MEHMET KILIÇ, Erdal TAŞKIN, Ahmet SELMANOĞLU

Primer immün yetmezlikli olgularımızın retrospektif değerlendirilmesi

Amaç : Bu çalışmada, kliniğimizde primer immün yetmezlik ta nısıyla izlenen hastaların klinik ve demografik özelliklerinin değerlendirmesi amaçlandı. Gereç ve Yöntem: Şubat 2008 -Mart 2014 tarihleri arasında pediatrik allerji ve immünoloji bölümünde primer immün yetmezlik tanısı konulan toplam 78 hastanın dosya kayıtları retrospektif olarak incelendi. Bulgular: Hastalarımızın %61.5i (n=48) erkek, %38.5i (n=30) kız idi. Hastaların tanı yaşları 5.4 ± 4.0 (0.4 -19) yıl ve şikayetlerinin başladığı yaş ise 3.0 ± 2.9 (0.1 -15) yıl olarak tespit edildi. Başvuru sebepleri sırasıyla; tekrarlayan alt solunum yolu enfeksiyonu %65.4 (n=51), üst solunum yolu enfeksiyonu %55.1 (n=43), gastroenterit %16.7 (n=13), oral kandidiyazis %9 (n=7), tekrarlayan idrar yolu enfeksiyonu %6.4 (n =5) ve egzema %6.4 (n=5) oranında kaydedildi. Hastalarımızın %71.8ini (n=56) antikor eksikliğine bağlı immün yetmezlikler içermekteydi. Diğer iyi tanımlanmış immün yetmezlik sendromları %10.3 (n=8), ağır kombine immün yetmezlikler %7.7 (n=6), diğer immün yetmezlikler %3.8 (n=3), kompleman eksiklikleri %3 .8 (n=3) ve fagositik sistem bozuklukları ise %1.3 (n= 1) oranında idi. Sonuç: Ülkemizde akraba evliliği oranının yüksek olmasından dolayı, primer immün yetmezlik hastalıkları sık görülmektedir. İmmün yet mezliklere bağlı komplikasyonların azaltılabilmesi için hekimlerin erken tanı ve tedavi açısından dikkatli olması gerekmektedir.

The retrospective evaluation of children with primary immunodeficiency disease

Objective: In this study, we aimed to investigate of clinical and demographic characteristics in children with the diagnosis of primary immunodeficiency our clinic. Material and Method : The medical records of 78 patients with primary immunodeficiency who are followed by departme nt of pediatric immunology and allergy between February 2008 and March 2014 were evaluated retrospectively. Results: 61.5% (n=48) of the patients were boys and 38.5% (n=30) were girls. The mean age at the diagnosis was 5.4 ± 4.0 (0.4 -19) years, the mean age at the beginning of symptoms was 3.0 ± 2.9 (0.1 - 15) years. The clinical presentations were lower respiratory tract infections 65.4% (n=51), upper respiratory tract infections 55.1% (n=43), gastroenteritis 16.7% (n=13), oral candidiasis 9% (n=7), recurrent urinary tract infections 6.4% (n=5), and eczema 6.4% (n=5), respectively. 71.8% of patients (n=56) had immunodeficiency due to antibody deficiency. The ratios of the other immunodeficiencies were other well -defined immunodeficiency syndromes 10.3% (n=8 ), severe combined immunodeficiency 7.7% (n=6), other immune deficiencies 3.8% (n=3), complement deficiency 3.8% (n=3), and defects of phagocytic system 1.3 % (n=1). Conclusion: The incidence of primary immunodeficiency is high in our country, due to the high rate of consanguineous marriages . We thought that physician must becareful in terms of early diagnosis and treatment is necessary for reducing the complications due to immune deficiency.

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