH), calcium, phosphorus, and parathyroid hormone (PTH) were also measured.

Results:

Growth delay was observed in sixteen cases (18.4%). Hypothyroidism was observed in sixteen cases (18.4%). Twenty-eight patients (32%) developed glucose regulation disorders, with 16 (18.4%) having moderate fasting hyperglycemia (HGM), nine (10.3%) showing glucose intolerance (IG), and 4 patients (4.6%) developing diabetes. Two patients (2.3%) had hypoparathyroidism. Hypogonadism was the most frequent complication, observed in 72% of the pubertal population. Over 90% of them experienced pubertal delay, 17.4% of patients had secondary amenorrhea, 13% had primary amenorrhea, and 8.7% experienced menstrual irregularities.

Conclusion

Despite chelation therapy, the risk of endocrine dysfunction remains common in patients who have transfusion-dependent β-thalassemia. Hypogonadism was the most common endocrine complication.