Brochhausen, C., Babel, M., Schmitt, V. H., Grevenstein, D., Schreml, S., Meyer-Scholten, C. and Klaus, G. (2018). Skin ulcerations due to CINCA syndrome and its successful treatment with prostaglandin E-1. Z. Rheumatol., 77 (7). S. 633 - 637. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1435-1250
Full text not available from this repository.Abstract
ZusammenfassungDas chronische infantile neurokutaneoartikulare Syndrom (CINCA) ist eine Erkrankung mit Defekt im CIAS1 (NLRP3)-Gen, welches uber das veranderte Genprodukt Cryopyrin zu einer Inflammasomaktivierung mit konsekutiv gesteigerter IL-1-Produktion fuhrt. Auch der Aktivierungsweg des Transkriptionsfaktors NF-B wird beeinflusst, der u.a. bei der Angiogenese eine Rolle spielt. Bei einer Patientin mit CINCA-Syndrom, die unter therapierefraktaren Hautulzera litt, konnten wir unter der Rationale eines angiogenen Effektes von Prostaglandin E-1 nach periodischen Infusionen (6 mu g/kg/24h/5Tage) uber den Zeitraum von 1Jahr eine Wundheilung mit dauerhaftem Wundverschluss erreichen. AbstractChronic infantile neurological cutaneous and articular syndrome (CINCA) is adisorder with a defect in the CIAS1 (NLRP3) gene and the altered gene product cryopyrin leads to inflammasome activation with increased IL-1beta synthesis. The activation pathway of the transcription factor NF-B is also affected, which plays arole in angiogenesis. With respect to the angiogenesis stimulating ability of prostaglandin E-1, we treated afemale patient with CINCA syndrome and conventionally non-responsive skin ulcers with prostaglandin E-1 infusions (6g/kg bw/24h/5day) followed by wound healing lasting over 3 weeks. After 1 year of periodic infusions, the skin defects were permanently closed.
Item Type: | Journal Article | ||||||||||||||||||||||||||||||||
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URN: | urn:nbn:de:hbz:38-175061 | ||||||||||||||||||||||||||||||||
DOI: | 10.1007/s00393-018-0515-5 | ||||||||||||||||||||||||||||||||
Journal or Publication Title: | Z. Rheumatol. | ||||||||||||||||||||||||||||||||
Volume: | 77 | ||||||||||||||||||||||||||||||||
Number: | 7 | ||||||||||||||||||||||||||||||||
Page Range: | S. 633 - 637 | ||||||||||||||||||||||||||||||||
Date: | 2018 | ||||||||||||||||||||||||||||||||
Publisher: | SPRINGER HEIDELBERG | ||||||||||||||||||||||||||||||||
Place of Publication: | HEIDELBERG | ||||||||||||||||||||||||||||||||
ISSN: | 1435-1250 | ||||||||||||||||||||||||||||||||
Language: | German | ||||||||||||||||||||||||||||||||
Faculty: | Unspecified | ||||||||||||||||||||||||||||||||
Divisions: | Unspecified | ||||||||||||||||||||||||||||||||
Subjects: | no entry | ||||||||||||||||||||||||||||||||
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Refereed: | Yes | ||||||||||||||||||||||||||||||||
URI: | http://kups.ub.uni-koeln.de/id/eprint/17506 |
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