title: Clostridium septicum myonecrosis in congenital neutropenia.
abstract: Severe congenital neutropenia (SCN) and Clostridium septicum myonecrosis is an uncommon and life-threatening association requiring urgent combined aggressive medical and surgical management. We report 2 cases of SCN (1 with known Kostmann's syndrome and 1 not known at presentation to have a congenital neutropenic disorder but subsequently received a diagnosis of cyclic neutropenia) who presented with spontaneous C septicum myonecrosis. The cases highlight the importance of response to recombinant human granulocyte colony-stimulating factor in obtaining a satisfactory outcome for these patients. Early, empirical use of recombinant human granulocyte colony-stimulating factor in patients who are suspected of having a congenital neutropenia and who present with life-threatening sepsis is recommended.
authors.affiliation: Division of Haematology/Oncology, Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Chris
authors.initials: C
authors.lastname: Barnes
authors.forename: J Ted
authors.initials: JT
authors.lastname: Gerstle
authors.forename: Melvin H
authors.initials: MH
authors.lastname: Freedman
authors.forename: Manuel D
authors.initials: MD
authors.lastname: Carcao
mesh_terms: D002675:Child, Preschool; D003013:Clostridium / Q000302:isolation & purification*; D003015:Clostridium Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D005738:Gas Gangrene / Q000209:etiology; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008297:Male; D009132:Muscles / Q000473:pathology*; D009336:Necrosis / Q000209:etiology; D009503:Neutropenia / Q000150:complications / Q000151:congenital* / Q000188:drug therapy; D011994:Recombinant Proteins
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor