scholarly journals Adrenal crisis: prevention and management in adult patients

2019 ◽  
Vol 10 ◽  
pp. 204201881984821 ◽  
Author(s):  
Rosemary Dineen ◽  
Christopher J Thompson ◽  
Mark Sherlock

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.

2010 ◽  
Vol 162 (3) ◽  
pp. 597-602 ◽  
Author(s):  
Stefanie Hahner ◽  
Melanie Loeffler ◽  
Benjamin Bleicken ◽  
Christiane Drechsler ◽  
Danijela Milovanovic ◽  
...  

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.


2015 ◽  
Vol 172 (3) ◽  
pp. R115-R124 ◽  
Author(s):  
Bruno Allolio

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.


Author(s):  
Sarpreet S Sekhon ◽  
Katelynn Crick ◽  
Tyler W Myroniuk ◽  
Kevin S C Hamming ◽  
Mahua Ghosh ◽  
...  

Abstract Introduction Adrenal insufficiency (AI) is an uncommon, life-threatening disorder requiring lifelong treatment with steroid therapy and special attention to prevent adrenal crisis. Little is known about the prevalence of AI in Canada or healthcare utilization rates by these patients. Objective We aimed to assess the prevalence and healthcare burden of AI in Alberta, Canada. Methods This study used a population-based, retrospective administrative health data approach to identify patients with a diagnosis of AI over a 5-year period and evaluated emergency and outpatient healthcare utilization rates, steroid dispense records and visit reasons. Results The period prevalence of AI was 839 per million adults. Patients made an average of 2.3 and 17.8 visits per year in the emergency department and outpatient settings, respectively. This was 3-4 times as frequent as the average Albertan and only 5% were coded as visits for AI. The majority of patients were dispensed glucocorticoid medications only. Conclusion The prevalence of AI in Alberta is higher than published data in other locations. The frequency of visits suggests a significant healthcare burden and emphasizes the need for a strong understanding of this condition across all clinical settings. Our most concerning finding is that 94.3% of visits were not labelled with AI, even though many of the top presenting complaints were consistent with adrenal crisis. Several data limitations were discovered that suggest improvements in the standardization of data submission and coding can expand the yield of future studies using this method.


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 ◽  
...  

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.


2021 ◽  
Vol 12 ◽  
Author(s):  
Chiara Sabbadin ◽  
Corrado Betterle ◽  
Carla Scaroni ◽  
Filippo Ceccato

Adrenal insufficiency (AI) is a life-threatening disorder, with increased morbidity and mortality, especially in case of an acute illness that can increase the requirement of cortisol. A novel infectious disease, termed Coronavirus Disease 2019 (COVID-19), appeared in 2020. Therefore, AI patients are experiencing a novel challenge: the risk of infection. In our experience, a prompt contact to the Endocrine center (with a telemedicine consultation) and a full awareness of diseases (cortisol deficiency, COVID-19 and the self-management of an adrenal crisis) are important to motivate patients. Vaccine is an effective treatment to prevent hospitalization and aggressive course of COVID-19. Some patients manifest challenges due to inequitable access and vaccine hesitancy, resulting in a delay in the acceptance of vaccines despite the availability of vaccination services. Therefore, an effort of all physicians must be conducted in order to advise patients with AI. In this short review, we try to answer some frequently asked questions regarding the management of patients with AI.


2013 ◽  
Vol 169 (6) ◽  
pp. R165-R175 ◽  
Author(s):  
Ashley Grossman ◽  
Gudmundur Johannsson ◽  
Marcus Quinkler ◽  
Pierre Zelissen

BackgroundConventional glucocorticoid (GC) replacement for patients with adrenal insufficiency (AI) is inadequate. Patients with AI continue to have increased mortality and morbidity and compromised quality of life despite treatment and monitoring.Objectivesi) To review current management of AI and the unmet medical need based on literature and treatment experience and ii) to offer practical advice for managing AI in specific clinical situations.MethodsThe review considers the most urgent questions endocrinologists face in managing AI and presents generalised patient cases with suggested strategies for treatment.ResultsOptimisation and individualisation of GC replacement remain a challenge because available therapies do not mimic physiological cortisol patterns. While increased mortality and morbidity appear related to inadequate GC replacement, there are no objective measures to guide dose selection and optimisation. Physicians must rely on experience to recognise the clinical signs, which are not unique to AI, of inadequate treatment. The increased demand for corticosteroids during periods of stress can result in a life-threatening adrenal crisis (AC) in a patient with AI. Education is paramount for patients and their caregivers to anticipate, recognise and provide proper early treatment to prevent or reduce the occurrence of ACs.ConclusionsThis review highlights and offers suggestions to address the challenges endocrinologists encounter in treating patients with AI. New preparations are being developed to better mimic normal physiological cortisol levels with convenient, once-daily dosing which may improve treatment outcomes.


Author(s):  
Leila Sozaeva ◽  
Nadezhda Makazan ◽  
Larisa Nikankina ◽  
Natalya Malysheva ◽  
Ekaterina Kuvaldina ◽  
...  

Primary adrenal insufficiency is manifested by a deficiency of adrenal cortex hormones and can lead to a life-threatening condition. Early diagnosis is key to patient survival. Auto-antibodies to one of the adrenal steroidogenesis enzymes, 21-hydroxylase, are an immunological marker of autoimmune adrenal insufficiency. On the one hand, the study of antibodies to 21-hydroxylase is a method that helps establish the etiology of the disease the autoimmune genesis of adrenal gland damage. On the other hand, the determination of autoantibodies to 21-hydroxylase is the only prognostic factor of the risk of adrenal insufficiency, which makes it possible to prevent the development of acute adrenal crisis. The article provides a brief literature review on autoantibodies to 21-hydroxylase and the pathogenesis of autoimmune adrenal insufficiency, and a series of clinical cases that illustrates the significant role of autoantibodies to 21-hydroxylase in diagnosis of adrenal insufficiency.


