title: Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation.
abstract: BACKGROUND
  With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infection, and cholestasis may all contribute. Acute pancreatitis is characterised by inflammation and activation of digestive proenzymes leading to autodigestive destruction of the pancreas and systemic activation of protease cascades including the complement system.
  
  
  OBJECTIVE
  To describe the effects of human C1 esterase inhibitor in two children, who developed severe acute pancreatitis with considerable complement activation after allogeneic haematopoietic stem cell transplantation.
  
  
  METHODS
  Both children showed clinical features resembling those observed in capillary leakage syndrome. In both patients, treatment with C1 esterase inhibitor concentrate contributed to a rapid clinical stabilisation.
  
  
  CONCLUSIONS
  These observations strongly support the proposed pathophysiological concept that early treatment with C1 esterase inhibitor interferes with the activation of the complement system in acute pancreatitis. Inhibition of complement activation prevents its adverse effects on vascular function and permeability, and thus stabilises intravascular fluid status and prevents multiorgan failure in acute pancreatitis.
authors.affiliation: Clinic of Paediatric Haematology and Oncology, University of Düsseldorf, Düsseldorf, Germany.
authors.forename: D T
authors.initials: DT
authors.lastname: Schneider
authors.forename: W
authors.initials: W
authors.lastname: Nürnberger
authors.forename: H
authors.initials: H
authors.lastname: Stannigel
authors.forename: H
authors.initials: H
authors.lastname: Bönig
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D000208:Acute Disease; D019559:Capillary Leak Syndrome / Q000188:drug therapy*; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D010195:Pancreatitis / Q000188:drug therapy*; D011183:Postoperative Complications / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D003174:Complement C1 Inactivator Proteins