Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi
The issue of contemporary diagnosis and correct management of patients with cystic fibrosis is relevant not only for Russia, but for the entire world. Despite achievements of the contemporary medical science, underdiagnosis of this systemic genetic disorder leading to severe incapacitation and death...
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| Format: | Article |
| Language: | Russian |
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Union of pediatricians of Russia
2015-12-01
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| Series: | Педиатрическая фармакология |
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| Online Access: | https://www.pedpharma.ru/jour/article/view/1063 |
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| author | A. A. Baranov L. S. Namazova-Baranova O. I. Simonova N. Y. Kashirskaya E. A. Roslavtseva Y. V. Gorinova S. A. Krasovskiy L. R. Selimzyanova |
| author_facet | A. A. Baranov L. S. Namazova-Baranova O. I. Simonova N. Y. Kashirskaya E. A. Roslavtseva Y. V. Gorinova S. A. Krasovskiy L. R. Selimzyanova |
| author_sort | A. A. Baranov |
| collection | DOAJ |
| description | The issue of contemporary diagnosis and correct management of patients with cystic fibrosis is relevant not only for Russia, but for the entire world. Despite achievements of the contemporary medical science, underdiagnosis of this systemic genetic disorder leading to severe incapacitation and death of pulmonary and heart failure in the vast majority of cases is still observed. The burden induced by this disease is so severe that neonatal screening has been introduced to suspect and detect disease and start pathogenetic treatment capable of stopping or slowing progression of the disease as soon as possible. Apart from the contemporary classification and diagnostic criteria, the article presents treatment goals and components requiring a multidisciplinary approach in detail. Specific attention is given to methods of kinesitherapy, use of antibacterial drugs and enzyme replacement therapy, which primarily determine prognosis of the disease. |
| format | Article |
| id | doaj-art-25b4323c3fe54c1cbf697cbaa9f40a59 |
| institution | DOAJ |
| issn | 1727-5776 2500-3089 |
| language | Russian |
| publishDate | 2015-12-01 |
| publisher | Union of pediatricians of Russia |
| record_format | Article |
| series | Педиатрическая фармакология |
| spelling | doaj-art-25b4323c3fe54c1cbf697cbaa9f40a592025-08-20T03:19:42ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892015-12-0112558960410.15690/pf.v12i5.14621058Contemporary Understanding of Diagnosis and Treatment of Children with Cystic FibrosiA. A. Baranov0L. S. Namazova-Baranova1O. I. Simonova2N. Y. Kashirskaya3E. A. Roslavtseva4Y. V. Gorinova5S. A. Krasovskiy6L. R. Selimzyanova7Scientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationScientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian Federation Pirogov Russian National Medical Research University, Moscow, Russian FederationScientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationFederal State Budgetary Research Institution «Medical/Genetic Scientific Center», Moscow, Russian FederationScientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian FederationScientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian FederationFederal State Institution «Research Institute of Pulmonology» of the Federal Biomedical Agency of Russia, Moscow, Russian FederationScientific Center of Children’s Health of the Ministry of Health of Russia, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationThe issue of contemporary diagnosis and correct management of patients with cystic fibrosis is relevant not only for Russia, but for the entire world. Despite achievements of the contemporary medical science, underdiagnosis of this systemic genetic disorder leading to severe incapacitation and death of pulmonary and heart failure in the vast majority of cases is still observed. The burden induced by this disease is so severe that neonatal screening has been introduced to suspect and detect disease and start pathogenetic treatment capable of stopping or slowing progression of the disease as soon as possible. Apart from the contemporary classification and diagnostic criteria, the article presents treatment goals and components requiring a multidisciplinary approach in detail. Specific attention is given to methods of kinesitherapy, use of antibacterial drugs and enzyme replacement therapy, which primarily determine prognosis of the disease.https://www.pedpharma.ru/jour/article/view/1063cystic fibrosisdiagnosispathogenesisclassificationtreatmentkinesitherapyenzyme replacement therapychildren |
| spellingShingle | A. A. Baranov L. S. Namazova-Baranova O. I. Simonova N. Y. Kashirskaya E. A. Roslavtseva Y. V. Gorinova S. A. Krasovskiy L. R. Selimzyanova Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi Педиатрическая фармакология cystic fibrosis diagnosis pathogenesis classification treatment kinesitherapy enzyme replacement therapy children |
| title | Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi |
| title_full | Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi |
| title_fullStr | Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi |
| title_full_unstemmed | Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi |
| title_short | Contemporary Understanding of Diagnosis and Treatment of Children with Cystic Fibrosi |
| title_sort | contemporary understanding of diagnosis and treatment of children with cystic fibrosi |
| topic | cystic fibrosis diagnosis pathogenesis classification treatment kinesitherapy enzyme replacement therapy children |
| url | https://www.pedpharma.ru/jour/article/view/1063 |
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