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lamine A/C-gerelateerd cardiocutaan progeriasyndroom (aandoening)
lamine A/C-gerelateerd cardiocutaan progeriasyndroom
LMNA-gerelateerd cardiocutaan progeriasyndroom
LCPS
LMNA-related cardiocutaneous progeria syndrome
Lamin A/C related cardiocutaneous progeria syndrome
A rare genetic premature aging syndrome characterized by adulthood-onset cutaneous manifestations that result in a prematurely aged appearance (such as premature thinning and graying of scalp hair, loss of subcutaneous fat, tightening of skin) associated with prominent cardiovascular manifestations, such as accelerated atherosclerosis, calcific valve disease, and cardiomyopathy. Patients present loss of eyebrows and eyelashes in childhood and have a predisposition to develop malignancies.
Id773426004
StatusPrimitive
Occurrencecongenitaal
Finding sitestructuur van huid
referentieset met complexe 'mapping' naar ICD-10
TargetE34.8
RuleTRUE
AdviceALWAYS E34.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified