Zasha Francheska Vazquez-Colon, MD
University of Florida
Gainesville, Florida
Disclosure information not submitted.
Timothy Foster, MD
Pediatric Endocrinology Fellow
University of Florida, United States
Disclosure information not submitted.
Laith Al Ejeilat, MD
Pediatric Resident
University of Florida, United States
Disclosure information not submitted.
Tanner McGill, MD
Emergency Medicine Resident
University of Florida, United States
Disclosure information not submitted.
.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.
Maritza Plaza-Verduin, MD
Clinical Assistant Professor
University of Florida, United States
Disclosure information not submitted.
Henry Rohrs, MD
Clinical Associate Professor
University of Florida, United States
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: Severe Adrenal Crisis With Wide Spectrum of Life-Threatening Complications in a Pediatric Patient
Introduction: Primary adrenal insufficiency (PAI) is a rare disease, most commonly caused by autoimmunity in the US, although worldwide it is caused by tuberculosis. It is characterized by the inability of the adrenal cortex to produce enough glucocorticoid and mineralocorticoid. Symptoms can start as nonspecific, which can delay diagnosis, but it can have a severe and life – threatening presentation including hyponatremia, hyperkalemia, hypotension, shock, and seizures.
Description: An 8 year old previously healthy male presented to his pediatrician with 5 day history of nausea, vomiting and long standing preference for pickle juice. Thought to be viral gastritis, was instructed to switch back to water. Two days later he developed confusion followed by a brief seizure without return to baseline. He was taken to the emergency department and was found to have sodium 101 mmol/L, hypochloremia (72 mmol/L), hyperkalemia (6.0 mmol/L). He received a 3% NaCl bolus and started on 0.9% NaCl infusion. His physical examination was remarkable for knee hyperpigmentation. Hydrocortisone stress dose was empirically given for high suspicion of adrenal insufficiency. Workup revealed CK 134,000 U/L with preserved renal function (creatinine 0.51 mg/dL), cortisol 1.4 mcg/dL, ACTH 3208.7 pg/mL, and later returned positive adrenocortical cytoplasmic antibodies. Findings were consistent with acute adrenal crisis secondary to PAI. Given the severity of his illness, hydrocortisone was increased to 200 mg/m2/day. Echocardiogram showed moderately decreased left ventricular systolic function, brain MRI was normal and very long chain fatty acids were not elevated. Further management included slow sodium correction to avoid osmotic demyelinization, dantrolene, epinephrine drip and milrinone infusion. Patient recovered cardiac function and was transitioned to oral maintenance hydrocortisone and fludrocortisone.
Discussion: Presentation of PAI varies from nonspecific with nausea, vomiting, and fatigue, to seizures secondary to severe hyponatremia and shock. This is a rare example of a pediatric patient having such a severe and wide spectrum of symptoms. Early recognition and prompt management is crucial to avoid increased morbidity. Higher hydrocortisone dose could be considered in extremis cases until stabilization and clinical improvement.