title: Cardiac failure in an infant with Chediak-Higashi syndrome: a hypothesis of the effect of diadenosine polyphosphates.
abstract: A 14-week-old boy with undiagnosed Chediak-Higashi syndrome developed fever with a high temperature and acute cardiac failure after having received a scheduled vaccination. We hypothesize that decreased concentrations and receptor binding of serum and tissue diadenosine polyphosphates, such as AP4A, AP5A, or AP6A, which are stored in various tissues and serve as extra-cellular signaling molecules or are secreted by cells in response to physiologically stressful stimuli, lead to the observed severe tachyarrhythmia. Diadenosine polyphosphates normally have a negative chronotropic and inotropic effect. This is the first report of severe cardiac failure in a child with Chediak-Higashi syndrome and we suggest that cardiac arrhythmias should be considered in such children in the event of high fever. Our hypothesis requires further investigation in other patients.
authors.affiliation: Department of Pediatrics, University of Hamburg, Hamburg, Germany, Children's Hospital Wilhelmstift, Hamburg, Germany. ganschow@uke.uni-hamburg.de
authors.forename: Rainer
authors.initials: R
authors.lastname: Ganschow
authors.forename: Enke
authors.initials: E
authors.lastname: Grabhorn
authors.forename: Joachim
authors.initials: J
authors.lastname: Lemke
authors.forename: Rudolf
authors.initials: R
authors.lastname: Lepler
mesh_terms: D002303:Cardiac Output, Low / Q000209:etiology* / Q000503:physiopathology*; D002609:Chediak-Higashi Syndrome / Q000503:physiopathology*; D015226:Dinucleoside Phosphates / Q000009:adverse effects*; D006801:Humans; D007223:Infant; D008297:Male
chemical_list: D015226:Dinucleoside Phosphates