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syndroom van hemihyperplasie met multipele lipomatose (aandoening)
syndroom van hemihyperplasie met multipele lipomatose
HHML-syndroom
syndroom van hemihyperplasie met meervoudige lipomatose
Hemihyperplasia with multiple lipomatosis syndrome
HHML (hemihyperplasia multiple lipomatosis) syndrome
A rare genetic overgrowth syndrome with characteristics of non-progressive, asymmetrical, moderate hemihyperplasia (frequently affecting the limbs) associated with slow growing, painless, multiple, recurrent, subcutaneous lipomatous masses distributed throughout entire body (in particular back, torso, extremities, fingers, axillae). Superficial vascular malformations may also be associated. Increased risk of intra-abdominal embryonal malignancies may be associated.
Id771440006
StatusPrimitive
Associated morphologylipomatose
Finding sitestructuur van subcutis
Associated morphologyhyperplasie
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetQ87.3
TermCongenitale gestoorde-ontwikkelingssyndromen met vroege versterkte groei
SNOMED CT to Orphanet simple map276280
SNOMED CT to ICD-10 extended map
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