Letter to the Editor: “Prevention of Adrenal Crisis: Cortisol Responses to Major Stress Compared to Stress Dose Hydrocortisone Delivery”

Introduction. Thyroid hormone is known to accelerate glucocorticoid turnover. In a thyrotoxic state, individuals with adrenal insufficiency are unable to increase endogenous cortisol production to compensate for increased turnover, placing them at risk for symptoms of glucocorticoid deficiency and adrenal crisis. In patients with salt-wasting congenital adrenal hyperplasia (SW-CAH), hyperandrogenemia is a measurable reflection of relative glucocorticoid insufficiency. Case Presentation. A 12-year-old girl with SW-CAH reported 3 recent episodes of vomiting without diarrhea, and accompanying tachycardia, responsive to stress dose steroids. In the previous 9 months, she unintentionally lost 2.6 kg. She had tachycardia and new thyromegaly. Labs showed suppressed TSH, elevated free T4 and total T3, and elevated thyroid stimulating immunoglobulin (TSI) consistent with Graves’ disease. Adrenal androgens were markedly elevated. Maintenance hydrocortisone dose was 25 mg/m2/day and was not changed. Methimazole was initiated. Four weeks later, free T4 and adrenal androgens normalized. She had no further vomiting episodes. Conclusions. Thyrotoxicosis must be included in the differential diagnosis of individuals with SW-CAH who present with episodes concerning for adrenal crises, escalating hydrocortisone requirements, and/or inadequate suppression of adrenal hormones.

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ENDOCRINOLOGY IN THE TIME OF COVID-19: Management of adrenal insufficiency

Acta Endocrinologica ◽

10.1530/eje-20-0361 ◽

2020 ◽

Vol 183 (1) ◽

pp. G25-G32 ◽

Cited By ~ 14

Author(s):

Wiebke Arlt ◽

Stephanie E Baldeweg ◽

Simon H S Pearce ◽

Helen L Simpson

Keyword(s):

Adrenal Insufficiency ◽

Clinical Deterioration ◽

Adrenal Crisis ◽

Modified Release ◽

Risk Of Infection ◽

Course Of Disease ◽

Restricted Access ◽

Increased Risk ◽

Sick Day Rules ◽

Stress Dose

We provide guidance on prevention of adrenal crisis during the global COVID-19 crisis, a time with frequently restricted access to the usual level of healthcare. Patients with adrenal insufficiency are at an increased risk of infection, which may be complicated by developing an adrenal crisis; however, there is currently no evidence that adrenal insufficiency patients are more likely to develop a severe course of disease. We highlight the need for education (sick day rules, stringent social distancing rules), equipment (sufficient glucocorticoid supplies, steroid emergency self-injection kit) and empowerment (steroid emergency card, COVID-19 guidelines) to prevent adrenal crises. In patients with adrenal insufficiency developing an acute COVID-19 infection, which frequently presents with continuous high fever, we suggest oral stress dose cover with 20 mg hydrocortisone every 6 h. We also comment on suggested dosing for patients who usually take modified release hydrocortisone or prednisolone. In patients with adrenal insufficiency showing clinical deterioration during an acute COVID-19 infection, we advise immediate (self-)injection of 100 mg hydrocortisone intramuscularly, followed by continuous i.v. infusion of 200 mg hydrocortisone per 24 h, or until this can be established, and administration of 50 mg hydrocortisone every 6 h. We also advise on doses for infants and children.

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Increased Frequency of Acute Illness and Hospitalizations in Infants and Toddlers with Congenital Adrenal Hyperplasia

10.1101/19005462 ◽

2019 ◽

Author(s):

Teresa Tseng ◽

Amy Seagroves ◽

Christina M. Koppin ◽

Madison Keenan ◽

Elana Putterman ◽

...

Keyword(s):

