DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME
Cryopyrin-Associated Periodic Syndrome (CAPS) refers to a group of autoinflammatory diseases and is caused by autosomal dominant mutations of the NLRP3 gene coding. Relapsing fever, rash, laboratory changes and inflammatory lesions of various organs are typical manifestations of CAPS. Inhibitors of...
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| Format: | Article |
| Language: | English |
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"Paediatrician" Publishers LLC
2015-04-01
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| Series: | Вопросы современной педиатрии |
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| Online Access: | https://vsp.spr-journal.ru/jour/article/view/73 |
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| author | A. L. Kozlova B. S. Pershin T. V. Varlamova A. Yu. Shcherbina |
| author_facet | A. L. Kozlova B. S. Pershin T. V. Varlamova A. Yu. Shcherbina |
| author_sort | A. L. Kozlova |
| collection | DOAJ |
| description | Cryopyrin-Associated Periodic Syndrome (CAPS) refers to a group of autoinflammatory diseases and is caused by autosomal dominant mutations of the NLRP3 gene coding. Relapsing fever, rash, laboratory changes and inflammatory lesions of various organs are typical manifestations of CAPS. Inhibitors of interleukin 1, including canakinumab, are modern means of pathogenetic therapy of patients with CAPS. The article presents the treatment results in 5 pediatric patients with CAPS. Application of canakinumab led to the relief of all major symptoms (fever, inflammatory changes in the blood lymphoproliferation, signs of organ damage), and no relevant side effects was detected. |
| format | Article |
| id | doaj-art-bd32ff1d04c4485dabdac74aa5150881 |
| institution | DOAJ |
| issn | 1682-5527 1682-5535 |
| language | English |
| publishDate | 2015-04-01 |
| publisher | "Paediatrician" Publishers LLC |
| record_format | Article |
| series | Вопросы современной педиатрии |
| spelling | doaj-art-bd32ff1d04c4485dabdac74aa51508812025-08-20T03:01:07Zeng"Paediatrician" Publishers LLCВопросы современной педиатрии1682-55271682-55352015-04-0114229229710.15690/vsp.v14i2.130173DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROMEA. L. Kozlova0B. S. Pershin1T. V. Varlamova2A. Yu. Shcherbina3D. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian FederationD. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian FederationD. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian FederationD. Rogachev Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology, Moscow, Russian FederationCryopyrin-Associated Periodic Syndrome (CAPS) refers to a group of autoinflammatory diseases and is caused by autosomal dominant mutations of the NLRP3 gene coding. Relapsing fever, rash, laboratory changes and inflammatory lesions of various organs are typical manifestations of CAPS. Inhibitors of interleukin 1, including canakinumab, are modern means of pathogenetic therapy of patients with CAPS. The article presents the treatment results in 5 pediatric patients with CAPS. Application of canakinumab led to the relief of all major symptoms (fever, inflammatory changes in the blood lymphoproliferation, signs of organ damage), and no relevant side effects was detected.https://vsp.spr-journal.ru/jour/article/view/73autoinflammatory syndromescryopyrin-associated periodic syndromeinterleukin 1inhibitorscanakinumab |
| spellingShingle | A. L. Kozlova B. S. Pershin T. V. Varlamova A. Yu. Shcherbina DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME Вопросы современной педиатрии autoinflammatory syndromes cryopyrin-associated periodic syndrome interleukin 1 inhibitors canakinumab |
| title | DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME |
| title_full | DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME |
| title_fullStr | DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME |
| title_full_unstemmed | DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME |
| title_short | DIAGNOSIS AND TREATMENT OF PATIENTS WITH CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME |
| title_sort | diagnosis and treatment of patients with cryopyrin associated periodic syndrome |
| topic | autoinflammatory syndromes cryopyrin-associated periodic syndrome interleukin 1 inhibitors canakinumab |
| url | https://vsp.spr-journal.ru/jour/article/view/73 |
| work_keys_str_mv | AT alkozlova diagnosisandtreatmentofpatientswithcryopyrinassociatedperiodicsyndrome AT bspershin diagnosisandtreatmentofpatientswithcryopyrinassociatedperiodicsyndrome AT tvvarlamova diagnosisandtreatmentofpatientswithcryopyrinassociatedperiodicsyndrome AT ayushcherbina diagnosisandtreatmentofpatientswithcryopyrinassociatedperiodicsyndrome |