title: Increased von Willebrand factor antigen and high molecular weight multimers in sickle cell disease associated with nocturnal hypoxemia.
abstract: We evaluated vWF profiles in children and adolescents with SCD and sleep hypoxemia. Mean vWF:Ag levels were significantly elevated in the SCD-hypoxemia group when compared with SCD-normoxia and control groups (p=0.007); and correlated inversely with pulse oximetry (r=-0.54, p=0.01). Densitographic analyses of vWF multimer distribution also showed an inverse correlation between %HMW-multimers and oxygen saturation (r=-0.62, p=0.03). The previously reported association between nocturnal desaturation and SCD vascular complications, including stroke, may be influenced by hypoxemic modulation of vWF as noted in this study.
authors.affiliation: Department of Pediatrics, Division of Hematology, Cardeza Foundation for Hematologic Research, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA. suba.krishnan@jefferson.edu
authors.forename: Suba
authors.initials: S
authors.lastname: Krishnan
authors.forename: Jamie
authors.initials: J
authors.lastname: Siegel
authors.forename: Gene
authors.initials: G
authors.lastname: Pullen
authors.forename: Megan
authors.initials: M
authors.lastname: Hevelow
authors.forename: Carlton
authors.initials: C
authors.lastname: Dampier
authors.forename: Marie
authors.initials: M
authors.lastname: Stuart
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000150:complications* / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D006801:Humans; D000860:Hypoxia / Q000097:blood* / Q000150:complications*; D008970:Molecular Weight; D010100:Oxygen / Q000737:chemistry; D010865:Pilot Projects; D020521:Stroke; D014842:von Willebrand Diseases / Q000097:blood* / Q000150:complications* / Q000276:immunology; D014841:von Willebrand Factor / Q000737:chemistry / Q000276:immunology*
chemical_list: D014841:von Willebrand Factor; D010100:Oxygen