Atilla ÇAYIR, Oğuzhan SEPETÇİĞİL, Avni KAYA, Celalettin KOŞAN, Behzat ÖZKAN

Ailevi Akdeniz Ateşi Olan Çocuklarda Kemik Metabolizmasının Değerlendirilmesi

Giriş-Amaç: Ailevi Akdeniz ateşi (AAA), tekrarlayan ateş ve poliserozit atakları ile kendini gösteren otozomal resesif geçişli, periyodik enflamatuvar bir hastalıktır. Ataklar dışındaki dönemde AAA’lı hastalar klinik olarak tamamen normale dönseler bile, ataksız dönemlerde de subklinik enflamasyonun devam ettiği gösterilmiştir. Bu çalışmada, AAA’lı çocuklarda AAA hastalığının kemik metabolizmasına olan etkilerinin değerlendirilmesi amaçlandı.Gereç ve Yöntem: Bu çalışmaya AAA tanısı ile takip edilen ve düzenli kolşisin tedavisi alan ataksız dönemdeki 35 puberte öncesi hasta ile yaş ve cinsiyeti benzer 30 sağlıklı çocuk kontrol grubu olarak alındı. Çalışma grubundaki tüm olguların fizik muayenesi yapılarak, hastalıkları ile bilgileri kaydedildi. Hastaların biyokimyasal tetkikleri, kemik döngüsü parametreleri, kemik yaşı ve lumbar 1-4 kemik mineral dansitesileri (KMD) değerlendirildi.Bulgular: Çalışma ve kontrol grubu arasında boy, kilo, kemik yaşı ve KMD açısından anlamlı bir farklılık yoktu (p>0,05). Olguların değerlendirilmesinde 25-OH D vitamini düzeyi ile kemik mineral içeriği arasında pozitif korelasyon tesbit edildi. AAA’lı olguların 5’inde (%14) kemik yaşının kronolojik yaşa göre geri olduğu ve ayrıca 2’sinde (%5) KMD Z skorunun -2’nin altında olduğu görüldü.Sonuçlar: Düzenli kolşisin tedavisi altındaki AAA’lı çocuklarda KMD ve kemik döngüsü parametrelerinin benzer olduğu saptandı. Bazı hastalarda görülen kemik yaşı geriliği ve KMD düşüklüğü, subklinik enflamasyona ikincil etkilenme olduğunu düşündürmektedir.

Anahtar Kelimeler:

Ailevi Akdeniz ateşi, kolşisin, kemik metabolizması

EVULATION BONE METABOLISM IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER

Introduction-Aim: Familial Mediterranean Fever (FMF) is a periodic inflammatory disease characterized by recurrent febrile attacks and polyserositis; inherited in an autosomal recessive manner. Recent studies confirmed that chronic subclinic inflammation is frequently present between febrile episodes in patients with FMF, although FMF patients are usually clinically asymptomatic. The aim of the present study was to investigate the effects of FMF on bone metabolism in children with FMF. Material-Methods: Thirty five prepubertal FMF patients in attack free period who were receiving regular colchicine therapy and 30 healthy children with similar age and gender were included in this study as study and control groups. All patients’ physical examination was performed and all data about symptoms at admission and positive findings in their physical examination were recorded. Additionally, biochemical parameters, bone turnover markers, bone age, and bone mineral density (BMD) obtained at lumbar 1-4 levels were evaluated for each patient. Findings: There was no statistically significant difference between height, weight, bone age, and BMD values of study and control groups (p>0.05). There was a positive correlation between vitamin D level and mean BMD values obtained at L1-L4 levels (p: 0.024, r: 0.38). A regression in bone age according to the chronologic age was detected in 5 (14%) and BMD Z scores lower than -2 SDS was detected in 2 (5%) FMF patients. Conclusions: BMD and bone turnover markers of children with FMF receiving regular colchicine therapy didn’t differ from those of healthy children. Bone age regression and low BMD Z scores in some FMF patients may be the secondary effects of subclinical inflammation

Keywords:

Familial Mediterranean fever, colchicine, bone mineralization,

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