The article considersthe etiological factors, clinical manifestations and diagnostic signs of the alveolar hemorrhagic syndrome, which is rare in the pediatric clinical practice, using the example of the group of the patients with orphan lung pathological condition observed in the Pulmonology Clinic of the Institute. The prevailing number of the patients consists of the children with the rare disease; that is the idiopathic pulmonary hemosiderosis. That is why the clinical manifestations of the alveolar hemorrhagic syndrome in the presence of this pathological condition are discussed, the algorithm of the diagnostic measures and the long-term observation results (including the disease outcomes) for the children received the different variants of the immunosuppressive therapy are presented.

Although bronchial asthma is a widespread disease in pediatric population, physicians still face difficulties in early diagnostics  of bronchial asthma in preschool children due to a high frequency of bronchial obstruction in this age group. This article covers key  points of the resolution adopted by the Panel of Experts of Pediatric Respiratory Society regarding standardization of the approach  to diagnostics and therapy of broncho-obstructive conditions, considering a cause of the disease, child’s age and condition severity,  as well as a place of nebulized budesonide suspension in maintenance treatment of bronchial asthma and acute exacerbations therapy.

This specific clinical example of a female patient with DiGeorge syndrome, a primary immunodeficiency disease, demonstrates difficulties in its timely diagnosis and in the choice of treatment policy in this category of patients. Lung injury in them is frequently the only manifestation of the underlying disease. Special attention is given to current diagnostic criteria (including genetic ones) and approaches to treatment and prognosis in patients with DiGeorge syndrome.

Aim. To analyze new possibilities of fractional determination of the expired air nitric oxide. Materials and methods. 120 children aged 2-18, ill with bronchial asthma, primary ciliary dyskinesia (PCD) and other diseases were examined. To carry out fractional determination of the expired air nitric oxide, Analyzer CLD88 (ECO MEDICS®, Switzerland) was used. Estimation of the results of alveolar test (subsequent thrice-repeated determination of Fe NO with the expiratory rate of 30, 100 and 300 l/min) was performed by the following parameters: Ca NO (alveolar NO concentration); Caw NO (NO concentration in the upper airway tissues); Daw NO (NO duffusion rate); Jaw NO (NO flow from the upper airway tissues); Fe NO50 (extrapolated Fe NO value with the expiratory rate of 50 ml/s). Results. Caw NO level moderately correlated (r=0,58) with the nasal nitric oxide level and ranged from 5,7 ppb (in PCD patients) to 473,5 ppb (in a child with severe BA associated with exacerbated allergic rhinitis). Higher correlation degree was revealed between Jaw NO level (166,6÷8815,8 pl/s) and Fe NO and Fe NO50 level ( r =0,82 and r =0,78, respectively). Conclusion. Differentially diagnostic significance of the determined indices in bronchial asthma and primary ciliary dyskinesia was shown.

Aim. To analyze new possibilities of fractional determination of the expired air nitric oxide. Materials and methods. 120 children aged 2-18, ill with bronchial asthma, primary ciliary dyskinesia (PCD) and other diseases were examined. To carry out fractional determination of the expired air nitric oxide, Analyzer CLD88 (ECO MEDICS®, Switzerland) was used. Estimation of the results of alveolar test (subsequent thrice-repeated determination of Fe NO with the expiratory rate of 30, 100 and 300 l/min) was performed by the following parameters: Ca NO (alveolar NO concentration); Caw NO (NO concentration in the upper airway tissues); Daw NO (NO duffusion rate); Jaw NO (NO flow from the upper airway tissues); Fe NO50 (extrapolated Fe NO value with the expiratory rate of 50 ml/s). Results. Caw NO level moderately correlated (r=0,58) with the nasal nitric oxide level and ranged from 5,7 ppb (in PCD patients) to 473,5 ppb (in a child with severe BA associated with exacerbated allergic rhinitis). Higher correlation degree was revealed between Jaw NO level (166,6÷8815,8 pl/s) and Fe NO and Fe NO50 level ( r =0,82 and r =0,78, respectively). Conclusion. Differentially diagnostic significance of the determined indices in bronchial asthma and primary ciliary dyskinesia was shown.

Among the causes of asthma-like diseases there is a rare congenital vascular pathology vascular tracheal ring clinically manifested by heavy breathing (sometimes associated with dysphagia). The reported clinical observation of a fifteen-year-old patient with vascular tracheal ring permits to accentuate attention of physicians, pediatricians, pulmonologists on peculiarity of diagnosis and optimal low-invasive surgery of such patients.

Bronchopulmonary pathology often dominates in patients with immunodeficiency states. Lung diseases in immunodeficient states are usually nonspecific. The article describes a case of chronic lung disease in child with primary immunodeficiency syndrome and Ehlers-Danlos syndrome.