{"orpha":97567,"name":"Immunotactoid glomerulopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Immunotactoid glomerulonephritis"],"definition":"Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP) are often grouped together as pathogenetically related diseases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97567/","orphanet_url":"https://www.orpha.net/en/disease/detail/97567","names":{"fr":{"name":"Glomérulonéphrite à dépôts organisés microtubulaires","synonyms":["Glomérulopathie à dépôts organisés microtubulaires"],"definition":"Maladie très rare caractérisée par une accumulation glomérulaire de microtubules dans le mésangium et la membrane basale glomérulaire, qui se manifeste principalement par une protéinurie, une microhématurie, un syndrome néphrotique, une insuffisance rénale et une hémopathie maligne. La glomérulonéphrite à dépôts organisés microtubulaires (GDOM) et la glomérulonéphrite à dépôts fibrillaires pseudoamyloïdes (GDF pseudoamyloïdes) sont souvent classées dans la même catégorie au vu de leurs similarités pathogénétiques."},"de":{"name":"Glomerulopathie, immunotaktoide","definition":"Die immunotaktische Glomerulopathie (ITG) ist eine sehr seltene Erkrankung, die durch eine glomeruläre Ansammlung von Mikrotubuli im Mesangium und in der glomerulären Basalmembran gekennzeichnet ist. Sie geht hauptsächlich mit Proteinurie, Mikrohämaturie, nephrotischem Syndrom, Niereninsuffizienz und hämatologischen Erkrankungen einher. ITG und die nicht amyloide fibrilläre Glomerulopathie (nicht amyloide FGP, s. dort) werden oft als pathogenetisch verwandte Erkrankungen zusammengefasst."},"es":{"name":"Glomerulopatía inmunotactoide","definition":"La glomerulopatía inmunotactoide (GIT) es una enfermedad muy poco frecuente caracterizada por una acumulación de microtúbulos en el mesangio y en la membrana basal glomerular, que se presenta principalmente con proteinuria, microhematuria, síndrome nefrótico, insuficiencia renal y neoplasia hematológica. La GIT y la glomerulopatía fibrilar no-amiloide (GPF no-amiloide) se agrupan normalmente como enfermedades patogenéticamente relacionadas."},"it":{"name":"Glomerulopatia immunotattoide","definition":"La glomerulopatia immunotattoide (ITG) è una malattia molto rara, caratterizzata dall'accumulo glomerulare di microtubuli nel mesangio e nella membrana basale glomerulare, che esordisce prevalentemente con proteinuria, micro-ematuria, sindrome nefrosica, insufficienza renale e tumori ematologici maligni. La ITG e la glomerulopatia fibrillare non amiloide (FGP non amiloide; si veda questo termine) vengono spesso raggruppate in quanto correlate a livello patogenetico."},"nl":{"name":"Immunotactoïde glomerulopathie","definition":"Immunotactoïde glomerulopathie (ITG) is een zeer zeldzame conditie die gekarakteriseerd wordt door glomerulaire accumulatie van microtubuli in het mesangium en de basale membraan van de glomeruli, en die zich meestal voordoet met proteïnurie, microhematurie, nefrotisch syndroom, nierinsufficiëntie en hematologische maligniteit. ITG en niet-amyloïde fibrillaire glomerulopathie (niet-amyloïde FGP; zie deze term) worden vaak samen gegroepeerd als pathogenetisch verwante ziekten."},"pt":{"name":"Glomerulopatia imunotactoide"},"pl":{"name":"Glomerulopatia immunotaktoidalna","synonyms":["Immunotactoid glomerulonephritis"],"definition":"Glomerulopatia immunotaktoidalna (Immunotactoid glomerulopathy – ITG) to bardzo rzadka choroba charakteryzująca się gromadzeniem mikrotubul w mezangium i błonie podstawnej kłębuszków nerkowych, objawiająca się białkomoczem pochodzenia nerkowego, mikrohematurią, zespołem nefrotycznym, niewydolnością nerek i zaburzeniami hematologicznymi. ITG i nieamyloidowa glomerulopatia włókienkowa (nieamloidowa FGP) są często zgrupowane razem ze względu na podobną patogenezę."},"cs":{"name":"Imunotaktoidní glomerulopatie","synonyms":["Imunotaktoidní glomerulonefritida"]},"uk":{"name":"Імунотактоїдна гломерулопатія","synonyms":["Імунотактоїдний гломерулонефрит"],"definition":"Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP, see this term) are often grouped together as pathogenetically related diseases."},"zh":{"name":"免疫触发性肾小球肾病","synonyms":["免疫触发性肾小球肾炎"],"definition":"免疫性乳突性肾小球病变(ITG)是一种非常罕见的疾病，主要表现为蛋白尿、微量血尿、肾病综合征、肾功能不全和血液系统恶性病变，其特征是肾小球系膜和肾小球基底膜微管堆积，主要表现为蛋白尿、微量血尿、肾病综合征、肾功能不全和血液系统恶性肿瘤。ITG和非淀粉样纤维性肾小球病变(非淀粉样蛋白FGP，见该词条)通常被归类为致病相关疾病。"},"tr":{"name":"İmmünotaktoid glomerülopati","synonyms":["İmmünotaktoid glomerülonefrit"]}},"xrefs":[{"source":"ICD-10","code":"N03.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5D00.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"144880802"},{"source":"MONDO","code":"0019991","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2242534","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12048","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10067871","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":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":200.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}