Non cystic fibrosis-related bronchiectasis in children: etiological structure, clinical and laboratory and computed tomographic characteristics

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Abstract

Aim. To establish the etiological structure and to present clinical and laboratory and instrumental characteristics of bronchiectasis (BE) not associated with cystic fibrosis (CF) in children.

Materials and methods. Sixty-seven hospitalised patients with BЕ not related to CF were followed up between 2017 and 2022. Examination methods: clinical-anamnestic method, general clinical laboratory investigations, investigation of allergological and immune status, phagocytosis system, determination of concentration of specific IgE and IgG to fungi of genus Aspergillus, sweat test, radiological examination and computed tomography (CT) of chest organs, bronchoscopy, Bacteriological examination of sputum and/or tracheobronchial aspirates, nasal and/or bronchial ciliary motility, esophagogastroduodenoscopy, 24-hour pH-metry, intra-esophageal combined impedance-pH-metry, genetic study, lung biopsy.

Results. Etiologic factors of BЕ not associated with CF in children were severe pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), Bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), prolonged bacterial bronchitis (1%), brain-lung-thyroid syndrome (1%). The clinical picture is characterized by cough (91%), shortness of breath (67%), fever during exacerbation (48%), chest pain (24%), exercise intolerance (55%), drumstick symptom (9%), moist (76%) and dry wheezing (37%). CT-semiotics of BЕ not associated with CF is characterized by localization in one (58%) or several (42%) lobes; traction (42%), non-traction (49%) B and their combination (9%); increased broncho-arterial ratio >0.9; thickening of bronchial wall; "mosaic perfusion"/"air-trap" symptom (9%); more frequent involvement of lower lungs (64%). The main infectious agents in BЕ not associated with CF were Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus.

Conclusion. On the basis of a multicentre study, the etiological structure, clinical and laboratory and CT-characteristics of non-CF ВE in children were established.

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About the authors

Pavel A. Frolov

People’s Friendship University of Russia (RUDN University); Morozov Children's City Clinical Hospital

Author for correspondence.
Email: 9715586@gmail.com
ORCID iD: 0000-0001-6564-9829

Аssistant, pulmonologist, People’s Friendship University of Russia (RUDN University), Morozov Children's City Clinical Hospital

Russian Federation, Moscow; Moscow

Mariya A. Zhestkova

People’s Friendship University of Russia (RUDN University)

Email: dr.zhestkova@gmail.com
ORCID iD: 0000-0003-4937-716X

Cand. Sci. (Med.), People’s Friendship University of Russia (RUDN University)

Russian Federation, Moscow

Dmitriy Yu. Ovsyannikov

People’s Friendship University of Russia (RUDN University); Morozov Children's City Clinical Hospital

Email: mdovsyannikov@yahoo.com
ORCID iD: 0000-0002-4961-384X

D. Sci. (Med.), People’s Friendship University of Russia (RUDN University), Morozov Children's City Clinical Hospital

Russian Federation, Moscow; Moscow

Maxim I. Ayrapetyan

Sechenov First Moscow State Medical University (Sechenov University)

Email: mdgkb@zdrav.mos.ru
ORCID iD: 0000-0002-0348-929X

Assoc. Prof., Sechenov First Moscow State Medical University (Sechenov University)

Russian Federation, Moscow

Oleg G. Topilin

Morozov Children's City Clinical Hospital

Email: mdgkb@zdrav.mos.ru
ORCID iD: 0000-0002-5302-0502

Department Head, Morozov Children's City Clinical Hospital

Russian Federation, Moscow

Anatoly A. Korsunskiy

Sechenov First Moscow State Medical University (Sechenov University); Speransky Children's City Clinical Hospital №9

Email: korsunskyAA@zdrav.mos.ru
ORCID iD: 0000-0002-9087-1656

D. Sci. (Med.), Prof., Sechenov First Moscow State Medical University (Sechenov University), Speransky Children's City Clinical Hospital №9

Russian Federation, Moscow; Moscow

Evgeniya V. Bojcova

Saint Petersburg State Pediatric Medical University

Email: evboitsova@mail.ru
ORCID iD: 0000-0002-3600-8405

D. Sci. (Med.), Saint Petersburg State Pediatric Medical University

Russian Federation, Saint Petersburg

Elena Yu. Zapevalova

Pavlov First Saint Petersburg State Medical University

Email: elena.zapevalova-13@yandex.ru
ORCID iD: 0000-0002-4337-3902

Res. Assist., Pavlov First Saint Petersburg State Medical University

Russian Federation, Saint Petersburg

Aleksander V. Orlov

Saint Olga Children's City Hospital

Email: orlovcf@yandex.ru
ORCID iD: 0000-0002-2069-7111

Cand. Sci. (Med.), Saint Olga Children's City Hospital

Russian Federation, Saint Petersburg

Helen V. Makarenko

People’s Friendship University of Russia (RUDN University)

Email: makelenavit@mail.ru
ORCID iD: 0000-0001-5598-8413

Аssistant, People’s Friendship University of Russia (RUDN University)

Russian Federation, Moscow

Yaroslav V. Marchenkov

Clinical and diagnostic center "Medsi"

Email: dr.marchenkov@gmail.com
ORCID iD: 0000-0002-5906-0230

Cand. Sci. (Med.), Clinical and diagnostic center "Medsi"

Russian Federation, Moscow

Pavel V. Berezhanskiy

People’s Friendship University of Russia (RUDN University); Morozov Children's City Clinical Hospital

Email: 9715586@gmail.com
ORCID iD: 0000-0001-5235-5303

Cand. Sci. (Med.), People’s Friendship University of Russia (RUDN University), Morozov Children's City Clinical Hospital

Russian Federation, Moscow; Moscow

Valerii V. Gorev

Morozov Children's City Clinical Hospital

Email: mdgkb@zdrav.mos.ru
ORCID iD: 0000-0001-8272-3648

Cand. Sci. (Med.), Morozov Children's City Clinical Hospital

Russian Federation, Moscow

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Supplementary files

Supplementary Files
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1. Fig. 1. Computed tomogram of chest organs of a 5-year-old boy with post-infectious bronchiolitis obliterans confirmed by lung biopsy: atelectasis (triangle-shaped area of shaded lung tissue with the base toward the heart) and ВЕ (small rounded lumens in this area) of the middle lobe, indirect CT signs of ВЕ (inhomogeneity of ventilation, "contour map" type perfusion mosaic pattern).

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2. Fig. 2. Computed tomograms of the chest organs of a patient with primary ciliary dyskinesia. Large BEs are identified in the upper lobe of the right lung (thick arrow, a, b); Fig. 2, b shows atelectasis of the middle (red triangle) and atelectasis of the upper lobe segment 2 (green triangle) of the right lung with BEs partially filled with secretion (thin arrow).

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