Abstract
The onset and progression of cardiac involvement in juvenile neuronal ceroid lipofuscinosis (Batten disease) are studied in 29 children and adolescents with genetically verified disease at Aarhus University Hospital, Skejby, Denmark.
Highlights
Children who undergo liver transplantation for VPA-associated acute liver failure have a significantly worse survival rate compared with children with LF caused by other drugs
Heart rate and variability were significantly reduced with increasing age, suggesting a decreased parasympathetic activity on the heart or negative influence on sinus node automaticity
Bradycardia, arrhythmia, sinus arrests and atrial flutter indicated an age-dependent decrease in sinus node activity
Summary
Children who undergo liver transplantation for VPA-associated acute liver failure have a significantly worse survival rate compared with children with LF caused by other drugs. The authors conclude that in a child with a progressive seizure disorder and acute liver failure after VPA therapy, the VPA should be discontinued and carnitine initiated while investigating a presumed mitochondrial disorder. Liver transplant should be contraindicated for VPA-associated liver failure, even when a mitochondrial disease is not confirmed. The onset and progression of cardiac involvement in juvenile neuronal ceroid lipofuscinosis (Batten disease) are studied in 29 children and adolescents with genetically verified disease at Aarhus University Hospital, Skejby, Denmark.
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