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

Authors: Ganschow, Rainer¹; Grabhorn, Enke¹; Lemke, Joachim²; Lepler, Rudolf²

Source: Pediatric Allergy and Immunology, Volume 13, Number 4, August 2002 , pp. 307-310(4)

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Abstract:

Ganschow R, Grabhorn E, Lemke J, Lepler R. 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 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.

Keywords: Chediak-Higashi syndrome; cardiac failure; diadenosine polyphosphates; fever

Document Type: Case report

DOI: http://dx.doi.org/10.1034/j.1399-3038.2002.01052.x

Affiliations: 1: Department of Pediatrics, University of Hamburg, Hamburg, Germany, 2: Children's Hospital Wilhelmstift, Hamburg, Germany

Publication date: 2002-08-01