scholarly journals EXTENSIVE EXPERTISE IN ENDOCRINOLOGY: Adrenal crisis

2015 ◽  
Vol 172 (3) ◽  
pp. R115-R124 ◽  
Author(s):  
Bruno Allolio
Keyword(s):  
Patient Education ◽  
Adrenal Insufficiency ◽  
Well Being ◽  
Stressful Events ◽  
Major Surgery ◽  
Adrenal Crisis ◽  
Self Administration ◽  
Necrosis Factor Alpha ◽  
Hydrocortisone Administration ◽  
Glucocorticoid Deficiency

Adrenal crisis is a life-threatening emergency contributing to the excess mortality of patients with adrenal insufficiency. Studies in patients on chronic replacement therapy for adrenal insufficiency have revealed an incidence of 5–10 adrenal crises/100 patient years and suggested a mortality rate from adrenal crisis of 0.5/100 patient years. Patients with adrenal crisis typically present with profoundly impaired well-being, hypotension, nausea and vomiting, and fever responding well to parenteral hydrocortisone administration. Infections are the major precipitating causes of adrenal crisis. Lack of increased cortisol concentrations during infection enhances pro-inflammatory cytokine release and sensitivity to the toxic effects of these cytokines (e.g. tumour necrosis factor alpha). Furthermore, pro-inflammatory cytokines may impair glucocorticoid receptor function aggravating glucocorticoid deficiency. Treatment of adrenal crisis is simple and highly effective consisting of i.v. hydrocortisone (initial bolus of 100 mg followed by 200 mg over 24 h as continuous infusion) and 0.9% saline (1000 ml within the first hour). Prevention of adrenal crisis requires appropriate hydrocortisone dose adjustments to stressful medical procedures (e.g. major surgery) and other stressful events (e.g. infection). Patient education is a key for such dose adjustments but current education concepts are not sufficiently effective. Thus, improved education strategies are needed. Every patient should carry an emergency card and should be provided with an emergency kit for parenteral hydrocortisone self-administration. A hydrocortisone pen would hold a great potential to lower the current barriers to hydrocortisone self-injection. Improved patient education and measures to facilitate parenteral hydrocortisone self-administration in impending crisis are expected to significantly reduce morbidity and mortality from adrenal crisis.

scholarly journals Standardised patient education in adrenal insufficiency: a prospective multi-centre evaluation

2020 ◽  
Vol 183 (2) ◽  
pp. 119-127
Author(s):  
Stephanie Burger-Stritt ◽  
Annemarie Eff ◽  
Marcus Quinkler ◽  
Tina Kienitz ◽  
Bettina Stamm ◽  
...  
Keyword(s):  
Patient Education ◽  
Adrenal Insufficiency ◽  
Well Being ◽  
Education Programme ◽  
Basic Knowledge ◽  
Adrenal Crisis ◽  
Data Sets ◽  
Subjective Well Being ◽  
Multi Centre Study ◽  
Repeated Training

Objective Patients with adrenal insufficiency (AI) suffer from impaired quality of life and are at risk of adrenal crisis (AC) despite established replacement therapy. Patient education is regarded an important measure for prevention of AC and improvement of AI management. A standardized education programme was elaborated for patients with chronic AI in Germany. Design Longitudinal, prospective, questionnaire-based, multi-centre study. Methods During 2-h sessions, patients (n = 526) were provided with basic knowledge on AI, equipped with emergency cards and sets and trained in self-injection of hydrocortisone. To evaluate the education programme, patients from eight certified centres completed questionnaires before, immediately after and 6–9 months after training. Results 399 completed data sets were available for analysis. Questionnaire score-values were significantly higher after patient education, indicating successful knowledge transfer (baseline: 17 ± 7.1 of a maximum score of 29; after training: 23 ± 4.2; P < 0.001), and remained stable over 6–9 months. Female sex, younger age and primary cause of AI were associated with higher baseline scores; after education, age, cause of AI and previous adrenal crisis had a significant main effect on scores. 91% of patients would dare performing self-injection after training, compared to 68% at baseline. An improvement of subjective well-being through participation in the education programme was indicated by 95% of the patients 6–9 months after participation. Conclusion Patient group education in chronic AI represents a helpful tool for the guidance of patients, their self-assurance and their knowledge on prevention of adrenal crises. Repeated training and adaptation to specific needs, for example, of older patients is needed.

