title: The effect of C1-esterase inhibitor in definite and suspected streptococcal toxic shock syndrome. Report of seven patients.
abstract: OBJECTIVE
  To evaluate the effect of adjunctive C1-esterase inhibitor substitution therapy on clinical characteristics and outcome of patients with streptococcal toxic shock syndrome (TSS).
  
  
  METHODS
  Observational.
  
  
  METHODS
  Medizinische Poliklinik, University of Bonn, Germany.
  
  
  METHODS
  Seven patients with direct or indirect evidence of streptococcal TSS.
  
  
  METHODS
  In addition to conventional and supportive therapy, all patients received 2-3 single doses of C1-esterase inhibitor totaling 6,000-10,000 U within the first 24 h after admission.
  
  
  RESULTS
  All patients developed fulminant septic shock, multiorgan failure and/or capillary leak syndrome and necrotizing fasciitis within 10-72 h following the onset of first symptoms. Between 1 and 4 days following administration of C1-esterase inhibitor, a marked shift of fluid from extravascular to intravascular compartments took place in all but one patient, accompanied by a transient intra-alveolar lung edema and rapidly decreasing need for adrenergic agents. Six of seven patients survived.
  
  
  CONCLUSIONS
  These clinical observations in a small series of patients and the favorable outcome point towards a positive effect of early and high-dose administration of C1-esterase inhibitor as adjunctive therapy in streptococcal TSS. The possible mechanism involved may be the attenuation of capillary leak syndrome (CLS) via early inactivation of complement and contact systems. Controlled studies are needed to establish an improvement of the survival rates of patients with streptococcal TSS following administration of C1-esterase inhibitor.
authors: Medizinische Universitäts-Poliklinik, University of Bonn, Germany.
authors: S
authors: S
authors: Fronhoffs
authors: J
authors: J
authors: Luyken
authors: K
authors: K
authors: Steuer
authors: M
authors: M
authors: Hansis
authors: H
authors: H
authors: Vetter
authors: P
authors: P
authors: Walger
mesh_terms: D000328:Adult; D000368:Aged; D019559:Capillary Leak Syndrome / Q000382:microbiology; D003131:Combined Modality Therapy; D003174:Complement C1 Inactivator Proteins / Q000494:pharmacology / Q000627:therapeutic use*; D003422:Critical Care / Q000379:methods; D005260:Female; D018495:Fluid Shifts; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012772:Shock, Septic / Q000188:drug therapy* / Q000378:metabolism / Q000401:mortality / Q000503:physiopathology; D013290:Streptococcal Infections / Q000188:drug therapy* / Q000378:metabolism / Q000401:mortality / Q000503:physiopathology; D013297:Streptococcus pyogenes; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D003174:Complement C1 Inactivator Proteins