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Cerebral events could be exacerbated or precipitated by hypertension, and it is unclear how these are related to arteriosclerotic changes as dyslipidemia is also a feature of SIOD. The correlation between hypercholesterolemia and cardiovascular risk in SIOD is unclear. Also, the etiology and management of headaches is not well characterized. Here we report our clinical observations in the management of SIOD in a patient who was diagnosed in school age despite early signs and symptoms. We describe biallelic variants, including a previously unreported c.1931G&gt;A (p.Arg644Gln) variant in <jats:italic>SMARCAL1<\/jats:italic>. We specifically investigated whether migraine\u2010like headaches and progressive nephropathy may be related to blood pressure dysregulation. We found a correlation between tighter blood pressure regulation using ambulatory blood pressure monitoring and a subjective decrease in headache symptoms. We discuss blood pressure medication management in SIOD. We also characterize dyslipidemia relative to atherosclerosis risks and provide new management strategies to consider for optimizing care.<\/jats:p>","DOI":"10.1002\/ajmg.a.61148","type":"journal-article","created":{"date-parts":[[2019,5,1]],"date-time":"2019-05-01T03:24:11Z","timestamp":1556681051000},"page":"1246-1252","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Schimke immunoosseous dysplasia and management considerations for vascular risks"],"prefix":"10.1002","volume":"179","author":[{"given":"Daniah T.","family":"Beleford","sequence":"first","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Institute for Human Genetics, University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad","family":"Diab","sequence":"additional","affiliation":[{"name":"Pediatric Orthopaedics, Department of Orthopaedic Surgery University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William F.","family":"Qubty","sequence":"additional","affiliation":[{"name":"Pediatric Headache, Division of Child Neurology, Department of Neurology University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary J.","family":"Malloy","sequence":"additional","affiliation":[{"name":"Departments of Medicine and Pediatrics Cardiovascular Research Institute, University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roger K.","family":"Long","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology, Department of Pediatrics University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0746-6084","authenticated-orcid":false,"given":"Joseph T.","family":"Shieh","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Institute for Human Genetics, University of California San Francisco  San Francisco California"},{"name":"Institute for Human Genetics and Department of Pediatrics, University of California San Francisco  San Francisco California"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,4,30]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.178459.111"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980630)78:2<118::AID-AJMG4>3.0.CO;2-K"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310050001"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng821"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/head.12960"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.3949\/ccjm.84a.16033"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0902604"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.044313"},{"issue":"2","key":"e_1_2_6_10_1","first-page":"89","article-title":"Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia","volume":"43","author":"Ehrich J. 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