{"orpha":329903,"name":"Immunoglobulin-mediated membranoproliferative glomerulonephritis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Ig-mediated MPGN","Ig-mediated membranoproliferative glomerulonephritis","Immunoglobulin-mediated MPGN"],"definition":"A primary form of membranoproliferative glomerulonephritis (MPGN) characterized by deposition in the renal glomeruli of immunoglobulin with complement fractions, especially C3. Clinical presentation may range from nephrotic syndrome and acute kidney injury to asymptomatic proteinuria and hematuria.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-329903/","orphanet_url":"https://www.orpha.net/en/disease/detail/329903","names":{"fr":{"name":"Glomérulonéphrite membranoproliférative médiée par les immunoglobulines","synonyms":["GNMP médiée par les Ig","GNMP médiée par les immunoglobulines","Glomérulonéphrite membranoproliférative médiée par les Ig","MPGN médiée par les Ig","MPGN médiée par les immunoglobulines"],"definition":"Forme primaire de glomérulonéphrite membranoproliférative (MPGN) pour membranoproliferative glomerulonephritis), caractérisée par des dépôts d'immunoglobulines dans les glomérules rénaux, avec des fractions du complément (en particulier C3). La présentation clinique peut aller du syndrome néphrotique et de la lésion rénale aiguë à la protéinurie et à l'hématurie asymptomatiques."},"de":{"name":"Glomerulonephritis, membranoproliferative, Immunoglobulin-vermittelte","synonyms":["Ig-mediated membranoproliferative Glomerulonephritis","Ig-vermittelte MPGN","Immunoglobulin-vermittelte MPGN"],"definition":"Eine primäre Form der membranoproliferativen Glomerulonephritis (MPGN), die durch Ablagerung von Immunglobulin mit Komplementanteilen, insbesondere C3, in den Nierenglomeruli gekennzeichnet ist. Das klinische Bild kann vom nephrotischen Syndrom und akuter Nierenschädigung bis hin zu asymptomatischer Proteinurie und Hämaturie reichen."},"es":{"name":"Glomerulonefritis membranoproliferativa mediada por inmunoglobulinas","synonyms":["Glomerulonefritis membranoproliferativa mediada por Ig","MPGN mediada por Ig","MPGN mediada por inmunoglobulinas"],"definition":"Es una forma primaria de glomerulonefritis membranoproliferativa (GNMP) caracterizada por el depósito en los glomérulos renales de inmunoglobulina con fracciones de complemento, especialmente de C3. La presentación clínica puede variar desde síndrome nefrótico y daño renal agudo hasta proteinuria y hematuria asintomáticas."},"it":{"name":"Glomerulonefrite membranoproliferativa mediata dalle immunoglobuline","synonyms":["Glomerulonefrite membranoproliferativa Ig-mediata","MPGN Ig-mediata","MPGN mediata dalle immunoglobuline"],"definition":"Si tratta di una forma primitiva di glomerulonefrite membranoproliferativa (MPGN), caratterizzata dal deposito nei glomeruli renali di immunoglobuline con frazioni del complemento, in particolare C3. Il quadro clinico comprende la sindrome nefrosica, il danno renale acuto, fino alla proteinuria e all'ematuria asintomatiche."},"nl":{"name":"Immunoglobuline-gemedieerde membranoproliferatieve glomerulonefritis","synonyms":["Ig-gemedieerde MPGN","Ig-gemedieerde membranoproliferatieve glomerulonefritis","Immunoglobuline-gemedieerde MPGN"],"definition":"Een primaire vorm van membranoproliferatieve glomerulonefritis (MPGN), gekenmerkt door depositie van immunoglobulinen met complementfracties, vooral C3, in glomeruli van nier. Klinische presentatie kan gaan van nefrotisch syndroom en acuut nierletsel, tot asymptomatische proteïnurie en hematurie."},"pt":{"name":"Glumerulonefrite membranosa proliferativa associada a imunoglobulina","definition":"Forma primária de glomerulonefrite membranoproliferativa (GNMP) caracterizada pela deposição nos glomérulos renais de imunoglobulina com frações do complemento, especialmente C3. A apresentação clínica pode variar de síndrome nefrótica e lesão renal aguda a proteinúria assintomática e hematúria."},"pl":{"name":"Błoniasto-rozplemowe kłębuszkowe zapalenie nerek wywoływane przez immunoglobuliny","synonyms":["Błoniasto-rozplemowe kłębuszkowe zapalenie nerek wywoływane przez Ig","MPGN wywołane przez Ig","MPGN wywołane przez immunoglobuliny"]},"cs":{"name":"Membranoproliferativní glomerulonefritida zprostředkovaná imunoglobuliny","synonyms":["MPGN zprostředkovaná Ig","Membranoproliferativní glomerulonefritida zprostředkovaná Ig","MPGN zprostředkovaná imunoglobuliny"]},"uk":{"name":"Імуноглобулін-опосередкований мембранопроліферативний гломерулонефрит","synonyms":["Ig-опосередкований мембранопроліферативний гломерулонефрит","Ig-опосередкований MPGN","Імуноглобулін-опосередкований MPGN"],"definition":"A primary form of membranoproliferative glomerulonephritis (MPGN) characterized by deposition in the renal glomeruli of immunoglobulin with complement fractions, especially C3. Clinical presentation may range from nephrotic syndrome and acute kidney injury to asymptomatic proteinuria and hematuria."},"zh":{"name":"免疫球蛋白介导膜性肾小球肾炎","synonyms":["Ig介导膜性肾小球肾炎"]},"tr":{"name":"İmmünoglobulin aracılı membranoproliferatif glomerülonefrit","synonyms":["Ig-aracılı MPGN","İmmünoglobulin-aracılı MPGN","Ig-aracılı membranoproliferatif glomerülonefrit"]}},"xrefs":[{"source":"OMIM","code":"615008","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"N03.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0014005","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679858","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":54370,"name":"Primary membranoproliferative glomerulonephritis","classifications":[156,188,233]}],"children":[],"genes":[{"symbol":"DGKE","name":"diacylglycerol kinase epsilon","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"23274426[PMID]","hgnc":"2852","ensembl":"ENSG00000153933","omim":"601440","loci":["17q22"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Unknown"],"onset":[],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":null,"source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2026-05-18","source":"35777834[PMID]_35777835[PMID]_42037281[PMID]_32622830[PMID]","terms":[{"hpo":"HP:0012574","label":"Mesangial hypercellularity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030888","label":"C3 nephritic factor positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005421","label":"Decreased circulating complement C3 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012593","label":"Nephrotic range proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000092","label":"Renal tubular atrophy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033495","label":"Segmental glomerulosclerosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0034001","label":"Anti-Complement factor H antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033319","label":"Fibrous crescent","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null}]}}