{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:53:47Z","timestamp":1790747627196,"version":"4.1.0"},"reference-count":29,"publisher":"American Academy of Pediatrics (AAP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,10,1]]},"abstract":"<jats:sec>\n                  <jats:title>OBJECTIVES:<\/jats:title>\n                  <jats:p>Individuals with Down syndrome (DS) are at risk for the development of moyamoya syndrome (MMS); MMS is often recognized only after a resulting stroke has occurred. Our goal with this study was to determine if elevations in blood pressure (BP) precede acute presentation of MMS in individuals with DS.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>METHODS:<\/jats:title>\n                  <jats:p>A single-center, retrospective case-control study was performed. Thirty patients with MMS and DS and 116 patients with DS only were identified retrospectively. Three BP recordings were evaluated at set intervals (18\u201324 months, 12\u201318 months, and 6\u201312 months before diagnosis of MMS). These were then compared against control averages from patients with DS only. To assess changes over the time, we used general linear model repeated measures analysis of variance. To identify independent predictors of MMS and DS, we used a multivariable analysis using generalized estimating equations accounting for repeated measures of BP.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>RESULTS:<\/jats:title>\n                  <jats:p>BP in patients with MMS and DS rose significantly over the 24-month period preceding presentation (34th, 42nd, and 70th percentiles at the 18\u201324\u2013month, 12\u201318\u2013month, and 6\u201312\u2013month periods, respectively). BPs in the patients with both MMS and DS were significantly higher than in the DS-only controls in the 6 to 12 (P &amp;lt; .001) and 12 to 18 months before presentation (P = .016). Higher Suzuki scores, bilateral disease, and posterior circulation involvement were also predictive of BP elevation before presentation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>CONCLUSIONS:<\/jats:title>\n                  <jats:p>Elevations in BP may foreshadow presentation of MMS in individuals with DS. This simple, low-cost screening measure may lead to early identification of at-risk patients in the medical home and prevent irreversible neurologic injury.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1542\/peds.2018-0840","type":"journal-article","created":{"date-parts":[[2018,9,6]],"date-time":"2018-09-06T07:24:22Z","timestamp":1536218662000},"source":"Crossref","is-referenced-by-count":24,"title":["Blood Pressure Elevation and Risk of Moyamoya Syndrome in Patients With Trisomy 21"],"prefix":"10.1542","volume":"142","author":[{"given":"Jonathan D.","family":"Santoro","sequence":"first","affiliation":[{"name":"Division of Child Neurology, Department of Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Neurology,"},{"name":"Division of Stroke and Neurocritical Care, Department of Neurology,"},{"name":"Departments of Neurology and"},{"name":"Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Mlynash","sequence":"additional","affiliation":[{"name":"Division of Stroke and Neurocritical Care, Department of Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thuy","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel V.","family":"Lazzareschi","sequence":"additional","affiliation":[{"name":"School of Medicine, Stanford University, Stanford, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lironn D.","family":"Kraler","sequence":"additional","affiliation":[{"name":"Departments of Neurology and"},{"name":"Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth W.","family":"Mayne","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary K.","family":"Steinberg","sequence":"additional","affiliation":[{"name":"Division of Stroke and Neurocritical Care, Department of Neurology,"},{"name":"Departments of Neurology and"},{"name":"Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2018,10,1]]},"reference":[{"issue":"1","key":"2021121023375589200_B1","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1161\/01.STR.14.1.104","article-title":"Moyamoya disease\u2013a review.","volume":"14","author":"Suzuki","year":"1983","journal-title":"Stroke"},{"issue":"3","key":"2021121023375589200_B2","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1001\/archneur.1969.00480090076012","article-title":"Cerebrovascular \u201cmoyamoya\u201d disease. Disease showing abnormal net-like vessels in base of brain.","volume":"20","author":"Suzuki","year":"1969","journal-title":"Arch Neurol"},{"key":"2021121023375589200_B3","doi-asserted-by":"crossref","first-page":"S238","DOI":"10.1016\/S0303-8467(97)00082-6","article-title":"Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (\u2018moyamoya\u2019 disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (moyamoya disease) of the Ministry of Health and Welfare, Japan.","volume":"99","author":"Fukui","year":"1997","journal-title":"Clin Neurol Neurosurg"},{"issue":"11","key":"2021121023375589200_B4","doi-asserted-by":"crossref","first-page":"2131","DOI":"10.1161\/01.STR.27.11.2131","article-title":"Moyamoya and Down syndrome. Clinical and radiological features.","volume":"27","author":"Cramer","year":"1996","journal-title":"Stroke"},{"issue":"2","key":"2021121023375589200_B5","first-page":"63","article-title":"Moyamoya disease and Down syndrome: case report and review of the literature.","volume":"102","author":"de Borchgrave","year":"2002","journal-title":"Acta Neurol Belg"},{"issue":"4","key":"2021121023375589200_B6","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/S0387-7604(12)80242-7","article-title":"Moyamoya disease (syndrome) and the Down syndrome.","volume":"14","author":"Fukuyama","year":"1992","journal-title":"Brain Dev"},{"issue":"2","key":"2021121023375589200_B7","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1227\/NEU.0b013e31827b9beb","article-title":"Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease.","volume":"72","author":"Kainth","year":"2013","journal-title":"Neurosurgery"},{"issue":"5","key":"2021121023375589200_B8","doi-asserted-by":"crossref","DOI":"10.1542\/peds.2005-0568","article-title":"Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization.","volume":"116","author":"Jea","year":"2005","journal-title":"Pediatrics"},{"issue":"1","key":"2021121023375589200_B9","first-page":"63","article-title":"Moyamoya syndrome in a child with Down syndrome.","volume":"9","author":"Fung","year":"2003","journal-title":"Hong Kong Med J"},{"issue":"1","key":"2021121023375589200_B10","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1186\/1757-1626-1-240","article-title":"Fluctuating hemiparesis secondary to moyamoya phenomenon in a child with Down syndrome: a case report.","volume":"1","author":"Rison","year":"2008","journal-title":"Cases J"},{"issue":"5","key":"2021121023375589200_B11","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1136\/jnnp-2011-301387","article-title":"Regional differences in incidence and patient characteristics of moyamoya disease: a systematic review.","volume":"83","author":"Kleinloog","year":"2012","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2021121023375589200_B12","doi-asserted-by":"crossref","first-page":"S45","DOI":"10.1016\/S0303-8467(97)00039-5","article-title":"Risk factors of moyamoya disease in Canada and the USA.","volume":"99","author":"Peerless","year":"1997","journal-title":"Clin Neurol Neurosurg"},{"issue":"4","key":"2021121023375589200_B13","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1161\/01.HYP.28.4.569","article-title":"Low blood pressure in Down\u2019s syndrome, a link with Alzheimer\u2019s disease?","volume":"28","author":"Morrison","year":"1996","journal-title":"Hypertension"},{"issue":"2","key":"2021121023375589200_B14","first-page":"123","article-title":"Blood pressure in Down\u2019s syndrome.","volume":"23","author":"Richards","year":"1979","journal-title":"J Ment Defic Res"},{"issue":"3","key":"2021121023375589200_B15","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1352\/0895-8017(2002)107<0201:DICDRB>2.0.CO;2","article-title":"Differences in cardiovascular disease risk between nondiabetic adults with mental retardation with and without Down syndrome.","volume":"107","author":"Draheim","year":"2002","journal-title":"Am J Ment Retard"},{"issue":"2","key":"2021121023375589200_B16","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1086\/519979","article-title":"Human microRNA-155 on chromosome 21 differentially interacts with its polymorphic target in the AGTR1 3\u2032 untranslated region: a mechanism for functional