High Risk ◽

Congenital Adrenal Hyperplasia ◽

Acute Illness ◽

Adrenal Crisis ◽

Infants And Toddlers ◽

Adrenal Hyperplasia ◽

Tertiary Center ◽

Ed Visits ◽

The Impact ◽

Stress Dose

AbstractPurposeInfants and toddlers with classical congenital adrenal hyperplasia (CAH) are at high risk for adrenal crisis and associated sequelae. To better understand acute illness at this early age, we determined the frequency and severity of acute illness and hospitalizations between 0-4 years of age, both within CAH and compared to controls. We also evaluated the impact of pre-hospital stress-dose hydrocortisone on Emergency Department (ED) visits and hospitalizations.MethodsWe performed a retrospective study of 40 CAH youth and 27 age-matched controls at a tertiary center. Characteristics of acute illnesses during the first 4 years of life were recorded, including fever, vomiting, diarrhea, ED visits, hospitalizations, abnormal electrolytes, and stress-dose hydrocortisone usage.ResultsCAH youth had more frequent illnesses requiring stress-dosing when they were younger than 2 years old [4.0 (1.0-6.0)] compared to when they were 2-4 years old [3.0 (1.0-4.0), P < 0.05], with the most illnesses during their first year of life. As well, CAH infants and toddlers had more hospitalizations younger than 2 years old compared to 2-4 years old (36 vs 2). 25% (3/12) of CAH youth with abnormal electrolytes in the ED did not receive any stress-dosing (oral/IM) prior to the ED, and only 25% (3/12) had received intramuscular hydrocortisone at home. CAH youth had more frequent ED visits (7.4 times as many) and hospitalizations (38 to 0) compared to controls.ConclusionsVery young children with classical CAH are at high risk for acute illness and hospitalizations during their first 2 years of life, and do not receive adequate stress-dosing prior to the ED despite appropriate education. Our findings underscore the need for earlier recognition of acute illness in this vulnerable population and improved education regarding administration of stress-dose hydrocortisone to prevent morbidity.

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Perioperative Adrenal Crisis

10.1093/med/9780190226459.003.0028 ◽

2017 ◽

Author(s):

Jing Tao ◽

Jeffrey J. Schwartz

Keyword(s):

Adrenal Insufficiency ◽

Adrenal Crisis ◽

Life Saving ◽

Distributive Shock ◽

Threatening Condition ◽

Life Threatening ◽

High Doses ◽

Patient At Risk ◽

Stress Dose ◽

Timely Treatment

Perioperative adrenal insufficiency (AI) (adrenal crisis) is an uncommon life-threatening condition manifested by distributive shock that is poorly responsive to the administration of fluids and vasopressors. Timely treatment with high doses of glucocorticoids can be life saving. The difficulty is in recognizing the patient at risk, suspecting the diagnosis, and distinguishing it from other forms of shock. The incidence of adrenal crisis is rare, in large part, due to the liberal use of perioperative “stress dose” steroids in patients already receiving steroids. In this chapter we review the pathophysiology involved in both primary and secondary AI, and provide step-by-step treatment recommendations for patients as risk for this condition.

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Perioperative glucocorticoid management based on current evidence

Anesthesia and Pain Medicine ◽

10.17085/apm.20089 ◽

2021 ◽

Vol 16 (1) ◽

pp. 8-15

Author(s):

Kwon Hui Seo

Keyword(s):

Adrenal Insufficiency ◽

Surgical Stress ◽

Perioperative Period ◽

Hemodynamic Instability ◽

Current Evidence ◽

Major Surgery ◽

Adrenal Crisis ◽

Number Of Patients ◽

Glucocorticoid Administration ◽

Stress Dose

Glucocorticoid preparations, adreno-cortical steroids, with strong anti-inflammatory and immunosuppressive effects, are widely used for treating various diseases. The number of patients exposed to steroid therapy prior to surgery is increasing. When these patients present for surgery, the anesthesiologist must decide whether to administer perioperative steroid supplementation. Stress-dose glucocorticoid administration is required during the perioperative period because of the possibility of failure of cortisol secretion to cope with the increased cortisol requirement due to surgical stress, adrenal insufficiency, hemodynamic instability, and the possibility of adrenal crisis. Therefore, glucocorticoids should be supplemented at the same level as that of normal physiological response to surgical stress by evaluating the invasiveness of surgery and inhibition of the hypothalamus-pituitary-adrenal axis. Various textbooks and research articles recommend the stress-dose of glucocorticoids during perioperative periods. It has been commonly suggested that glucocorticoids should be administered in an amount equivalent to about 100 mg of cortisol for major surgery because it induces approximately 5 times the normal secretion. However, more studies, with appropriate power, regarding the administration of stress-dose glucocorticoids are still required, and evaluation of patients with possible adrenal insufficiency and appropriate glucocorticoid administration based on surgical stress will help improve the prognosis.

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Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings

Journal of Investigative Medicine ◽

10.1136/jim-2019-000999 ◽

2019 ◽

Vol 68 (1) ◽

pp. 16-25 ◽

Cited By ~ 3

Author(s):

Bradley S Miller ◽

Sandra P Spencer ◽

Mitchell E Geffner ◽

Evgenia Gourgari ◽

Amit Lahoti ◽

...

