Mitigating Alarm Fatigue and Improving the Bedside Experience by Reducing Nonactionable Alarms.

Objective: To assess whether conditional bedside alarm triggers can reduce the frequency of nonactionable alarms without compromising patient safety and enhance nursing and family satisfaction.

Study Design: Single-center, quality improvement initiative in an acute care cardiac unit and pediatric intensive care unit. Following the 4-week preintervention baseline period, bedside monitors were programmed with hierarchical time delay and conditional alarm triggers.

Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.

Only a single patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency has been described in the literature, and the molecular basis of this inborn error of valine catabolism has remained unknown until now. Here, we present a second patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency, who was identified through blood spot acylcarnitine analysis showing persistently increased levels of hydroxy-C(4)-carnitine. Both patients manifested hypotonia, poor feeding, motor delay, and subsequent neurological regression in infancy.