{"orpha":314709,"name":"Primary localized amyloidosis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Localized AL amyloidosis"],"definition":null,"note":"This disease is described under AL amyloidosis","url":"https://raremap.alftech.space/d/ORPHA-314709/","orphanet_url":"https://www.orpha.net/en/disease/detail/314709","names":{"fr":{"name":"Amylose primitive localisée","synonyms":["Amylose AL localisée"]},"de":{"name":"Amyloidose, lokalisierte primäre","synonyms":["AL-Amyloidose, lokalisierte","Immunoglobulinamyloidose, lokalisierte"]},"es":{"name":"Amiloidosis localizada primaria","synonyms":["Amiloidosis de cadena ligera localizada","Amiloidosis inmunoglobulínica localizada"]},"it":{"name":"Amiloidosi localizzata primitiva","synonyms":["Amiloidosi AL localizzata","Amiloidosi immunoglobulinica localizzata"]},"nl":{"name":"Primaire gelokaliseerde amyloïdose","synonyms":["Gelokaliseerde AL amyloïdose","Gelokaliseerde amyloïdose van de immunoglobulinen"]},"pt":{"name":"Amiloidose localizada primária","synonyms":["Amiloidose AL localizada","Amiloidose imunoglobulinica localizada"]},"pl":{"name":"Pierwotna amyloidoza zlokalizowana","synonyms":["Zlokalizowana amyloidoza AL.","Zlokalizowana amyloidoza immunoglobulinowa"]},"cs":{"name":"Primární lokalizovaná amyloidóza","synonyms":["Lokalizovaná AL amyloidóza"]},"uk":{"name":"Первинний локалізований амілоїдоз","synonyms":["Локалізований амілоїдоз AL"]},"zh":{"name":"原发性局灶性淀粉样变","synonyms":["局灶性AL淀粉样变"],"definition":"原发性局限性淀粉样变性是一种轻链淀粉样变性（见本术语），由不溶性淀粉样纤维聚集而成，这些淀粉样纤维来源于错误折叠的单克隆免疫球蛋白轻链，通常由浆细胞瘤产生（见本术语），其特征是局限性淀粉样沉积，临床表现局限于器官受累，最常见的是尿路（膀胱）、眼睛、呼吸道（喉、肺）和皮肤。"},"tr":{"name":"Primer lokalize amiloidoz","synonyms":["Lokalize AL amiloidoz"]}},"xrefs":[{"source":"ICD-10","code":"E85.4","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"5D00.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1376966206"},{"source":"MONDO","code":"0017817","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679901","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":102069,"name":"OBSOLETE: Hepatic amyloidosis with intrahepatic cholestasis","status":"inactive"}],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":85443,"name":"AL amyloidosis","classifications":[146,181,188,194,196,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}