Keyword(s):

Emergency Management ◽

Adrenal Insufficiency ◽

Early Recognition ◽

Action Plan ◽

Sodium Succinate ◽

Urgent Care ◽

Adrenal Crisis ◽

Care Providers ◽

Endocrine Society ◽

Stress Dose

Adrenal insufficiency (AI) remains a significant cause of morbidity and mortality in children with 1 in 200 episodes of adrenal crisis resulting in death. The goal of this working group of the Pediatric Endocrine Society Drug and Therapeutics Committee was to raise awareness on the importance of early recognition of AI, to advocate for the availability of hydrocortisone sodium succinate (HSS) on emergency medical service (EMS) ambulances or allow EMS personnel to administer patient’s HSS home supply to avoid delay in administration of life-saving stress dosing, and to provide guidance on the emergency management of children in adrenal crisis. Currently, hydrocortisone, or an equivalent synthetic glucocorticoid, is not available on most ambulances for emergency stress dose administration by EMS personnel to a child in adrenal crisis. At the same time, many States have regulations preventing the use of patient’s home HSS supply to be used to treat acute adrenal crisis. In children with known AI, parents and care providers must be made familiar with the administration of maintenance and stress dose glucocorticoid therapy to prevent adrenal crises. Patients with known AI and their families should be provided an Adrenal Insufficiency Action Plan, including stress hydrocortisone dose (both oral and intramuscular/intravenous) to be provided immediately to EMS providers and triage personnel in urgent care and emergency departments. Advocacy efforts to increase the availability of stress dose HSS during EMS transport care and add HSS to weight-based dosing tapes are highly encouraged.

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Corticosteroids Replacement in a Patient With Panhypopituitarism and COVID-19 Infection

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1180 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A578-A579

Author(s):

Gowri Karuppasamy ◽

Zaina Abdelhalim Alamer ◽

Samman Rose ◽

Ibrahim Abdulla Al-Janahi

Keyword(s):

Pituitary Adenoma ◽

Adrenal Insufficiency ◽

Hormone Replacement ◽

Hypogonadotropic Hypogonadism ◽

Adrenal Crisis ◽

High Dose ◽

Pituitary Hormone ◽

Secondary Hypothyroidism ◽

Stress Dose

Abstract Background: Hypopituitarism refers to complete or partial insufficiency of pituitary hormone secretion and patients require lifelong hormone replacement. Those with ACTH deficiency rely on exogenous glucocorticoids and at times of intercurrent illness require stress doses to prevent an adrenal crisis. The benefits and adverse effects of corticosteroids for treatment of COVID-19 pneumonia are currently under investigation. We report our experience in a patient with COVID-19 pneumonia who received high dose corticosteroids for panhypopituitarism. Clinical Case: A 51-year-old man presented with one-week history of fever and generalized weakness. He had been diagnosed with a non-functional pituitary macro-adenoma causing panhypopituitarism 1 year ago when he developed generalized tonic-clonic seizures. He underwent trans-sphenoidal resection of the pituitary adenoma. However, he then discontinued his hormonal therapy and was lost to follow up. He had postural hypotension but was not tachypneic or hypoxemic. He tested positive for COVID-19 and chest x-ray showed prominent bilateral broncho-vascular markings; he was hospitalized as mild COVID-19 pneumonia. Laboratory investigations revealed secondary adrenal insufficiency, secondary hypothyroidism and hypogonadotropic hypogonadism. MRI of the pituitary now showed persistence of the pituitary macroadenoma, measuring 3.5 x 3.7 x 2.4 cm in dimensions, causing sellar obliteration and left cavernous sinus invasion. Treatment with stress dose steroids, Hydrocortisone 50 mg 4 times daily was initiated, followed by thyroid hormone replacement with Levothyroxine 125 mcg daily. He also received antivirals and supportive care for COVID-19, guided by local hospital protocol. After significant clinical improvement, steroids were tapered down and he was discharged on a maintenance dose of 20 mg hydrocortisone per day in divided doses. The patient was stable at outpatient follow up after one month. He was started on testosterone replacement for erectile dysfunction due to hypogonadotropic hypogonadism. He was offered surgery for complete resection of the residual pituitary adenoma, but he declined and preferred to continue medical therapy. Conclusion: Hypopituitarism is associated with significant morbidity and premature mortality, a key risk factor being cortisol deficiency. Adrenal crisis is a life-threatening medical emergency and remains an important cause of death in patients with adrenal insufficiency. These patients are also vulnerable to develop severe complications from COVID-19 infection due to the absence of normal cortisol responses to stress. Despite receiving stress dose corticosteroids, this high-risk patient recovered from COVID-19 pneumonia without complications. These findings support the use of corticosteroids when necessary for treatment of coexisting conditions in patients with COVID-19.

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