Cough in childhood is one of the most common reasons for and symptoms when contacting a pediatric physician. Chronic (lasting over four weeks) wet/productive cough is of particular diagnostic difficulty when it is caused by protracted bacterial bronchitis (PBB). The purpose of this research was to study the etiology, clinical options, X-ray semiotics and comorbid diseases in children with PBB. Materials and methods used: clinical observation and analysis of case histories of 76 children aged starting 4.5 months up to 16 years old with PBB were carried out from Mar. 2017 to Jun. 2022. Research methods: microbiological, radiography and computed tomography (CT) of the chest, bronchoscopy, allergy diagnostics, spirometry. The design of the study was as follows: multicentre, non-comparative, open-label, non-randomized, ambispective pilot study. Results: PBB is more common in boys (57%) aged 3 to 7 y/o (59%) and is rare in infancy (7%). The majority of patients met the clinical definition of PBB (86%) and responded to 2 weeks of antibiotic therapy with amoxicillin/clavulanate (78%). The microbiological diagnosis of PBB was confirmed in 14% of children based on bronchoscopy with microbiological examination of bronchoalveolar lavage (BAL) fluid or sputum examination. The most common pathogens encountered in BAL, sputum and discharge from the nasopharynx, oropharynx are as follows: S. aureus, M. catarrhalis, H. influenzae and S. pneumoniae. The chest X-ray showed bronchitis changes; the chest CT, which was performed in 10 patients, had showed thickening of the bronchial walls. Bronchial dilatation was found in 3 children, bronchiectasis was found in a single patient. Bronchial asthma was comorbid with PBB, occurred in 22% of PBB cases. Conclusion: it is necessary for a pediatric physician to keep PBB in mind as one of the reasons for chronic wet/productive cough in children in order to correctly and timely diagnose the disease and prescribe its adequate treatment.
Primary ciliary dyskinesia (PCD) is an orphan disease associated with mutations in several genes. It is a ciliopathy, an abnormality of the cilia and flagella. Ciliopathies include the extremely rare Simpson - Golabi - Bemel syndrome (SSGB) type II.The aim of this article is to familiarize the reader with the possibility of simultaneous presence of type II SSGB and PCD in a patient with bronchiectasis (BE).Results. The first clinical observation in the Russian literature is presented withhistory, physical examination, including clinical and morphologic examination, results of additional investigations and initiation of therapy. The case describes a 15-year-old patient with BE and other lesions typical of PCD confirmed on the basis of structural changes in the cilia of the respiratory epithelium of the trachea detected by transmission electron microscopy. The patient had a pathogenic mutation of the OFD1 gene responsible for the development of both type II SSGB and PCD.Conclusion. Several variants of ciliopathies may occur in one patient, and PCD may present as a syndrome.
Despite the manifestations of primary ciliary dyskinesia (PCD) in the neonatal period or in the first year of life, the diagnosis of this rare disease is usually established at the age of 4–7 years. The aim of the study was to search for reserves for early diagnosis of PCD. Materials and methods of research: 17 patients were observed with PCD, confirmed on the basis of the PICADAR scale, highspeed light video microscopy (all patients) and transmission electron microscopy (in 3 patients) of the mucous biopsy of the respiratory membrane using a genetic study (in one patient). Results: all patients were born full-term; neonatal respiratory distress syndrome (RDS) was observed in 71% of patients; the median duration of mechanical ventilation/oxygen therapy was 14 [7; 21] days; lateralization abnormalities were found in 35% of patients; all patients had persistent nasal congestion and/or rhinorrhea, 94% had chronic or recurrent sinusitis, otitis, recurrent pneumonia, chest x-ray and computed tomography (CT) scans in 71% had atelectasis with constant localization in the middle lobe, 12 of 15 patients, traced in the follow-up, – bronchiectasis (BE), also mainly in the middle lobe (in 9 patients). The average age at diagnosis was 5 [1.75; 7] years, patients with an established diagnosis over the age of 3 years were more often diagnosed with BE. The median of PICADAR scores was 7 [6; 8] points. Conclusions: to establish early diagnosis of PCD, it is important to consider neonatal RDS, lateralization of organs, difficulty in nasal breathing, starting from the first half of life.
В статье представлено клиническое наблюдение недоношенного ребенка с бронхолегочной дисплазией, перенесшего тяжелый острый бронхиолит (ОБ), вызванный респираторно-синцитиальным вирусом (РСВ). Приведены сведения об эпидемиологии, этиологии, диагностике, терапии ОБ, пассивной иммунопрофилактике РСВ-инфекции с помощью паливизумаба, количестве его назначений в Москве, эффективности паливизумаба для профилактики инфекций нижних дыхательных путей, вызванных РСВ. Проведен подробный анализ течения, клинических, лабораторных и рентгенологических данных, терапии РСВ-ОБ у наблюдавшегося пациента с сопоставлением с современными данными литературы.Финансирование. Работа не имела спонсорской поддержки. Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов.
Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease of unknown etiology that develops in the first year of life and manifests itself as persistent tachypnea syndrome. The aim of the study was to determine the diagnostic value of the clinical scale for the diagnosis of NEHI in comparison with computed tomography (CT) data of the chest organs and for differential diagnosis with acute bronchiolitis (AB)/community-acquired pneumonia (CAP). Materials and methods of research: a multicenter case-control study of children aged 21 days of life to 12 months was carried out: – 83 children with NEHI and 83 infants with AB or CAP. To determine the sensitivity and specificity of this scale for differential diagnosis with AB/CAP, the results of the assessment according to the proposed scale were compared in patients with NEHI (study group) and patients with AB/CAP (control group) on the day of treatment and after 3 weeks. Results: the sensitivity of the clinical scale for the diagnosis of NEHI when compared with the CT data of the chest organs was 85.5%. In the differential diagnosis of NEHI with AB/CAP on the day of treatment, the sensitivity of the clinical scale for the diagnosis of NEHI was 85.5%, the specificity was 80.7%; when re-evaluated after 3 weeks, the accuracy of the scale increased from 83.1% to 91.6%, the specificity – from 80.7% to 97.6%. Diagnosis on a scale after 3 weeks reduced the possibility of overdiagnosis of NEHI from 19.3% to 2.4%. Conclusions: the clinical scale for the diagnosis of NEHI has a high diagnostic value in comparison with the results of CT scan of the chest organs and for differential diagnosis with AB/CAP, especially when re-evaluating.
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