Universität zu Köln

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

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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
Creators:	Creators Email ORCID ORCID Put Code Brochhausen, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Babel, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmitt, V. H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Grevenstein, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schreml, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Meyer-Scholten, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Klaus, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED
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
Uncontrolled Keywords:	Keywords Language ANGIOGENESIS; CELLS; INHIBITION; GROWTH Multiple languages Rheumatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/17506