Neslihan Önenli MUNGAN, Fatih GÜRBÜZ, Eda MENGEN, Özden ÖZGÜR, Ali Kemal TOPALOĞLU, Bilgin YÜKSEL

Osteogenezis İmperfekta Olan Çocuklarda İki Farklı Pamidronat Protokolünün Değerlendirilmesi

Amaç: Osteogenezis imperfekta bağ dokusunun kalıtsal bir hastalığıdır. Bu durumdaki çocuklar tekrarlayan kırıklar, deformiteler, osteoporosis ve ağrıdan yakınırlar. Son yıllarda pamidronat standart tedavi tercihi olmuştur. Ancak, optimal doz ve aralık henüz tam olarak belirlenmemiştir. Bu çalışmada amaç, iki farklı pamidronat tedavi rejiminin karşılaştırılmasıdır. Materyal ve Metod: Yaşları 42.3 ± 37.4 ay arasında değişen toplam 12 hastada yapıldı. Başlangıçta her iki ayda bir defa 1.5 mg/kg/gün dozunda 23.5 ± 9.0 ay pamidromat infüzyonu uygulandı (ilk protokol), daha sonra üçer aylık aralarla 3 gün üstüste 1 mg/kg/gün 18.5 ± 5.1 ay dozuna geçildi (ikinci protokol). Kemik mineral dansitesi z-skoru yıllık değerlendirildi. Bulgular: Yıllık kırık oranı ilk protokolde 6.3 ± 5.5"den 1.1 ± 1.3"e (p=0.001), ikinci protokolde 1.1 ± 1.3"den 0.0 ± 0.0"a (p

Anahtar Kelimeler:

Osteogenesiz imperfekta, pamidronat, çocuklar

Evaluation of Two Different Pamidronate Treatment Protocols in Children with Osteogenesis Imperfecta

Purpose: Osteogenesis imperfecta is an inherited disorder of connective tissue. Children with this condition suffer from recurrent fractures, deformities, osteoporosis and pain. Over the recent years, pamidronate became the standard treatment choice. However the optimal dose and interval have not been defined yet. The main of this study was to compare of two different pamidronate regime. Materials and Methods: 12 patients aged 42.3 ± 37.4 months were studied. At the beginning patients had received pamidronate infusion at a dose of 1.5 mg/kg/day once, every two months with duration of 23.5 ± 9.0 months (first protocol), than switched to a dose of 1mg/kg/day for three consecutive days, every three months with duration of 18.5 ± 5.1 months (second protocol). The bone mineral density Z-score was evaluated yearly. Results: Annual fracture rate decreased from 6.3 ± 5.5 to 1.1 ± 1.3 (p=0.001) in the first and from 1.1 ± 1.3 to 0.0 ± 0.0 (p

Keywords:

Osteogenesis imperfecta, pamidronate, children.,

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Yazışma Adresi / Address for Correspondence: Dr. Fatih GÜRBÜZ Çukurova Üniversitesi Tıp Fakültesi, Çocuk Endokrinolojisi Bilim Dalı, ADANA Email: fggurbuz@yahoo.com G eliş tarihi/Received on: 20.01.2014
Kabul tarihi/Accepted on:27.02.2014