The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study
Background. Data on the efficacy of enzyme replacement therapy (ERT) in relation to the pathology of ENT organs and respiratory performance in sleep in children with mucopolysaccharidosis (MPS) is poorly presented in the literature. Our aim was to assess the effect of ERT on the upper respiratory tr...
Saved in:
| Main Authors: | , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | Russian |
| Published: |
Union of pediatricians of Russia
2018-05-01
|
| Series: | Педиатрическая фармакология |
| Subjects: | |
| Online Access: | https://www.pedpharma.ru/jour/article/view/1615 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849395866112098304 |
|---|---|
| author | Liliya A. Osipova Ludmila M. Kuzenkova Leyla S. Namazova-Baranova Anait K. Gevorkyan Tatiana V. Podkletnova Viktor V. Altunin Olga V. Kozhevnikova Liya V. Toropchina Tatiana A. Polunina E. V. Zelenkova Nato D. Vashakmadze |
| author_facet | Liliya A. Osipova Ludmila M. Kuzenkova Leyla S. Namazova-Baranova Anait K. Gevorkyan Tatiana V. Podkletnova Viktor V. Altunin Olga V. Kozhevnikova Liya V. Toropchina Tatiana A. Polunina E. V. Zelenkova Nato D. Vashakmadze |
| author_sort | Liliya A. Osipova |
| collection | DOAJ |
| description | Background. Data on the efficacy of enzyme replacement therapy (ERT) in relation to the pathology of ENT organs and respiratory performance in sleep in children with mucopolysaccharidosis (MPS) is poorly presented in the literature. Our aim was to assess the effect of ERT on the upper respiratory tract in children with MPS. Methods. According to the case histories, we studied treatment results of children with MPS type I and II who received ERT in the Research Center of Children’s Health from January 2007 to November 2016. The severity of upper airway obstruction and its change during ERT was assessed according to indices of apnea-hypopnea and desaturation (SpO2), average/minimal SpO2, duration of SpO2 episodes < 90%, and hypertrophy degree of palatine tonsils and adenoids. Results. The severity of the obstructive sleep apnea syndrome did not progress in children (n = 15) with MPS against the background of ERT with a median duration of 38 (23; 48) months: initially, the apnea-hypopnea index was 3 (1.3; 7.7), while the repeated study — 2.6 (0.9; 13.5) (p = 0.507). There was also no statistically significant change in cardiorespiratory monitoring values. Conclusion. Long-term ERT in children with MPS type I and II interferes with the progression of airway obstruction. |
| format | Article |
| id | doaj-art-a7bb8f3e83444eb7a078a8fa7e48b853 |
| institution | Kabale University |
| issn | 1727-5776 2500-3089 |
| language | Russian |
| publishDate | 2018-05-01 |
| publisher | Union of pediatricians of Russia |
| record_format | Article |
| series | Педиатрическая фармакология |
| spelling | doaj-art-a7bb8f3e83444eb7a078a8fa7e48b8532025-08-20T03:39:29ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892018-05-0115213514010.15690/pf.v15i2.18701571The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort StudyLiliya A. Osipova0Ludmila M. Kuzenkova1Leyla S. Namazova-Baranova2Anait K. Gevorkyan3Tatiana V. Podkletnova4Viktor V. Altunin5Olga V. Kozhevnikova6Liya V. Toropchina7Tatiana A. Polunina8E. V. Zelenkova9Nato D. Vashakmadze10National Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, Moscow; Sechenov First Moscow State Medical University, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, Moscow; Sechenov First Moscow State Medical University, Moscow; N.I. Pirogov Russian National Medical Research University, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, Moscow; Sechenov First Moscow State Medical University, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowRussian Medical Academy of Continuous Professional Education, Moscow; Moscow Regional Consultative and Diagnostic Center for Children, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, MoscowNational Medical Research Center of Children’s Health of the Ministry of Health of the Russian Federation, Moscow; N.I. Pirogov Russian National Medical Research University, MoscowBackground. Data on the efficacy of enzyme replacement therapy (ERT) in relation to the pathology of ENT organs and respiratory performance in sleep in children with mucopolysaccharidosis (MPS) is poorly presented in the literature. Our aim was to assess the effect of ERT on the upper respiratory tract in children with MPS. Methods. According to the case histories, we studied treatment results of children with MPS type I and II who received ERT in the Research Center of Children’s Health from January 2007 to November 2016. The severity of upper airway obstruction and its change during ERT was assessed according to indices of apnea-hypopnea and desaturation (SpO2), average/minimal SpO2, duration of SpO2 episodes < 90%, and hypertrophy degree of palatine tonsils and adenoids. Results. The severity of the obstructive sleep apnea syndrome did not progress in children (n = 15) with MPS against the background of ERT with a median duration of 38 (23; 48) months: initially, the apnea-hypopnea index was 3 (1.3; 7.7), while the repeated study — 2.6 (0.9; 13.5) (p = 0.507). There was also no statistically significant change in cardiorespiratory monitoring values. Conclusion. Long-term ERT in children with MPS type I and II interferes with the progression of airway obstruction.https://www.pedpharma.ru/jour/article/view/1615childrenmucopolysaccharidosisenzyme replacement therapyairway obstructionapnea-hypopnea indexhypertrophy of palatine tonsilshypertrophy of adenoids |
| spellingShingle | Liliya A. Osipova Ludmila M. Kuzenkova Leyla S. Namazova-Baranova Anait K. Gevorkyan Tatiana V. Podkletnova Viktor V. Altunin Olga V. Kozhevnikova Liya V. Toropchina Tatiana A. Polunina E. V. Zelenkova Nato D. Vashakmadze The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study Педиатрическая фармакология children mucopolysaccharidosis enzyme replacement therapy airway obstruction apnea-hypopnea index hypertrophy of palatine tonsils hypertrophy of adenoids |
| title | The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study |
| title_full | The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study |
| title_fullStr | The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study |
| title_full_unstemmed | The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study |
| title_short | The Impact of Enzyme-Replacement Therapy on Upper Airway Obstruction in Children with Mucopolysaccharidoses: Retrospective Cohort Study |
| title_sort | impact of enzyme replacement therapy on upper airway obstruction in children with mucopolysaccharidoses retrospective cohort study |
| topic | children mucopolysaccharidosis enzyme replacement therapy airway obstruction apnea-hypopnea index hypertrophy of palatine tonsils hypertrophy of adenoids |
| url | https://www.pedpharma.ru/jour/article/view/1615 |
| work_keys_str_mv | AT liliyaaosipova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT ludmilamkuzenkova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT leylasnamazovabaranova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT anaitkgevorkyan theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT tatianavpodkletnova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT viktorvaltunin theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT olgavkozhevnikova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT liyavtoropchina theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT tatianaapolunina theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT evzelenkova theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT natodvashakmadze theimpactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT liliyaaosipova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT ludmilamkuzenkova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT leylasnamazovabaranova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT anaitkgevorkyan impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT tatianavpodkletnova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT viktorvaltunin impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT olgavkozhevnikova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT liyavtoropchina impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT tatianaapolunina impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT evzelenkova impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy AT natodvashakmadze impactofenzymereplacementtherapyonupperairwayobstructioninchildrenwithmucopolysaccharidosesretrospectivecohortstudy |