Diagnosis and Management of Genetic Causes of Middle Aortic Syndrome in Children: A Comprehensive Literature Review

被引：4
|
作者：
Lazea, Cecilia [1 ,2 ]
Al-Khzouz, Camelia [1 ,3 ]
Sufana, Crina [2 ]
Miclea, Diana [3 ,4 ]
Asavoaie, Carmen [5 ]
Filimon, Ioana [5 ]
Fufezan, Otilia [5 ]
机构：
[1] Univ Med & Pharm Iuliu Hatieganu, Dept Mother & Child, 68 Motilor St, Cluj Napoca 400370, Romania
[2] Emergency Pediat Hosp, Dept Pediat 1, Cluj Napoca, Romania
[3] Emergency Pediat Hosp, Dept Med Genet, Cluj Napoca, Romania
[4] Univ Med & Pharm Iuliu Hatieganu, Dept Mol Sci, Cluj Napoca, Romania
[5] Emergency Pediat Hosp, Dept Radiol & Med Imaging, Cluj Napoca, Romania
来源：
THERAPEUTICS AND CLINICAL RISK MANAGEMENT | 2022年 / 18卷
关键词：
middle aortic syndrome; hypertension; neurofibromatosis type 1; Williams syndrome; Alagille syndrome; tuberous sclerosis; mucopolysaccharidoses; RENAL-ARTERY STENOSIS; WILLIAMS-BEUREN-SYNDROME; MIDAORTIC SYNDROME; ALAGILLE-SYNDROME; RENOVASCULAR HYPERTENSION; ABDOMINAL-AORTA; CARDIOVASCULAR MANIFESTATIONS; NEUROFIBROMATOSIS TYPE-1; TUBEROUS SCLEROSIS; SURGICAL-TREATMENT;
D O I：
10.2147/TCRM.S348366
中图分类号：
R19 [保健组织与事业（卫生事业管理）];
学科分类号：
摘要：
Middle aortic syndrome (MAS) is a rare vascular disease representing an important cause of severe hypertension in children. MAS is characterized by segmental or diffuse narrowing of the abdominal and/or distal descending aorta with involvement of the renal and visceral branches. Most cases of MAS are idiopathic, but MAS may occur in genetic and acquired disorders. The most common genetic causes of MAS are neurofibromatosis type I, Williams syndrome, Alagille syndrome, tuberous sclerosis and mucopolysaccharidosis. This review article discusses the pathophysiological aspects, distinctive associated features, and management of genetic forms of MAS in children.
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收藏
页码：233 / 248
页数：16
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