Amiodarone for the emergency care of children.

Amiodarone is a class 3 antiarrhythmic agent used for a broad range of arrhythmias including adenosine-resistant supraventricular tachycardia, junctional ectopic tachycardia, and ventricular tachycardia. Compared with adults, there are few data on its use in children with arrhythmias resistant to conventional therapy. National and international guidelines for cardiopulmonary resuscitation and emergency cardiovascular care recommend its use for a variety of arrhythmias based on case reports, cohort studies, and extrapolation from adult data. This article will review the historical development, chemical properties, metabolism, indications and contraindications, and adverse effects of amiodarone in infants and children. After completing this CME activity, the reader should be able to utilize amiodarone in the pediatric population for arrhythmias and identify complications associated with its use.

Segmental neurofibromatosis associated with renal angiomyolipomas.

Segmental neurofibromatosis (SN) is a rare disorder characterized by neurofibromas or neurofibromas with café-au-lait spots limited to one region of the body without crossing the midline. Renal angiomyolipomas (AMLs) are rare benign neoplasms usually found in association with tuberous sclerosis (TS). Similar to neurofibromatosis (NF), TS has a high spontaneous mutation rate and a family history often is absent. Although both are autosomal dominant diseases with neural involvement, there are few reports in the literature demonstrating a link between the 2 disorders. We report a case of SN associated with renal AMLs. To our knowledge, there has been only 1 prior report of renal AML associated with NF type 1 (NF1), and there have been no prior reports of SN associated with AML.