single-nucleotide polymorphisms related to phenotypes.","volume":"81","author":"Sethupathy","year":"2007","journal-title":"Am J Hum Genet"},{"issue":"7","key":"2021121023375589200_B17","doi-asserted-by":"crossref","first-page":"1318","DOI":"10.3174\/ajnr.A3883","article-title":"Cerebrovascular collaterals correlate with disease severity in adult North American patients with Moyamoya disease.","volume":"35","author":"Strother","year":"2014","journal-title":"AJNR Am J Neuroradiol"},{"key":"2021121023375589200_B18","volume-title":"Fourth Report of the Expert Panel on Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents","author":"US Department of Health and Human Services","year":"2005"},{"issue":"35","key":"2021121023375589200_B19","first-page":"1","article-title":"Mean systolic and diastolic blood pressure in adults aged 18 and over in the United States, 2001-2008.","author":"Wright","year":"2011","journal-title":"Natl Health Stat Report"},{"issue":"12","key":"2021121023375589200_B20","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1056\/NEJMra0804622","article-title":"Moyamoya disease and moyamoya syndrome.","volume":"360","author":"Scott","year":"2009","journal-title":"N Engl J Med"},{"issue":"6","key":"2021121023375589200_B21","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1111\/pan.12140","article-title":"Cerebrovascular autoregulation in pediatric moyamoya disease.","volume":"23","author":"Lee","year":"2013","journal-title":"Paediatr Anaesth"},{"key":"2021121023375589200_B22","first-page":"142","article-title":"Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis.","volume":"100","author":"Scott","year":"2004","journal-title":"J Neurosurg"},{"issue":"2","key":"2021121023375589200_B23","doi-asserted-by":"crossref","first-page":"E17","DOI":"10.3171\/FOC\/2008\/24\/2\/E17","article-title":"Progression of disease in unilateral moyamoya syndrome.","volume":"24","author":"Smith","year":"2008","journal-title":"Neurosurg Focus"},{"key":"2021121023375589200_B24","first-page":"1077","volume-title":"Pediatric Neurosurgery: Surgery of the Developing Nervous System","author":"Ohaegbulam","year":"2001","edition":"4th ed."},{"issue":"2","key":"2021121023375589200_B25","doi-asserted-by":"crossref","first-page":"E16","DOI":"10.3171\/FOC\/2008\/24\/2\/E16","article-title":"Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions.","volume":"24","author":"Veeravagu","year":"2008","journal-title":"Neurosurg Focus"},{"issue":"5","key":"2021121023375589200_B26","doi-asserted-by":"crossref","first-page":"927","DOI":"10.3171\/2009.4.JNS081649","article-title":"Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article.","volume":"111","author":"Guzman","year":"2009","journal-title":"J Neurosurg"},{"issue":"11","key":"2021121023375589200_B27","doi-asserted-by":"crossref","first-page":"3304","DOI":"10.1161\/STROKEAHA.110.598565","article-title":"Neurosurgical advances in the treatment of moyamoya disease.","volume":"42","author":"Pandey","year":"2011","journal-title":"Stroke"},{"issue":"2\u20133","key":"2021121023375589200_B28","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1159\/000093238","article-title":"Progression of unilateral moyamoya disease: a clinical series.","volume":"22","author":"Kelly","year":"2006","journal-title":"Cerebrovasc Dis"},{"issue":"11","key":"2021121023375589200_B29","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1177\/0883073817718730","article-title":"Moyamoya disease in children: results from the international pediatric stroke study.","volume":"32","author":"Lee","year":"2017","journal-title":"J Child Neurol"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2018-0840\/1065431\/peds_20180840.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2018-0840\/1065431\/peds_20180840.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T08:04:20Z","timestamp":1639209860000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/142\/4\/e20180840\/37419\/Blood-Pressure-Elevation-and-Risk-of-Moyamoya"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10,1]]},"references-count":29,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2018,10,1]]},"published-print":{"date-parts":[[2018,10,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2018-0840","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10,1]]},"article-number":"e20180840"}}