The Impact of the COVID-19 Pandemic on Self-Reported Outcomes in Patients With Adrenal Insufficiency.

CONTEXT: The COVID-19 pandemic has impacted healthcare environment. OBJECTIVE: To determine the impact of the pandemic on self-reported outcomes in patients with adrenal insufficiency (AI). DESIGN AND SETTING: Prospective longitudinal survey study at 2 tertiary centers. PARTICIPANTS: Patients with AI. INTERVENTION: Patient-centered questionnaire. MAIN OUTCOME MEASURES: Depression Anxiety Stress Scales-21, Short Form-36, and AI self-management. RESULTS: Of 342 patients, 157 (46%) had primary AI, 109 (32%) had secondary AI, and 76 (22%) had glucocorticoid-induced AI. When compared to prepandemic, daily glucocorticoid dose and number of adrenal crises did not change. However, patients reported a higher financial impact from AI (34% vs 23%, P = 0.006) and difficulty accessing medical care (31% vs 7%, P < 0.0001) during the pandemic. A third of patients reported difficulty managing AI during the pandemic. After adjusting for duration and subtypes of AI, younger patients [odds ratio (OR) 2.3, CI 95% 1.3-4.1], women (OR 3.7, CI 95% 1.9-7.1), poor healthcare access(OR 4.2, CI 95% 2.3-7.7), lack of good insurance support (OR 2.8, CI 95% 1.3-5.9), and those with a higher financial impact (OR 2.3, CI 95% 1.3-4.3) reported greater difficulties managing AI. Patients were more likely to report a higher anxiety score (≥8) if they found managing AI challenging during the pandemic (OR 3.0, CI 95% 1.3-6.9), and had lower Physical Component Summary (OR 4.9, CI 95% 2.2-11.0) and Mental Component Summary (OR 4.1, CI 95% 1.8-9.5) scores prior to the pandemic. CONCLUSIONS: A third of patients with AI reported difficulties with management of AI during the pandemic, particularly in younger patients, women, and those with poor healthcare access.

Cardiometabolic and psychological effects of dual-release hydrocortisone: a cross-over study.

BACKGROUND: Adrenal insufficiency (AI) is associated with increased cardiovascular morbidity and mortality and reduced quality of life (QoL). Optimum glucocorticoid (GC) dosing and timing are crucial in the treatment of AI, yet the natural circadian secretion of cortisol is difficult to mimic. The once-daily dual-release hydrocortisone (DR-HC) preparation (Plenadren®), offers a more physiological cortisol profile and may address unmet needs. METHODS: An investigator-initiated, prospective, cross-over study in patients with AI. Following baseline assessment of cardiometabolic risk factors and QoL, patients switched from their usual hydrocortisone regimen to a once-daily dose equivalent of DR-HC and were reassessed after 12 weeks of treatment. RESULTS: Fifty-one patients (21 PAI/30 SAI) completed the study. Mean age was 41.6 years (s.d. 13), and 58% (n = 30) were male. The median daily HC dose before study entry was 20 mg (IQR 15-20 mg). After 3 months on DR-HC, the mean SBP decreased by 5.7 mmHg, P = 0.0019 and DBP decreased by 4.5 mmHg, P = 0.0011. There was also a significant reduction in mean body weight (-1.23 kg, P = 0.006) and BMI (-0.3 kg/m2, P = 0.003). In a sub-analysis, there was a greater reduction in SBP observed in patients with SAI when compared to PAI post-DR-HC. Patients reported significant improvements in QoL using three validated QoL questionnaires, with a greater improvement in PAI. CONCLUSION: Dual-release hydrocortisone decreases BP, weight and BMI compared with conventional HC treatment, even at physiological GC replacement doses. Additionally, DR-HC confers significant improvements in QoL compared to immediate-release HC, particularly in patients with PAI, which is also reflected in the patient preference for DR-HC.

Impact of COVID-19 pandemic on psychophysical stress in patients with adrenal insufficiency: the CORTI-COVID study.

