Cardiac failure in an infant with Chediak–Higashi syndrome: A hypothesis of the effect of diadenosine polyphosphates.

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 AP₄ A, AP₅ A, or AP₆ A, 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. [ABSTRACT FROM AUTHOR]