FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3040463386> ?p ?o ?g. }

• W3040463386 endingPage "2131" @default.
• W3040463386 startingPage "2131" @default.
• W3040463386 abstract "Hypocomplementemic urticarial vasculitis syndrome (HUVS), or McDuffie syndrome, is a rare small vessel vasculitis associated with urticaria, hypocomplementemia and positivity of anti-C1q antibodies. In rare cases, HUVS can manifest as an immune-complex mediated glomerulonephritis with a membranoproliferative pattern of injury. Due to the rarity of this disorder, little is known about the clinical manifestation, pathogenesis, treatment response and outcome of such patients. We describe here three cases of HUVS with severe renal involvement. These patients had a rapidly progressive form of glomerulonephritis with severe nephrotic syndrome against a background of a membranoproliferative pattern of glomerular injury with extensive crescent formation. Therefore, these patients required aggressive induction and maintenance immunosuppressive therapy, with a clinical and renal response in two patients, while the third patient progressed to end-stage renal disease. Because of the rarity of this condition, there are few data regarding the clinical presentation, pathology and outcome of such patients. Accordingly, we provide an extensive literature review of cases reported from 1976 until 2020 and place them in the context of the current knowledge of HUVS pathogenesis. We identified 60 patients with HUVS and renal involvement that had adequate clinical data reported, out of which 52 patients underwent a percutaneous kidney biopsy. The most frequent renal manifestation was hematuria associated with proteinuria (70% of patients), while one third had abnormal kidney function on presentation (estimated glomerular filtration (GFR) below 60 mL/min/1.73 m2). The most frequent glomerular pattern of injury was membranoproliferative (35%), followed by mesangioproliferative (21%) and membranous (19%). Similar to other systemic vasculitis, renal involvement carries a poorer prognosis, but the outcome can be improved by aggressive immunosuppressive treatment." @default.
• W3040463386 created "2020-07-10" @default.
• W3040463386 creator A5002604187 @default.
• W3040463386 creator A5012174065 @default.
• W3040463386 creator A5029584267 @default.
• W3040463386 creator A5044192397 @default.
• W3040463386 creator A5050154347 @default.
• W3040463386 creator A5071844231 @default.
• W3040463386 creator A5074786320 @default.
• W3040463386 date "2020-07-06" @default.
• W3040463386 modified "2023-10-18" @default.
• W3040463386 title "Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review" @default.
• W3040463386 cites W1574593386 @default.
• W3040463386 cites W1644867893 @default.
• W3040463386 cites W168720297 @default.
• W3040463386 cites W1777816980 @default.
• W3040463386 cites W1965852727 @default.
• W3040463386 cites W1968937582 @default.
• W3040463386 cites W1970172000 @default.
• W3040463386 cites W1973213373 @default.
• W3040463386 cites W1978155311 @default.
• W3040463386 cites W1979612556 @default.
• W3040463386 cites W1981913695 @default.
• W3040463386 cites W1982522274 @default.
• W3040463386 cites W1982983726 @default.
• W3040463386 cites W2000574489 @default.
• W3040463386 cites W2014303963 @default.
• W3040463386 cites W2015618907 @default.
• W3040463386 cites W2018579962 @default.
• W3040463386 cites W2026281013 @default.
• W3040463386 cites W2026765255 @default.
• W3040463386 cites W2028590571 @default.
• W3040463386 cites W2029222345 @default.
• W3040463386 cites W2031517139 @default.
• W3040463386 cites W2050195386 @default.
• W3040463386 cites W2052663745 @default.
• W3040463386 cites W2059397286 @default.
• W3040463386 cites W2063495043 @default.
• W3040463386 cites W2065858204 @default.
• W3040463386 cites W2067217824 @default.
• W3040463386 cites W2067274685 @default.
• W3040463386 cites W2070909340 @default.
• W3040463386 cites W2077507687 @default.
• W3040463386 cites W2079317312 @default.
• W3040463386 cites W2079966908 @default.
• W3040463386 cites W2082440340 @default.
• W3040463386 cites W2083319127 @default.
• W3040463386 cites W2083437660 @default.
• W3040463386 cites W2086081331 @default.
• W3040463386 cites W2105009819 @default.
• W3040463386 cites W2105549572 @default.
• W3040463386 cites W2107504209 @default.
• W3040463386 cites W2111605985 @default.
• W3040463386 cites W2113209071 @default.
• W3040463386 cites W2127321441 @default.
• W3040463386 cites W2138171304 @default.
• W3040463386 cites W2140623520 @default.
• W3040463386 cites W2157745361 @default.
• W3040463386 cites W2207440368 @default.
• W3040463386 cites W2240795355 @default.
• W3040463386 cites W2322953276 @default.
• W3040463386 cites W2322968580 @default.
• W3040463386 cites W2409209259 @default.
• W3040463386 cites W2411180117 @default.
• W3040463386 cites W2418275968 @default.
• W3040463386 cites W2418532437 @default.
• W3040463386 cites W2551696561 @default.
• W3040463386 cites W2604550155 @default.
• W3040463386 cites W2612163713 @default.
• W3040463386 cites W2762247077 @default.
• W3040463386 cites W2769560628 @default.
• W3040463386 cites W2801034911 @default.
• W3040463386 cites W2890201507 @default.
• W3040463386 cites W2899799909 @default.
• W3040463386 cites W2904185114 @default.
• W3040463386 cites W2948756275 @default.
• W3040463386 cites W2977479222 @default.
• W3040463386 cites W360128320 @default.
• W3040463386 cites W4232872423 @default.
• W3040463386 cites W4243314081 @default.
• W3040463386 cites W60956676 @default.
• W3040463386 cites W2462946607 @default.
• W3040463386 doi "https://doi.org/10.3390/jcm9072131" @default.
• W3040463386 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7408727" @default.
• W3040463386 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32640739" @default.
• W3040463386 hasPublicationYear "2020" @default.
• W3040463386 type Work @default.
• W3040463386 sameAs 3040463386 @default.
• W3040463386 citedByCount "17" @default.
• W3040463386 countsByYear W30404633862020 @default.
• W3040463386 countsByYear W30404633862021 @default.
• W3040463386 countsByYear W30404633862022 @default.
• W3040463386 countsByYear W30404633862023 @default.
• W3040463386 crossrefType "journal-article" @default.
• W3040463386 hasAuthorship W3040463386A5002604187 @default.
• W3040463386 hasAuthorship W3040463386A5012174065 @default.
• W3040463386 hasAuthorship W3040463386A5029584267 @default.
• W3040463386 hasAuthorship W3040463386A5044192397 @default.

• next