{"orpha":86861,"name":"Non-amyloid monoclonal immunoglobulin deposition disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Non-amyloid MIDD","Randall disease"],"definition":"A rare, secondary glomerular disease characterized by proteinuria, dysproteinemias, nephrotic syndrome, and nodular glomerulopathy leading to renal failure, with or without extra-renal manifestations. The renal biopsy shows typical deposits of monoclonal immunoglobulins that do not show a fibrillar organization and are negative for Congo red staining. Associated signs and symptoms depend on the involvement of other organs, liver, heart, nerve fibers, gastrointestinal tract, or skin.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86861/","orphanet_url":"https://www.orpha.net/en/disease/detail/86861","names":{"fr":{"name":"Maladie des dépôts d'immunoglobuline monoclonale non-amyloïde","synonyms":["MDIM non-amyloïde","MIDD non-amyloïde","Maladie de Randall"],"definition":"Glomérulopathie secondaire rare, caractérisée par une protéinurie, une dysprotéinémie, un syndrome néphrotique et une glomérulosclérose nodulaire, entraînant une insuffisance rénale, avec ou sans manifestations extra-rénales. La biopsie rénale met en évidence des dépôts caractéristiques d'immunoglobuline monoclonale sans organisation fibrillaire et négatifs au rouge Congo. Les signes et symptômes associés dépendent de l'atteinte d'autres organes, tels que le foie, le coeur, les fibres nerveuses, le tractus gastro-intestinal ou la peau."},"de":{"name":"Nicht-amyloide monoklonale Immunglobulin-Ablagerungskrankheit","synonyms":["Immunoglobulin-Speicherkrankheit, monoklonale nichtamyloide","MIDD, nichtamyloide","Randall-Krankheit"],"definition":"Eine sekundäre glomeruläre Erkrankung, die durch Proteinurie, Dysproteinämie, nephrotisches Syndrom und noduläre Glomerulopathie gekennzeichnet ist und zu Nierenversagen mit oder ohne extra renale Manifestationen führt. Die Nierenbiopsie zeigt typische Ablagerungen von monoklonalen Immunglobulinen, die keine fibrilläre Organisation aufweisen und für die Kongorot-Färbung negativ sind. Die begleitenden Anzeichen und Symptome hängen von der Beteiligung anderer Organe, der Leber, des Herzens, der Nervenfasern, des Gastrointestinaltraktes oder der Haut ab."},"es":{"name":"Enfermedad por depósito no amiloides de inmunoglobulinas monoclonales","definition":"Es una enfermedad glomerular secundaria poco frecuente caracterizada por proteinuria, disproteinemia, síndrome nefrótico y glomerulopatía nodular que progresa a insuficiencia renal, con o sin manifestaciones extrarrenales. La biopsia renal muestra típicos depósitos de inmunoglobulinas monoclonales sin organización fibrilar y negativos en la tinción con rojo Congo. Los signos y síntomas asociados dependen de la afectación de otros órganos, tales como el hígado, el corazón, las fibras nerviosas, el tracto gastrointestinal o la piel."},"it":{"name":"Malattia da deposito di immunoglobuline monoclonali non amiloidi","synonyms":["MIDD non amiloide","Malattia da depositi d'immunoglobulina monoclonale non amiloide","Malattia di Randall"],"definition":"Si tratta di una glomerulopatia rara secondaria, caratterizzata da proteinuria, disproteinemia, sindrome nefrosica e glomerulopatia nodulare, che esitano nell'insufficienza renale, con o senza sintomi extrarenali. La biopsia renale evidenzia depositi caratteristici di immunoglobuline monoclonali prive di una organizzazione fibrillare e negative alla colorazione rosso Congo. I segni clinici dipendono dal coinvolgimento degli altri organi (fegato, cuore, fibre nervose, tratto gastrointestinale o cute)."},"nl":{"name":"Ziekte van niet-amyloïde depositie van monoklonaal immunoglobuline","synonyms":["Niet-amyloïde MIDD","Ziekte van Randall"],"definition":"Een zeldzame, secundaire glomerulaire aandoening, gekenmerkt door proteïnurie, dysproteïnemie, nefrotisch syndroom, en nodulaire glomerulopathie die leidt tot nierfalen, met of zonder extrarenale manifestaties. Biopsie van nier toont typische afzettingen van monoklonale immunoglobulinen die geen fibrillaire organisatie vertonen en die negatief zijn voor kleuring met Congorood. Geassocieerde verschijnselen en symptomen hangen af van de betrokkenheid van andere organen, lever, hart, zenuwvezels, maag-darmstelsel, of huid."},"pt":{"name":"Doença de deposição de imunoglobulinas monoclonais","definition":"Doença glomerular secundária, rara caracterizada por proteinúria, disproteinemias, síndrome nefrótica e glomerulopatia nodular levando a insuficiência renal, com ou sem manifestações extra-renais. A biópsia renal evidencia depósitos típicos de imunoglobulinas monoclonais que não apresentam organização fibrilar e são negativas para coloração com vermelho do Congo. Os sinais e sintomas associados dependem do envolvimento de outros órgãos, fígado, coração, fibras nervosas, trato gastrointestinal ou pele."},"pl":{"name":"Nieamyloidowa choroba monoklonalnych depozytów immunoglobulinowych","synonyms":["Choroba Randalla","Nieamyloidowa MIDD"]},"cs":{"name":"Non-amyloidní onemocnění z depozice monoklonálních imunoglobulinů","synonyms":["Non-amyloidní MIDD","Randallova nemoc"]},"uk":{"name":"Неамілоїдна хвороба відкладення моноклональних імуноглобулінів","synonyms":["Неамілоїдна МІДД","Хвороба Рендела"],"definition":"A rare, secondary glomerular disease characterized by proteinuria, dysproteinemias, nephrotic syndrome, and nodular glomerulopathy leading to renal failure, with or without extra-renal manifestations. The renal biopsy shows typical deposits of monoclonal immunoglobulins that do not show a fibrillar organization and are negative for Congo red staining. Associated signs and symptoms depend on the involvement of other organs, liver, heart, nerve fibers, gastrointestinal tract, or skin."},"zh":{"name":"非淀粉样单克隆免疫球蛋白沉积病","synonyms":["非淀粉样MIDD"]},"tr":{"name":"Amiloid olmayan monoklonal immünoglobulin birikimi hastalığı","synonyms":["Randall hastalığı","Amiloid olmayan MIDD"]}},"xrefs":[{"source":"ICD-10","code":"D89.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019463","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5394673","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":567558,"name":"Non-genetic systemic disease with glomerulopathy as a major feature","classifications":[188,233]},{"orpha":98282,"name":"Plasma cell tumor","classifications":[194,202,233]}],"children":[{"orpha":93556,"name":"Heavy chain deposition disease","type":"Clinical subtype"},{"orpha":93557,"name":"Light and heavy chain deposition disease","type":"Clinical subtype"},{"orpha":93558,"name":"Light chain deposition disease","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[],"phenotypes":null}