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Hastaneye Başvuran Malnutrisyonu ve/veya Tekrarlayan Akciğer Enfeksiyonu Olan Çocuklarda Kistik Fibrozis Sıklığı Araştırılması

The Eveluation of Cystic Fibrosis Frequency in Children with Malnutrition and/ or Recurrent Pulmonary Infection

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Abstract (2. Language): 
Cystic fibrosis (CF) which is occurred by mutations in the gene regulation of transmembrane message shows autosomal recessive inheritance. It is also the most common fatal diseases in white race. The frequency of disease is estimated as 1/2500-1/3500 in white race, 1/1700 in African Americans. But the frequency of CF is not known for our countries. In this study, the frequency of disease was tried to find in children with malnutrition and/or recurrent pulmonary infections who admitted to hospital. This prospective study was conducted between February 2007 and January 2010 and children with malnutrition and/or recurrent pulmonary infections were enrolled the study. The diagnosis CF was made by using positive sweat test and appropriate clinical findings and rule out of other diseases which also caused recurrent pulmonary infections or malnutrition. A total of 491 children were enrolled the study. The mean age of children was 26,1±35.01 months with the range of 2-216 months. Of all children, 335 (68.3%) were male and 156 (31.7%) were female. Positive sweat test was found in 35 (7.1 %) children. The frequency of CF was found to be 5.3 % in children with malnutrition and 8.8 % in children with recurrent pulmonary infections. Finally we emphasized one more times that CF should be keep in mind in children with malnutrition and/or recurrent pulmonary infections due to its early diagnosis and prevention of late complications.
Abstract (Original Language): 
Kistik fibroz (KF), transmembran ileti regülasyonu genindeki mutasyon sonucu oluşur ve otozomal resesif kalıtım gösteren beyaz ırkın en sık rastlanan ölümcül hastalığıdır. Hastalığın sıklığı beyaz ırkta 1/2500-1/3500, Afrika kökenli Amerikalılarda 1/1700 civarındadır. KF’nin ülkemizdeki sıklığı ise bilinmemektedir. Bu çalışmada, hastaneye başvuran tekrarlayan akciğer enfeksiyonu ve/veya malnutisyonu olan çocuklarda Kistik fibrozis sıklığının bulunması amaçlanmıştır. Çalışmaya Şubat 2007 ile Ocak 2010 tarihleri arasında kliniğimize başvuran tekrarlayan akciğer ve/ veya malnutrisyonu olan vakalar alındı. Vakalarda kistik fibrozis tanısı, kistik fibrozis kliniği ile uyumlu bulgulara sahip olma, diğer hastalıkların dışlanması ve ter testi pozitifliği esaslarına göre kondu. Çalışmaya 491 çocuk vaka alındı. Vakaların yaşları minimum 2, maksimum 216 ay olup ortalama 26,1±35.01 ay idi. Vakaların 335 (%68.3)’si erkek, 156 (%31.7)’ü kız idi. Çalışmaya alınan vakalar ter testi sonucuna göre değerlendirildiğinde pozitif ter testi vaka sayısı 35 (%7.1) idi. Tekrarlayan akciğer enfeksiyonu olan çocuklarda KF sıklığı %5.3, malnutrisyonu olanlarda %8.8, malnutrisyonu ve/ veya tekrarlayan akciğer enfeksiyonu olan çocuklarda ise %7.1 (%95 Confidence interval 3.9 -9.2) olarak bulundu. Bu çalışma ile biz hastaneye başvuran malnutrisyon ve/veya tekrarlayan akciğer enfeksiyonu olan olgularda Kistik Fibrozis tanısının erken konulması ve geç komplikasyonlarının önlenmesi için mutlaka akılda tutulması gerektiğini vurgulamak istedik.
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