Ruhuşen KUTLU, Ayşe Özlem KILIÇASLAN, Mustafa KULAKSIZOĞLU

Beta-talasemi major komplikasyonu olarak gelişen diabetes mellitus ve hipoparatiroidi

Beta-talasemi major ilk defa Cooley ve Lee tarafından tanımlanmış olup, ?-globin zincir sentezinde azalma ile giden, resesif karakterli bir hemoglobin bozukluğudur. Homozigot olgular düzenli kan transfüzyonu gerektiren ağır anemi ile seyreder. Şelasyon ve transfüz- yon tedavisi kombinasyonu yaşam beklentisini 40-50'li yaşlara kadar dramatik olarak uzatır. Diğer yandan sık kan transfüzyonları vücutta fazla demir birikimine yol açarak hipogonadizm, diabetes mellitus, hipotiroid, hipoparatiroid ve diğer endokrin bozukluklara neden olabilir. Bu yazıda talasemi major komplikasyonu olarak diabetes mellitus ve hipoparatiroidi gelişen bir olgu sunumu takdim edilmiştir.

Diabetes mellitus and hypoparathyroidism developed as a complication of beta-thalassemia major

Beta-thalassemia major, first described by Cooley and Lee, is a recessively inherited hemoglobin disorder and characterized by redu- ced synthesis of ?-globin chain. The homozygous state results in severe anemia, which needs regular blood transfusion. The combi- nation of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients who can recently survive into their fourth and fifth decades of life. On the other hand, frequent blood transfusion in turn can lead to iron overload whi- ch may result in hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism and other endocrine abnormalities. In this paper, a case with diabetes mellitus and hypoparathyroidism developing as complications of beta-thalassemia major was presented.

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