scholarly journals Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies

2010 ◽  
Vol 162 (3) ◽  
pp. 597-602 ◽  
Author(s):  
Stefanie Hahner ◽  
Melanie Loeffler ◽  
Benjamin Bleicken ◽  
Christiane Drechsler ◽  
Danijela Milovanovic ◽  
...  
Keyword(s):  
Risk Factors ◽  
Adrenal Insufficiency ◽  
Educational Status ◽  
Cross Sectional Study ◽  
Sudden Onset ◽  
Stressful Events ◽  
Adrenal Crisis ◽  
Cross Sectional ◽  
Crisis Prevention ◽  
Dhea Treatment

ObjectiveAdrenal crisis (AC) is a life-threatening complication of adrenal insufficiency (AI). Here, we evaluated frequency, causes and risk factors of AC in patients with chronic AI.MethodsIn a cross-sectional study, 883 patients with AI were contacted by mail. Five-hundred and twenty-six patients agreed to participate and received a disease-specific questionnaire.ResultsFour-hundred and forty-four datasets were available for analysis (primary AI (PAI), n=254; secondary AI (SAI), n=190). Forty-two percent (PAI 47% and SAI 35%) reported at least one crisis. Three hundred and eighty-four AC in 6092 patient years were documented (frequency of 6.3 crises/100 patient years). Precipitating causes were mainly gastrointestinal infection and fever (45%) but also other stressful events (e.g. major pain, surgery, psychic distress, heat and pregnancy). Sudden onset of apparently unexplained AC was also reported (PAI 6.6% and SAI 12.7%). Patients with PAI reported more frequent emergency glucocorticoid administration (42.5 vs 28.4%, P=0.003). Crisis incidence was not influenced by educational status, body mass index, glucocorticoid dose, DHEA treatment, age at diagnosis, hypogonadism, hypothyroidism or GH deficiency. In PAI, patients with concomitant non-endocrine disease were at higher risk of crisis (odds ratio (OR)=2.02, 95% confidence interval (CI) 1.05–3.89, P=0.036). In SAI, female sex (OR=2.18, 95% CI 1.06–4.5, P=0.035) and diabetes insipidus (OR=2.71, 95% CI 1.22–5.99, P=0.014) were associated with higher crisis incidence.ConclusionAC occurs in a substantial proportion of patients with chronic AI, mainly triggered by infectious disease. Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI.

scholarly journals The incidence of adrenal crisis in the postoperative period of HPA axis insufficiency after surgical treatment for Cushing’s syndrome

2019 ◽  
Vol 181 (2) ◽  
pp. 201-210 ◽  
Author(s):  
Leonie H A Broersen ◽  
Femke M van Haalen ◽  
Tina Kienitz ◽  
Olaf M Dekkers ◽  
Christian J Strasburger ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Crisis ◽  
Pituitary Hormone ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Life Threatening ◽  
Glucocorticoid Deficiency

Background Adrenal crisis, the most feared complication of adrenal insufficiency, is a potentially life-threatening state of acute glucocorticoid deficiency. After successful surgery for Cushing’s syndrome, many patients develop (transient) adrenal insufficiency. The incidence of adrenal crisis in patients treated for hypercortisolism is unknown. Methods Cohort study included consecutive patients with Cushing’s syndrome with adrenal insufficiency after surgery from Leiden and Berlin from 2000 to 2015. We summarized the incidence of adrenal crisis, compared patients with and without adrenal crisis regarding potential risk factors for its occurrence and assessed the effect of better education in time on incidence of adrenal crisis. Results We included 106 patients, of whom 19 patients had a total of 41 adrenal crises. There were 9.0 crises per 100 patient-years at risk (95% confidence interval (CI): 6.7–12.0). All crises occurred while on hydrocortisone replacement. The risk ratio for a recurrent crisis was 2.3 (95% CI: 1.2–4.6). No clear change in incidence of adrenal crisis due to better education in time was observed. There was no difference in recurrence rate between patients with, and without any crisis, but patients with adrenal crisis had more often pituitary deficiencies. Conclusions The incidence of adrenal crises after treatment for Cushing’s syndrome is substantial, and patients who suffered from an adrenal crisis have higher risk for recurrent crisis. Adrenal crisis tends to present early after remission of Cushing’s syndrome, which is probably the period of severest HPA axis suppression, despite in general higher hydrocortisone replacement doses for withdrawal complaints in this period. Additional pituitary hormone deficiencies may be a risk marker for increased risk of adrenal crisis. However, further risk factor analysis is needed to identify risks for a first crisis. Effective education methods to prevent adrenal crises should be identified and implemented, including stress instructions by trained nursing staff before hospital discharge.

scholarly journals Adrenoleukodystrophy as a Cause of Spastic Paraparesis and Hyperpigmentation

