Osteochondritis dissecans
Synonyms: König disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Osteochondritis dissecans (OCD) is a rare bone disease characterized by an acquired idiopathic necrotic lesion of subchondral bone with the formation of a sequestrum, which may detach to form loose bodies in joints. OCD mainly affects the knee, ankle and elbow joints and can lead to pain, functional limitations and secondary osteoarthritis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Osteochondritis dissecans | König disease |
| fr | Ostéochondrite disséquante | Maladie de König |
| de | Osteochondrosis dissecans | König-Krankheit · Osteochondrosis dissecans (König) |
| es | Osteocondritis disecante | Enfermedad de Kônig |
| it | Osteocondrite dissecante | Malattia di Kônig |
| nl | Osteochondritis dissecans | Ziekte van König |
| pt | Osteocondrite dissecante | |
| pl | Oddzielająca martwica kostno-chrzęstna | Choroba Kôniga |
| cs | Osteochondritis dissecans | Königova nemoc |
| uk | Розсікаючий остеохондрит | Хвороба Кеніга |
| zh | 剥脱性骨软骨炎 | König病 |
| tr | Osteokondrit dissekans | König hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M93.2 | E | specific | validated |
| ICD-11 | FB82.1 | NTBT | index | validated |
| MONDO | 0017178 | E | validated | |
| MeSH | D010008 | E | validated | |
| UMLS | C0029421 | E | validated | |
| GARD | 12703 | E | validated | |
| MedDRA | 10031231 | 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:399158Osteonecrosis
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 | 1-5 / 10 000 | 35 / 100 000 | Europe | Value and class | Not yet validated | 16958015[PMID] |
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
18 annotations · validated 2024-02-21| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002815 | Abnormality of the knee | |
| HP:0002829 | Arthralgia | |
| HP:0001387 | Joint stiffness | |
| HP:0001386 | Joint swelling | |
| HP:0001376 | Limitation of joint mobility | |
| Frequent (79-30%) | ||
| HP:0001367 | Abnormal joint morphology | |
| HP:0011843 | Abnormality of skeletal physiology | |
| HP:0010885 | Avascular necrosis | |
| HP:6000917 | Bone marrow edema | |
| HP:0003184 | Decreased hip abduction | |
| HP:0001382 | Joint hypermobility | |
| HP:0001377 | Limited elbow extension | |
| HP:0006376 | Limited elbow flexion | |
| HP:0000938 | Osteopenia | |
| HP:0003088 | Premature osteoarthritis | |
| Occasional (29-5%) | ||
| HP:0002992 | Abnormality of tibia morphology | |
| HP:0001288 | Gait disturbance | |
| HP:0009050 | Quadriceps muscle atrophy | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 34109940[PMID]_28639852[PMID]_21979791[PMID]_16794036[PMID]_30252347[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2764
- This record as JSON: /data/d/2764.json