Humoral immun yetmezlikli hastalarda akciğer bulgularının değerlendirilmesi
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Date
2010
Authors
Karalı, Zuhal
Journal Title
Journal ISSN
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Publisher
Uludağ Üniversitesi
Abstract
Humoral immün yetmezlikler, primer immün yetmezliklerin %50-60'ını oluşturur. Klinik olarak tekrarlayan üst ve alt solunum yolu enfeksiyonuna neden olarak, akciğer harabiyetine yol açabilmektedir Bu hastalıkta tanı ve tedavinin erken dönemde yapılması, özellikle akciğer hasarının gelişimi ve ilerlemesini engeller.Çalışmamızın amacı humoral immün yetmezlikli hastalarımızın arşiv kayıtlarından geçirilen sinopulmoner enfeksiyonların sıklığı ve bunların sonucunda akciğerlerde meydana gelen parankimal, plevral ve mediastinal patolojik değişiklikleri, görüntüleme yöntemleri ile saptamak, aynı zamanda akciğer fonksiyonel kapasitelerini solunum fonksiyon testleri ile değerlendirmektir.Bu çalışmada, Uludağ Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Pediatrik İmmunoloji polikliniğinden humoral immun yetmezlik tanısı ile takip edilen bazı hastaların arşiv kayıtlarından demografik, klinik, laboratuvar, radyolojik görüntüleme bulguları ve solunum fonksiyon testleri verileri retrospektif olarak incelenmiştir.Çalışmamıza toplam 56 humoral immün yetmezlikli hasta alındı. Çalışma grubumuz; Yaygın Değişken İmmün Yetmezlik (YDİY)'li 25 hasta, X'e bağlı agamaglobulinemi'li (XLA) 3 hasta, Hiper immünglobulin M sendrom'lu 5 hasta, immünglobulin G alt grup eksikliği tanılı 19, selektif IgA eksikliği tanılı 4 hastadan oluşmaktaydı.Hastalardan 54'ünün akciğer HRCT bulguları değerlendirildi. Akciğer tomografilerinde en sık görülen patoloji %27.7 ile (15/54) atelektazi olup bunu %25.9 (14/54) oranıyla bronşektazi izlemektedir. Çalışmamızdaki tüm hastalar değerlendirildiğinde bronşektazi en sık (%57.5) alt loblarda görüldü. Özellikle YDİY'li hastalarımızın çoğunun HRCT`lerinde patoloji saptandı.Hastaların 48'ine (%85.7) solunum fonksiyon testi yapıldı ve 20'sinin (%41.7) solunum fonksiyon testinin normal olmadığı saptandı. En sık görülen bozukluk orta obstrüktif ve orta restriktif patern birlikteliğiydi.YDİY`li 4, Hiper IgM tanılı 2 hastanın akciğer tomografilerinde bronşektazi, ?tree in bud?, buzlu cam görünümü gibi patolojik bulgular olmasına rağmen SFT'leri normal saptandı. Benzer şekilde SFT'de, YDİY`li 3 hastada restriktif, Hiper IgM tanılı 1 hastada restriktif ve obstrüktif patern birlikteliği varken, akciğer tomografileri normal saptandı. Çalışmamızda solunum fonksiyon testi (SFT) ile radyolojik bulguların her zaman tutarlı olmadığı görüldü.Sonuç olarak; humoral immün yetmezliği olan hastaların akciğer bulgularının değerlendirilmesi ve buna yönelik tedavi planlanması prognoz açısından son derece önemlidir. İmmün yetmezlikli hastalarda, solunum sistemi ile ilgili semptom olmaksızın belli aralıklarla yapılan HRCT ve SFT, sessiz seyreden patolojik bulguları ortaya koyan ve tedaviyi yönlendirebilen önemli testlerdir. Bu nedenle bu hastalarda akciğer bulgularının değerlendirilmesinde klinik bulgular olmasa da mutlaka belirli aralıklarla akciğerler görüntülenmeli ve eş zamanlı olarak SFT yapılmalıdır.