PURPOSE: COVID-19 is a novel threat to patients with adrenal insufficiency (AI), whose life expectancy and quality (QoL) are impaired by an increased risk of infections and stress-triggered adrenal crises (AC). If infected, AI patients require prompt replacement tailoring. We assessed, in a cohort of AI patients: prevalence and clinical presentation of COVID-19; prevalence of AC and association with intercurrent COVID-19 or pandemic-related psychophysical stress; lockdown-induced emotional burden, and health-related QoL. METHODS: In this monocentric (Ancona University Hospital, Italy), cross-sectional study covering February-April 2020, 121 (40 primary, 81 secondary) AI patients (59 males, 55 ± 17 years) completed telematically three questionnaires: the purpose-built "CORTI-COVID", assessing medical history and concern for COVID-19-related global health, AI-specific personal health, occupational, economic, and social consequences; the AddiQoL-30; the Short-Form-36 (SF-36) Health Survey. RESULTS: COVID-19 occurred in one (0·8% prevalence) 48-year-old woman with primary AI, who promptly tailored her replacement. Dyspnea lasted three days, without requiring hospitalization. Secondary AI patients were not involved. No AC were experienced, but pandemic-related stress accounted for 6/14 glucocorticoid up-titrations. Mean CORTI-COVID was similar between groups, mainly depending on "personal health" in primary AI (ρ = 0.888, p < 0.0001) and "economy" in secondary AI (ρ = 0.854, p < 0.0001). Working restrictions increased occupational concern. CORTI-COVID correlated inversely with QoL. AddiQoL-30 and SF-36 correlated strongly. Comorbidities worsened patients' QoL. CONCLUSION: If educational efforts are made in preventing acute events, AI patients seem not particularly susceptible to COVID-19. The novel "CORTI-COVID" questionnaire reliably assesses the pandemic-related emotional burden in AI. Even under unconventional stress, educated AI patients preserve a good QoL.

Bilateral adrenalectomy in Cushing's disease: Altered long-term quality of life compared to other treatment options.

OBJECTIVE: Bilateral adrenalectomy (BADX) has become an important treatment of Cushing's disease (CD), especially when other treatment options have failed. The aim of this study was to evaluate the long-term quality of life (QoL) of patients having undergone BADX for CD, in comparison to other therapeutic options. METHODS: Thirty-four patients with CD were identified in two French centers: 17 underwent BADX and the remaining 17 one or more of the following treatments: surgery, medical therapy or radiotherapy. Three questionnaires were filled in by each patient in order to evaluate their QoL: Short Form-36 Health Survey (SF-36), Cushing QoL questionnaire and Beck depression inventory (BDI). RESULTS: The mean age of patients was 49.3±15.2 years. Average time lapse between diagnosis and BADX was 6.1 years. Results from each questionnaire adjusted to age showed a lower QoL among patients who underwent BADX. These were significant in most aspects of the SF-36 questionnaire (bodily pain P<0.01, general health P<0.01, vitality P≤0.05, social functioning P≤0.05), as well as in the Cushing QoL questionnaire (P<0.05) and BDI (P≤0.05). Adrenal insufficiency appeared to be the major predictor of poor QoL whatever their initial treatment. CONCLUSIONS: Despite their clinical remission, patients who undergo BADX appear to be at a greater risk of suffering an impaired QoL due to more prolonged period of time with imperfectly controlled hypercortisolism combined with definitive adrenal insufficiency.

Group 6. Modalities and frequency of monitoring of patients with adrenal insufficiency. Patient education.

Patients with adrenal insufficiency require regular, specialised monitoring in order to optimise their replacement therapy, to detect signs of under- and over-dosage, and to examine for possible associated disorders (auto-immune disorders in the case of auto-immune primary adrenal insufficiency either isolated or as part of auto-immune polyendocrinopathy syndrome type 1; illnesses with underlying monogenic causes). The transition period between adolescence and adulthood represents an added risk of a breakdown in monitoring which requires particular attention from medical teams and coordination between adult and pediatric medical teams. It is essential to encourage patient autonomy in the management of their illness, notably their participation in treatment education programs, in particular programs that target avoidance of, or early treatment of acute adrenal insufficiency. The principal educational objectives for patients in such programs are: to be in possession of, and carry the necessary tools for their treatment in an emergency; to be able to identify situations of increased risk and the early signs of adrenal crisis; to know how to adjust their oral glucocorticoid treatment; to be capable of administering hydrocortisone by subcutaneous injection; to be able to predict and therefore adjust treatment to different situations (heat, physical exercise, travel) and to be able to correctly use the appropriate resources of the healthcare services. Other programs could also be developed to respond to needs and expectations of patients, notably concerning the adjustment of hydrocortisone dosage to avoid overdose in the context of chronic fatigue syndrome.

