||||
syndroom van progressieve segmentale overgroei met fibroadipeuze hyperplasie (aandoening)
syndroom van progressieve segmentale overgroei met fibroadipeuze hyperplasie
Segmental progressive overgrowth syndrome with fibroadipose hyperplasia
A rare PIK3CA (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) related overgrowth syndrome disease with characteristics of segmental and progressive overgrowth, predominantly involving the adipose tissue, or a mixture of adipose and fibrous tissue, with variable involvement of subcutaneous and muscular tissue, as well as skeletal overgrowth. Overgrowth severity and range is highly variable although frequently it is asymmetric and disproportionate, it affects lower extremities more than the upper ones and progresses in a distal to proximal patten. Congenital overgrowth is typically associated.
Id787094005
StatusPrimitive
Associated morphologymorfologische afwijking
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyhyperplasie
Finding sitestructuur van bindweefsel
Clinical courseprogressief
referentieset met complexe 'mapping' naar ICD-10
TargetQ87.3
RuleTRUE
AdviceALWAYS Q87.3 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified