{"orpha":93558,"name":"Light chain deposition disease","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["LCDD"],"definition":"A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by deposition of abnormal immunoglobulin light chains in the kidneys, resulting in nephrotic syndrome and renal failure. Symptomatic extrarenal deposition is uncommon, although hepatic, cardiac, and neural deposits have been reported. The condition frequently occurs in association with multiple myeloma or in patients with M protein and marrow plasma cells at monoclonal gammopathy of undetermined significance levels.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-93558/","orphanet_url":"https://www.orpha.net/en/disease/detail/93558","names":{"fr":{"name":"Maladie de dépôt des chaînes légères","synonyms":["LCDD"],"definition":"Maladie rare des dépôts d'immunoglobuline monoclonale non-amyloïde, caractérisée par des dépôts de chaînes légères d'immunoglobulines anormales dans les reins, entraînant un syndrome néphrotique et une insuffisance rénale. La présence de dépôts extra-rénaux symptomatiques est peu courante, bien que des cas de dépôts neuraux, cardiaques et hépatiques aient été rapportés. La maladie survient fréquemment en association avec un myélome multiple ou chez les patients atteints de gammapathie monoclonale de signification indéterminée (prolifération de plasmocytes dans la moelle osseuse caractérisée par la production de la protéine M)."},"de":{"name":"Leichtketten-Speicherkrankheit","synonyms":["LCDD"],"definition":"Eine nicht-amyloide monoklonale Immunglobulin-Speicherkrankheit, die durch die Ablagerung abnormaler Immunglobulin-Leichtketten in den Nieren gekennzeichnet ist und zu einem nephrotischen Syndrom und Nierenversagen führt. Symptomatische extrarenale Ablagerungen sind selten, obwohl über hepatische, kardiale und neurale Ablagerungen berichtet wurde. Die Erkrankung tritt häufig in Verbindung mit einem multiplen Myelom oder bei Patienten mit M-Protein und Plasmazellen im Knochenmark auf, die eine monoklonale Gammopathie von unbestimmter Bedeutung aufweisen."},"es":{"name":"Enfermedad por depósito de cadenas ligeras","definition":"Es una enfermedad poco frecuente por depósito de inmunoglobulina monoclonal no amiloide caracterizada por el depósito de cadenas ligeras anómalas de inmunoglobulina en los riñones, que resulta en síndrome nefrótico e insuficiencia renal. El depósito extrarrenal sintomático es poco común, aunque se ha descrito la ocurrencia de depósitos hepáticos, cardíacos y neurales. La afección se asocia frecuentemente a mieloma múltiple o a pacientes con proteína M y células plasmáticas de la médula ósea en gammapatía monoclonal de significado incierto."},"it":{"name":"Malattia da deposito di catene leggere","synonyms":["LCDD"],"definition":"Si tratta di una malattia rara da deposito di immunoglobuline monoclonali non amiloidi, caratterizzata dal deposito delle catene leggere delle immunoglobuline anomale nei reni, che esita nella sindrome nefrosica e nell'insufficienza renale. Il deposito extrarenale sintomatico è raro, ma sono stati descritti depositi epatici, cardiaci e nel sistema nervoso. Spesso la malattia colpisce i pazienti affetti da mieloma multiplo o con proteina M e plasmacellule midollari nel contesto di una gammopatia monoclonale di significato incerto."},"nl":{"name":"Lichteketendepositieziekte","synonyms":["LCDD"],"definition":"Een zeldzame aandoening met depositie van niet-amyloïde monoklonale immunoglobulinen, gekarakteriseerd door afzetting van abnormale lichte ketens van immunoglobulinen in nier, wat resulteert in nefrotisch syndroom en nierfalen. Symptomatische extrarenale depositie komt niet vaak voor, maar afzettingen in lever, hart en hersenen weren reeds gerapporteerd. De aandoening komt vaak voor in associatie met multipel myeloom of bij patiënten met M-proteïne en plasmacellen in beenmerg op een niveau zoals bij monoklonale gammopathie van onbepaalde significantie."},"pt":{"name":"Doença de deposição de cadeias leves","definition":"Doença rara de acúmulo de imunoglobulina monoclonal não amilóide caracterizada pela deposição de cadeias leves de imunoglobulina anómalas nos rins, resultando em síndrome nefrótica e insuficiência renal. A deposição extra-renal sintomática é incomum, embora depósitos hepáticos, cardíacos e neuronais tenham sido relatados. A doença ocorre frequentemente em associação com mieloma múltiplo ou em doentes com proteína M e células plasmáticas da medula com gamopatia monoclonal em níveis de significância indeterminados."},"pl":{"name":"Choroba deponowania łańcuchów lekkich","synonyms":["LCDD"]},"cs":{"name":"Nemoc z depozit lehkých řetězců","synonyms":["LCDD"]},"uk":{"name":"Хвороба відкладення легких ланцюгів","synonyms":["LCDD"],"definition":"A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by deposition of abnormal immunoglobulin light chains in the kidneys, resulting in nephrotic syndrome and renal failure. Symptomatic extrarenal deposition is uncommon, although hepatic, cardiac, and neural deposits have been reported. The condition frequently occurs in association with multiple myeloma or in patients with M protein and marrow plasma cells at monoclonal gammopathy of undetermined significance levels."},"zh":{"name":"轻链沉积病","synonyms":["LCDD"]},"tr":{"name":"Hafif zincir birikimi hastalığı","synonyms":["LCDD"]}},"xrefs":[{"source":"ICD-10","code":"D89.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A83.52","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1612001446"},{"source":"MONDO","code":"0019730","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0238239","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6906","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":86861,"name":"Non-amyloid monoclonal immunoglobulin deposition disease","classifications":[188,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}