Exploration of knowledge and understanding in patients with primary adrenal insufficiency: a mixed methods study.

BACKGROUND: Primary adrenal insufficiency (PAI) is a rare and severe condition requiring lifelong steroid replacement. During acute illness or stressful events, it is important to appropriately adjust glucocorticoid dose; failure to do so may lead to an adrenal crisis. The aim of the study was to explore patients PAI knowledge and understanding of the condition, steroid replacement adjustment during acute illness or stress and provided education. METHODS: Ten adult patients with PAI were purposefully recruited from two hospitals in a tertiary NHS Trust in England, UK. Data was collected using a mixed method approach utilising semi-structured audio-recorded interviews and hospital case note review. Interviews were transcribed verbatim and analysed using Burnard's content analysis framework. Information from the hospital case note review was captured using a matrix table based on pre-defined criteria. RESULTS: Four key themes emerged: 'Addison's disease and hydrocortisone replacement'; 'stress and corticosteroids'; 'patient compliance/adherence' and 'transition'. Patients reported feelings of 'going through a transition from uncertainty to adaption' following diagnosis. All participants had a good level of knowledge and understanding of required medication however application in times of need was poor. Medication adherence and prevention of a crisis relied not only on patient knowledge and application but also the support of family and health professionals. Health care professional knowledge required improvement to aid diagnosis and management of PAI. CONCLUSION: Patients with PAI did not apply existing knowledge to adjust steroid dose during acute illness or stress. Although a sample of limited size, our study identified there is a need to further explore why patients with Addison's disease do not apply existing knowledge during times of increased need. Future research should consider appropriate behaviour change interventions to promote medication adherence to reduce risk of an adrenal crisis.

Self-management in adrenal insufficiency - towards a better understanding.

Patients with adrenal insufficiency (AI) require life-long glucocorticoid (GC) replacement treatment and dose adjustment in stress situations to prevent life-threatening adrenal crises. Herein this study we evaluated the patients' healthcare situation and their knowledge on AI, comparing various aspects to a prior survey in 209 physicians. Using a questionnaire, we conducted a comprehensive survey among 33 AI patients who were treated at the endocrine outpatient clinics of two University Hospitals in Germany. The majority of AI patients (97%) named their treating physician as main source for information. Overall, 89.7% of interviewees were satisfied with their medical treatment; however, about 1/3 reported controversies with healthcare professionals regarding GC replacement in various situation. Two thirds of AI patients increased their substitution dose temporarily within the last 12 months. However, not all patients had an emergency ID, and only 64.5% an emergency kit. None of the interviewed patients identified the need for adjustment in all given situations correctly. Almost 80% of patients did not correctly identify all symptoms of GC over- and under-replacement. Interestingly, we found no significant differences between patients and physicians regarding specific aspects of GC replacement. We showed that: (i) AI patients have some knowledge gaps on modalities and adequacy of GC replacement therapy; (ii) long-term management of patients with AI remains a challenge requiring an experienced specialist; and (iii) further education of physicians as primary source of information is necessary. Additional education may help AI patients to empower them to adequate self-treatment.

Relative hypocortisolism is associated with obesity and the metabolic syndrome in recurrent affective disorders.

BACKGROUND: Cardiovascular disease (CVD) is one of the main causes of excess deaths in affective disorders. Affective disorders are associated with increased frequencies of CVD risk-factors such as obesity, dyslipidemia, and metabolic syndrome. Stress-induced chronic cortisol excess has been suggested to promote obesity and metabolic syndrome. Chronic stress with frequent or persisting hypothalamic-pituitary-adrenal-axis (HPA-axis) hyperactivity may, over time, lead to a state of low HPA-axis activity, also denoted hypocortisolism. A low-dose weight-adjusted dexamethasone-suppression-test (DST) is considered to be a sensitive measure of hypocortisolism. METHODS: 245 patients with recurrent depression or bipolar disorder and 258 controls participated in a low-dose DST and were also examined with regard to metabolic status. RESULTS: Patients with hypocortisolism (low post-DST cortisol) compared with patients without hypocortisolism (normal or high post-DST cortisol) exhibited increased odds ratios (OR) for obesity (OR=4.0), overweight (OR=4.0), large waist (OR=2.7), high LDL (OR=4.2), low HDL (OR=2.4), high LDL/HDL ratio (OR=3.3), high TC/HDL ratio (OR=3.4) and metabolic syndrome (OR=2.0). A similar pattern but less pronounced was also found in the control sample. LIMITATIONS: The cross sectional study design and absence of analyses addressing lifestyle factors. CONCLUSIONS: Our findings suggest that a substantial portion of the metabolic disorders and cardiovascular risk factors seen in recurrent affective disorders are found among individuals exhibiting hypocortisolism. This might indicate that long-term stress is a central contributor to metabolic abnormalities and CVD mortality in recurrent affective disorders.

