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Hydrocephalus in CINCA syndrome treated with anakinra
Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome is a rare congenital autoinflammatory disease characterized by neonatal-onset chronic meningitis, hydrocephalus, sensorineural hearing loss, persistent urticarial rash, deforming arthritis, and recurrent fever. This clinical entity...
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| Published in: | Child's nervous system 2006-04, Vol.22 (4), p.334-337 |
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| container_end_page | 337 |
| container_issue | 4 |
| container_start_page | 334 |
| container_title | Child's nervous system |
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| creator | Rigante, D Ansuini, V Caldarelli, M Bertoni, B La Torraca, I Stabile, A |
| description | Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome is a rare congenital autoinflammatory disease characterized by neonatal-onset chronic meningitis, hydrocephalus, sensorineural hearing loss, persistent urticarial rash, deforming arthritis, and recurrent fever. This clinical entity is believed to result from dysregulation of cytokine production. No recommended treatment protocol exists so far for CINCA syndrome.
We report a 7-year-old child affected with CINCA syndrome in whom no therapy had resulted effective. Anakinra, an interleukin-1-receptor antagonist, was administered in a 1-year period with complete inflammatory symptom remission and dramatically ameliorated laboratory tests. This optimal response has been supported by the demonstration of a stabilized hydrocephalus upon magnetic resonance imaging and by an overall improvement of the neurodevelopmental issues.
This paper emphasizes and discusses the medical approach with anakinra in CINCA syndrome presenting with hydrocephalus in which a consistent control of the neurological picture can be obtained. |
| doi_str_mv | 10.1007/s00381-006-1280-3 |
| format | article |
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We report a 7-year-old child affected with CINCA syndrome in whom no therapy had resulted effective. Anakinra, an interleukin-1-receptor antagonist, was administered in a 1-year period with complete inflammatory symptom remission and dramatically ameliorated laboratory tests. This optimal response has been supported by the demonstration of a stabilized hydrocephalus upon magnetic resonance imaging and by an overall improvement of the neurodevelopmental issues.
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We report a 7-year-old child affected with CINCA syndrome in whom no therapy had resulted effective. Anakinra, an interleukin-1-receptor antagonist, was administered in a 1-year period with complete inflammatory symptom remission and dramatically ameliorated laboratory tests. This optimal response has been supported by the demonstration of a stabilized hydrocephalus upon magnetic resonance imaging and by an overall improvement of the neurodevelopmental issues.
This paper emphasizes and discusses the medical approach with anakinra in CINCA syndrome presenting with hydrocephalus in which a consistent control of the neurological picture can be obtained.</description><subject>Arthritis - complications</subject><subject>Arthritis - immunology</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Chronic Disease</subject><subject>Fever - complications</subject><subject>Fever - immunology</subject><subject>Hearing Loss, Sensorineural - complications</subject><subject>Hearing Loss, Sensorineural - immunology</subject><subject>Humans</subject><subject>Hydrocephalus - complications</subject><subject>Hydrocephalus - drug therapy</subject><subject>Hydrocephalus - immunology</subject><subject>Immune System Diseases - complications</subject><subject>Immune System Diseases - drug therapy</subject><subject>Immune System Diseases - immunology</subject><subject>Immunologic Factors - therapeutic use</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Interleukin 1 Receptor Antagonist Protein - therapeutic use</subject><subject>Male</subject><subject>Meningitis - complications</subject><subject>Meningitis - immunology</subject><subject>Syndrome</subject><subject>Treatment Outcome</subject><subject>Urticaria - complications</subject><subject>Urticaria - immunology</subject><issn>0256-7040</issn><issn>1433-0350</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><recordid>eNpF0EtLAzEUBeAgiq3VH-BGZuUuejN5L8tgbaHoRtchM0no6DxqMoP03zulBVcXLuecxYfQPYEnAiCfEwBVBAMITHIFmF6gOWGUYqAcLtEcci6wBAYzdJPSFwDhKtfXaEYEz7liao74-uBiX_n9zjZjyuouKzZvxTJLh276tz4boreDd9lvPewy29nvuov2Fl0F2yR_d74L9Ll6-SjWePv-uimWW1zlWg9YOykVByhFGYQNunSUC8dzTyoXNKEOpKpKJZyygQPTkuocmGPSQahoaekCPZ5297H_GX0aTFunyjeN7Xw_JiOkAsU4n4LkFKxin1L0wexj3dp4MATM0cqcrMxkZY5Whk6dh_P4WLbe_TfOOPQP9HJjkQ</recordid><startdate>200604</startdate><enddate>200604</enddate><creator>Rigante, D</creator><creator>Ansuini, V</creator><creator>Caldarelli, M</creator><creator>Bertoni, B</creator><creator>La