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A113-A113
Author(s):  
Priyanka Kesavan Chary ◽  
Aziz Ur Rehman ◽  
Tamis Marie Bright
Keyword(s):  
Erectile Dysfunction ◽  
Lower Extremity ◽  
Adrenal Insufficiency ◽  
Adrenal Glands ◽  
Spastic Paraparesis ◽  
Well Being ◽  
Adrenal Crisis ◽  
Lower Extremity Weakness ◽  
Mri Brain ◽  
Work Up

Abstract Background: X-linked Adrenoleukodystrophy (X-ALD) is caused by an abnormal gene mutation on the X chromosome which codes for an abnormal ABC transporter, preventing transport of very long chain fatty acids (VLCFAs) into peroxisomes for beta oxidation. These VLCFAs, unable to be broken down, thus accumulate in the CNS, adrenal cortex, and Leydig cells of the testes. The clinical presentation depends upon the anatomical involvement, which most often includes the CNS and adrenal glands, and can be a cause of adrenomyeloneuropathy (AMN) or adrenal insufficiency. Case Report: We report a 44 year old male who presented with 9 years of progressive spastic paraparesis. Physical exam was significant for lower extremity spasticity, 3/5 bilateral lower extremity weakness, and hyperpigmentation. His initial work-up for autoimmune disease, MRI brain, and lumbar puncture were unremarkable, and genetic testing for Hereditary Spastic Paraparesis was negative. However, due to advancing symptoms over the years with progression of spasticity, polyneuropathy, development of erectile dysfunction and significant hyperpigmentation, re-evaluation with MRI brain and EMG revealed diffuse symmetrical cerebral and cerebellar volume loss and axonal motor peripheral neuropathy, respectively. Due to hyperpigmentation, further work-up for VLCFAs was positive and presence of the ABCD1 mutation confirmed the diagnosis of X-ALD. Evaluation of adrenal glands yielded high ACTH of &gt;2000 pg/mL (normal: 7.2–63.3) and low AM cortisol of 2.5 ug/dL (normal: 6.2–19.4). Insignificant increase of cortisol from 2.5 ug/dL to 2.7 ug/dL with cosyntropin confirmed primary adrenal insufficiency as the cause of hyperpigmentation with elevated ACTH. Surprisingly, his testosterone level was normal at 542 (normal: 250–1100 ng/dL) and suggestive of Leydig cell-sparing and neuropathy-induced erectile dysfunction. The patient was started on hydrocortisone for adrenal insufficiency with improvement in his fatigue and feelings of well-being. He received physical therapy and is being monitored closely for progression of AMN by Neurology. Conclusion: Our case suggests that ALD should be considered in the differential diagnosis of unexplained paraparesis with associated hyperpigmentation in males. Moreover, timely diagnosis and medical intervention can prevent life-threatening adrenal crisis in such patients.

scholarly journals Adrenal crisis: prevention and management in adult patients

2019 ◽  
Vol 10 ◽  
pp. 204201881984821 ◽  
Author(s):  
Rosemary Dineen ◽  
Christopher J Thompson ◽  
Mark Sherlock
Keyword(s):  
Adrenal Insufficiency ◽  
Signs And Symptoms ◽  
Adrenal Crisis ◽  
Self Administration ◽  
Steroid Dependency ◽  
Delayed Treatment ◽  
Crisis Prevention ◽  
Clinician Education ◽  
Life Threatening ◽  
Life Threatening Event

Adrenal crisis is an acute life-threatening emergency contributing to the excess mortality that is reported in patients with adrenal insufficiency. The incidence of adrenal crisis is estimated to be 8 per 100 patient years in patients with adrenal insufficiency. Patients with adrenal crisis present systemically unwell with nonspecific signs and symptoms often leading to misdiagnosis and delayed treatment. An adrenal crisis may be the first presentation of adrenal insufficiency or can occur in patients who have been established on glucocorticoid replacement therapy. Infections are the major precipitating factor, but other causes include physical stress such as a surgical procedure or trauma, forgetting or discontinuing glucocorticoid therapy, pronounced physical activity, and psychological stress. The emergency treatment involves prompt recognition and administration of parenteral hydrocortisone, rehydration and management of electrolyte abnormalities. Prevention is centred around patient education. All patients should be educated on stress dosing and parenteral glucocorticoid administration. They should carry a steroid dependency alert card and wear a medical alert bracelet or similar identification. Despite many improvements in the management of patients with adrenal insufficiency, adrenal crisis continues to occur and represents a major source of morbidity, mortality and distress for patients. Improved patient and clinician education and measures to facilitate parenteral hydrocortisone self-administration in impending crisis are central to the management of this life-threatening event.

scholarly journals Perioperative glucocorticoid management based on current evidence

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.

scholarly journals MON-160 The Burdens of Adrenal Insufficiency: A Survey Study from Two Tertiary Care Centers in the United States

