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Neonatal treatment of CINCA syndrome
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نویسنده
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paccaud y. ,berthet g. ,von scheven-gête a. ,vaudaux b. ,mivelaz y. ,hofer m. ,roth-kleiner m.
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منبع
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pediatric rheumatology - 2014 - دوره : 12 - شماره : 1
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چکیده
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Chronic infantile neurological cutaneous articular (cinca) syndrome,also called neonatal onset multisystem inflammatory disease (nomid) is a chronic disease with early onset affecting mainly the central nervous system,bones and joints and may lead to permanent damage. we report two preterm infants with severe cinca syndrome treated by anti-interleukin-1 in the neonatal period,although,so far,no experience with this treatment in infants younger than three months of age has been reported. a review of the literature was performed with focus on treatment and neonatal features of cinca syndrome. case report: two cases suspected to have cinca syndrome were put on treatment with anakinra in the early neonatal period due to severe clinical presentation. we observed a rapid and persistent decline of clinical signs and systemic inflammation and good drug tolerance. diagnosis was confirmed in both cases by mutations in the nlrp3/cias1-gene coding for cryopyrin. as particular neonatal clinical signs polyhydramnios and endocardial overgrowth are to be mentioned. conclusion: we strongly suggest that specific treatment targeting interleukin-1 activity should be started early. being well tolerated,it can be introduced already in neonates presenting clinical signs of severe cinca syndrome in order to rapidly control inflammation and to prevent life-long disability. © 2014 paccaud et al.
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کلیدواژه
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Anakinra; CINCA; IL-1 receptor; IL-1β; Neonatal treatment; NOMID
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آدرس
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clinic of neonatology,department of pediatrics,university hospital and university of lausanne,lausanne, Switzerland, clinic of pediatrics,cantonal hospital of aarau,aarau, Switzerland, unit of pediatric rheumatology of western switzerland,departments of pediatrics,university hospital and university of lausanne,university hospital of geneva,geneva, Switzerland, pediatric infectiology unit,department of pediatrics,university hospital and university of lausanne,lausanne, Switzerland, pediatric cardiology unit,department of pediatrics,university hospital and university of lausanne,lausanne, Switzerland, unit of pediatric rheumatology of western switzerland,departments of pediatrics,university hospital and university of lausanne,university hospital of geneva,geneva, Switzerland, clinic of neonatology,department of pediatrics,university hospital and university of lausanne,lausanne,switzerland,clinic of neonatology,centre hospitalier universitaire vaudois,chuv,av pierre decker 2,lausanne,ch-1011, Switzerland
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Authors
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