Torraca, I</creator><creator>Stabile, A</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200604</creationdate><title>Hydrocephalus in CINCA syndrome treated with anakinra</title><author>Rigante, D ; Ansuini, V ; Caldarelli, M ; Bertoni, B ; La Torraca, I ; Stabile, A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c299t-9d778500b6bf6af9bd356d52e1cdf913d078cb86d8af5049739204d47d0fc3ba3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2006</creationdate><topic>Arthritis - complications</topic><topic>Arthritis - immunology</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Chronic Disease</topic><topic>Fever - complications</topic><topic>Fever - immunology</topic><topic>Hearing Loss, Sensorineural - complications</topic><topic>Hearing Loss, Sensorineural - immunology</topic><topic>Humans</topic><topic>Hydrocephalus - complications</topic><topic>Hydrocephalus - drug therapy</topic><topic>Hydrocephalus - immunology</topic><topic>Immune System Diseases - complications</topic><topic>Immune System Diseases - drug therapy</topic><topic>Immune System Diseases - immunology</topic><topic>Immunologic Factors - therapeutic use</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Interleukin 1 Receptor Antagonist Protein - therapeutic use</topic><topic>Male</topic><topic>Meningitis - complications</topic><topic>Meningitis - immunology</topic><topic>Syndrome</topic><topic>Treatment Outcome</topic><topic>Urticaria - complications</topic><topic>Urticaria - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Rigante, D</creatorcontrib><creatorcontrib>Ansuini, V</creatorcontrib><creatorcontrib>Caldarelli, M</creatorcontrib><creatorcontrib>Bertoni, B</creatorcontrib><creatorcontrib>La Torraca, I</creatorcontrib><creatorcontrib>Stabile, A</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Child's nervous system</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Rigante, D</au><au>Ansuini, V</au><au>Caldarelli, M</au><au>Bertoni, B</au><au>La Torraca, I</au><au>Stabile, A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Hydrocephalus in CINCA syndrome treated with anakinra</atitle><jtitle>Child's nervous system</jtitle><addtitle>Childs Nerv Syst</addtitle><date>2006-04</date><risdate>2006</risdate><volume>22</volume><issue>4</issue><spage>334</spage><epage>337</epage><pages>334-337</pages><issn>0256-7040</issn><eissn>1433-0350</eissn><notes>ObjectType-Case Study-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-4</notes><notes>content type line 23</notes><notes>ObjectType-Report-1</notes><notes>ObjectType-Article-3</notes><abstract>Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome is a rare congenital autoinflammatory disease characterized by neonatal-onset chronic meningitis, hydrocephalus, sensorineural hearing loss, persistent urticarial rash, deforming arthritis, and recurrent fever. This clinical entity is believed to result from dysregulation of cytokine production. No recommended treatment protocol exists so far for CINCA syndrome.
We report a 7-year-old child affected with CINCA syndrome in whom no therapy had resulted effective. Anakinra, an interleukin-1-receptor antagonist, was administered in a 1-year period with complete inflammatory symptom remission and dramatically ameliorated laboratory tests. This optimal response has been supported by the demonstration of a stabilized hydrocephalus upon magnetic resonance imaging and by an overall improvement of the neurodevelopmental issues.
This paper emphasizes and discusses the medical approach with anakinra in CINCA syndrome presenting with hydrocephalus in which a consistent control of the neurological picture can be obtained.</abstract><cop>Germany</cop><pmid>16525848</pmid><doi>10.1007/s00381-006-1280-3</doi><tpages>4</tpages></addata></record> |
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| ispartof | Child's nervous system, 2006-04, Vol.22 (4), p.334-337 |
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| language | eng |
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| subjects | Arthritis - complications Arthritis - immunology Child Child, Preschool Chronic Disease Fever - complications Fever - immunology Hearing Loss, Sensorineural - complications Hearing Loss, Sensorineural - immunology Humans Hydrocephalus - complications Hydrocephalus - drug therapy Hydrocephalus - immunology Immune System Diseases - complications Immune System Diseases - drug therapy Immune System Diseases - immunology Immunologic Factors - therapeutic use Infant Infant, Newborn Interleukin 1 Receptor Antagonist Protein - therapeutic use Male Meningitis - complications Meningitis - immunology Syndrome Treatment Outcome Urticaria - complications Urticaria - immunology |
| title | Hydrocephalus in CINCA syndrome treated with anakinra |
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