Servet YEL, Osman EVLİYAOĞLU, Yaşar BOZKURT, Selvi KELEKÇİ, Murat TUTANÇ

Ailesel Akdeniz Ateşi Bulunan Çocuklarda İdrarla Kalsiyum Atılımı

Giriş ve amaç: Genetik geçişli multisistemik bir hastalık olan Ailevi Akdeniz Ateşi (Familial Mediterranean fever, FMF) Türkiye’de seyrek değildir. FMF hastalarında hiperkalsiüri sıklığını belirleyerek bu grup hastalarda böbrek taşı riskini değerlendirmeyi amaçladık.Gereç ve yöntemler: Klinik bulgular ve moleküler genetik çalışmalarla FMF tanısı konup mutasyonları belirlenen 23 atak dönemi FMF’li, 58 remisyonda FMF’li hasta ve 25 adet yaş, cins ve antropometrik ölçümler bakımından benzer sağlıklı kontrol grubu çalışmaya alındı. Öykü, klinik muayene ve akut faz reaktanları (kan sedimantasyon hızı, C-reaktif protein ve fibrinojen düzeyleri) ile atak dönemleri tespit edildi. Olgulara diyet kısıtlanması yapılmadan 24 saatlik idrar kalsiyum düzeyleri ölçüldü.Bulgular: Atak dönemi FMF’li çocuklarda akut faz reaktanları (AFR) düzeyleri, hem ataksız dönem (P<0.001) hem de kontrol grubundan (P<0.001) anlamlı yüksek bulunurken; ataksız dönem ile sağlıklı kontroller arasında AFR düzeyleri bakımından anlamlı fark saptanmadı (P>0.05). Atak dönemlerinde FMF’li hastaların idrar kalsiyum düzeyleri (4.63±1.75 mg/kg/gün), hem ataksız dönemden (3.88±0.97 mg/kg/gün) hem de kontrollerden (3.30±0.82 mg/kg/gün) daha yüksek bulundu. Ancak, sadece atak dönemi FMF’liler ile kontrol grubu arasındaki fark istatistiksel olarak anlamlı düzeye ulaştı (P=0.013). Hiperkalsiüri sıklığı atak sırasında %30, ataklar arasında %26 ve kontrol grubunda %4.5 olarak bulundu.Sonuçlar: Pediatrik FMF hastalarında idrarla kalsiyum atılım fazlalığı dikkate alınıp, kalsiyum fazlalığının metabolik olduğu düşünülerek böbrek taşı riskine karşı dikkatli olunması uygun olacaktır.

Anahtar Kelimeler:

Çocuk, FMF, üriner sistem taş hastalığı, hiperkalsiüri

URINARY CALCIUM EXCRETION IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER

Background and aim: Familial Mediterranean Fever (FMF) is a hereditary multi-systemic disease that is frequent in Turkey. Our aim is to determine the frequency of hypercalciuria and the risk of urinary stone disease in children with FMF. Materials and methods: In our study twenty-three children with acute phase FMF disease and 58 children with remission phase FMF disease were included. They are diagnosed by anamnesis, clinical and molecular genetic studies. 25 age- and gender-matched healthy control children were included. Acute phase reaction was defined by increased acute phase reactants (AFR) including erythrocyte sedimentation rate, C-reactive protein and fibrinogen levels. Results: Children with acute phase FMF had higher AFR levels when compared with both remission phase and the control subjects (P<0.001 for each). However, no difference was found in AFR levels between remission phase FMF and the control subjects (P>0.05). Urinary daily calcium excretion was higher during acute attack (4.63±1.75 mg/kg/day) than those of both remission phase (3.88±0.97 mg/kg/day) and the control group (3.30±0.82 mg/kg/day). However the difference reached to a significant level between acute attack phase and the control subjects (P=0.013). The frequency of hypercalciuria was 30% in acute phase, 26% in remission phase and 4.5% in control subjects.Conclusions: High urinary calcium excretion of FMF children should be taken into account considering elevated urinary calcium as a metabolic risk factor for urinary stone formation

Keywords:

Children, FMF, urinary stone disease, hypercalciuria,

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