Life-threatening human herpes virus-6 infection in early childhood: presenting symptom of a primary immunodeficiency?
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.
Hennus
Marije P
MP
Department of Pediatric Intensive Care, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands. m.p.hennus@umcutrecht.nl
van Montfrans
Joris M
JM
van Vught
Adrianus J
AJ
Tesselaar
Kiki
K
Boelens
Jaap-Jan
JJ
Jansen
Nicolaas J
NJ
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
D004279:DNA, Viral