.jpg "Charlene Pringle, CPNP, FCCM (she/her/hers) photo")
Charlene Pringle, CPNP, FCCM (she/her/hers)
Pediatric Critical Care Nurse Practitioner
University of Florida
Gainesville, Florida
Disclosure information not submitted.
Zasha Francheska Vazquez-Colon, MD
University of Florida
Gainesville, Florida
Disclosure information not submitted.
Lara Nicolas, MD
Clinical Assistant Professor, Department of Pediatrics, Critical Care Medicine
University of Florida Health Shands Children's Hospital, United States
Disclosure information not submitted.
Title: Dantrolene as treatment for hyperpyrexia and rhabdomyolysis due to life-threatening adrenal crisis
Case Report Body: INTRODUCTION: Acute adrenal insufficiency is a life-threatening condition that may follow a longer state of non-specific malaise due to unrecognized chronic adrenal insufficiency. Presentation may be extremis with severe hyponatremia, seizures, and shock. Rarely, presentation may be complicated by high fever and rhabdomyolysis.
Description: An 8-year-old child without significant past history developed acute confusion followed by a brief seizure after 5 days of nausea, vomiting and malaise. Evaluation in emergency department was significant for severe electrolyte derangement with hyponatremia (101 mmol/L), hypochloremia (72 mmol/L) and hyperkalemia (6 mmol/L). He received a bolus of hypertonic saline and was admitted for hyponatremic seizures and altered mental status. During stabilization in the PICU there was noted hyperpigmentation of bilateral knees. He received empiric hydrocortisone for suspicion of adrenal insufficiency. Cortisol drawn prior to steroid administration subsequently resulted as 1.4 mcg/dL and ACTH 3208.7 ng/mL. Adrenocortical cytoplasmic antibodies later returned positive. Four hours after arrival he developed hyperpyrexia unresponsive to all typical cooling measures. CK, which had been initially mildly elevated at 9388 U/L rapidly rose to 134,000 U/L with myoglobinuria. Due to failure to control hyperpyrexia for over 4 hours with rapidly worsening rhabdomyolysis dantrolene 2.5 mg/kg IV was administered for malignant hyperthermia (MH) type syndrome in an effort to stop skeletal muscle hypermetabolism. Body temperature improved from 39.2 to 37.4 within 60 minutes of dantrolene administration. CK peaked at 134,000 U/L and was improved to 88000 U/L within 12 hours and 5431 U/L within 24 hours, with resolution of myoglobinuria. Renal function remained preserved. There was no family history of MH or neuroleptic malignant syndrome.
Discussion: Classically triggered by volatile anesthetics and succinylcholine, malignant hyperthermia with rhabdomyolysis has also been attributed to viral infections. There are a few case reports describing a MH-type syndrome with rhabdomyolysis during primary adrenal crisis, with only one prior case described in a child. To our knowledge ours is the first report of successful use of dantrolene to stabilize skeletal muscle metabolism in this scenario in a child.