| 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. |
| | DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | E24.8 | | Term | Overige gespecificeerde vormen van syndroom van Cushing |
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| SNOMED CT to Orphanet simple map | 189427 |
| SNOMED CT to ICD-10 extended map | | Target | E24.8 | | Rule | TRUE | | Advice | ALWAYS E24.8 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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