Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi
Yıl: 2020 Cilt: 55 Sayı: 2 Sayfa Aralığı: 174 - 183 Metin Dili: Türkçe DOI: 10.14744/TurkPediatriArs.2020.46656 İndeks Tarihi: 19-08-2020
Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi
Öz: 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
Anahtar Kelime: Evaluation of pulmonary findings in patients with humoral immunodeficiency
Öz: 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.
Anahtar Kelime: Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
- 1. McCusker C, Upton J, Warrington R. Primary immunodeficiency. Allergy Asthma Clin Immunol 2018; 14: 61.
- 2. Stiehm ER, Ochs HD, Winkelstein J. Immunodeficiency disorders; general consideration. In: Ochs HD, Stiehm ER, Winkelstein J, editors. Immunologic disorders in infant and children. 5th ed. Pennsylvania: Elsevier Saunders Company; 2004.p.652–84.
- 3. Gathmann B, Grimbacher B, Beauté J, et al; ESID Registry Working Party. The European internet-based patient and research database for primary immunodeficiencies: results 2006-2008. Clin Exp Immunol 2009; 157: 3−11.
- 4. Kilic SS, Ozel M, Hafizoglu D, Karaca NE, Aksu G, Kutukculer N. The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey--two centers study. J Clin Immunol 2013; 33: 74−83.
- 5. Yorulmaz A, Artaç H, Kara R, Keleş S, Reisli İ. Primer immün yetmezlikli 1054 olgunun retrospektif değerlendirilmesi. Astım Allerji İmmünoloji 2008; 6: 127−34.
- 6. Bousfiha A, Jeddane L, Picard C, et al. The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies. J Clin Immunol 2018; 38: 129–43.
- 7. Yazdani R, Abolhassani H, Asgardoon MH, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol 2017; 27: 2130−224.
- 8. Alkan G, Keles S, Reisli İ. Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency. Int J Pediatr 2018; 2018: 3527480.
- 9. Çalişkaner AZ, Reisli İ, Arslan Ş, Uçar R, Ataseven H, Selçuk NY. Common variable immunodeficiency in adults requires reserved protocols for long-term follow-up. Turk J Med Sci 2016; 46: 430−6.
- 10. Cinetto F, Scarpa R, Rattazzi M, Agostini C. The broad spectrum of lung diseases in primary antibody deficiencies. Eur Respir Rev 2018; 27: 180019.
- 11. Sperlich JM, Grimbacher B, Workman S, et al. Respiratory Infections and Antibiotic Usage in Common Variable Immunodeficiency. J Allergy Clin Immunol Pract 2018; 6: 159–68.e3.
- 12. Mooney D, Edgar D, Einarsson G, Downey D, Elborn S, Tunney M. Chronic lung disease in common variable immune deficiency (CVID): A pathophysiological role for microbial and non-B cell immune factors. Crit Rev Microbiol 2017; 43: 508−19.
- 13. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J 2005; 26: 948−68.
- 14. Jesenak M, Banovcin P, Jesenakova B, Babusikova E. Pulmonary manifestations of primary immunodeficiency disorders in children. Front Pediatr 2014; 2: 77.
- 15. Rusconi F, Panisi C, Dellepiane RM, et al. Pulmonary and sinus diseases in primary humoral immunodeficiencies with chronic productive cough. Arch Dis Child 2003; 88: 1101−5.
- 16. Thickett KM, Kumararatne DS, Banerjee AK, Dudley R, Stableforth DE. Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings. QJM 2002; 95: 655−62.,
- 17. Aghamohammadi A, Allahverdi A, Abolhassani H, et al. Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia. Respirology 2010; 15: 289−95.
- 18. Costa-Carvalho BT, Wandalsen GF, Pulici G, Aranda CS, Solé D. Pulmonary complications in patients with antibody deficiency. Allergol Immunopathol (Madr) 2011; 39: 128−32.
- 19. Papadopoulou A, Mermiri D, Taousani S, Triga M, Nicolaidou P, Priftis KN. Bronchial hyper-responsiveness in selective IgA deficiency. Pediatr Allergy Immunol 2005; 16: 495−500.
