Diagnosis and management of pediatric adrenal crisis
Adrenal Insufficiency (AI) and Adrenal crisis (AC) are two critical diagnoses in pediatrics that can be devastating if missed and thus require prompt recognition and treatment. Adrenal crisis can be the first presentation of conditions associated with adrenal insufficiency. The adage, “if you don’t think about it, you can’t diagnose it” holds especially true for this diagnosis and reminds us to keep it on our differential for many different conditions as adrenal crisis can have a myriad of presentations as well.
The true incidence of AC in childhood is not known, but extrapolation from adult data suggests a frequency of 5-10ACs/100 patient years, and of those cases 1 in 200 will die (2-3). The episodes are more common in primary AI compared to secondary AI. Congenital Adrenal Hyperplasia (CAH) represents the most common cause of adrenal insufficiency in infancy and childhood. Bilateral adrenal hemorrhage and autoimmune adrenalitis (Addison disease) are two other primary AI diseases. Secondary AI diagnoses involve a problem in the hypothalamus and/or pituitary whether it be a receptor problem or a hormone problem. The most common cause of secondary AI is actually iatrogenic from cessation of high dose or chronic glucocorticoids. This is where being an astute clinician and taking a good history can clue you in to the diagnosis by recognizing nonspecific illness in the setting of chronic steroid use.
Children who carry the diagnosis of AI (and those who haven’t been officially diagnosed) are at risk for AC during any physiologic stress, particularly with co-occurring illness or injury. Viral rather than bacterial illnesses are more common in children, and acute gastroenteritis is a common precipitant to AC. Vomiting and diarrhea associated with gastroenteritis leads to dehydration, electrolyte disturbances, and poor absorption or inability to tolerate oral steroid replacement (2). Inadequate steroid coverage prior to surgical procedures as well as anesthesia during said procedures have been associated with increased risk of AC in pediatrics. There is also risk of AC surrounding compliance with routine glucocorticoid replacement and improper implementation of stress dosing protocols (2).
When is it actually a crisis?
There is not a concise definition in the literature for pediatrics, but the one that I think represents the severity and broad scope of the diagnosis is “An acute critical illness out of proportion in severity to the current illness and manifested by any of the following: hypotension/shock, decreased level of consciousness/lethargy, unexplained hypoglycemia or hyponatremia, or seizure.” (1) To further complicate things, other definitions include nausea, vomiting, diarrhea, dehydration, and weight loss. These kids are SICK and usually have a serious hemodynamic or electrolyte abnormalities. You may be seeing a theme here, but this is why prompt recognition and treatment is so important.
There are some lab studies that can help for these patients, including a complete metabolic panel, serum cortisol, and plasma ACTH level. However, the diagnosis is CLINICAL and should not be made based on labs! (Think tension pneumothorax diagnosed on CXR). The lab abnormalities you would expect to see with this patient population includes hyponatremia, hyperkalemia, and hypoglycemia. If you see a shock state not responding to fluid resuscitation, do not delay steroids (more on that below) if there is any difficulty obtaining labs.
Don’t fret, here is where we try and break things down and make things a bit easier. Parents will often come with an action plan from their friendly neighborhood endocrinologist and if that’s the case follow what is on the paper! For those that don’t come with treatment in hand, let’s breakdown the common treatment pathways. For oral stress dosing it is typically Triple their normal oral dose. Some parents will have IM hydrocortisone for home use and may have given that prior to arrival to the hospital.
Once in the Emergency Department, the preferred route and medication is IV hydrocortisone (unless you can’t get an IV, then give it IM). Methylprednisolone can be used if hydrocortisone is not readily available, but dexamethasone cannot be used for these patients since it has no mineralocorticoid action.
General dosing by age(IV/IM) is 25mg for <3, 50 mg 3-12, and 100mg for 12 and older. Once the stress dose has been given some fuzzy math using mg/m^2/day is used to determine ongoing dosing. This is typically 50-100mg/m^2/day divided every 6 hours for 24-48hrs.
Again, these kids often present in a shock state and are sick. They will likely need aggressive volume resuscitation with your fluid de jour, electrolyte correction, as well as glucose monitoring and replacement as necessary. They may require admission to the ICU depending on their level of illness.
Get the point
- These kids are sick! Consider adrenal insufficiency/adrenal crisis on your differential for patients in profound shock and treat 1st (steroids) and figure out the cause 2nd
- Don’t forget there are a constellation symptoms and presentations that can still be adrenal crisis, nonspecific symptoms of N/V/D with someone on chronic steroids should make you consider adrenal crisis!
-Use IV(Preferred) or IM hydrocortisone: 25 mg <3, 50mg 3-12, 100mg >12 years old
1. Goldbloom EB., Mokashi A., Cumming EA., Abish S., Benseler SM., Huynh HQ. Symptomatic adrenal suppression among children in Canada. Arch Dis Child. 2017. Apr;102(4):338-339.
2. Rushworth R., Torphy D., Stratakis C., Falhammar H. Adrenal Crises in Children: Perspectives and Research Directions. Hormone Research in Pediatrics. 2018; 89: 341-351.