A 29 year old female with a history of a congenital endocrinological deficiency (on chronic hormonal replacement therapy) is brought into your critical care area as a “sepsis code”.
She tells you that for the past week she has had a productive cough and fever, but did not take the extra “hormones” that she was told to take when she is sick or under a lot of stress.
Amidst her hypotensive hallucinations, she tells you that she was born with ambiguous genitalia (and if this wasn’t reminiscent of a Step 1 question about 21 something or other deficiency, you would think she was tripping on “trainwreck”).
On physical exam: VS: 78/54, HR: 109, T: 101.3F, O2Sat: 98% on RA.
Otherwise, she has hyperpigmented macules in her oral mucosa and signs of an upper respiratory infection.
Labs:
CBC: WBC:13.04 Hb:14.2 Hct:42.7 Plt:248
CMP: Na:129 K:5.7 Cl:116 CO2:18 BUN:9 Cr:0.86 Glu:98, Arterial PH: 7.35
CXR: neg.
What endocrinological emergency does this patient have?
Acute on chronic adrenal insufficiency. The patient has congenital adrenal hyperplasia (21 hydroxylase deficiency: ambiguous genitalia, salt wasting, life long systemic steroids) and did not take larger doses of steroids during an acute infection.
The most common manifestations of acute adrenal insufficiency are:
- Hypotension and shock out of proportion to infection, in particular postural.
- Abdominal pain, nausea and vomiting.
- Laboratory: hyponatremia, hyperkalemia, mild hyperchloremia.
- Depending on etiology: hyperpigmentation (due to elevated ACTH) / vitiligo.
The most common etiologies are:
Primary adrenal insufficiency: adrenal dysfunction: congenital adrenal hyperplasia, infection, drugs, adrenal hemorrhage, sarcoid or metastases.
Secondary adrenal insufficiency: hypothalamic-pituitary dysfunction: iatrogenic (withdrawal of chronic steroids), pituitary pathology, Sheehan syndrome.
** The most common infectious cause of adrenal insufficiency in the world is tuberculosis and in the US, HIV.
How do you diagnose this condition in the ED?
You don’t. Leave it for the medicine people to do their cosyntropin stimulation test and other medieval alchemy to differentiate primary versus secondary adrenal insufficiency. But if you have a situation where shock is out of proportion to the sepsis, and the blood pressure is refractory to fluids you should have to treat aggressively.
Certainly, you should obtain extensive imaging and laboratory tests, including a cortisol level of which a level >18mcg virtually rules out an adrenal crisis.
How do you treat?
“Prognosis is related to rapidity of treatment”
Crystaloids + dextrose.
Stress dose steroids 100 mg bolus of hydrocortisone (mineralo and corticosteroids) or 4 mg dexamethasone. Then place on maintenance.
For refractory hypotension, use pressors.
Lastly, where did Gauze get its name?
Depends whether you ask the French government or English dictionaries, though they both lead to the same place; Gaza. According to English dictionaries, the word “Gauze” is derived from a form of silk weaving that was popular in the middle ages in Gaza, Palestine. However, according to the French government (ooh lala), the english word comes from the French word “gaze”, which in turn likely originated from the Arabic and Persian word “qazz”, which means “raw silk”; it too originated from the name of the town of Gaza.
Tintinalli, Judith E., and J. Stephan. Stapczynski. “225.” Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. New York: McGraw-Hill, 2011. 1526+. Print.
Nieman, Lynnette K., MD. “UpToDate.” UpToDate. N.p., Feb. 2013. Web. 20 July 2015.
Written by Itamar Goldstein MD
And thanks to Dr. James Willis for his guidance.
Itamar Goldstein
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