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Natalia Genere ◽  
Dingfeng Li ◽  
Majlinda Xhikola ◽  
Emma Behnken ◽  
Tiffany Abbondanza ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Burden Of Disease ◽  
General Health ◽  
Well Being ◽  
The United States ◽  
Comfort Level ◽  
Survey Study ◽  
Adrenal Crisis ◽  
Glucocorticoid Replacement Therapy

Abstract Background: Adrenal insufficiency (AI) is a chronic disorder necessitating life-long replacement. Patients’ quality of life and health outcomes depend on knowledge and comfort level with self-management. Objective: To determine patients’ knowledge in regards to diagnosis and management, estimate burden of disease and to identify predictors of adverse outcomes in patients with AI Methods: Survey study of patients with AI evaluated at two tertiary medical centers between 2015 and 2019. Collected variables included data on circumstances of AI diagnosis, symptoms, management, burden of disease, and overall well-being. Results: Among 785 patients (mean age at diagnosis 44.2 ± 18.0, 64% women, and 92% Caucasian), 310 (40%) had primary AI (PAI), 255 (33%) had secondary AI (SAI) not related to glucocorticoid use, and 211 (27%) had steroid-induced AI (SIAI). Patients were diagnosed with AI after presenting with symptoms for a median of 1 year (0-6), 28% with symptoms lasting &gt;2 years, 44% visiting emergency room (ER) at least once prior to diagnosis. A third of patients reported a discordant diagnosis from their medical record. Baseline glucocorticoid replacement therapy included hydrocortisone (HC) in 447 (59%), median of 20 mg (IQR 15 - 25mg), prednisone in 190 (25%), median of 5 mg (IQR 4 - 7.5mg), other regimens in 38 (5%), and no steroids in the remainder (85, 11%); 197 (26%) patients reported daily equivalent HC dose of &gt;25 mg. Overall, 549 (73%) of patients reported use of stress dose steroids at least once per year, higher in patients taking HC &gt;25 mg/day (3.2 vs 2.7 times per year if HC&lt;25mg/day, p=0.01). Improper use of stress steroids was reported in 193, 25% patients. Patients taking HC&gt;25 mg /day reported a higher number of adrenal crises (1.6 vs 1.3 in patients on HC&lt;25 mg/day, p=0.04). Among 314 (41%) patients who reported ER visits due to adrenal crisis, only a third received prompt glucocorticoids. One third of patients described their general health as fair or poor. Predictors of negative perceptions of overall health included SIAI (OR 6.2 and 2.5, vs PAI and SAI respectively), poor understanding of diagnosis (OR 2.6), daily HC&gt;25 mg (OR 2.1), and presence of at least one adrenal crisis (OR 2.3) (p&lt;0.001 for all). Conclusion: Patients with AI experience delay in diagnosis, and a third do not fully understand their diagnosis. In addition to patient education, interventions to improve general health and outcomes may include selecting a physiological glucocorticoid replacement therapy, prevention of adrenal crisis, and improving ER care.

Differences in the Way to Conceive Happiness Relate to Different Reactions to Negative Events

2018 ◽  
Vol 39 (1) ◽  
pp. 27-38 ◽  
Author(s):  
Giulia Fuochi ◽  
Chiara A. Veneziani ◽  
Alberto Voci
Keyword(s):  
Positive Emotions ◽  
Satisfaction With Life ◽  
Well Being ◽  
Protective Role ◽  
Stressful Events ◽  
Interactive Effects ◽  
Negative Events ◽  
Orientations To Happiness ◽  
Specific Reactions ◽  
The Way

Abstract. This paper aimed to assess whether differences in the way to conceive happiness, measured by the Orientations to Happiness measure, were associated with specific reactions to negative events. We hypothesized that among orientations to pleasure (portraying hedonism), to meaning (representing a eudaimonic approach to life), and to engagement (derived from the experience of flow), orientation to meaning would have displayed a stronger protective role against recent negative and potentially stressful events. After providing a validation of the Italian version of the Orientations to Happiness measure (Study 1), we performed regression analyses of the three orientations on positive and negative emotions linked to a self-relevant negative event (Study 2), and moderation analyses assessing the interactive effects of orientations to happiness and stressful events on well-being indicators (Study 3). Our findings supported the hypotheses. In Study 2, meaning was associated with positive emotions characterized by a lower activation (contentment and interest) compared to the positive emotions associated with pleasure (amusement, eagerness, and happiness). In Study 3, only meaning buffered the effect of recent potentially stressful events on satisfaction with life and positive affect. Results suggest that orientation to meaning might help individuals to better react to negative events.

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