{"orpha":97332,"name":"Kienbock disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Aseptic necrosis of the lunate bone","Lunatomalacia","Osteochondrosis of the lunate bone","Progressive avascular necrosis of the lunate bone"],"definition":"Kienbock disease is a rare bone disorder of unknown etiology characterized clinically by osteonecrosis of the carpal lunate, eventually leading to collapse of the lunate bone impacting wrist function.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97332/","orphanet_url":"https://www.orpha.net/en/disease/detail/97332","names":{"fr":{"name":"Maladie de Kienbock","synonyms":["Nécrose aseptique du semi-lunaire","Nécrose avasculaire du semi-lunaire","Ostéochondrite du semi-lunaire"],"definition":"Maladie osseuse rare de cause inconnue qui se caractérise cliniquement par une ostéonécrose du semi-lunaire du carpe, entraînant à terme un affaissement de l'os semi-lunaire et affectant le fonctionnement du poignet."},"de":{"name":"Kienböck-Krankheit","synonyms":["Aspetische Mondbeinnekrose","Lunatummalazie","Lunatumnekrose","Osteochondritis des Mondbeins","Progressive avaskuläre Mondbeinnekrose"],"definition":"Die Kienböck-Krankheit ist eine seltene Knochenkrankheit mit unbekannter Ursache. Sie ist klinisch gekennzeichnet durch Osteonekrose des karpalen Lunatum-Knochens. Dessen möglicher Zusammenbruch beeinträchtigt die Funktion des Handgelenks."},"es":{"name":"Enfermedad de Kienbock","synonyms":["Lunatomalacia","Necrosis aséptica del hueso semilunar","Necrosis avascular progresiva del hueso semilunar","Osteocondrosis del hueso semilunar"],"definition":"La enfermedad de Kienbock es un trastorno óseo poco frecuente y de etiología desconocida que se caracteriza clínicamente por osteonecrosis del semilunar carpiano, lo que finalmente conduce al colapso del hueso semilunar afectando a la función de la muñeca."},"it":{"name":"Malattia di Kienbock","synonyms":["Lunatomalacia","Necrosi asettica dell'osso semilunare","Osteocondrite dell'osso semilunare","Osteocondrosi dell'osso semilunare"],"definition":"La malattia di Kienbock è una patologia scheletrica rara, ad eziologia sconosciuta, caratterizzata da osteonecrosi del semilunare carpale, che esita eventualmente nel collasso dell'osso semilunare, compromettendo la funzionalità del polso."},"nl":{"name":"Ziekte van Kienbock","synonyms":["Aseptische necrose van os lunatum","Lunatomalacie","Osteochondrose van maanbeen","Progressieve avasculaire necrose van maanbeen"],"definition":"Een zeldzame aandoening van bot met ongekende etiologie, klinisch gekenmerkt door osteonecrose van os lunatum (maanbeen), wat uiteindelijk leidt tot collaps van dit bot en aantasting van polsfunctie."},"pt":{"name":"Doença de Kienbock"},"pl":{"name":"Choroba Kienbocka","synonyms":["Jałowa martwica kości księżycowatej","Lunatomalacja","Osteochondroza kości księżycowatej"]},"cs":{"name":"Kienboeckova nemoc","synonyms":["Aseptická nekróza os lunatum","Lunatomalacie","Osteochondritida os lunatum","Osteochondróza os lunatum"]},"uk":{"name":"Хвороба Кіенбока","synonyms":["Асептичний некроз півмісяцевої кістки","Лунатомаляція","Остеохондроз півмісяцевої кістки","Прогресуючий аваскулярний некроз півмісяцевої кістки"],"definition":"Kienbock disease is a rare bone disorder of unknown etiology characterized clinically by osteonecrosis of the carpal lunate, eventually leading to collapse of the lunate bone impacting wrist function."},"zh":{"name":"Kienbock病","synonyms":["月骨无菌性坏死"],"definition":"Kienbock病是一种少见的病因不明的骨疾病，临床表现为腕月骨坏死，最终导致月骨塌陷，影响腕关节功能。"},"tr":{"name":"Kienbock hastalığı","synonyms":["Lunatomalazya","Lunat kemiğin osteokondrozu","Lunat kemiğin aseptik nekrozu"]}},"xrefs":[{"source":"ICD-10","code":"M92.2","qualifier":"BTNT","icd_relation":"inclusion","validated":true},{"source":"ICD-10","code":"M93.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"FB81.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1867483039"},{"source":"MONDO","code":"0019967","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0022682","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9690","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10064242","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93419,"name":"Rare bone disease"},"parents":[{"orpha":399319,"name":"Osteochondrosis","classifications":[196,199]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":6.6,"area":"Worldwide","status":"Validated","source":"34861414[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0003019","label":"Abnormality of the wrist","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010885","label":"Avascular necrosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002653","label":"Bone pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001376","label":"Limitation of joint mobility","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010886","label":"Osteochondritis Dissecans","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002758","label":"Osteoarthritis","frequency":"Frequent (79-30%)","criteria":null}]}}