Humoral immunodeficiencies with antibody production defects constitude 50-60% of all primary immunodeficiencies. Inclinically, they cause to recurrent upper and lower respiratory infections and subsequent structural lung damage immediate diagnosis and treatment prevents development and progression of lung damage. Our objective is to analyze clinical records of our humoral immunodeficiency patients and describe frequency of sinopulmonary infections and consequencies such as paranchymal, pleural, mediastinal pathological changes. At the same time, we aimed to evaluate functional capacity of lungs by pulmonary function tests. We retrospectively assessed demographic, clinical, laboratory, radiological findings and pulmonary function tests of humoral immunodeficiency patients who are followed in Pediatric Immunology Outpatient Clinics in Uludag University Faculty of Medicine. The study comprised 56 patients; 25 with common variable immunodeficiency (CVĠD), 3 with X linked agammaglobulinemia, 5 with Hyper Immunglobulin M (IgM) syndrome, 19 with Immunoglobulin subgroup deficiencies and 4 with selective Immunglobulin A deficiency. Among the 54 patients who were analyzed with high resolution computed tomography (HRCT), the leading finding was atelectasis (27.7%- 15/54), followed by bronchiectasis (25.9%-14/54). Bronchiectasis mostly affected the lower lobes (57.5%) in whole patient group. Particulary, most of the common variable immunodeficiency patients had some patologies in their high resolution computed tomography. Fourty-eight patients (85.7%) were evaluated by pulmonary function tests (PFT) in which twenty of them (41.7%) had abnormal results. The most frequent finding was moderate obstructive with moderate restrictive pattern. Four patients with CVĠD and two patients with Hyper IgM syndrome had bronchiectasis, tree in bud and ground-glass opacities in HRCT while they had normal pulmonary function tests. Similarly, three patients with CVĠD had restrictive, one patient with Hyper IgM syndrome had moderate restrictive with moderate obstructive pattern findings in pulmonary function tests whereas they had normal HRCT. Pulmonary function tests were not consistent with radiological findings in all cases. In conclusion, evaluation of pulmonary findings and planning of the treatment according to these findings is very important in patients with humoral immunodeficiency. Serially taken HRCT‟s and PFT‟s, even if the absence of symptoms, tools which may reveal silent pathological findings and thus may guide the treatment approaches. Consequently, serial pulmonary imaging studies and PFT‟s should be considered for the evaluations of pulmonary findings in patients with humoral immune deficiency, even in the abscence of clinical findings.
Humoral immunodeficiencies with antibody production defects constitude 50-60% of all primary immunodeficiencies. Inclinically, they cause to recurrent upper and lower respiratory infections and subsequent structural lung damage immediate diagnosis and treatment prevents development and progression of lung damage. Our objective is to analyze clinical records of our humoral immunodeficiency patients and describe frequency of sinopulmonary infections and consequencies such as paranchymal, pleural, mediastinal pathological changes. At the same time, we aimed to evaluate functional capacity of lungs by pulmonary function tests. We retrospectively assessed demographic, clinical, laboratory, radiological findings and pulmonary function tests of humoral immunodeficiency patients who are followed in Pediatric Immunology Outpatient Clinics in Uludag University Faculty of Medicine. The study comprised 56 patients; 25 with common variable immunodeficiency (CVĠD), 3 with X linked agammaglobulinemia, 5 with Hyper Immunglobulin M (IgM) syndrome, 19 with Immunoglobulin subgroup deficiencies and 4 with selective Immunglobulin A deficiency. Among the 54 patients who were analyzed with high resolution computed tomography (HRCT), the leading finding was atelectasis (27.7%- 15/54), followed by bronchiectasis (25.9%-14/54). Bronchiectasis mostly affected the lower lobes (57.5%) in whole patient group. Particulary, most of the common variable immunodeficiency patients had some patologies in their high resolution computed tomography. Fourty-eight patients (85.7%) were evaluated by pulmonary function tests (PFT) in which twenty of them (41.7%) had abnormal results. The most frequent finding was moderate obstructive with moderate restrictive pattern. Four patients with CVĠD and two patients with Hyper IgM syndrome had bronchiectasis, tree in bud and ground-glass opacities in HRCT while they had normal pulmonary function tests. Similarly, three patients with CVĠD had restrictive, one patient with Hyper IgM syndrome had moderate restrictive with moderate obstructive pattern findings in pulmonary function tests whereas they had normal HRCT. Pulmonary function tests were not consistent with radiological findings in all cases. In conclusion, evaluation of pulmonary findings and planning of the treatment according to these findings is very important in patients with humoral immunodeficiency. Serially taken HRCT‟s and PFT‟s, even if the absence of symptoms, tools which may reveal silent pathological findings and thus may guide the treatment approaches. Consequently, serial pulmonary imaging studies and PFT‟s should be considered for the evaluations of pulmonary findings in patients with humoral immune deficiency, even in the abscence of clinical findings.
Description
Keywords
Humoral immün yetmezlik, Akciğer HRCT, Solunum fonksiyon testi, Alerji, Humoral immunodeficiency, Lung high resolution computed tomography, Pulmonary function tests, Allergy
Citation
Karalı, Z. (2010). Humoral immun yetmezlikli hastalarda akciğer bulgularının değerlendirilmesi. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.