title: Life-threatening human herpes virus-6 infection in early childhood: presenting symptom of a primary immunodeficiency?
abstract: OBJECTIVE
  To report two previously healthy children with a life-threatening course of human herpes virus type 6 (HHV-6) infection and prolonged pediatric intensive care treatment.
  
  
  METHODS
  Case reports.
  
  
  METHODS
  A 16 bed pediatric intensive care unit at a tertiary care children's hospital.
  
  
  METHODS
  Two children with life-threatening HHV-6 disease.
  
  
  METHODS
  Both children were mechanically ventilated because of respiratory failure. A detailed viral and immunologic workup was performed and treatment with antiviral medication started.
  
  
  METHODS
  Polymerase chain reaction assays of plasma, cerebrospinal fluid, bronchoalveolar lavage, and lung biopsies yielded HHV-6 in both patients. Immunophenotyping and lymphocyte stimulation tests with both mitogens and antigens indicated an immunodeficiency in both patients.
  
  
  CONCLUSIONS
  HHV-6 infection should be considered in infants and young children with respiratory failure or meningo-encephalitis without clear causative agent or failure to respond to empirical treatment. A thorough immunologic workup and early start with antiviral therapy in any patient with a life-threatening course of HHV-6 infection is mandatory, because a severe HHV-6 infection can be the first indication of a primary immunodeficiency.
authors.affiliation: Department of Pediatric Intensive Care, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands. m.p.hennus@umcutrecht.nl
authors.forename: Marije P
authors.initials: MP
authors.lastname: Hennus
authors.forename: Joris M
authors.initials: JM
authors.lastname: van Montfrans
authors.forename: Adrianus J
authors.initials: AJ
authors.lastname: van Vught
authors.forename: Kiki
authors.initials: K
authors.lastname: Tesselaar
authors.forename: Jaap-Jan
authors.initials: JJ
authors.lastname: Boelens
authors.forename: Nicolaas J
authors.initials: NJ
authors.lastname: Jansen
mesh_terms: D001992:Bronchoalveolar Lavage Fluid; D002675:Child, Preschool; D004279:DNA, Viral / Q000032:analysis; D005260:Female; D015654:Herpesvirus 6, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000503:physiopathology*; D015278:Intensive Care Units, Pediatric; D008297:Male; D016133:Polymerase Chain Reaction; D019349:Roseolovirus Infections / Q000175:diagnosis* / Q000276:immunology / Q000821:virology; D012720:Severity of Illness Index
chemical_list: D004279:DNA, Viral