We evaluated growth parameters and endocrine disorders in relation to the quantity of liver iron (LIC) measured by the Ferriscan method and insulin-like growth factor 1 (IGF-I) level in a cohort of adolescents with sickle cell disease (SCD) (n=40) and beta thalassemia major (BTM) (n=52) receiving nearly the same protocol of transfusion and iron chelation therapy since early childhood. Before transfusion, hemoglobin concentration had not been less than 9 g/dl in the past 12 years; subcutaneous daily desferrioxamine was administered for all of them since early childhood (25 years of age). All patients were shifted to oral therapy with Deferasirox iron chelation, 20 mg oral daily for the past 5 years.
Results: BTM patients with severe hepatic iron overload had significantly shorter stature, lower IGFISDS and higher ALT and AST and ferritin concentration compared to thalassemic patients with lower LIC. Patients with SCD with significantly higher LIC had significantly shorter stature, lower IGFISDS and higher ALT compared to SCD patients with lower LIC. Linear regression studies showed significant correlation between LIC and ferritin level in SCD and BTM. LIC and serum ferritin level were correlated significantly with IGF-I level in patients with BTM. LIC was correlated significantly with ALT in patients with BTM and serum ferritin was correlated significantly with ALT in both groups. Patients with BTM had significantly lower HtSDS, IGF-I SDS and FT4 level compared to patients with SCD. LIC and mean FBG were significantly higher in patients with BTM compared to those with SCD. Serum Ferritin and hepatic enzyme concentrations did not differ between the two groups of patients. (Table 1)
|IGF-I SDS <−2||27.5%||40.4%||0.2|
|SCD= Sickle cell disease, BTM = beta thalassemia major, IGF-ISDS = insulin-like growth factor 1 SDS, DM = diabetes mellitus, IFG= impaired fasting glucose.|
Conclusion: The Prevalence of endocrinopathies especially hypothyroidism, DM, and hypogonadism were significantly higher in BTM patients versus SCD patients and higher in patients with higher LIC versus those with lower LIC.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology