FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2063760967> ?p ?o ?g. }

• W2063760967 endingPage "148" @default.
• W2063760967 startingPage "143" @default.
• W2063760967 abstract "The immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) is a rare disorder caused by mutations of the FOXP3 gene. The FOXP3 gene encodes a DNA-binding protein of the forkhead/winged-helix family and is the central controller of the development of CD4+CD25+ regulatory T cells. CD4+CD25+ regulatory T cells help prevent autoimmune disease; a deficiency of these cells causes increased immunologic reactivity and autoimmunity. We describe a 14-year-old boy with IPEX syndrome confirmed by mutation analysis of the FOXP3 gene. The patient had chronic dermatitis and later developed bullous pemphigoid. He subsequently formed diffuse prurigo nodularis–like lesions resistant to multiple topical and systemic immunosuppressive medications. These lesions were confirmed by biopsy, direct immunofluorescence, and enzyme-linked immunosorbent assay of the 180 kd bullous pemphigoid antigen to be pemphigoid nodularis. He recently responded to rituximab, allowing discontinuation of his oral prednisone. The immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) is a rare disorder caused by mutations of the FOXP3 gene. The FOXP3 gene encodes a DNA-binding protein of the forkhead/winged-helix family and is the central controller of the development of CD4+CD25+ regulatory T cells. CD4+CD25+ regulatory T cells help prevent autoimmune disease; a deficiency of these cells causes increased immunologic reactivity and autoimmunity. We describe a 14-year-old boy with IPEX syndrome confirmed by mutation analysis of the FOXP3 gene. The patient had chronic dermatitis and later developed bullous pemphigoid. He subsequently formed diffuse prurigo nodularis–like lesions resistant to multiple topical and systemic immunosuppressive medications. These lesions were confirmed by biopsy, direct immunofluorescence, and enzyme-linked immunosorbent assay of the 180 kd bullous pemphigoid antigen to be pemphigoid nodularis. He recently responded to rituximab, allowing discontinuation of his oral prednisone." @default.
• W2063760967 created "2016-06-24" @default.
• W2063760967 creator A5037631413 @default.
• W2063760967 creator A5051726843 @default.
• W2063760967 creator A5052932911 @default.
• W2063760967 creator A5057822789 @default.
• W2063760967 creator A5060863731 @default.
• W2063760967 date "2006-07-01" @default.
• W2063760967 modified "2023-09-23" @default.
• W2063760967 title "Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) associated with pemphigoid nodularis: A case report and review of the literature" @default.
• W2063760967 cites W1486722793 @default.
• W2063760967 cites W1499151513 @default.
• W2063760967 cites W1564285195 @default.
• W2063760967 cites W1574744856 @default.
• W2063760967 cites W1611284225 @default.
• W2063760967 cites W1966289025 @default.
• W2063760967 cites W1972187865 @default.
• W2063760967 cites W1973059694 @default.
• W2063760967 cites W1980201274 @default.
• W2063760967 cites W1983339176 @default.
• W2063760967 cites W1992950426 @default.
• W2063760967 cites W1996902432 @default.
• W2063760967 cites W2000831718 @default.
• W2063760967 cites W2002726823 @default.
• W2063760967 cites W2008011653 @default.
• W2063760967 cites W2015937151 @default.
• W2063760967 cites W2020731438 @default.
• W2063760967 cites W2025766796 @default.
• W2063760967 cites W2038625225 @default.
• W2063760967 cites W2045252184 @default.
• W2063760967 cites W2061811357 @default.
• W2063760967 cites W2064149316 @default.
• W2063760967 cites W2064976599 @default.
• W2063760967 cites W2067108551 @default.
• W2063760967 cites W2071352985 @default.
• W2063760967 cites W2072447036 @default.
• W2063760967 cites W2088622301 @default.
• W2063760967 cites W2091065682 @default.
• W2063760967 cites W2094430543 @default.
• W2063760967 cites W2097811455 @default.
• W2063760967 cites W2113499607 @default.
• W2063760967 cites W2115356027 @default.
• W2063760967 cites W2117486036 @default.
• W2063760967 cites W2152298295 @default.
• W2063760967 cites W2163510549 @default.
• W2063760967 cites W2166725781 @default.
• W2063760967 cites W2415998525 @default.
• W2063760967 cites W2564152524 @default.
• W2063760967 cites W3148978848 @default.
• W2063760967 cites W4214725158 @default.
• W2063760967 cites W4229637961 @default.
• W2063760967 cites W4232515381 @default.
• W2063760967 cites W4232547926 @default.
• W2063760967 cites W4241121236 @default.
• W2063760967 cites W4256247450 @default.
• W2063760967 doi "https://doi.org/10.1016/j.jaad.2005.08.047" @default.
• W2063760967 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16781310" @default.
• W2063760967 hasPublicationYear "2006" @default.
• W2063760967 type Work @default.
• W2063760967 sameAs 2063760967 @default.
• W2063760967 citedByCount "76" @default.
• W2063760967 countsByYear W20637609672012 @default.
• W2063760967 countsByYear W20637609672013 @default.
• W2063760967 countsByYear W20637609672014 @default.
• W2063760967 countsByYear W20637609672015 @default.
• W2063760967 countsByYear W20637609672016 @default.
• W2063760967 countsByYear W20637609672017 @default.
• W2063760967 countsByYear W20637609672018 @default.
• W2063760967 countsByYear W20637609672019 @default.
• W2063760967 countsByYear W20637609672020 @default.
• W2063760967 countsByYear W20637609672021 @default.
• W2063760967 countsByYear W20637609672022 @default.
• W2063760967 countsByYear W20637609672023 @default.
• W2063760967 crossrefType "journal-article" @default.
• W2063760967 hasAuthorship W2063760967A5037631413 @default.
• W2063760967 hasAuthorship W2063760967A5051726843 @default.
• W2063760967 hasAuthorship W2063760967A5052932911 @default.
• W2063760967 hasAuthorship W2063760967A5057822789 @default.
• W2063760967 hasAuthorship W2063760967A5060863731 @default.
• W2063760967 hasConcept C142724271 @default.
• W2063760967 hasConcept C159654299 @default.
• W2063760967 hasConcept C203014093 @default.
• W2063760967 hasConcept C2778075117 @default.
• W2063760967 hasConcept C2778426112 @default.
• W2063760967 hasConcept C2779134260 @default.
• W2063760967 hasConcept C2779252107 @default.
• W2063760967 hasConcept C2779727006 @default.
• W2063760967 hasConcept C2780130043 @default.
• W2063760967 hasConcept C2780194698 @default.
• W2063760967 hasConcept C71924100 @default.
• W2063760967 hasConcept C8891405 @default.
• W2063760967 hasConceptScore W2063760967C142724271 @default.
• W2063760967 hasConceptScore W2063760967C159654299 @default.
• W2063760967 hasConceptScore W2063760967C203014093 @default.
• W2063760967 hasConceptScore W2063760967C2778075117 @default.
• W2063760967 hasConceptScore W2063760967C2778426112 @default.
• W2063760967 hasConceptScore W2063760967C2779134260 @default.
• W2063760967 hasConceptScore W2063760967C2779252107 @default.

• next