Modern views on ?hildren's interstitial lung disease

Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. ?hildren's interstitial lung diseases is an topicalproblem of pediatric pulmonology. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children's ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail.Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children's ILD, therapy options are presented in this article

Respiratory diseases in children with down syndrome

Down syndrome (DS) is one of the most common hereditary human diseases and the most common chromosomal abnormality (trisomy of chromosome 21). Besides the typical appearance, multiple anomalies of the cardiovascular, central nervous and immune systems, gastrointestinal tract, locomotor system, patients with DS commonly have respiratory tract diseases. The article presents current literature data on the causes of recurrent respiratory infections, upper and lower respiratory tract obstruction and obstructive sleep apnea. It provides data on the consequences of such respiratory tract abnormality as tracheal bronchus. It also describes interstitial lung diseases with DS (pulmonary hypoplasia, subpleural cysts) and their association with congenital heart diseases and pulmonary hypertension. It presents observations of patients with DS and pulmonary subpleural cysts. It discusses Palivizumab use for severe respiratory syncytial virus infection in children with DS. © 2017, Pediatria Ltd. All rights reserved

Respiratory diseases in children with down syndrome

Down syndrome (DS) is one of the most common hereditary human diseases and the most common chromosomal abnormality (trisomy of chromosome 21). Besides the typical appearance, multiple anomalies of the cardiovascular, central nervous and immune systems, gastrointestinal tract, locomotor system, patients with DS commonly have respiratory tract diseases. The article presents current literature data on the causes of recurrent respiratory infections, upper and lower respiratory tract obstruction and obstructive sleep apnea. It provides data on the consequences of such respiratory tract abnormality as tracheal bronchus. It also describes interstitial lung diseases with DS (pulmonary hypoplasia, subpleural cysts) and their association with congenital heart diseases and pulmonary hypertension. It presents observations of patients with DS and pulmonary subpleural cysts. It discusses Palivizumab use for severe respiratory syncytial virus infection in children with DS. © 2017, Pediatria Ltd. All rights reserved

Modern views on ?hildren's interstitial lung disease

Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. ?hildren's interstitial lung diseases is an topicalproblem of pediatric pulmonology. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children's ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail.Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children's ILD, therapy options are presented in this article

Jeune syndrome: Case series report

Jeune syndrome (synonym: asphyxiating thoracic dystrophy) is a rare genetically heterogeneous autosomal recessive disease, characterized by the development of generalized osteochondrodysplasia, anomalies of skeletal system with multi-organ involvement. This syndrome is based on mutations of the IFT80, DYNC2H1, WDR19, TTC21B genes localized in the locus of chromosomes 3q25.33, 11q22.3, 4p14, 2q24.3 respectively and mutation in the locus of chromosome 15q13 too. These genes play an important role in functioning of cilia in the mammalian body. These organelles participate in the development of the skeletal and hepatobiliary system, nephrons, retina, and other structures of the body. Thurs Jeune syndrome belongs to the ciliopathies. The article presents modern literature findings on genetics, pathogenesis, its clinical, X-ray presentations and CT patterns, diagnosis and treatment of this rare disease. The article also presents the first Russian case series including 7 patients with this disease. Combinations of such symptoms as narrow bell-shaped thorax, variable limb shortness, respiratory failure, including oxygen dependence, recurrent respiratory infections, and motor development delay in observed patients helped to diagnose Jeune syndrome in all cases

Jeune syndrome: Case series report

Jeune syndrome (synonym: asphyxiating thoracic dystrophy) is a rare genetically heterogeneous autosomal recessive disease, characterized by the development of generalized osteochondrodysplasia, anomalies of skeletal system with multi-organ involvement. This syndrome is based on mutations of the IFT80, DYNC2H1, WDR19, TTC21B genes localized in the locus of chromosomes 3q25.33, 11q22.3, 4p14, 2q24.3 respectively and mutation in the locus of chromosome 15q13 too. These genes play an important role in functioning of cilia in the mammalian body. These organelles participate in the development of the skeletal and hepatobiliary system, nephrons, retina, and other structures of the body. Thurs Jeune syndrome belongs to the ciliopathies. The article presents modern literature findings on genetics, pathogenesis, its clinical, X-ray presentations and CT patterns, diagnosis and treatment of this rare disease. The article also presents the first Russian case series including 7 patients with this disease. Combinations of such symptoms as narrow bell-shaped thorax, variable limb shortness, respiratory failure, including oxygen dependence, recurrent respiratory infections, and motor development delay in observed patients helped to diagnose Jeune syndrome in all cases

Long-term home oxygen therapy in children: To whom, when, how?

Chronic hypoxemia in children worsens the course of chronic lung and heart diseases, a number of other organs and systems in children, causes development of pulmonary hypertension and pulmonary heart, disrupts growth and development, increases the risk of death. Correction of hypoxemia with long-term oxygen therapy, incl. at home, seems to be the most pathogenetically justified method of therapy for these disorders. The review, based on current literature data and authors own experience, provides information on the indications and epidemiology of home oxygen therapy (HOT), the mechanisms of its action in various clinical situations, the impact ongrowth and development of a child. It discusses normative values and necessary monitoring of oxygenation, management tactics for children requiring HOT and the method of weaning from oxygen. The important technical characteristics of providing long-term oxygen therapy at home are described in detail. © 2018, Pediatria Ltd. All rights reserved

Actual problems of neonatal pulmonology

The article presents discussion questions, problems and achievements in newborns pulmonology and contribution of Russian researches to this section of neonatology and pediatrics. Authors assess prospects of obtained results implementation and their scientific and practical significance. The article discusses in detail the most common newborns lung diseases-respiratory distress syndrome, bronchopulmonary dysplasia (BPD) and pneumonia. It illuminates the actual problem of complications and differential diagnosis of BPD with other chronic and interstitial newborns lung diseases, occurring under its «disguise». © 2016, Pediatria Ltd. All rights reserved