Journals / Türk Pediatri Arşivi / 2020 / Cilt: 55 - Sayı: 2

Evaluation of pulmonary findings in patients with humoral immunodeficiency

Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi

Pages
174–183
Publication Date
2022-11-05
DOI
—
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AI Summary & Key Takeaways

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⚡ TL;DR(Too Long; Didn't Read)

Conclusion: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.

📌 3 Key Takeaways

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Objective & Hypothesis:Aim: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency.
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Key Findings & Data:Results: The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency.
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Conclusion & Impact:Conclusion: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Aim: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency. Material and Methods: Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively. Results: The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency. The most common symptom of the patients was chronic cough (n=47, 83.9%). The most common pathologies on high-resolution computed tomography of the chest were atelectasis and bronchiectasis (27.7%). The most common pathology in pulmonary function tests was the presence of moderate obstructive patterns along with restrictive patterns (n=6,12.5%). The FEV1, FVC, and FEF 25–75 values were significantly lower in patients with common variable immunodeficiency compared with the patients who had IgG subset deficiencies (p=0.001, p=0.01, p=0.01). Among the patients who were treated with intravenous immunoglobulin, the age at the diagnosis of immunodeficiency was higher in patients with bronchiectasis (14.2±8.4 years) compared with those without bronchiectasis (10.1±11.4 years) (p=0.04). Conclusion: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.

Özet

Amaç: Humoral immün yetmezlik tanılı olgularımızda, sinopulmonerenfeksiyonların sıklığı ve bunların sonucunda solunum sistemindemeydana gelen değişikliklerin saptanması ve fonksiyonel akciğer kapasitelerinin ölçülmesi amaçlanmıştır.Gereç ve Yöntemler: Çalışmaya humoral immün yetmezlik tanılı 56 olgualındı. Olguların dosya kayıtlarından klinik, laboratuvar, radyolojik görüntüleme bulguları ve solunum fonksiyon testi verileri geriye dönükolarak incelendi.Bulgular: Olguların tanılara göre dağılımı; yaygın değişken immün yetmezlik tanılı 25 olgu, X’e bağlı agamaglobulinemi tanılı üç olgu, Hiperimmunoglobulin M sendromu tanılı beş olgu, İgG alt grup eksikliğitanılı 19 olgu ve selektif immunoglobulin A eksikliği tanılı dört olguşeklindedir. Olguların 37’si (%66,1) erkek 19’u (%33,9) kadın, yaş ortalaması 14,1±10,6 yıldı. Olguların en sık yakınması kronik öksürük idi (n=47,%83,9). Akciğerlerin yüksek çözünürlüklü bilgisayarlı tomografilerindeen sık görülen patoloji atelektazi ve bronşiektaziydi (%27,7). Solunumfonksiyon testlerinde en sık görülen anormallik orta obstrüktif ve ortarestriktif patern birlikteliğiydi (n=6,%12,5). Solunum fonksiyon testibulguları ile radyolojik bulguların her zaman tutarlı olmadığı görüldü.İntravenöz immünglobulin alan olgular arasında yaygın değişken immün yetmezlik tanılı olgularda, immünglobulin G alt grup eksikliği olanolgulara göre solunum fonksiyon testlerinde; FEV 1, FVC ve FEF 25–75değerlerinin anlamlı düşük olduğu saptandı (p=0,001, p=0,01, p=0,01).İntravenöz immünglobulin tedavisi alan olgularda; bronşiektazisi olanların immün yetmezlik tanı yaşı (14,2±8,4 yıl), olmayanlara (10,1±11,4 yıl)göre anlamlı olarak büyüktü (p=0,04).Çıkarımlar: Klinik bulgular solunum sistemindeki yapısal ve fonksiyoneldeğişiklikleri izlemek için yeterli değildir ve olgular gereğinde akciğeryüksek çözünürlüklü bilgisayarlı tomografi ve solunum fonksiyon testleri ile değerlendirilmelidir

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Cite This Article

Yazıcı, Z., Karalı, Y., Karalı, Z., Çekiç, Ş., Canıtez, Y., Sapan, N., Gültekin, S. Ş. K. (2022). Evaluation of pulmonary findings in patients with humoral immunodeficiency. Türk Pediatri Arşivi, Cilt: 55 - Sayı: 2, 174–183. https://ajindex.com/articles/640948

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