- 20. Milota T, Bloomfield M, Parackova Z, Sediva A, Bartunkova J, Horvath R. Bronchial Asthma and Bronchial Hyperresponsiveness and Their Characteristics in Patients with Common Variable Immunodeficiency. Int Arch Allergy Immunol 2019; 178: 192−200.
- 21. Busse PJ, Farzan S, Cunningham-Rundles C. Pulmonary complications of common variable immunodeficiency. Ann Allergy Asthma Immunol 2007; 98: 1−43.
- 22. Agondi RC, Barros MT, Rizzo LV, Kalil J, Giavina-Bianchi P. Allergic asthma in patients with common variable immunodeficiency. Allergy 2010; 65: 510−5.
- 23. Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract 2016; 4: 38−59.
- 24. Loftus BG, Price JF, Lobo-Yeo A, Vergani D. IgG subclass deficiency in asthma. Arch Dis Child 1988; 63: 1434−7.
- 25. Kim JH, Ye YM, Ban GY, et al. Effects of Immunoglobulin Replacement on Asthma Exacerbation in Adult Asthmatics with IgG Subclass Deficiency. Allergy Asthma Immunol Res 2017; 9: 526−33.
- 26. de Moraes Lui C, Oliveira LC, Diogo CL, Kirschfink M, Grumach AS. Immunoglobulin G subclass concentrations and infections in children and adolescents with severe asthma. Pediatr Allergy Immunol 2002; 13: 195−202.
- 27. Ballow M. Practical aspects of immunoglobulin replacement. Ann Allergy Asthma Immunol. 2017; 119: 299−303.
- 28. Kainulainen L, Varpula M, Liippo K, Svedström E, Nikoskelainen J, Ruuskanen O. Pulmonary abnormalities in patients with primary hypogammaglobulinemia. J Allergy Clin Immunol 1999; 104: 1031−6.
- 29. Curtin JJ, Webster AD, Farrant J, Katz D. Bronchiectasis in hypogammaglobulinaemia--a computed tomography assessment. Clin Radiol 1991; 44: 82−4.
- 30. Dukes RJ, Rosenow EC 3rd, Hermans PE. Pulmonary manifestations of hypogammaglobulinaemia. Thorax 1978; 33: 603−7.
- 31. Martínez García MA, de Rojas MD, Nauffal Manzur MD, et al. Respiratory disorders in common variable immunodeficiency. Respir Med. 2001; 95: 191−5.
- 32. Feydy A, Sibilia J, De Kerviler E, et al. Chest high resolution CT in adults with primary humoral immunodeficiency. Br J Radiol 1996; 69: 1108−16.
- 33. Sweinberg SK, Wodell RA, Grodofsky MP, Greene JM, Conley ME. Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia. J Allergy Clin Immunol 1991; 88: 96−104.
- 34. Obregon RG, Lynch DA, Kaske T, Newell JD Jr, Kirkpatrick CH. Radiologic findings of adult primary immunodeficiency disorders. Contribution of CT. Chest 1994; 106: 490−5.
- 35. Reiff DB, Wells AU, Carr DH, Cole PJ, Hansell DM. CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types. AJR Am J Roentgenol 1995; 165: 261−7.
- 36. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. International Union of Immunological Societies. Clin Exp Immunol 1999; 118: 1−28.
- 37. Cunningham-Rundles C. Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. J Clin Immunol 1989; 9: 22–33.
- 38. Cunningham-Rundles C, Bodian C. Common Variable imunodeficiency: clinical and immunological features of 248 patients. Clin Immunol 1999; 92: 34−48.
- 39. Mullighan CG, Fanning GC, Chapel HM, Welsh KI. TNF and lymphotoxin-alpha polymorphisms associated with common variable immunodeficiency: role in the pathogenesis of granulomatous disease. J Immunol 1997; 159: 6236−41.
- 40. Mechanic LJ, Dikman S, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Intern Med 1997; 127: 613−7.
- 41. Park JE, Beal I, Dilworth JP, Tormey V, Haddock J. The HRCT appearances of granulomatous pulmonary disease in common variable immune deficiency. Eur J Radiol 2005; 54: 359−64.
