Sinding-Larsen-Johansson disease
Synonyms: Aseptic necrosis of patella · Osteochondrosis of patella
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Childhood
- Status
- active
A rare osteochrondrosis characterized by chronic traction injury of the immature osteotendinous junction where the patellar tendon inserts into the inferior pole of the patella. It is typically seen in active adolescents. Patients present with point tenderness at the inferior pole of the patella associated with focal swelling and restriction of knee joint range of movement. Radiographic features include thickening of the proximal patellar tendon (potentially followed by dystrophic calcification and/or ossification) and fragmentation of the inferior pole of the patella.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Sinding-Larsen-Johansson disease | Aseptic necrosis of patella · Osteochondrosis of patella |
| fr | Maladie de Sinding-Larsen-Johansson | Nécrose aseptique de la rotule · Ostéochondrose de la rotule |
| de | Sinding-Larsen-Johansson-Syndrom | Osteochondrosis der Patella · Patellanekrose, aseptische |
| es | Enfermedad de Sinding-Larsen-Johansson | Necrosis aséptica de la rótula · Necrosis aséptica de rótula · Osteocondrosis de la rótula · Osteocondrosis de rótula |
| it | Malattia di Sinding-Larsen-Johansson | Necrosi asettica della rotula · Osteocondrosi della rotula |
| nl | Ziekte van Sinding-Larsen-Johansson | Aseptische necrose van patella · Osteochondrose van patella |
| pt | Doença Sinding-Larsen-Johansson | |
| pl | Choroba Sindinga, Larsena i Johanssona | Jałowa martwica rzepki · Osteochondroza rzepki |
| cs | Sinding-Larsenova-Johanssonova nemoc | Aseptická nekróza apexu pately · Osteochondróza čéšky · Sinding-Larsen-Johanssonova nemoc |
| uk | Хвороба Сіндінга-Ларсена-Йоханссона | Асептичний некроз надколінка · Остеохондроз надколінка |
| zh | Sinding-Larsen-Johansson病 | 髌骨无菌性坏死 |
| tr | Sinding-Larsen-Johansson hastalığı | Patella osteokondrozu · Patellanın aseptik nekrozu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M92.4 | NTBT | inclusion | validated |
| ICD-11 | FB82.1 | NTBT | index | validated |
| MONDO | 0019970 | E | validated | |
| UMLS | C1504517 | E | validated | |
| MedDRA | 10063585 | 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 | Unknown | — | Worldwide | Class only | Not yet validated | 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
5 annotations · validated 2019-06-14| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0030839 | Knee pain | |
| Frequent (79-30%) | ||
| HP:0001386 | Joint swelling | |
| HP:0010501 | Limitation of knee mobility | |
| HP:0040188 | Osteochondrosis | |
| Occasional (29-5%) | ||
| HP:0002661 | Painless fractures due to injury | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 721864[PMID]_26399331[PMID]_18936034[PMID]_23396316[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97337
- This record as JSON: /data/d/97337.json