Research Topics
Genomes and Genes | Nazli B McDonnellSummaryAffiliation: National Institutes of Health Country: USA Publications
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Detail Information
Publications
Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissueThomas H Milhorat
Department of Neurosurgery, The Chiari Institute, Harvey Cushing Institute of Neuroscience, North Shore Long Island Jewish Health System, Manhasset, New York 11030, USA
J Neurosurg Spine 7:601-9. 2007..The authors report an association between CM-I and hereditary disorders of connective tissue (HDCT) that can present with lower brainstem symptoms attributable to occipitoatlantoaxial hypermobility and cranial settling...
Echocardiographic findings in classical and hypermobile Ehlers-Danlos syndromesNazli B McDonnell
Human Genetics and Integrative Medicine Section, Laboratory of Genetics, National Institute on Aging, National Institutes of Health, Baltimore, Maryland 21224, USA
Am J Med Genet A 140:129-36. 2006..The study is ongoing and will accrue longitudinal data on 100 subjects with classical and hypermobile EDSs at 2-year intervals...
Junction site analysis of chimeric CYP21A1P/CYP21A2 genes in 21-hydroxylase deficiencyWuyan Chen
Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, MD 21224, USA
Clin Chem 58:421-30. 2012..A comprehensive junction site analysis of chimeric CYP21A1P/CYP21A2 genes is needed for optimizing genetic analysis strategy and determining clinical relevance...
Comprehensive genetic analysis of 182 unrelated families with congenital adrenal hyperplasia due to 21-hydroxylase deficiencyGabriela P Finkielstain
Program in Developmental Endocrinology and Genetics, National Institutes of Health, Bethesda, Maryland 20892 1932, USA
J Clin Endocrinol Metab 96:E161-72. 2011..Genetic analysis is commonly performed in patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency...
Circulating transforming growth factor-beta in Marfan syndromePeter Matt
602 Mason F Lord Bldg, Center Tower, Johns Hopkins University, Baltimore, MD 21239, USA
Circulation 120:526-32. 2009..We hypothesized that dysregulation of TGF-beta might be mirrored in circulating TGF-beta concentrations...
The phenotypic spectrum of contiguous deletion of CYP21A2 and tenascin XB: quadricuspid aortic valve and other midline defectsWuyan Chen
Laboratory of Clinical Investigation, National Institute on Aging, National Institutes of Health, Baltimore, Maryland, USA
Am J Med Genet A 149:2803-8. 2009..These findings further delineate the phenotype of the CAH-TNX contiguous gene deletion syndrome and point to an intersection of connective tissue dysplasias with a common gene-mediated endocrine disorder...
Doxycycline ameliorates the susceptibility to aortic lesions in a mouse model for the vascular type of Ehlers-Danlos syndromeWilfried Briest
Laboratory of Cardiovascular Sciences, National Institute on Aging, Baltimore, Maryland, USA
J Pharmacol Exp Ther 337:621-7. 2011..The results suggest that doxycycline merits clinical testing as a treatment for vEDS...
Contemporary management of vascular complications associated with Ehlers-Danlos syndromeBenjamin S Brooke
Division of Vascular and Endovascular Surgery, Johns Hopkins Hospital, Baltimore, MD 21287, USA
J Vasc Surg 51:131-8; discussion 138-9. 2010..The purpose of this study was to review the surgical management and clinical outcomes of EDS patients undergoing vascular procedures at a tertiary medical center with multimodality expertise in connective tissue disorders...
