Kienbock disease
Synonyms: Aseptic necrosis of the lunate bone · Lunatomalacia · Osteochondrosis of the lunate bone · Progressive avascular necrosis of the lunate bone
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kienbock disease | Aseptic necrosis of the lunate bone · Lunatomalacia · Osteochondrosis of the lunate bone · Progressive avascular necrosis of the lunate bone |
| fr | Maladie de Kienbock | Nécrose aseptique du semi-lunaire · Nécrose avasculaire du semi-lunaire · Ostéochondrite du semi-lunaire |
| de | Kienböck-Krankheit | Aspetische Mondbeinnekrose · Lunatummalazie · Lunatumnekrose · Osteochondritis des Mondbeins · Progressive avaskuläre Mondbeinnekrose |
| es | Enfermedad de Kienbock | Lunatomalacia · Necrosis aséptica del hueso semilunar · Necrosis avascular progresiva del hueso semilunar · Osteocondrosis del hueso semilunar |
| it | Malattia di Kienbock | Lunatomalacia · Necrosi asettica dell'osso semilunare · Osteocondrite dell'osso semilunare · Osteocondrosi dell'osso semilunare |
| nl | Ziekte van Kienbock | Aseptische necrose van os lunatum · Lunatomalacie · Osteochondrose van maanbeen · Progressieve avasculaire necrose van maanbeen |
| pt | Doença de Kienbock | |
| pl | Choroba Kienbocka | Jałowa martwica kości księżycowatej · Lunatomalacja · Osteochondroza kości księżycowatej |
| cs | Kienboeckova nemoc | Aseptická nekróza os lunatum · Lunatomalacie · Osteochondritida os lunatum · Osteochondróza os lunatum |
| uk | Хвороба Кіенбока | Асептичний некроз півмісяцевої кістки · Лунатомаляція · Остеохондроз півмісяцевої кістки · Прогресуючий аваскулярний некроз півмісяцевої кістки |
| zh | Kienbock病 | 月骨无菌性坏死 |
| tr | Kienbock hastalığı | Lunatomalazya · Lunat kemiğin osteokondrozu · Lunat kemiğin aseptik nekrozu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M92.2 | BTNT | inclusion | validated |
| ICD-10 | M93.1 | BTNT | specific | validated |
| ICD-11 | FB81.0 | NTBT | index | validated |
| MONDO | 0019967 | E | validated | |
| UMLS | C0022682 | E | validated | |
| GARD | 9690 | E | validated | |
| MedDRA | 10064242 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:399319Osteochondrosis
rare systemic and rheumatological diseases; rare bone diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 6.6 / 100 000 | Worldwide | Value and class | Validated | 34861414[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
7 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0003019 | Abnormality of the wrist | |
| HP:0002829 | Arthralgia | |
| HP:0010885 | Avascular necrosis | |
| HP:0002653 | Bone pain | |
| HP:0001376 | Limitation of joint mobility | |
| HP:0010886 | Osteochondritis Dissecans | |
| Frequent (79-30%) | ||
| HP:0002758 | Osteoarthritis | |
Run the differential on the 6 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97332
- This record as JSON: /data/d/97332.json