ESPE Abstracts

Introduction: β-thalassemia is a hereditary chronic hemolytic anemia characterized by a defect in the synthesis of beta-globin chains, particularly common in the Mediterranean region, southern Asia, and the Middle East (1) Transfusion programs and chelating therapy have greatly extended the life expectancy of patients (2). This has led to an increase in the prevalence of endocrine complications, linked to iron overload (3) including abnormalities in glucose homeostasis (4). Glycoregulation disturbances range from insulin resistance and glucose intolerance to obvious diabetes mellitus. Regular follow up of ferritinemia is the most commonly biological component used to assess the degree of iron overload. The correlation between the ferritinemia rate and glycoregulation abnormalities is not well studied and deserves to be specified (5-6)

Objectives: Main: Describe the frequency of glucose homeostasis abnormalities in patients with polytransfused β-thalassemia

Secondary: study the relationship between abnormal glycoregulation and : • The ferritinemia level • Average transfusion volume (MTV) • Duration of transfusion therapy • Splenectomy • Compliance with chelating treatment

Method: It is a descriptive, analytical and mono-centric cross-sectional study which was carried out in the pediatric department of CHU Mustapha and which involved 87 patients (46 Girls and 41 Boys), followed for several years on a regular basis and treated by a transfusion regimen (more than 10 transfusions) combined with a chelating treatment. All patients were assessed by a clinical history, physical examination, fasting blood glucose and oral hyperglycemia test (HGPO) combined with a test of HOMA-IR (Homeostasis Model Accessment of insulin resistance).

Results: 28 (32.2%) patients presented glycoregulation disorders with 17 (60.7%) girls and 11 (39.3%) boys, there is no statistically significant relationship between the two genders (P = 0.31), The average age of patients with glycoregulation disorders is 13.4 ± 6.58 years,16 (18.4%) patients had moderate fasting hyperglycemia (MGH), 8 (9.2%) patients had glucose intolerance (GI), and 4 (4.6%) patients had diabetes (3 patients diagnosed before the start of the study).10 (12%) patients presented a positive HOMA test, there is no statistically significant relationship between ferritinemia and glycoregulation disorders (P = 0.65).

Conclusion: Our results suggest that children with β-thalassemia have a high incidence of glycoregulation disorder in the second decade of life or later.