Talasemi Majorlü Vakalarda Endokrin Komplikasyonların Değerlendirilmesi

Öz

Amaç: Kan transfüzyonları beta talasemi major hastalarının dokularında demir birikimine yol açarak, endokrin sistem organları da dahil olmak üzere birçok organda çeşitli derecelerde fonksiyon kayıpları oluşturur. Bu çalışmanınamacı; hastanemiz Çocuk Hematoloji Polikliniğinde beta talasemi major tanısıyla izlenen hastalardaki endokrinolojik komplikasyonları saptamaktır.Gereç ve Yöntem: Uludağ Üniversitesi Tıp Fakültesi Çocuk Hematoloji BilimDalında Ocak 1976-Ağustos 2008 tarihleri arasında beta talasemi major tanısıyla takip edilen hastaların dosyaları retrospektif olarak incelenmiş ve tiroidbezi palpasyonu ile puberte değerlendirmesini de içeren ayrıntılı fizik muayeneleri yapılmıştır. Konsultasyon istenen hastalar Çocuk Endokrinoloji BilimDalında değerlendirilmiştir.Bulgular: Toplam 44 hastanın 20’si %45,5 kız, 24’ü %54,5 erkek olup hastaların ortalama kronolojik yaşı 13,54±7,32 2,75-35,2 yıl olarak hesaplanmıştır.“Osteoporoz” ve “büyüme hormonu eksikliği dışındaki büyüme geriliği olguları” hariç tutulduğunda toplam 12 %27,2 olguda, dahil edildiğinde ise 40 %90,9 olguda bir ya da birden fazla endokrin patoloji mevcuttu. Kırkdört olgunun 27’sinde %61,3 osteoporoz, 17’sinde %38,6 büyüme geriliği, 11’inde %25 osteopeni, 6’sında %13,6 hipogonadizm, 3’ünde %6,8 hipotiroidizm,2’sinde %4,5 hipoparatirodizm, 1’inde %2,3 büyüme hormonu eksikliği ve1’inde 2,3 tip I diabetes mellitus mevcuttu. Olguların ortalama ferritin düzeyi1976,15±1494,75 ng/ml, aylık ortalama transfüzyon sayısı ise 1,46±0,34 olaraksaptandı. Ferritin düzeyi ve aylık transfüzyon ihtiyacı ile endokrin komplikasyonlar arasında istatistiksel açıdan anlamlı birliktelik yoktu. Farklı şelasyontedavisi alan gruplardaki endokrin patolojiler farklılık göstermedi. Çalışmada0-9 yaş gurubu ile 10-19 yaş gurubu arasında büyüme geriliği açısından anlamlı fark bulunmuştur %8,3’e karşılık %30,6; p=0,049 . Sonuç: Talasemi majorlu hastalar endokrinolojik fonksiyon bozukluğu açısından risk altındadırlar ve kemik sağlıkları ciddi derecede bozulmuştur. On yaşından küçükler özellikle büyüme geriliği ve osteoporoz açısından, 10 yaş veüzerinde olanlar ise hipogonadizm, büyüme geriliği ve osteoporoz başta olmak üzere tüm endokrinolojik komplikasyonlar yönünden yakın izlenmelidirler

Anahtar Kelimeler

• Talasemi major,endokrinolojik komplikasyon,çocuklukçağı,ferritin,kan transfüzyonu

Evaluation of Endocrine Complications in Patients with Thalassemia Major

Abstract

Aim: Multiple blood transfusions in beta thalassemia patients causes iron overload in various tissues including endocrine glands thereby leading to multiple endocrine dysfunction. The aim of this study was to determine the endocrine complications seen in beta thalassemia patients followed-up in outpatient clinics of Pediatric Hematology Division of Uludag University Faculty of Medicine. Materials and Methods: The files of patients with thalassemia major followed-up in outpatient clinics of Pediatric Hematology Division of Uludag University Faculty of Medicine from January 1976 to August 2008 were retrospectively evaluated for endocrine disorders. All patients had a detailed physical examination including palpation of thyroid gland and pubertal staging. Endocrine evaluation was performed in the Division of Pediatric Endocrinology. Results: A total of 44 [20 female 45.5% ; 24 male 54.5% ; and mean chronological age 13.54±7.32 2.75-35.2 years] patients were evaluated. The ratios of patients with endocrine dysfunction were 27.2 % and 90.9%, respectively, when we exclude or include those with osteoporosis/osteopenia or growth failure other than growth hormone deficiency. Of all patients, 27 61.3% had osteoporosis, 17 38.6% had growth retardation, 11 25% had osteopenia, 6 13.6% had hypogonadism, 3 6.8% had hypothyroidism, 2 4.5% had hypoparathyroidism, 1 2.3 had growth hormone deficiency, and 1 2.3 had type 1 diabetes mellitus. Mean ferritin levels and monthly transfusion numbers were 1976.15±1494.75 ng/ml and 1.46±0.34, respectively. There were no significant association between ferritin levels, monthly transfusion needs, and endocrine dysfunctions studied. Endocrine dysfunctions did not differ significantly amongst those having different chelating agents. The ratio of patients with growth retardations in 10 to 19-age-group was significantly higher than those in 0 to 9-age-group 30.6% vs 8.3%; p=0.049 . Conclusion: Patients with thalassemia major are under increased risk of various endocrine dysfunction. Bone health is significantly compromised. Those younger than 10 years should be closely followed for especially growth retardation and osteoporosis/osteopenia and those who are 10 years of age or older should be followed for all endocrine pathologies, especially for hypogonadism, growth retardation, and osteoporosis. Journal of Current Pediatrics 2008; 6: 58-65

Keywords

• Thalassemia major,endocrinologic complications,childhood,ferritin,blood transfusion

Kaynakça

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