- 42. Gibson M, Hansell DM. Lymphocytic disorders of the chest: pathology and imaging. Clin Radiol 1998; 53: 469−80.
- 43. Davies CW, Juniper MC, Gray W, Gleeson FV, Chapel HM, Davies RJ. Lymphoid interstitial pneumonitis associated with common variable hypogammaglobulinaemia treated with cyclosporin A. Thorax 2000;55: 88−90.
- 44. Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med 1993; 86: 31−42.
- 45. Buckley RH. Pulmonary complications of primary immunodeficiencies. Paediatr Respir Rev 2004; 5: S225−33.
- 46. Tuncel E. Klinik radyoloji. Ankara: Nobel Tıp Güneş Yayınları; 2008.p. 278−9.
- 47. Boloursaz MR, Khalilzadeh S, Nezhad PR, et al. Chest CT Manifestations in Children with CVID: A 10-Year Report. Tanaffos 2012; 11: 56−9.
- 48. Webb WR, Higgins CB. Thoracic Imaging: Pulmonary and Cardiovascular Radiology. Philadelphia: Lippincott Williams and Wilkins; 2005.p.321−2.
- 49. Miller WT Jr, Panosian JS. Causes and imaging patterns of tree-in-bud opacities. Chest 2013; 144: 1883−92.
- 50. Watts WJ, Watts MB, Dai W, Cassidy JT, Grum CM, Weg JG. Respiratory dysfunction in patients with common variable hypogammaglobulinemia. Am Rev Respir Dis 1986; 134: 699−703.
| APA | Karalı Z, Karali Y, ÇEKİÇ Ş, yazıcı z, CANITEZ Y, Sapan N, GÜLTEKİN S (2020). Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. , 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| Chicago | Karalı Zuhal,Karali Yasin,ÇEKİÇ Şükrü,yazıcı zeynep,CANITEZ Yakup,Sapan Nihat,GÜLTEKİN Sara Şebnem Kılıç Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. (2020): 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| MLA | Karalı Zuhal,Karali Yasin,ÇEKİÇ Şükrü,yazıcı zeynep,CANITEZ Yakup,Sapan Nihat,GÜLTEKİN Sara Şebnem Kılıç Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. , 2020, ss.174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| AMA | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. . 2020; 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| Vancouver | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. . 2020; 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| IEEE | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S "Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi." , ss.174 - 183, 2020. 10.14744/TurkPediatriArs.2020.46656 |
| ISNAD | Karalı, Zuhal vd. "Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi". (2020), 174-183. https://doi.org/10.14744/TurkPediatriArs.2020.46656 |
| APA | Karalı Z, Karali Y, ÇEKİÇ Ş, yazıcı z, CANITEZ Y, Sapan N, GÜLTEKİN S (2020). Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. Türk Pediatri Arşivi, 55(2), 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| Chicago | Karalı Zuhal,Karali Yasin,ÇEKİÇ Şükrü,yazıcı zeynep,CANITEZ Yakup,Sapan Nihat,GÜLTEKİN Sara Şebnem Kılıç Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. Türk Pediatri Arşivi 55, no.2 (2020): 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| MLA | Karalı Zuhal,Karali Yasin,ÇEKİÇ Şükrü,yazıcı zeynep,CANITEZ Yakup,Sapan Nihat,GÜLTEKİN Sara Şebnem Kılıç Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. Türk Pediatri Arşivi, vol.55, no.2, 2020, ss.174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| AMA | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. Türk Pediatri Arşivi. 2020; 55(2): 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| Vancouver | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi. Türk Pediatri Arşivi. 2020; 55(2): 174 - 183. 10.14744/TurkPediatriArs.2020.46656 |
| IEEE | Karalı Z,Karali Y,ÇEKİÇ Ş,yazıcı z,CANITEZ Y,Sapan N,GÜLTEKİN S "Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi." Türk Pediatri Arşivi, 55, ss.174 - 183, 2020. 10.14744/TurkPediatriArs.2020.46656 |
| ISNAD | Karalı, Zuhal vd. "Humoral immün yetmezlikli olgularda akciğer bulgularının değerlendirilmesi". Türk Pediatri Arşivi 55/2 (2020), 174-183. https://doi.org/10.14744/TurkPediatriArs.2020.46656 |
