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Association between neurofibromatosis type 1 and cerebrovascular diseases in children: A systematic review.


ABSTRACT:

Background

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disease that affects one in every 3000 individuals. This disease can present a wide range of clinical manifestations, ranging from skin abnormalities to severe vascular damage. Although not commonly recognized in the context of NF-1, cerebrovascular disease (CVD), can be often present since childhood and diagnosed just later in life. When present, NF-1-associated CVD clinical manifestations may include headache, cognitive deficits and ultimately aneurysm rupture, causing death. Thus, CVD plays an important role in the clinical manifestations, disease severity and prognosis of patients with NF-1. This systematic review aims to summarize the body of evidence linking NF-1 and CVD in children.

Methods

Two indepe

SUBMITTER: Barreto-Duarte B 

PROVIDER: S-EPMC7781472 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature