Modern Clinical Guidelines for the Management of Patients with Gaucher Disease
This article presents data on the epidemiology, pathogenesis, modern classification and the main clinical manifestations of Gaucher disease in children. The criteria for differential diagnosis with other diseases are given. It has been shown that the only effective method of treating Gaucher disease...
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| Format: | Article |
| Language: | Russian |
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Union of pediatricians of Russia
2024-12-01
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| Series: | Педиатрическая фармакология |
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| Online Access: | https://www.pedpharma.ru/jour/article/view/2555 |
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| author | Inga V. Anisimova Galina V. Baydakova Aleksander A. Baranov Nato D. Vashakmadze Elena A. Vishneva Olga S. Gundobina Elena A. Dobrynina Ekaterina Yu. Zaharova Elena V. Kaytukova Elena V. Komarova Sergey I, Kutsev Alla E. Lavrova Svetlana V. Mihaylova Goar B. Movsisyan Lyudmila P. Nazarenko Leyla S. Namazova-Baranova Ekaterina A. Nikolaeva Tatiana M. Pervunina Natalia L. Pechatnikova Nelya S. Pogosyan Aleksander S. Potapov Aleksander A. Pushkov Svetlana A. Repina Kirill V. Savostyanov Liliya R. Selimzyanova Olga Ya. Smirnova Natalia S. Smetanina Tatiana V. Strokova Andrey N. Surkov Marina V. Fedoseenko Tatiana T. Batysheva Olga V. Bykova Yuriy A. Klimov Sergey V. Tihonov |
| author_facet | Inga V. Anisimova Galina V. Baydakova Aleksander A. Baranov Nato D. Vashakmadze Elena A. Vishneva Olga S. Gundobina Elena A. Dobrynina Ekaterina Yu. Zaharova Elena V. Kaytukova Elena V. Komarova Sergey I, Kutsev Alla E. Lavrova Svetlana V. Mihaylova Goar B. Movsisyan Lyudmila P. Nazarenko Leyla S. Namazova-Baranova Ekaterina A. Nikolaeva Tatiana M. Pervunina Natalia L. Pechatnikova Nelya S. Pogosyan Aleksander S. Potapov Aleksander A. Pushkov Svetlana A. Repina Kirill V. Savostyanov Liliya R. Selimzyanova Olga Ya. Smirnova Natalia S. Smetanina Tatiana V. Strokova Andrey N. Surkov Marina V. Fedoseenko Tatiana T. Batysheva Olga V. Bykova Yuriy A. Klimov Sergey V. Tihonov |
| author_sort | Inga V. Anisimova |
| collection | DOAJ |
| description | This article presents data on the epidemiology, pathogenesis, modern classification and the main clinical manifestations of Gaucher disease in children. The criteria for differential diagnosis with other diseases are given. It has been shown that the only effective method of treating Gaucher disease is pathogenetic enzyme replacement therapy, which relieves the main clinical manifestations of the disease, improving the quality of life of patients and without having pronounced side effects. The main errors of diagnosis and management of patients, as well as unjustified prescriptions for the treatment of this disease, are indicated. |
| format | Article |
| id | doaj-art-aef7e75a2d1f4182ae1f2f5185c04a3a |
| institution | Kabale University |
| issn | 1727-5776 2500-3089 |
| language | Russian |
| publishDate | 2024-12-01 |
| publisher | Union of pediatricians of Russia |
| record_format | Article |
| series | Педиатрическая фармакология |
| spelling | doaj-art-aef7e75a2d1f4182ae1f2f5185c04a3a2025-08-20T03:59:21ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892024-12-0121655156810.15690/pf.v21i6.28422143Modern Clinical Guidelines for the Management of Patients with Gaucher DiseaseInga V. Anisimova0Galina V. Baydakova1Aleksander A. Baranov2Nato D. Vashakmadze3Elena A. Vishneva4Olga S. Gundobina5Elena A. Dobrynina6Ekaterina Yu. Zaharova7Elena V. Kaytukova8Elena V. Komarova9Sergey I, Kutsev10Alla E. Lavrova11Svetlana V. Mihaylova12Goar B. Movsisyan13Lyudmila P. Nazarenko14Leyla S. Namazova-Baranova15Ekaterina A. Nikolaeva16Tatiana M. Pervunina17Natalia L. Pechatnikova18Nelya S. Pogosyan19Aleksander S. Potapov20Aleksander A. Pushkov21Svetlana A. Repina22Kirill V. Savostyanov23Liliya R. Selimzyanova24Olga Ya. Smirnova25Natalia S. Smetanina26Tatiana V. Strokova27Andrey N. Surkov28Marina V. Fedoseenko29Tatiana T. Batysheva30Olga V. Bykova31Yuriy A. Klimov32Sergey V. Tihonov33N.P. BoNot declaredchkov Medical Genetics Research CentreN.P. Bochkov Medical Genetics Research CentreResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; I.M. Sechenov First Moscow State Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of SurgeryResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of SurgeryN.P. Bochkov Medical Genetics Research CentreResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of SurgeryN.P. Bochkov Medical Genetics Research CentrePrivolzhsky Research Medical UniversityRussian Children’s Clinical HospitalNational Medical Research Center for Children’s HealthResearch Institute of Medical GeneticsResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityYu. E. Veltischev Research and Clinical Institute for PediatricsAlmazov National Medical Research CentreMorozov Children’s City Clinical HospitalAll-Russian Society of Orphan DiseasesI.M. Sechenov First Moscow State Medical University; National Medical Research Center for Children’s HealthNational Medical Research Center for Children’s HealthN.P. Bochkov Medical Genetics Research CentreNational Medical Research Center for Children’s HealthResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; I.M. Sechenov First Moscow State Medical University; Pirogov Russian National Research Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of SurgeryDmitry Rogachev National Medical Research Center of pediatric Hematology, Oncology and ImmunologyFederal Research Centre of Nutrition, Biotechnology and Food SafetyResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityResearch Institute of Pediatrics and Children’s Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityScientific and Practical Center for Pediatric Psychoneurology; Peoples’ Friendship University of RussiaScientific and Practical Center for Pediatric Psychoneurology; Peoples’ Friendship University of RussiaScientific and Practical Center for Pediatric PsychoneurologyScientific and Practical Center for Pediatric Psychoneurology; Peoples’ Friendship University of RussiaThis article presents data on the epidemiology, pathogenesis, modern classification and the main clinical manifestations of Gaucher disease in children. The criteria for differential diagnosis with other diseases are given. It has been shown that the only effective method of treating Gaucher disease is pathogenetic enzyme replacement therapy, which relieves the main clinical manifestations of the disease, improving the quality of life of patients and without having pronounced side effects. The main errors of diagnosis and management of patients, as well as unjustified prescriptions for the treatment of this disease, are indicated.https://www.pedpharma.ru/jour/article/view/2555gaucher diseased-glucosidasechitotriosidasehepatosplenomegalyenzyme replacement therapyimiglucerasechildren |
| spellingShingle | Inga V. Anisimova Galina V. Baydakova Aleksander A. Baranov Nato D. Vashakmadze Elena A. Vishneva Olga S. Gundobina Elena A. Dobrynina Ekaterina Yu. Zaharova Elena V. Kaytukova Elena V. Komarova Sergey I, Kutsev Alla E. Lavrova Svetlana V. Mihaylova Goar B. Movsisyan Lyudmila P. Nazarenko Leyla S. Namazova-Baranova Ekaterina A. Nikolaeva Tatiana M. Pervunina Natalia L. Pechatnikova Nelya S. Pogosyan Aleksander S. Potapov Aleksander A. Pushkov Svetlana A. Repina Kirill V. Savostyanov Liliya R. Selimzyanova Olga Ya. Smirnova Natalia S. Smetanina Tatiana V. Strokova Andrey N. Surkov Marina V. Fedoseenko Tatiana T. Batysheva Olga V. Bykova Yuriy A. Klimov Sergey V. Tihonov Modern Clinical Guidelines for the Management of Patients with Gaucher Disease Педиатрическая фармакология gaucher disease d-glucosidase chitotriosidase hepatosplenomegaly enzyme replacement therapy imiglucerase children |
| title | Modern Clinical Guidelines for the Management of Patients with Gaucher Disease |
| title_full | Modern Clinical Guidelines for the Management of Patients with Gaucher Disease |
| title_fullStr | Modern Clinical Guidelines for the Management of Patients with Gaucher Disease |
| title_full_unstemmed | Modern Clinical Guidelines for the Management of Patients with Gaucher Disease |
| title_short | Modern Clinical Guidelines for the Management of Patients with Gaucher Disease |
| title_sort | modern clinical guidelines for the management of patients with gaucher disease |
| topic | gaucher disease d-glucosidase chitotriosidase hepatosplenomegaly enzyme replacement therapy imiglucerase children |
| url | https://www.pedpharma.ru/jour/article/view/2555 |
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