Agammaglobulinemik hastalarımızın retrospektif olarak değerlendirilmesi
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Tarih
2024
Yazarlar
Dergi Başlığı
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Cilt Başlığı
Yayıncı
Necmettin Erbakan Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Agammaglobulinemi, serum immünoglobulin düzeylerinde ciddi düşüklüğün saptandığı ve sıklıkla tekrarlayan enfeksiyon kliniği ile başvuruya neden olan bir durumdur. Çalışmamızın amacı agammaglobulinemi saptanan çocuk olguların demografik, klinik ve laboratuvar bulgularını retrospektif olarak inceleyerek agammaglobulinemik hastaların özelliklerinin daha iyi anlaşılmasına katkı sağlamaktır. Çalışmamıza Necmettin Erbakan Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Alerji ve İmmunoloji Bilim Dalı bölümünde takip edilen ve serum IgG değeri 400 mg/dl'nin altı olup, IgA ve IgM değerleri yaşa göre olması gereken değerin 2 standart sapmasının altında ölçülerek agammaglobulinemi tanısı konulan 25 hasta dahil edilmiştir.
Agammaglobulinemik hastalarımızın %80’i erkek olup, ortalama tanı yaşı 62,5±65,1 ay ve ortalama izlem süresi 9,1±5,8 yıl idi. Hastaların %36’sı klasik agammaglobulinemik hastalar (grup 1) ve %64’ü diğer agammaglobulinemik hastalar (grup 2) olarak değerlendirildi. Grup 2'deki hastalarımızın %40’ı yaygın değişken immün yetmezlik (YDİY) (grup 2a) ve %24'ü sendromik özellikli agammaglobulinemi (grup 2b) idi. Grup 2’de ebeveyn akrabalığı anlamlı derecede yüksekken (p=0,021), ailede nedeni bilinmeyen ölüm öyküsü grup 1’de anlamlı derecede yüksekti (p=0,033). Başvurudaki boy ve vücut ağırlığı, grup 2b'de anlamlı derecede düşüktü (p=0,013 ve p=0,012). Tekrarlayan enfeksiyonlardan otitis media öyküsü grup 1’de anlamlı derecede yüksekti (p=0,016). Gruplar arasında sendromik görünüm, bronşiektazi, lenfadenopati, eşlik eden otoimmün hastalık açısından anlamlı fark izlenmedi. Grup 2b’de trombosit sayısı diğer gruplardan düşük bulundu (p<0,05). İmmünglobulin replasman tedavisi alan hastaların tedavi süresi grup 1’de daha uzundu (p=0,049). Dokuz yıllık takip süremizde agammaglobulinemik hastalarımızda ölüm oranı %20 olup gruplar arasında fark saptanmadı.
Sonuç olarak Bruton hastalığı dışında YDİY ve sendromik immün yetmezliklerin de agammaglobulinemi ile seyredebileceğini vurgulamak ve agammaglobulinemik seyreden hastalıkların daha iyi anlaşılmasına katkı sağlamak istiyoruz.
Agammaglobulinemia is a condition in which serum immünoglobulin levels are severely low and frequently leads to presentation with recurrent infection clinic. The aim of our study was to contribute to a better understanding of the characteristics of agammaglobulinemic patients by retrospectively analyzing the demographic, clinical and laboratory findings of pediatric patients with agammaglobulinemia. The study included 25 patients who were followed up in Necmettin Erbakan University, Faculty of Medicine, Department of Pediatrics, Division of Pediatric Allergy and İmmünology and diagnosed with agammaglobulinemia with serum IgG values below 400 mg/dl and IgA and IgM values below 2 standard deviations of the age-appropriate value. Eighty percent of our agammaglobulinemic patients were male, the mean age at diagnosis was 62.5±65.1 months and the mean follow-up period was 9.1±5.8 years. Of the patients, 36% were classical agammaglobulinemic patients (group 1) and 64% were other agammaglobulinemic patients (group 2). In group 2, 40% of our patients had diffuse variable immünodeficiency (DVID) (group 2a) and 24% had agammaglobulinemia with syndromic features (group 2b). Parental consanguinity was significantly higher in group 2 (p=0.021), while family history of death of unknown cause was significantly higher in group 1 (p=0.033). Height and body weight at presentation were significantly lower in group 2b (p=0.013 and p=0.012). History of otitis media among recurrent infections was significantly higher in group 1 (p=0.016). No significant difference was observed between the groups in terms of syndromic appearance, bronchiectasis, lymphadenopathy and accompanying autoimmüne disease. Platelet count in group 2b was lower than the other groups (p<0.05). The duration of immünoglobulin replacement therapy was longer in group 1 (p=0.049). The mortality rate was 20% in our agammaglobulinemic patients during our nine-year follow-up period and no difference was found between the groups. In conclusion, we would like to emphasize that besides Bruton's disease, SIDS and syndromic immunodeficiencies may also present with agammaglobulinemia and contribute to a better understanding of diseases with agammaglobulinemic course.
Agammaglobulinemia is a condition in which serum immünoglobulin levels are severely low and frequently leads to presentation with recurrent infection clinic. The aim of our study was to contribute to a better understanding of the characteristics of agammaglobulinemic patients by retrospectively analyzing the demographic, clinical and laboratory findings of pediatric patients with agammaglobulinemia. The study included 25 patients who were followed up in Necmettin Erbakan University, Faculty of Medicine, Department of Pediatrics, Division of Pediatric Allergy and İmmünology and diagnosed with agammaglobulinemia with serum IgG values below 400 mg/dl and IgA and IgM values below 2 standard deviations of the age-appropriate value. Eighty percent of our agammaglobulinemic patients were male, the mean age at diagnosis was 62.5±65.1 months and the mean follow-up period was 9.1±5.8 years. Of the patients, 36% were classical agammaglobulinemic patients (group 1) and 64% were other agammaglobulinemic patients (group 2). In group 2, 40% of our patients had diffuse variable immünodeficiency (DVID) (group 2a) and 24% had agammaglobulinemia with syndromic features (group 2b). Parental consanguinity was significantly higher in group 2 (p=0.021), while family history of death of unknown cause was significantly higher in group 1 (p=0.033). Height and body weight at presentation were significantly lower in group 2b (p=0.013 and p=0.012). History of otitis media among recurrent infections was significantly higher in group 1 (p=0.016). No significant difference was observed between the groups in terms of syndromic appearance, bronchiectasis, lymphadenopathy and accompanying autoimmüne disease. Platelet count in group 2b was lower than the other groups (p<0.05). The duration of immünoglobulin replacement therapy was longer in group 1 (p=0.049). The mortality rate was 20% in our agammaglobulinemic patients during our nine-year follow-up period and no difference was found between the groups. In conclusion, we would like to emphasize that besides Bruton's disease, SIDS and syndromic immunodeficiencies may also present with agammaglobulinemia and contribute to a better understanding of diseases with agammaglobulinemic course.
Açıklama
Anahtar Kelimeler
İmmün yetmezlikler, İmmundeficiency, agammaglobulinemi, agammaglobulinemia, çocuk, children
Kaynak
WoS Q Değeri
Scopus Q Değeri
Cilt
Sayı
Künye
Akkuş, T. (2024). Agammaglobulinemik hastalarımızın retrospektif olarak değerlendirilmesi. (Yayınlanmamış tıpta uzmanlık tezi) Necmettin Erbakan Üniversitesi, Tıp Fakültesi Dahili Tıp Bilimleri Bölümü Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Konya.
