{"orpha":84087,"name":"Collagen type III glomerulopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Collagenofibrotic glomerulopathy"],"definition":"A rare non-immune-mediated glomerular disease characterized by abnormal accumulation of type III collagen within the mesangium and subendothelial space of the glomerulus. Clinically it usually manifests with proteinuria (often in the nephrotic range), microscopic hematuria, peripheral edema and/or hypertension. Progression to end-stage kidney failure is possible.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-84087/","orphanet_url":"https://www.orpha.net/en/disease/detail/84087","names":{"fr":{"name":"Glomérulopathie à dépôts de collagène de type III","definition":"Glomérulopathie rare sans médiation immunitaire, caractérisée par l'accumulation anormale de collagène de type III dans le mésangium et l'espace sous-endothélial du glomérule. Sur le plan clinique, la maladie se manifeste habituellement par une protéinurie (souvent néphrotique), une hématurie microscopique, un oedème périphérique et/ou une hypertension. Certains cas progressent vers l'insuffisance rénale terminale."},"de":{"name":"Kollagen Typ III-Glomerulopathie","synonyms":["Kollagenofibrotische Glomerulopathie"],"definition":"Eine seltene, nicht immunvermittelte glomeruläre Erkrankung, die durch eine abnorme Ansammlung von Typ-III-Kollagen im Mesangium und im subendothelialen Raum des Glomerulus gekennzeichnet ist. Klinisch manifestiert sie sich meist mit Proteinurie (oft im nephrotischen Bereich), mikroskopischer Hämaturie, peripheren Ödemen und/oder Hypertonie. In einigen Fällen wird eine Progression zum terminalem Nierenversagen beobachtet."},"es":{"name":"Glomerulopatía por colágeno tipo III","synonyms":["Glomerulopatía colagenofibrótica"],"definition":"Es una enfermedad glomerular no inmunomediada caracterizada por un acúmulo anómalo de colágeno de tipo III en el mesangio y en el espacio subendotelial del glomérulo. Clínicamente suele manifestarse con proteinuria (a menudo en el rango nefrótico), hematuria microscópica, edema periférico y/o hipertensión. La progresión a insuficiencia renal terminal es posible."},"it":{"name":"Glomerulopatia da deposito di collagene tipo 3","definition":"Si tratta di una glomerulopatia rara non immuno-mediata, caratterizzata da depositi anomali di collagene di tipo III nel mesangio e nello spazio subendoteliale del glomerulo. Dal punto di vista clinico, la malattia in genere si manifesta con proteinuria (spesso nel range nefrosico), ematuria microscopica, edema periferico e/o ipertensione. È possibile la progressione verso l'insufficienza renale terminale."},"nl":{"name":"Collageen type III-glomerulopathie","synonyms":["Collagenofibrotische glomerulopathie"],"definition":"Een zeldzame niet-immuungemedieerde glomerulaire aandoening, gekenmerkt door abnormale accumulatie van type III collageen in mesangium en subendotheliale ruimte van glomerulus. Klinisch manifesteert de aandoening zich meestal met proteïnurie (vaak in het nefrotische bereik), microscopische hematurie, perifeer oedeem, en/of hypertensie. Progressie naar terminaal nierfalen is mogelijk."},"pt":{"name":"Glomerulopatia de colagénio tipo III"},"pl":{"name":"Glomerulopatia kolagenu typu III","synonyms":["Collagenofibrotic glomerulopathy"]},"cs":{"name":"Glomerulopatie související s kolagenem typu III","synonyms":["Collagenofibrotic glomerulopathy"]},"uk":{"name":"Колагенова гломерулопатія, тип III","synonyms":["Колагенофіброзна гломерулопатія"],"definition":"A rare non-immune-mediated glomerular disease characterized by abnormal accumulation of type III collagen within the mesangium and subendothelial space of the glomerulus. Clinically it usually manifests with proteinuria (often in the nephrotic range), microscopic hematuria, peripheral edema and/or hypertension. Progression to end-stage kidney failure is possible."},"zh":{"name":"胶原3型肾小球病","synonyms":["胶原纤维性肾小球病"]},"tr":{"name":"Kollajen tip III glomerülopati","synonyms":["Kolajenofibrotik glomerülopati"]}},"xrefs":[{"source":"ICD-10","code":"N07.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB4Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019396","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3872695","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":93548,"name":"Glomerular disease","classifications":[188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}