Özet:

Aim: Early diagnosis and treatment of endocrine dysfunction, the most frequent complication seen in thalassemia major (TM) patients is important for improving their quality of life. Our aim in this study was to find out the ineidence and the distribution of endocrin dysfunction in our TM patients. Methods: Out of 252 patients with hemoglobinopathy, 161 TM patients were evaluated for growth retardation within the 0-3, 4-9, 10-14, 15-20 and 21-30 year age groups and 75 patients over 10 years old were alsa evaluated for hypogonadism, hypothyroidism, growth hormon deficiency, glucose metabolism impairment and hypoparathyroidism. Height percentile and standard deviation scores (SDS) of the patients were obtained; the ones with height < 3 percentile and height velocity /year<10 percentile were tested for growth hormon deficiency / insufficiency. Gonadotropin releasing hormone (GnRh), thyrotropin releasing hormone (TRH) stimulation tests and oral glucose tolerance testing (OGTT) were alsa done. Results: The incidence of growth retardation for the overall TM patients studied was found 20%; whereas within the age groups over 3 years of age it was 12%, 30%, 47% and 27%, respectively. The lowest height SDS was seen during the pubertal stage showing that delayed puberty was the major factor for stunted growth in TM patients. Growth hormon deficiency was found in only one patient whereas insufficiency in two patients. Most frequent endocrine complication seen over 10 years of age was found to be hypogonadism (78%). Incidence of clinical primary hypothyroidism, insulin dependent diabetes mellitus (IDDM) and hypoparathyroidsm were 21%, 9.3% and 4% respectively. While 64% of the 75 patients had one endocrine complication, 25.3% were associated with multiple endocrine complications. Ages of the 63.6% of the latter group were over 20 years old. Conclusion: Endocrine evaluation must be carried out regularly in TM patients for a better quality of life.

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