{"orpha":97566,"name":"Non-amyloid fibrillary glomerulopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Congo red-negative amyloidosis-like glomerulopathy","Non-amyloid fibrillary glomerulonephritis"],"definition":"Non-amyloid fibrillary glomerulopathy (non-amyloid FGP) is a rare cause of glomerulonephritis (GN) characterized by glomerular accumulation of non-amyloid fibrils in the mesangium and the glomerular (and rarely tubular) basement membrane, that mainly presents with renal insufficiency, micro-hematuria and nephrotic range proteinuria. Non-amyloid FGP and immunotactoid glomerulopathy (ITG) are often grouped together as pathogenetically related diseases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97566/","orphanet_url":"https://www.orpha.net/en/disease/detail/97566","names":{"fr":{"name":"Glomérulonéphrite à dépôts fibrillaires pseudoamyloïdes","synonyms":["Glomérulopathie à dépôts fibrillaires pseudoamyloïdes"],"definition":"Rare cause de glomérulonéphrite caractérisée par des dépôts fibrillaires pseudoamyloïdes dans la membrane basale du mésangium et du glomérule rénal (plus rarement du tubule rénal), qui se manifeste principalement par une insuffisance rénale, une hématurie microscopique et une protéinurie néphrotique. La glomérulonéphrite à dépôts fibrillaires pseudoamyloïdes (GDF pseudoamyloïdes) et la glomérulonéphrite à dépôts organisés microtubulaires sont souvent regroupées au vu de leurs similitudes pathogénétiques."},"de":{"name":"Glomerulopathie, fibrilläre nicht-amyloide","definition":"Die nicht-amyloide fibrilläre Glomerulopathie (nicht-amyloide FGP) ist eine seltene Ursache der Glomerulonephritis (GN), die gekennzeichnet ist durch eine glomeruläre Ansammlung von nicht-amyloiden Fibrillen im Mesangium und der glomerulären (und selten tubulären) Basalmembran, die sich hauptsächlich mit Niereninsuffizienz, Mikrohämaturie und nephrotischer Proteinurie manifestiert. Die nicht-amyloide FGP und die immunotaktische Glomerulopathie (ITG, s. dort) werden oft als pathogenetisch verwandte Krankheiten zusammengefasst."},"es":{"name":"Glomerulopatía fibrilar no amiloide","definition":"La glomerulopatía fibrilar no-amiloide (GPF no-amiloide) es un caso poco frecuente de glomerulonefritis (GN) caracterizado por una acumulación de fibrillas no amiloides en el mesangio y en la membrana basal glomerular (y raramente tubular), que principalmente se presenta con insuficiencia renal, micro-hematuria y proteinuria en rango nefrótico. La GPF no-amiloide y la glomerulopatía inmunotactoide (GIT) están frecuentemente agrupadas como enfermedades patogenéticamente relacionadas."},"it":{"name":"Glomerulopatia fibrillare non amiloide","definition":"La glomerulopatia fibrillare non amiloide (FGP non amiloide) è una forma rara di glomerulonefrite (GN) ed è caratterizzata dall'accumulo glomerulare di fibrille non amiloidi nel mesangio e nella membrana basale glomerulare (di rado in quella tubulare), che si manifesta prevalentemente con insufficienza renale, micro-ematuria e proteinuria di tipo nefrosico. La FGP non amiloide e la glomerulopatia immunotattoide (ITG; si veda questo termine) vengono spesso raggruppate in quanto correlate a livello patogenetico."},"nl":{"name":"Niet-amyloïde fibrillaire glomerulopathie","definition":"Niet-amyloïde fibrillaire glomerulopathie (niet-amyloïde FGP) is een zeldzame oorzaak van glomerulonefritis (GN) die wordt gekarakteriseerd door glomerulaire accumulatie van niet-amyloïde fibrillen in het mesangium en de glomerulaire (en zelden tubulaire) basale membraan, en die zich meestal voordoet met nierinsufficiëntie, microhematurie en proteïnurie in het nefrotische bereik. Niet-amyloïde FGP en immunotactoïde glomerulopathie (ITG; zie deze term) worden vaak samen gegroepeerd als pathogenetisch verwante ziekten."},"pt":{"name":"Glomerulopatia fibrilar, não-amilóide"},"pl":{"name":"Nieamyloidowa glomerulopatia włókienkowa","synonyms":["Congo red-negative amyloidosis-like glomerulopathy","Non-amyloid fibrillary glomerulonephritis"],"definition":"Nieamyloidowa glomerulopatia włókienkowa (non-amyloid fibrillary glomerulopathy – non-amyloid FGP) to rzadka przyczyna kłębuszkowego zapalenia nerek (glomerulonephriris – GN) charakteryzująca się gromadzeniem się nieamyloidowych włókienek w mezangium i błonie podstawnej kłębuszków nerkowych (i rzadko w kanalikach), która głównie objawia się niewydolnością nerek, mikrohematurią i białkomoczem pochodzenia nerkowego. Nieamyloidowa glomerulopatia włókienkowa i glomerulopatia immunotaktoidalna (ITG, sprawdź ten termin) są często zgrupowane razem ze względu na podobną patogenezę."},"cs":{"name":"Neamyloidová fibrilární glomerulopatie","synonyms":["Neamyloidová fibrilární glomerulonefritida","Kongo-červeň negativní glomerulopatie podobná amyloidóze"]},"uk":{"name":"Неамілоїдна фібрилярна гломерулопатія","synonyms":["Конго червоно-негативна амілоїдозоподібна гломерулопатія","Неамілоїдний фібрилярний гломерулонефрит"],"definition":"Non-amyloid fibrillary glomerulopathy (non-amyloid FGP) is a rare cause of glomerulonephritis (GN) characterized by glomerular accumulation of non-amyloid fibrils in the mesangium and the glomerular (and rarely tubular) basement membrane, that mainly presents with renal insufficiency, micro-hematuria and nephrotic range proteinuria. Non-amyloid FGP and immunotactoid glomerulopathy (ITG, see this term) are often grouped together as pathogenetically related diseases."},"zh":{"name":"非淀粉样纤维样肾小球病","synonyms":["刚果红阴性淀粉样变性样肾小球病"],"definition":"非淀粉样纤维状肾小球病变是肾小球肾炎(GN)的一种罕见病因，主要表现为肾功能不全、微量血尿和肾病范围蛋白尿，其特征是肾小球系膜和肾小球(很少为管状)非淀粉样纤维沉积。非淀粉样蛋白FGP和免疫乳样肾小球病变(ITG，见该词条)通常被归类为相关疾病。"},"tr":{"name":"Amiloid olmayan fibriller glomerülopati","synonyms":["Amiloid olmayan fibriller glomerülonefrit","Kongo kırmızısı-negatif amiloidoz benzeri glomerülopati"]}},"xrefs":[{"source":"ICD-10","code":"N03.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB4Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019990","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273674","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12740","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":91137,"name":"Immunotactoid or fibrillary glomerulopathy","classifications":[188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[],"phenotypes":null}