Primary Immunodeficiency Disorders in children with Non-Cystic Fibrosis BronchiectasisD. Çağdaş email@example.com, M. Pehlivantürk Kızılkan2, A. Tagiyev2, N. Emiralioğlu3, A. Keleş4, E. Yalçın3, D. Doğru3, U. Özçelik3, N. Kiper3, İ. Tezcan1Show more: Authors information and Publication history
Department of Pediatrics, Division of Pediatric Immunology, Hacettepe University Medical School, Ankara, Turkey2
Department of Pediatrics, Hacettepe University Medical School, Ankara, Turkey3
Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University Medical School, Ankara, Turkey4
Hacettepe University Medical School, Ankara, TurkeyHistory:
Published online: 02 November 2020
Accepted: 28 April 2020
Received: 21 March 2020
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. Methods.
Seventy NCFB patients followed up in a two-year period were enrolled. Results.
Median age was 14 years (min-max: 6-30). Male/female ratio was 39/31; parental consanguinity, 38.6%. Most patients with NCFB (84.28%) had their first pulmonary infection within the first year of their lives. 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). PID, primary ciliary dyskinesia (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), 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.Key words
Non-cystic fibrosis bronchiectasis; bronchiectasis; respiratory infections; common variable immunodeficiency; combined immunodeficiency; RASGRP1.
Cite this article as:
D Çağdaş, M Pehlivantürk Kizılkan, A Tagiyev, N Emiralioğlu, A Keleş, E Yalçın, D Doğru, U Özçelik, N Kiper, İ Tezcan, Primary Immunodeficiency Disorders in children with Non-Cystic Fibrosis Bronchiectasis, Eur Ann Allergy Clin Immunol 2020;52(6):271-276. doi:10.23822/EurAnnACI.1764-1489.151.