Talasemi majorlu hastalarda kemik mineral yoğunluğu ile hipogonadizm arasındaki ilişki

Öz

Introduction ve Aim: The lifetime expectancy in patients with B-thalassemia major has significantly been extended after the introduction of hypertransfusion protocols. However these applications caused an increase of endocrine complications such as osteoporosis and hypogonadism. It's believed that the excessive iron load is responsible for osteoporosis and other complications as many other different factors. The aim of this research is to exemine the factors which may impact the bone turn over and compare bone mineral density of patients with B-thalassemia major either hypogonadism developed and undeveloped. Material and Methods: 43 thalassemia major patients who have the physical signs of puberty or in the ages of puberty have been examined. All patients' bone mineral density measurements have been made and they're classified as the patient group with hypogonadism and as the control group without hypogonadism. The results are analysed in accordance with the data used in pediatric endocrinology. Results: It'sfound that the incidence of hypogonadism is 48% (21 patients out of 43) and the incidence of bone disease is 30% (13 patients out of 43). There hasn't been any significant statistical difference between the types of chelation and the levels of ferritin. Conclusion: Although in literature it's been confrated in some research that there is a remarkable relationship between bone mineral density and hypogonadism existences, we haven't found any significant difference in bone mineral density between the patient group and the control group, evertheless, the level of vit D3 is higher in the control group than the patient group. Vit D3 levels can be impacted by many other factors. That s why vit D3 levels are lower in thalassemia major patients with hypogonadism and in order to determine as vit D3 supplement must be given those patients, more multicenter prospective studies are needed.

Anahtar Kelimeler

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Talasemi majorlu hastalarda kemik mineral yoğunluğu ile hipogonadizm arasındaki ilişki

Öz

Giriş ve Amaç: Beta-Talasemi majörlü hastalarda yaşam süresi hipertransfüzyon protokollerinin uygulanmasından sonra belirgin olarak uzamıştır. Bununla birlikte, bu osteoporoz, hipogonadizm gibi endokrin komplikasyonların artmasına yol açmıştır. Komplikasyonların gelişiminde olduğu gibi osteoporozda da aşırı demir birikimi sorumlu tutulmakla birlikte aynı zamanda çok çeşitli faktörlerinde bu konuda etkili olabileceğine inanılmaktadır. Bu çalışmanın amacı beta-talasemi majörlü hastalardan, hipogonadizm gelişen ve gelişmeyen olguların, kemik mineral yoğunluklarını karşılaştırmak ve kemik yapım yıkımını etkileyebilecek faktörleri araştırmaktır. Materyal ve Metod: Talasemi majör tanısı ile izlenen hastalardanpuberteyaş sınırını dolduran veyapuberte muayenesi ile puberteye girdikleri belirlenen, 43 hasta değerlendirmeye alındı. Tüm hastaların kemik mineral yoğunluğu ölçümleri yapıldı. Hormonal değerlerine göre hipogonad olanlar (hasta grubu) ve hipogonad olmayanlar (kontrol grubu) olarak ikiye ayrıldı. Hastaların sonuçları pediatrik endokrinolojide kullanılan kaynak veriler ile değerlendirildi. Bulgular: Hipogonadizm sıklığı % 48 (43 hastanın 21 'i), kemik hastalığı ise % 30 (43 hastanın 13 u) olarak bulundu. Şelasyon tipleri ile ferritin düzeyleri arasında istatistiksel olarak anlamlı bir fark bulunmadı. Sonuç: Bu çalışmada literatürleri incelediğimizde bazı çalışmalarda hipogonadizm ile kemik mineral yoğunluğu arasında anlamlı bir ilişki bulunmuş olmasına rağmen bizim yaptığımız bu çalışmada hipogonadizm gelişmiş olan ile hipogonadizm gelişmemiş olan hasta grupları arasında kemik mineral yoğunluğu açısından anlamlı bir fark olmadığı görülmüştür. Fakat çalışmamızda vit D3 düzeyleri kontrol grubunda hasta grubuna göre daha yüksek bulunmuştur. Bilindiği gibi vit D3 düzeyini etkileyen çok fazla faktör bulunmaktadır. Bu nedenledir ki hipogonadizm gelişmiş olan talasemi majorlu hastalarda vit D3 düzeyi daha düşüktür ve bu hastalara vit D3 desteği yapılmalıdır diyebilmek için diğer-faktörlerin elimine edildiği, çok merkezli prospektif çalışmalara ihtiyaç vardır.

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Kaynakça

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