2019 ◽  
Vol 58 (4) ◽  
pp. 406-412
Author(s):  
Ladan Davallow Ghajar ◽  
Lauren K. Wood Heickman ◽  
Mark Conaway ◽  
Alan D. Rogol

Our objective was to assess the risk of adrenal insufficiency (AI) with short-term use of low- to moderate-potency topical corticosteroids (TCS) for treatment of atopic dermatitis. Our systematic literature search revealed 9 studies (n = 371) that evaluated AI using adrenocorticotropic hormone stimulation testing, with measures of serum cortisol levels at baseline and following at least 2 weeks of TCS application. Biochemical AI was defined by a stimulated cortisol level of ≤18.0 µg/dL (~500 nmol/L). The overall proportion of AI with low-to-moderate TCS use was 2.7% (95% confidence interval = 1.47% to 4.89%). None of the children showed any clinical evidence of AI or adrenal crisis. Short-term use of low- to moderate-potency TCS for the treatment of atopic dermatitis is associated with a low risk of adrenal suppression. General practitioners do not need to test these patients for adrenal suppression in the absence of concerning signs and symptoms of AI.


2021 ◽  
Vol 12 ◽  
Author(s):  
Nada Younes ◽  
Isabelle Bourdeau ◽  
Andre Lacroix

Primary adrenal insufficiency (PAI) is a rare disease and potentially fatal if unrecognized. It is characterized by destruction of the adrenal cortex, most frequently of autoimmune origin, resulting in glucocorticoid, mineralocorticoid, and adrenal androgen deficiencies. Initial signs and symptoms can be nonspecific, contributing to late diagnosis. Loss of zona glomerulosa function may precede zona fasciculata and reticularis deficiencies. Patients present with hallmark manifestations including fatigue, weight loss, abdominal pain, melanoderma, hypotension, salt craving, hyponatremia, hyperkalemia, or acute adrenal crisis. Diagnosis is established by unequivocally low morning serum cortisol/aldosterone and elevated ACTH and renin concentrations. A standard dose (250 µg) Cosyntropin stimulation test may be needed to confirm adrenal insufficiency (AI) in partial deficiencies. Glucocorticoid and mineralocorticoid substitution is the hallmark of treatment, alongside patient education regarding dose adjustments in periods of stress and prevention of acute adrenal crisis. Recent studies identified partial residual adrenocortical function in patients with AI and rare cases have recuperated normal hormonal function. Modulating therapies using rituximab or ACTH injections are in early stages of investigation hoping it could maintain glucocorticoid residual function and delay complete destruction of adrenal cortex.


2021 ◽  
Vol 31 (5) ◽  
pp. 96-99
Author(s):  
Martynas Judickas

Antinksčių krizė − tai ūmi gyvybei pavojinga būklė, le­mianti pacientų, kuriems patvirtintas antinksčių nepa­kankamumas, didesnį mirštamumą. Nepaisant didelės antinksčių nepakankamumo gydymo pažangos, antinks­čių krizė vis dar išlieka viena iš pagrindinių šios grupės pacientų mirties priežasčių, nes jai būdingas sisteminis organizmo pažeidimas, nesant specifinių simptomų, to­dėl sunku diagnozuoti ir neretai pavėluojama gydyti. Šiuo Straipsniuyje plačiau bus aptartasiamas antinksčių krizės aktualumas, ligai būdingąa klinika, gydymo bei prevencijos gairės. Tyrimo tikslas − apžvelgti ir aptarti retos, tačiau gyvy­bei itin pavojingos būklės – antinksčių krizės epidemi­ologijos, diagnostikos bei gydymo naujoves. Atlikta naujausių prevencinių programų bei gydymo gairių, ku­rios leistų sumažinti šios klinikinės būklės atvejų skaičių ateityje, paieška mokslinėje literatūroje. Literatūros šal­tinių paieška vykdyta anglų kalba PubMed, UpToDate, Medscape ir Cohrane medicinos duomenų bazėse. Nau­doti raktiniai žodžiai ir jų deriniai: adrenal crisis, adrenal insufficiency, hydrocortisone, treatment guidelines for adrenal crisis, prevention of adrenal crisis syndrome. Atrinkti 24 viso teksto moksliniai straipsniai, atitikę ty­rimo tikslą ir atlikta jų analizė. Tyrimo rezultatai parodė, kad pagrindinis šią patologiją išprovokuojantis veiksnys yra infekcija, tačiau antinksčių krizės priežastis gali būti operacija, trauma, gliukokortikoidų terapijos plano nesi­laikymas ar itin sunkus fizinis stresas. Pirmoji pagalba, nustačius antinksčių krizę, apima: parenterinį hidrokor­tizono skyrimą, rehidrataciją ir elektrolitų homeostazės atkūrimą. Pagrindinė prevencijos priemonė – pacientų edukacija. Išvados: 1) pirmo pasirinkimo vaistas gydyti antinksčių krizę yra hidrokortizonas; 2) pirmos kartos ir ilgo veikimo gliukokortikoidai prednizolonas ir dek­sametazonas nerekomenduojami, išskyrus atvejus, kai hidrokortizonu nepasiekiama efektyvaus gydymo; 3) laiku pradėtas adekvatus gretutinių ligų, tokių kaip vėžys, autoimuniniai susirgimai, antinksčių nepakankamumas bei infekcijos, gydymas gali sumažinti antinksčių krizės atvejų skaičių; 4) sergantieji Adisono liga, reguliariai naudojantys specialius klausimynus savo būklei įver­tinti, gali reikšmingai sumažinti antinksčių krizės riziką.


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