familiaire osteonecrose van femurkop (aandoening)
familiaire osteonecrose van femurkop
familiaire avasculaire necrose van caput femoris
familiale aseptische osteonecrose van caput femoris
familiaire botnecrose van femurkop
Familial avascular necrosis of femoral head
Familial avascular necrosis of head of femur
A severely disabling disease with manifestation of progressive groin pain, a limping gait, leg length discrepancy, collapse of the subchondral bone, limitation of hip function and eventual degeneration of the hip joint requiring total hip arthroplasty. Familial forms appear to be very rare, with only three families identified so far. Age of onset in these familial cases ranges from 15-48. Transmission in familial cases is autosomal dominant and mutations in the type II collagen gene (COL2A1) have been detected in affected family members.
Associated morphologyavasculaire necrose
Finding sitestructuur van caput femoris
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TermOverige gespecificeerde vormen van botnecrose van bekkengebied en bovenbeen
SNOMED CT to Orphanet simple map86820
SNOMED CT to ICD-10 extended map
AdviceALWAYS M87.85
CorrelationSNOMED CT source code to target map code correlation not specified