Background: The estimated prevalence of beta thalassemia is 38% in India, Pakistan, Bangladesh and China. The combination of regular blood transfusions and chelation therapy has dramatically increased the life expectancy of thalassemics, but it has led to iron overload and chelation toxicity, with many complications including growth failure, gonadal dysfunction, hypothyroidism, hypoparathyroidism, DM, etc.
Objective and hypotheses: To study the incidence and prevalence of endocrine disturbances in thalassemia major children, receiving regular blood transfusions. Also to study the correlation between-Age of starting transfusion/Average Sr. Ferritin levels/Age of starting chelation AND incidence of Endocrine problems in these children.
Method: Basic information for each child was determined age of presentation, onset of transfusion and chelation/Anthropometric data/Pubertal status(tanner staging). Blood investigations-Sr. Ferritin and average ferritin levels, Hb level, Sr. Calcium/Phosphorus/Alkaline Phosphatase, PTH, Thyroid profile, OGTT, LH/FSH, Sr. Estradiol (girls), Sr. Testosterone (boys).
Results: See Table 1 below.
(% in both sexes)
|Cyprus (435)||Greece (262)||Italy (1861)||Tehran (220)||N. Am (262)||Our study (225)|
|Hypogonadism Delayed Pub/Arrested Pub||32.5||42||49||17.5/35.1||42||13.8/4.9/5.8|
Conclusion: In our study endocrine complications increased with Age maximum seen in age group>15 years.
No association was found between age of onset of transfusion and chelation with incidence of endocrinal problems.
Increased incidence of Hypogonadism, Hypothyroidism, Hypoparathyroidism was found with high average ferritin levels- at levels >2500 ng/ml.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology