scholarly journals Acute Illness and Death in Children With Adrenal Insufficiency

2021 ◽  
Vol 12 ◽  
Author(s):  
Chris Worth ◽  
Avni Vyas ◽  
Indraneel Banerjee ◽  
Wei Lin ◽  
Julie Jones ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Parent Perceptions ◽  
Patient Characteristics ◽  
Adrenal Crisis ◽  
Children And Young People ◽  
Life Threatening ◽  
Training Programmes ◽  
Available Information ◽  
New Strategies ◽  
Missed Opportunity

BackgroundAdrenal Insufficiency (AI) can lead to life-threatening Adrenal Crisis (AC) and Adrenal Death (AD). Parents are trained to prevent, recognise and react to AC but there is little available information on what parents are actually doing at home to manage symptomatic AI.MethodsThree approaches were taken: (A) A retrospective analysis of patient characteristics in children and young people with AD over a 13-year period, (B) An interview-aided questionnaire to assess the circumstances around AC in children currently in our adrenal clinic, and (C) a separate study of parent perceptions of the administration of parenteral hydrocortisone.ResultsThirteen patients died (median age 10 years) over a thirteen-year period resulting in an estimated incidence of one AD per 300 patient years. Those with unspecified adrenal insufficiency were overrepresented (P = 0.004). Of the 127 patients contacted, thirty-eight (30%) were identified with hospital attendance with AC. Responses from twenty patients (median age 7.5 years) with AC reported nausea/vomiting (75%) and drowsiness (70%) as common symptoms preceding AC. All patients received an increase in oral hydrocortisone prior to admission but only two received intramuscular hydrocortisone. Questionnaires revealed that 79% of parents reported confidence in the administration of intramuscular hydrocortisone and only 20% identified a missed opportunity for injection.ConclusionsIn children experiencing AC, parents followed ‘sick day’ guidance for oral hydrocortisone, but rarely administered intramuscular hydrocortisone. This finding is discrepant from the 79% of parents who reported confidence in this task. Local training programmes for management of AC are comprehensive, but insufficient to prevent the most serious crises. New strategies to encourage use of parenteral hydrocortisone need to be devised.

scholarly journals Adrenal Insufficiency: Investigating Prevalence and Healthcare Utilization Using Administrative Data

2021 ◽  
Author(s):  
Sarpreet S Sekhon ◽  
Katelynn Crick ◽  
Tyler W Myroniuk ◽  
Kevin S C Hamming ◽  
Mahua Ghosh ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Healthcare Utilization ◽  
Population Based ◽  
Adrenal Crisis ◽  
Published Data ◽  
Clinical Settings ◽  
Healthcare Burden ◽  
Data Limitations ◽  
Life Threatening ◽  
Outpatient Settings

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.

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 Frequently Asked Questions in Patients With Adrenal Insufficiency in the Time of COVID-19

2021 ◽  
Vol 12 ◽  
Author(s):  
Chiara Sabbadin ◽  
Corrado Betterle ◽  
Carla Scaroni ◽  
Filippo Ceccato
Keyword(s):  
Adrenal Insufficiency ◽  
Short Review ◽  
Acute Illness ◽  
Self Management ◽  
Vaccine Hesitancy ◽  
Adrenal Crisis ◽  
Risk Of Infection ◽  
Telemedicine Consultation ◽  
Life Threatening ◽  
Cortisol Deficiency

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.

scholarly journals THERAPY OF ENDOCRINE DISEASE: Perspectives on the management of adrenal insufficiency: clinical insights from across Europe

2013 ◽  
Vol 169 (6) ◽  
pp. R165-R175 ◽  
Author(s):  
Ashley Grossman ◽  
Gudmundur Johannsson ◽  
Marcus Quinkler ◽  
Pierre Zelissen
Keyword(s):  
Adrenal Insufficiency ◽  
Clinical Signs ◽  
Adrenal Crisis ◽  
Dose Selection ◽  
Endocrine Disease ◽  
Once Daily ◽  
Mortality And Morbidity ◽  
Medical Need ◽  
Life Threatening

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.

Assessment of autoantibodies against 21-hydroxylase in the diagnosis of primary autoimmune adrenal insufficiency

2020 ◽  
Author(s):  
Leila Sozaeva ◽  
Nadezhda Makazan ◽  
Larisa Nikankina ◽  
Natalya Malysheva ◽  
Ekaterina Kuvaldina ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Patient Survival ◽  
Adrenal Crisis ◽  
Primary Adrenal Insufficiency ◽  
Adrenal Steroidogenesis ◽  
Threatening Condition ◽  
Life Threatening ◽  
The One

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.

Perioperative Adrenal Crisis

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.

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 Challenging Diagnosis of Addison’s Disease Presenting with Adrenal Crisis

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Ni Wayan Wina Dharmesti ◽  
Made Ratna Saraswati ◽  
Ketut Suastika ◽  
Wira Gotera ◽  
I Made Pande Dwipayana
Keyword(s):  
Adrenal Insufficiency ◽  
Adrenal Cortex ◽  
Addison’S Disease ◽  
Adrenal Crisis ◽  
Addison's Disease ◽  
Rapid Progression ◽  
Circulatory Collapse ◽  
Emergency Ward ◽  
Primary Adrenal Insufficiency ◽  
Life Threatening

Primary adrenal insufficiency, also known as Addison’s disease, is a rare but potentially fatal condition resulting from the failure of the adrenal cortex to produce glucocorticoid and/or mineralocorticoid hormones. Unfortunately, the clinical manifestation of primary adrenal insufficiency is not specific and often progresses insidiously, resulting in late diagnosis, or in severe cases, life-threatening circulatory collapse. Adrenal insufficiency should be considered in patients with unexplained vascular collapse. We report the case of a woman who presented to the emergency ward with unexplainable shock that was later diagnosed as adrenal crisis due to Addison’s disease. The presence of hyperpigmentation in patients with rapid progression of adrenal insufficiency suggests the diagnosis of Addison’s disease presenting with adrenal crisis.

scholarly journals Type II polyglandular autoimmune syndrome: a case of Addison’s disease precipitated by use of levothyroxine

2019 ◽  
Vol 12 (8) ◽  
pp. e230760 ◽  
Author(s):  
Katherine Hoener ◽  
Tulsi Sharma
Keyword(s):  
Adrenal Insufficiency ◽  
Autoimmune Disorder ◽  
Adrenal Crisis ◽  
Antibody Treatment ◽  
Primary Adrenal Insufficiency ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
Life Threatening ◽  
Endocrinology Clinic

A 46-year-old woman was referred to the endocrinology clinic for evaluation of progressive fatigue, dizziness and treatment-resistant hypothyroidism. Initial laboratory results revealed hypothyroidism, hyponatraemia and hyperkalaemia. Liothyronine sodium (Cytomel) was initiated, which exacerbated her fatigue and dizziness. Suspecting adrenal insufficiency, an 08:00 cortisol level was obtained and found to be low with failure to increase following cosyntropin stimulation test. Diagnosis of primary adrenal insufficiency was confirmed via CT abdomen and pelvis revealing diminutive adrenal glands and elevated 21-hydroxylase antibody. Treatment was initiated with hydrocortisone 10 mg every morning and 5 mg at 16:00/day, with plan for patient follow-up in 3 weeks to assess need for mineralocorticoid replacement. Polyglandular syndromes are rare and have a wide variety of presentation. Thus, we recommend screening patients with a single autoimmune disorder who do not respond to conventional therapy to prevent possible life-threatening adrenal crisis.

scholarly journals Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group

2017 ◽  
Vol 6 (8) ◽  
pp. 935-942 ◽  
Author(s):  
Lia Ferreira ◽  
João Silva ◽  
Susana Garrido ◽  
Carlos Bello ◽  
Diana Oliveira ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Adult Population ◽  
Serum Cortisol ◽  
High Plasma ◽  
Adrenal Crisis ◽  
Primary Adrenal Insufficiency ◽  
Electrolyte Disturbances ◽  
Threatening Condition ◽  
Life Threatening ◽  
Portuguese Study

Introduction Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI. Aims To characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI. Methods This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals. Results We investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 µg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment, 17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances. Conclusion This is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.

Sign in / Sign up

Export Citation Format

Share Document