Primary immunodeficiency disorders in children with Non-Cystic Fibrosis Bronchiectasis
D. Çağdaş cagdasdenizna@yahoo.com1, M. Pehlivantürk Kızılkan2, A. Tagiyev2, N. Emiralioğlu3, A. Keleş4, E. Yalçın3, D. Doğru3, U. Özçelik3, N. Kiper3, İ. Tezcan1Show more: Authors information and Publication history1Hacettepe University Medical School, Department of Pediatric Immunology, Ankara, Turkey
2Hacettepe University Medical School, Department of Pediatrics, Ankara, Turkey
3Hacettepe University Medical School, Department of Pediatric Pulmonology, Ankara, Turkey
4Hacettepe University Medical School, Ankara, Turkey
History:
Published online: 30 April 2020
Accepted:28 April
Received: 21 March 2020
Doihttp://doi.org/10.23822/EurAnnACI.1764-1489.151 SummaryIntroduction. Primary Immunodeficiency diseases(PID) are common in patients with non-cystic fibrosis bronchiectasis(NCFB). Our objective was to determine ratio/types of PID in NCFB.
Patients. Seventy NCFB patients followed up in a two-year period were enrolled.
Results. Median age was 14 years (min.-max., 6-30). Patients had their first pulmonary infection at a median age of 6 months(min.-max., 0.5-84), were diagnosed with bronchiectasis at about 9 years(114 months)(min.-max., 2-276)). Male/female ratio was 39/31; parental consanguinity, 38.6%. PID, primary ciliary dyskinesis (PCD), bronchiolitis obliterans, rheumatic/autoimmune diseases, severe congenital heart disease and tuberculosis were evaluated as the most common causes of NCFB. About 40% of patients (n=16) had bronchial hyperreactivity(BH) and asthma. Twenty-nine patients(41.4%) had a PID, and nearly all (n=28) had primary antibody deficiency, including patients with combined T and B cell deficiency. PID and non-PID groups did not differ according to gender, parental consanguinity, age at first pneumonia, age of onset of chronic pulmonary symptoms, bronchiectasis, presence of gastroesophageal reflux disease(GERD), bronchial hyperreactivity(BH) and asthma (p>0.05). Admission to immunology clinic was about 3 years later in PID compared with non-PID group(p<0.001). Five patients got molecular diagnosis, X-linked agammaglobulinemia(n=2), LRBA deficiency(n=1), RASGRP1 deficiency(n=1), MHC Class II deficiency(n=1). They were given monthly IVIG and HSCT was performed for three patients.
Conclusions. PID accounted for about 40% of NCFB. Early diagnosis/appropriate treatment have impact on clinical course of a PID patient. Thus, follow-up in also immunology clinics should be a routine for patients who experience pneumonia in the first year of their lives and those with NCFB. Most patients with NCFB (84.28%) had their first pulmonary infection within the first year of their lives.
Key wordsNon-Cystic Fibrosis Bronchiectasis, primary immunodeficiency, bronchiectasis, respiratory infections
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