Fibromuscular dysplasia, arterial

Preferred Label : Fibromuscular dysplasia, arterial;

Symbol : FMDA;

CISMeF acronym : FMDA; FMD;

Type : Other, mainly phenotypes with suspected mendelian basis;

Alternative titles and symbols : Fibromuscular dysplasia of arteries;

Description : Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4 in 1,000 and the prevalence of cervicocranial FMD is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007);

Inheritance : Autosomal dominant;

Prefixed ID : 135580;

Details

Origin ID

135580;

UMLS CUI

C0016052;

Automatic exact mappings (from CISMeF team)
- FLNA wt Allele [NCIt concept]
- Flow-Mediated Vasodilation [NCIt concept]
- frontometaphyseal dysplasia [DO Concept]
Currated CISMeF NLP mapping
- Arterial fibromuscular dysplasia [ICD-10 Sub-category]
- Arterial fibromuscular dysplasia [ICD-11 Subcategory]
- Arterial fibromuscular dysplasia [ICD-10 Sub-category (who)]
- Arterial fibromuscular dysplasia [HPO term]
- Fibromuscular Dysplasia [NCIt concept]
- Fibromuscular dysplasia [MedDRA Preferred Term]
- Fibromuscular dysplasia (morphologic abnormality) [SNOMED CT concept]
- Fibromuscular dysplasia of arteries [ORDO Disease]
- Fibromuscular hyperplasia of artery (disorder) [Inactive SNOMED CT concept]
- fibromuscular dysplasia [Disease (Nov.)]
- fibromuscular dysplasia [Radlex concept]
- fibromuscular dysplasia [SNOMED Notion]
- fibromuscular dysplasia [MeSH concept]
HPO term(s)
- Aortic dissection [HPO term]
- Arterial fibromuscular dysplasia [HPO term]
- Autosomal dominant inheritance [HPO term]
- Hypertension due to renal artery hyperplasia [HPO term]
- Intermittent claudication [HPO term]
- Myocardial infarction [HPO term]
- Renovascular hypertension [HPO term]
- Stroke [HPO term]
ORDO concept(s)
- Fibromuscular dysplasia of arteries [ORDO Disease]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Arterial fibromuscular dysplasia [HPO term]
- Arterial fibromuscular dysplasia [ICD-10 Sub-category]
- Arterial fibromuscular dysplasia [ICD-11 Subcategory]
- Fibromuscular Dysplasia [NCIt concept]
- Fibromuscular dysplasia [MedDRA Preferred Term]
- Fibromuscular dysplasia (morphologic abnormality) [SNOMED CT concept]
- Fibromuscular dysplasia of arteries [ORDO Disease]
- Fibromuscular dysplasia of wall of artery (disorder) [SNOMED CT concept]
- Fibromuscular hyperplasia (morphologic abnormality) [Inactive SNOMED CT concept]
- Fibromuscular hyperplasia of artery (disorder) [Inactive SNOMED CT concept]
- fibromuscular dysplasia [MeSH Descriptor]
- fibromuscular dysplasia [MeSH concept]
- fibromuscular dysplasia [Radlex concept]
- fibromuscular dysplasia [SNOMED Notion]
- fibromuscular dysplasia [Disease (Nov.)]

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