raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:399180

Secondary non-traumatic avascular necrosis

Synonyms: Secondary non-traumatic AVN · Secondary non-traumatic osteonecrosis

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adult
Status
active

A rare osteonecrosis disease characterized by death of bone cellular components secondary to an interruption of the subchondral blood supply, typically manifesting with unilateral or bilateral, unifocal or multifocal lesions usually located on the epiphysis, metaphysis and/or diaphysis of the femoral heads, knees, shoulders, ankles and/or wrists, leading to gradual onset of pain and progressive joint degeneration resulting in loss of function. Association with corticosteroid usage, alcoholism, hyperbaric events, radiation or cytotoxic agent exposure, hemoglobinopathies, and/or underlying autoimmune or metabolic disease, amongst others, has been observed.

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Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enSecondary non-traumatic avascular necrosisSecondary non-traumatic AVN · Secondary non-traumatic osteonecrosis
frNécrose avasculaire secondaire non traumatiqueAVN secondaire non traumatique · Ostéonécrose secondaire non traumatique
deAvaskuläre Nekrose, nicht-traumatische sekundäreSekundäre nicht-traumatische AVN · Sekundäre nicht-traumatische Osteonekrose
esNecrosis avascular no traumática secundariaAVN no traumática secundaria · Osteonecrosis no traumática secundaria
itNecrosi secondaria non traumatica avascolareAVN non traumatica secondaria
nlSecundaire niet-traumatische avasculaire necroseSecundaire niet-traumatische AVN · Secundaire niet-traumatische osteonecrose
ptNecrose avascular secundária não-traumática
plWtórna martwica awaskularna niezwiązana z urazemWtórna AVN
csSekundární netraumatická avaskulární nekrózaSekundární netraumatická AVN
ukВторинний нетравматичний аваскулярний некрозВторинний нетравматичний АВН · Вторинний нетравматичний остеонекроз
zh继发性非缺血性坏死继发性非创伤性骨坏死
trSekonder travmatik olmayan avasküler nekrozSekonder travmatik olmayan AVN · Sekonder travmatik olmayan osteonekroz

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10M87.1NTBTattributedvalidated
ICD-10M87.3NTBTattributedvalidated
ICD-11FB81.YNTBTattributedvalidated
MONDO0018376Evalidated
UMLSC5192430Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

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Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

13 annotations · validated 2019-10-24
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0010885Avascular necrosis
Frequent (79-30%)
HP:0030955Alcoholism
HP:0002960Autoimmunity
HP:0002653Bone pain
HP:0001376Limitation of joint mobility
Occasional (29-5%)
HP:0003549Abnormality of connective tissue
HP:0001288Gait disturbance
HP:0031520Groin pain
HP:0004377Hematological neoplasm
HP:0002664Neoplasm
HP:0001370Rheumatoid arthritis
HP:0002725Systemic lupus erythematosus
Very rare (<4-1%)
HP:0100724Hypercoagulability

Run the differential on the 1 obligate / very frequent terms

Annotation source: 19294340[PMID]_18524806[PMID]_9779852[PMID]_3768054[PMID]_8235665[PMID]

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Sources for this card