ESPE Abstracts (2019) 92 P3-188

Endocrinopathies and Linear Growth in Adolescents with β-Thalassemia Intermedia in Relation to Liver Iron Content

ASHRAF TAWFIK SOLIMAN, Mohamed Yassin, ashraf adel

hamad medical centre, Doha, Qatar

We evaluated the linear growth and some endocrine function in 10 adolescents with Thalassemia Intermedia (TI) followed at Hematology Section, Doha (Qatar) in relation to the liver iron content (LIC).

Patients and Methods: This retrospective cohort study was performed on 10 adolescents with TI who were randomly selected from the Hematology clinic, National Centre for Cancer Care and Research, Hamad Medical Corporation of Doha (Qatar). 8 infrequently received a blood transfusion. And 2 did not receive blood transfusion.

Data recorded from charts included transfusion frequency, history of chelation therapy, and splenectomy. Auxological data were recorded as well as the pubertal stage. Laboratory data and target organ complications (including endocrinopathies and liver disease were recorded at the last clinic visit. Iron overload was assessed by FerriScan (liver iron content; (LIC) and indirectly by measuring serum ferritin concentration (SF). Bone mineral density (BMD) was assessed by dual-energy X-ray absorptiometry (DXA).

Results: Short stature [(Ht SDS < -2] occurred in 3/10 (30%) with a mean HtSDS = -1.95 +/- 0.7. (IGF-1) SDS was < -2 in 3/10 with a mean = -1.12 +/- 0.9. Impaired fasting blood glucose occurred in 2/10 and diabetes mellitus in 1/10. Delayed puberty occurred in 1/10. Morning 8 AM cortisol was normal (mean = 398 +/- 111) . No thyroid or hypo-parathyroid abnormalities were detected in any patient. Free T4 was normal in all patients (mean = 13.2 +/- 1.19 pmol/L). Liver iron content (LIC) > 15 mg/g dry weight and SF > 2,000 ng/mL were detected in 7/10 of patients. High liver enzyme level (ALT) was detected in 3/10 of patients. Osteopenia was diagnosed in one patient. Ht-SDS was correlated significantly with IGF-1 SDS (r = 0.45, P < 0.05). LIC was correlated with IGF-I SDS and HtSDS (r = 0.51 and 0.5 respectively) . ALT concentrations were correlated significantly with IGF-I levels (r = 0.8, P <0.01) .

Conclusions: A significant number of TI adolescents on occasional blood transfusion have high LIC, short stature and dysglycemia. Regular monitoring for these abnormalities is highly recommended.

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