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.

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.

The article is devoted to bronchiectases issue in children and reflects the most actual approaches to the diagnosis and management of patients with this pathology. Information on the true prevalence of bronchiectases both abroad and in Russia varies. Wherein, bronchiectases can be either an independent disease or a manifestation of another pathology, which necessitates an integrated multidisciplinary approach not only for diagnosis but also for patient management with this pathology. The material presented by the authors is based on clinical guidelines developed and approved by the profile association of the Union of Pediatricians of Russia and aimed at raising awareness of specialists providing medical care to children.

Hypersensitive pneumonitis (HP) is an interstitial lung disease occuring in childhood. This article demonstrates own long-running monitoring of a girl with HP at the age of 8. The development of the disease is linked to the home ecology. The diagnosis was based on data from the history, clinical symptoms with respiratory deficiency and crepitating wheezing in the lungs, characteristicfunctional and X-ray manifestations; it was proved by detection in the plasma of the blood of specific immunoglobulins of class G to cause-significant allergens. Elimination interventions and adequate treatment have enabled positive dynamics to be achieved during the disease

The article is devoted to bronchiectases issue in children and reflects the most actual approaches to the diagnosis and management of patients with this pathology. Information on the true prevalence of bronchiectases both abroad and in Russia varies. Wherein, bronchiectases can be either an independent disease or a manifestation of another pathology, which necessitates an integrated multidisciplinary approach not only for diagnosis but also for patient management with this pathology. The material presented by the authors is based on clinical guidelines developed and approved by the profile association of the Union of Pediatricians of Russia and aimed at raising awareness of specialists providing medical care to children.

The article presents the most modern positions of healthcare delivery for children with primary ciliary dyskinesia. Symptoms of this pathology in clinical practice vary that is conditioned by genetic heterogeneity of the disease. The most common disease manifestation in children is frequent inflammatory diseases of the upper and lower respiratory tract. They are recorded in most patients, especially in young children, and the diagnosis is often determined untimely due to a low awareness of specialists about this nosology. Differential diagnostic approach is described in detail, peculiarities of treatment and management of children with this nosology are specified. The material is based on clinical guidelines developed and approved by the professional association «Union of Pediatricians of Russia».

The paper deals with interstitial lung diseases in children. It gives an update and the results of the authors’ observations of different forms of interstitial lung diseases. Particular emphasis is placed on hypersensitive pneumonitis as the most common nosological entity among childhood interstitial lung diseases. The authors followed up 186 children with hypersensitive pneumonitis. They present the most important clinical, functional, radiological, and immunological diagnostic signs of this disease and consider its prognosis. In addition, there is evidence for other rare forms of interstitial lung diseases (idiopathic interstitial pneumonia, idiopathic pulmonary hemosiderosis, etc.) in children.

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.