Biomaterial Database

Familial fibromuscular dysplasia presenting as sudden death. 1988

F E Dominguez, and L G Tate, and M J Robinson

Mount Sinai Medical Center, Miami Beach, Florida.

Fibromuscular dysplasia involving the aorta, its main arch branches, and the coronary arteries is infrequent. Familial occurrence of arterial fibromuscular dysplasia is rarely recognized in infancy. We report three infant siblings with this disorder who presented with sudden unexpected death secondary to acute myocardial infarction as a complication of fibromuscular dysplasia involving the aorta, its main arch branches, and the major coronary arteries. This condition must be considered in the differential diagnosis of identifiable causes of the sudden infant death syndrome.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D009203	Myocardial Infarction	NECROSIS of the MYOCARDIUM caused by an obstruction of the blood supply to the heart (CORONARY CIRCULATION).	Cardiovascular Stroke,Heart Attack,Myocardial Infarct,Cardiovascular Strokes,Heart Attacks,Infarct, Myocardial,Infarction, Myocardial,Infarctions, Myocardial,Infarcts, Myocardial,Myocardial Infarctions,Myocardial Infarcts,Stroke, Cardiovascular,Strokes, Cardiovascular
D003327	Coronary Disease	An imbalance between myocardial functional requirements and the capacity of the CORONARY VESSELS to supply sufficient blood flow. It is a form of MYOCARDIAL ISCHEMIA (insufficient blood supply to the heart muscle) caused by a decreased capacity of the coronary vessels.	Coronary Heart Disease,Coronary Diseases,Coronary Heart Diseases,Disease, Coronary,Disease, Coronary Heart,Diseases, Coronary,Diseases, Coronary Heart,Heart Disease, Coronary,Heart Diseases, Coronary
D003645	Death, Sudden	The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions.	Sudden Death
D005260	Female		Females
D005352	Fibromuscular Dysplasia	An idiopathic, segmental, nonatheromatous disease of the musculature of arterial walls, leading to STENOSIS of small and medium-sized arteries. There is true proliferation of SMOOTH MUSCLE CELLS and fibrous tissue. Fibromuscular dysplasia lesions are smooth stenosis and occur most often in the renal and carotid arteries. They may also occur in other peripheral arteries of the extremity.	Fibromuscular Dysplasia of Arteries,Arteries Fibromuscular Dysplasia,Arteries Fibromuscular Dysplasias,Dysplasia, Fibromuscular,Fibromuscular Dysplasias
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D001018	Aortic Diseases	Pathological processes involving any part of the AORTA.	Aortic Disease,Disease, Aortic,Diseases, Aortic
D001157	Arterial Occlusive Diseases	Pathological processes which result in the partial or complete obstruction of ARTERIES. They are characterized by greatly reduced or absence of blood flow through these vessels. They are also known as arterial insufficiency.	Arterial Obstructive Diseases,Arterial Occlusion,Arterial Obstructive Disease,Arterial Occlusions,Arterial Occlusive Disease,Disease, Arterial Obstructive,Disease, Arterial Occlusive,Obstructive Disease, Arterial,Occlusion, Arterial,Occlusive Disease, Arterial