title: Leukocyte adhesion deficiency in children and Irish setter dogs.
abstract: Children with the genetic immunodeficiency disease leukocyte adhesion deficiency, or LAD, develop life-threatening bacterial infections as a result of the inability of their leukocytes to adhere to the vessel wall and migrate to the sites of infection. Recently, the canine counterpart to LAD, known as canine leukocyte adhesion deficiency, or CLAD, has been described in Irish setter dogs. This review describes how the clinical phenotype of dogs with CLAD closely parallels that of children with the severe deficiency phenotype of LAD, thus enabling the CLAD dog to provide a disease-specific, large-animal model for testing novel hematopoietic stem cell and gene therapy strategies before their translation to children with LAD.
authors.affiliation: Experimental Transplantation and Immunology Branch, National Cancer Institute, National Institutes of Health, 10 Center MSC1907, Building 10, Room 12C116, Bethesda, MD 20892, USA.
authors.forename: Thomas R
authors.initials: TR
authors.lastname: Bauer
authors.forename: Yu-Chen
authors.initials: YC
authors.lastname: Gu
authors.forename: Kate E
authors.initials: KE
authors.lastname: Creevy
authors.forename: Laura M
authors.initials: LM
authors.lastname: Tuschong
authors.forename: Lisa
authors.initials: L
authors.lastname: Embree
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.forename: Robert A
authors.initials: RA
authors.lastname: Sokolic
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Hickstein
mesh_terms: D000818:Animals; D002648:Child; D004195:Disease Models, Animal; D004285:Dogs; D015316:Genetic Therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000503:physiopathology* / Q000628:therapy; D010641:Phenotype