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Cushing-syndroom door macronodulaire bijnierhyperplasie (aandoening)
Cushing-syndroom door macronodulaire bijnierhyperplasie
syndroom van Cushing door macronodulaire bijnierhyperplasie
Hypercortisolism due to macronodular adrenal hyperplasia
Cushing syndrome due to macronodular adrenal hyperplasia
Adrenocorticotropic hormone independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing syndrome with characteristics of nodular enlargement of both adrenal glands that produce excess cortisol. The disease presents a bimodal age distribution with a rare subset presenting in the first years of life, particularly associated to McCune-Albright syndrome. Most patients present in their fifth or sixth decade. The adrenal glands can be massively enlarged bilaterally with the presence of numerous macronodules; however diffuse adrenal enlargement without nodules has been described. AIMAH is most often reported as sporadic but there are increasing reports of familial cases with autosomal dominant transmission.
Id720459002
StatusDefined
DHD Diagnosethesaurus-referentieset
referentieset met complexe door RIVM geautoriseerde nationale 'mapping' naar ICD-10 voor diagnosethesaurus
TargetE24.8
TermOverige gespecificeerde vormen van syndroom van Cushing
referentieset met complexe 'mapping' naar ICD-10
TargetE24.8
RuleTRUE
AdviceALWAYS E24.8
CorrelationSNOMED CT source code to target map code correlation not specified