Excellent Grand Rounds presentation today by Dr. Joseph Garcia, focused on HIF-2 signaling and the use of acetate to increase epo production in treatment of anemia. In his slides, he discussed the use of biomarkers beyond traditional iron studies (ferritin, transferrin, iron, etc.) to determine the etiology of a patient’s anemia. Among these, the soluble transferrin receptor (sTfR), is useful because it does not change in response to acute inflammation, making it useful in the diagnosis of anemia of chronic disease. The chart below summarizes the changes in sTfR levels in different settings of anemia:
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Review of the Physiology
- CRH –> pituitary ACTH –> adrenals to make glucocorticoids, mineralcorticoids and androgens
- Adrenal Gland
- Cortex makes cortisol, adrenal androgens and aldosterone
- Glomerulosa (aldosterone – salt)
- Fasciculata (cortisol – sugar)
- Reticularis (androgens – sex)
- Medulla makes epinephrine
- Autoimmune (Addison’s disease)
- Polyglandular syndromes
- Granulomatous infections (sarcoid, fungal infections, etc)
- Infiltrative diseases (HIV, CMV, amyloid)
- High ACTH
- Rapid withdrawal of exogenous steroids
- Low or inappropriately normal ACTH
Signs and Symptoms
Acute adrenal insufficiency/adrenal crisis can be an endocrine emergency!
- All causes: weakness, weight loss, NV, fatigue, vague abd pain, hypercalcemia (20%)
- Primary: hypotension and hyperpigmentation, hyperkalemia and hyponatremia (due to affecting both the aldo and cortisol)
- Secondary: hypotension but not associated with hyperkalemia (because aldo is still released by the renin-angiotension response)
- If acute AI suspected with s/sx of shock, must give steroids right away – Dexamethasone will not interfere with cortisol test but will affect ACTH.
- If stable, check Cosyntropin stim test
- Check baseline cortisol
- Give 0.25 mg cosyntropin
- Recheck cortisol at 30 and 60 mins
- Expected stimulation >18-20
- Check ACTH
- Check aldo level
- Expected findings:
- Low stim test
- High ACTH
- Low aldo
- Secondary or tertiary
- Low stim test
- Low or normal ACTH
- Normal aldo
- Acute adrenal insufficiency (ADRENAL CRISIS!)
- General idea: this is a medical emergency! High fatality rate if not treated (approaching 100%). If adrenal crisis is suspected, start treatment before labs have resulted!
- IV Fluids: 2-3 L NS or d5NS bolus as quickly as possible to correct hypotension and hyponatremia. Continue IVF for the next 24-48 hours or until stabilization.
- Dexamethasone: use if cosyntropin stim test has NOT been performed, as it does not interfere with the measurement of plasma cortisol. Give 4 mg decadron IV q12h.
- Hydrocortisone: use if the diagnosis has already been made or decadron is not available. Give 100mg q6h for 2-3 days.
- Continue IV steroids for 2-3 days or until stabilization – then taper to PO hydrocortisone.
- Needed only in primary adrenal insuffi ciency
- Not needed if hydrocortisone dose >50 mg per 24 h, as this will activate the mineralcorticoid receptor
- Chronic adrenal insufficiency:
- Glucocorticoid: hydrocortisone split into 2-3 doses daily, with 2/3 in the AM and 1/3 in the PM. Aim for the lowest possible steroid dose to relieve signs and symptoms.
- Primary adrenal insufficiency: start on 20–25 mg hydrocortisone per 24 h
- Secondary adrenal insufficiency: 15–20 mg hydrocortisone per 24 h
- Increase to stress dose for acute illnesses
- Mineralcorticoid: Needed only in primary adrenal insufficiency, and not if total hydrocortisone dose is greater than 50mg/day.
- Fludrocortisone oral 0.05-0.1 mg/day (lower dose may be sufficient in patients receiving hydrocortisone).
- Sex Steroids: consider in patients who complain of decreased energy, sexual dysfunction, or impaired mood despite optimum replacement therapy with glucocorticoids and mineralocorticoids.
- Start with DHEA 25–50 mg as a single morning dose
- Charmandari, Evangelia et al. Adrenal insufficiency. The Lancet , Volume 383 , Issue 9935, 2152 – 2167.
- Arlt, Wiebke et al. Adrenal insufficiency. The Lancet , Volume 361, Issue 9372 , 1881 – 1893.
- Jadoul, et al. Mineralocorticoids in the management of primary adrenocortical insufficiency. Journal of Endocrinological Investigation. February 1991, Volume 14, Issue 2, pp 87-91.
- Arlt, Wiebke et al. The Approach to the Adult with Newly Diagnosed Adrenal Insufficiency. The Journal of Clinical Endocrinology & Metabolism 2009 94:4, 1059-1067
This past week at Morning Report we discussed about cystic lung disease. Below is pertinent information regarding lung cysts and what to include in the differential:
- Lung cyst is defined as a round parenchymal lucency or area of low attenuation with a thin wall
- Cysts can be found on CT scans (particularly high-res) and requires a work-up as they are never normal
- Differential for diseases characterized by lung cysts is broad ranging from isolated chest disorders to rare multisystem diseases
- Characteristic findings on imaging, clinical history, and pathological findings (i.e. biopsy) may help determine the diagnosis
- Important to distinguish pulmonary cyst from cavity
- Cysts usually are thin walled, <2-3mm thick) and contain air but occassionally fluid or solid material
- Cavities are defined as gas-filled spaces that develop in an area of pulmonary consolidation, mass, or nodule
- Differential to consider in patient who presents with cysts:
- Centrilobular emphysema
- Lymphangioleiomyomatosis (LAM)
- Langerhans cell histiocytosis
- Lymphocytic interstitial pneumonia (LIP)
- Pulmonary metastasis
- Birt Hogg Dube syndrome
- Desquamative interstitial pneumonia (DIP)
- End stage IPF with honeycombing
Case Challenge #6 presented a 34 yo male with AIDS and disseminated histoplasmosis who presents with fever, hypotension, eosinophilia, hyponatremia, and hyperkalemia.
Which of the following is most likely to make the diagnosis?
The correct answer is:
Cosyntropin Stimulation Test
The patient has ADRENAL CRISIS!
- Can mimic sepsis with fevers, nausea, abdominal pain, and hypotension
- Typical scenario: Patient presents in shock after recent stressor (surgery, acute illness) in individual with risk factors for adrenal insufficiency (recent steroids, infection such as TB or Histo with predilection for adrenals)
- Classic labs: Hyponatremia, hyperkalemia, hypoglycemia, eosinophilia
Great job! A review of adrenal insufficiency is on the way. Challenge # 7 is coming up!
How much prednisone do I give my patient that I am tapering off of solumedrol?! Take a look at the handy chart below to learn more about steroid equivalencies.
Sandeep Mukherjee and Urmila Mukherjee, “A Comprehensive Review of Immunosuppression Used for Liver Transplantation,” Journal of Transplantation, vol. 2009, Article ID 701464, 20 pages, 2009. doi:10.1155/2009/701464
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