title: FcgammaRIIIb and complement component C7 codeficiency in a patient with recurrence of fulminant meningococcal septic shock.
abstract: Individuals with deficiencies of the late components of complement exhibit a susceptibility to the recurrence of meningococcal disease with a usually mild clinical presentation. We report the recurrence of fulminant meningococcal disease in a complement component C7-deficient patient. We found a total deficiency of FcgammaRIIIb on neutrophils, which could partially explain the unusually severe clinical presentation.
authors.affiliation: Immunology Laboratory, Lyon-Sud University Hospital, Pierre-Benite, France.
authors.forename: Anne-Lise
authors.initials: AL
authors.lastname: Debard
authors.forename: Brigitte
authors.initials: B
authors.lastname: Lamy
authors.forename: Guillaume
authors.initials: G
authors.lastname: Monneret
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Mira
authors.forename: Alexandre
authors.initials: A
authors.lastname: Pachot
authors.forename: Marion
authors.initials: M
authors.lastname: Kleijer
authors.forename: Marie-Françoise
authors.initials: MF
authors.lastname: Aillaud
authors.forename: André
authors.initials: A
authors.lastname: Boibieux
authors.forename: Jacques
authors.initials: J
authors.lastname: Bienvenu
authors.forename: Gérard
authors.initials: G
authors.lastname: Carret
authors.forename: Gérard
authors.initials: G
authors.lastname: Fournier
authors.forename: Julien
authors.initials: J
authors.lastname: Bohé
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D016470:Bacteremia; D003184:Complement C7 / Q000172:deficiency* / Q000235:genetics; D005260:Female; D058851:GPI-Linked Proteins; D015870:Gene Expression; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006801:Humans; D008589:Meningococcal Infections / Q000235:genetics / Q000378:metabolism*; D009504:Neutrophils / Q000378:metabolism; D017452:Receptors, IgG / Q000172:deficiency* / Q000378:metabolism; D012008:Recurrence; D012772:Shock, Septic / Q000235:genetics / Q000378:metabolism*
chemical_list: D015703:Antigens, CD; D003184:Complement C7; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; D017452:Receptors, IgG