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Review
. 2022 Mar 24:13:843927.
doi: 10.3389/fneur.2022.843927. eCollection 2022.

Ring Finger Protein 213 in Moyamoya Disease With Pulmonary Arterial Hypertension: A Mini-Review

Affiliations
Review

Ring Finger Protein 213 in Moyamoya Disease With Pulmonary Arterial Hypertension: A Mini-Review

Yuting Luo et al. Front Neurol. .

Abstract

Moyamoya disease (MMD), most often diagnosed in children and adolescents, is a chronic cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Recently, many investigators show a great interest in MMD with pulmonary arterial hypertension (PAH). Ring finger protein 213 (RNF213) is a major susceptibility gene for MMD and also has strong correlations with PAH. Therefore, this review encapsulates current cases of MMD with PAH and discusses MMD with PAH in the aspects of epidemiology, pathology, possible pathogenesis, clinical manifestations, diagnosis, and treatment.

Keywords: Moyamoya disease; RNF213; idiopathic pulmonary arterial hypertension; peripheral pulmonary artery stenosis; pulmonary arterial hypertension.

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Conflict of interest statement

The authors declare that the review was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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References

    1. Shang S, Zhou D, Ya J, Li S, Yang Q, Ding Y, et al. . Progress in Moyamoya disease. Neurosurg Rev. (2020) 43:371–82. 10.1007/s10143-018-0994-5 - DOI - PubMed
    1. Ahn IM, Park DH, Hann HJ, Kim KH, Kim HJ, Ahn HS. Incidence, prevalence, and survival of Moyamoya disease in Korea: a nationwide, population-based study. Stroke. (2014) 45:1090–5. 10.1161/STROKEAHA.113.004273 - DOI - PubMed
    1. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘Moyamoya’ disease) Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg. (1997) 99:S238–40. 10.1016/S0303-8467(97)00082-6 - DOI - PubMed
    1. Tho-Calvi SC, Thompson D, Saunders D, Agrawal S, Basu A, Chitre M, et al. . Clinical features, course, and outcomes of a UK cohort of pediatric Moyamoya. Neurology. (2018) 90:e763–70. 10.1212/WNL.0000000000005026 - DOI - PubMed
    1. Zhang X, Xiao W, Zhang Q, Xia D, Gao P, Su J, et al. . Progression in Moyamoya Disease: Clinical Feature, Neuroimaging Evaluation and Treatment. Curr Neuropharmacol. (2021). - PMC - PubMed

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