FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4366991290> ?p ?o ?g. }

• W4366991290 endingPage "644" @default.
• W4366991290 startingPage "644" @default.
• W4366991290 abstract "Cutaneous manifestations are one of the most common presentations among patients with inborn errors of immunity (IEI). These skin manifestations are often among the first presenting features in the majority of patients preceding the IEI diagnosis. We studied 521 available monogenic patients with IEI listed in the Iranian IEI registry up to November 2022. We extracted each patient's demographic information, detailed clinical history of cutaneous manifestations, and immunologic evaluations. The patients were then categorized and compared based on their phenotypical classifications provided by the International Union of Immunological Societies. Most patients were categorized into syndromic combined immunodeficiency (25.1%), non-syndromic combined immunodeficiency (24.4%), predominantly antibody deficiency (20.7%), and diseases of immune dysregulation (20.5%). In total, 227 patients developed skin manifestations at a median (IQR) age of 2.0 (0.5-5.2) years; a total of 66 (40.7%) of these patients initially presented with these manifestations. Patients with cutaneous involvement were generally older at the time of diagnosis [5.0 (1.6-8.0) vs. 3.0 (1.0-7.0) years; p = 0.022]. Consanguinity was more common among patients who developed skin disorders (81.4% vs. 65.2%, p < 0.001). The overall skin infection rate and the type of dominant pathogens were significantly different among the IEI patients in different phenotypical classifications (p < 0.001). Atopic presentation, including urticaria, was highly prevalent among patients with congenital defects of phagocytes (p = 0.020). The frequency of eczema was also significantly higher among cases with both syndromic and non-syndromic combined immunodeficiency (p = 0.009). In contrast, autoimmune cutaneous manifestations, including alopecia and psoriasis, were most common in patients with immune dysregulation (p = 0.001) and defects in intrinsic or innate immunity (p = 0.031), respectively. The presence of autoimmune cutaneous complications significantly improved the survival rate of IEI patients (p = 0.21). In conclusion, cutaneous manifestations were observed in nearly 44% of Iranian patients with monogenic IEI. A considerable number of patients with cutaneous involvements developed these disorders as their first manifestation of the disease, which was particularly noticeable in patients with non-syndromic combined immunodeficiency and phagocytic defects. The neglected skin disorders in IEI patients might delay diagnosis, which is generally established within a 3-year interval from the development of skin-related problems. Cutaneous disorders, especially autoimmune features, might indicate a mild prognosis in IEI patients." @default.
• W4366991290 created "2023-04-27" @default.
• W4366991290 creator A5002799085 @default.
• W4366991290 creator A5004388838 @default.
• W4366991290 creator A5014068059 @default.
• W4366991290 creator A5018220598 @default.
• W4366991290 creator A5024541850 @default.
• W4366991290 creator A5025067610 @default.
• W4366991290 creator A5026108376 @default.
• W4366991290 creator A5028354492 @default.
• W4366991290 creator A5032866577 @default.
• W4366991290 creator A5036184465 @default.
• W4366991290 creator A5041361661 @default.
• W4366991290 creator A5043131199 @default.
• W4366991290 creator A5051258190 @default.
• W4366991290 creator A5051539580 @default.
• W4366991290 creator A5057262090 @default.
• W4366991290 creator A5063588114 @default.
• W4366991290 creator A5063627272 @default.
• W4366991290 creator A5084770398 @default.
• W4366991290 creator A5087698983 @default.
• W4366991290 creator A5090636400 @default.
• W4366991290 date "2023-04-24" @default.
• W4366991290 modified "2023-10-14" @default.
• W4366991290 title "Autoimmune versus Non-autoimmune Cutaneous Features in Monogenic Patients with Inborn Errors of Immunity" @default.
• W4366991290 cites W1541934272 @default.
• W4366991290 cites W1969451697 @default.
• W4366991290 cites W2013136108 @default.
• W4366991290 cites W2031213205 @default.
• W4366991290 cites W2051978340 @default.
• W4366991290 cites W2065284615 @default.
• W4366991290 cites W2080625195 @default.
• W4366991290 cites W2104147879 @default.
• W4366991290 cites W2158564200 @default.
• W4366991290 cites W2310664088 @default.
• W4366991290 cites W2756116231 @default.
• W4366991290 cites W2809153463 @default.
• W4366991290 cites W2897055301 @default.
• W4366991290 cites W2898617924 @default.
• W4366991290 cites W2903532032 @default.
• W4366991290 cites W2914208637 @default.
• W4366991290 cites W2918426287 @default.
• W4366991290 cites W2998817435 @default.
• W4366991290 cites W3005664566 @default.
• W4366991290 cites W3043146115 @default.
• W4366991290 cites W3111249725 @default.
• W4366991290 cites W3127088777 @default.
• W4366991290 cites W3134508234 @default.
• W4366991290 cites W3165749521 @default.
• W4366991290 cites W3202768221 @default.
• W4366991290 cites W3205938183 @default.
• W4366991290 cites W4210833938 @default.
• W4366991290 cites W4225982713 @default.
• W4366991290 cites W4283363193 @default.
• W4366991290 cites W4306646032 @default.
• W4366991290 cites W2743402386 @default.
• W4366991290 doi "https://doi.org/10.3390/biology12050644" @default.
• W4366991290 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37237458" @default.
• W4366991290 hasPublicationYear "2023" @default.
• W4366991290 type Work @default.
• W4366991290 citedByCount "0" @default.
• W4366991290 crossrefType "journal-article" @default.
• W4366991290 hasAuthorship W4366991290A5002799085 @default.
• W4366991290 hasAuthorship W4366991290A5004388838 @default.
• W4366991290 hasAuthorship W4366991290A5014068059 @default.
• W4366991290 hasAuthorship W4366991290A5018220598 @default.
• W4366991290 hasAuthorship W4366991290A5024541850 @default.
• W4366991290 hasAuthorship W4366991290A5025067610 @default.
• W4366991290 hasAuthorship W4366991290A5026108376 @default.
• W4366991290 hasAuthorship W4366991290A5028354492 @default.
• W4366991290 hasAuthorship W4366991290A5032866577 @default.
• W4366991290 hasAuthorship W4366991290A5036184465 @default.
• W4366991290 hasAuthorship W4366991290A5041361661 @default.
• W4366991290 hasAuthorship W4366991290A5043131199 @default.
• W4366991290 hasAuthorship W4366991290A5051258190 @default.
• W4366991290 hasAuthorship W4366991290A5051539580 @default.
• W4366991290 hasAuthorship W4366991290A5057262090 @default.
• W4366991290 hasAuthorship W4366991290A5063588114 @default.
• W4366991290 hasAuthorship W4366991290A5063627272 @default.
• W4366991290 hasAuthorship W4366991290A5084770398 @default.
• W4366991290 hasAuthorship W4366991290A5087698983 @default.
• W4366991290 hasAuthorship W4366991290A5090636400 @default.
• W4366991290 hasBestOaLocation W43669912901 @default.
• W4366991290 hasConcept C159654299 @default.
• W4366991290 hasConcept C16005928 @default.
• W4366991290 hasConcept C187212893 @default.
• W4366991290 hasConcept C203014093 @default.
• W4366991290 hasConcept C2776433325 @default.
• W4366991290 hasConcept C2777607303 @default.
• W4366991290 hasConcept C2778329239 @default.
• W4366991290 hasConcept C2779019163 @default.
• W4366991290 hasConcept C2779923321 @default.
• W4366991290 hasConcept C2780194698 @default.
• W4366991290 hasConcept C71924100 @default.
• W4366991290 hasConcept C86803240 @default.
• W4366991290 hasConcept C8891405 @default.
• W4366991290 hasConceptScore W4366991290C159654299 @default.
• W4366991290 hasConceptScore W4366991290C16005928 @default.

• next