Exploring the Impact of Short- and Long-Term Hydrocortisone Replacement on Cognitive Function, Quality of Life and Catecholamine Secretion: A Pilot Study.

Hydrocortisone (HC) substitution is essential in the treatment for patients with adrenal insufficiency (AI). Current replacement regimens however only incompletely mimic the physiological circadian rhythm of cortisol secretion, thereby resulting in subclinical temporary hypo- and hypercortisolism. Several studies point toward impairment of cognitive functions under these conditions, in part due to affected catecholamine secretion. Aim of this study was to evaluate the influence of long-term versus short-term HC replacement therapy on the adrenomedullary system and cognitive functions. Fourteen patients with primary or secondary AI were divided into two groups, depending on the duration of disease and HC replacement therapy (<15 years). All subjects underwent standardized neurocognitive testing; in addition, cortisol and catecholamine levels as well as physiological parameters and quality of life (QoL) were assessed. Patients with HC replacement therapy ≥15 years (n = 7) received significantly higher equivalent glucocorticoid doses than those with a shorter lasting therapy (n = 7; p = 0.048). Neuropsychological tests, QoL, physiological parameters, and cortisol levels did not differ significantly between both groups. Of note, norepinephrine levels were significantly lower in patients on short-term HC replacement therapy (p = 0.025). However, there were no significant differences in catecholamines with respect to the underlying pathophysiology, gender, or age. Irrespective of the duration of use, male patients scored significantly better for single aspects of QoL, whereas females performed significantly better in the attention test. Overall, we showed that duration of cortisol replacement therapy may have an impact on catecholamine release, but does not seem to affect cognitive functions and QoL.

Hydrocortisone Dose Influences Pain, Depressive Symptoms and Perceived Health in Adrenal Insufficiency: A Randomized Controlled Trial.

BACKGROUND: There is a major lack of randomized controlled trials (RCTs) evaluating the effects of hydrocortisone (HC) substitution therapy in patients with secondary adrenal insufficiency. Therefore, we evaluated the effects of two different replacement doses of HC on health-related quality of life (HRQoL) in a RCT. METHODS: This RCT with a double-blind cross-over design was performed at the University Medical Center Groningen. Forty-seven patients (29 men, age 51 ± 14 years, range 19-73 years) with secondary adrenal insufficiency participated. Patients received both a lower and a higher dose of HC (0.2-0.3 and 0.4-0.6 mg/kg body weight/day) for 10 weeks in random order. HRQoL was assessed with a daily mood and symptom checklist (Patient Health Questionnaire-15 [PHQ-15], Generalized Anxiety Disorder-7 [GAD-7], Patient Health Questionnaire-9 [PHQ-9]) and with questionnaires assessing general well-being (RAND 36-Item Health Survey [RAND-36]), mood (Hospital Anxiety and Depression Scale [HADS]) and fatigue (Multidimensional Fatigue Inventory-20 [MFI-20]). ClinicalTrials.gov identifier: NCT01546922. RESULTS: Patients receiving the higher dose of HC reported significantly fewer symptoms of depression (p = 0.016 and p = 0.045 for HADS and PHQ-9, respectively), less general and mental fatigue (p = 0.004 and p = 0.003, respectively, both MFI-20), increased motivation (p = 0.021, MFI-20), better physical functioning (p = 0.041), better general health (p = 0.013) and more vitality (p = 0.025) (all RAND-36). In addition, while on the higher dose, fewer somatic symptoms (p = 0.022) and less pain (p < 0.001) (both PHQ-15) were experienced. CONCLUSIONS: On the higher dose of HC, patients reported a better HRQoL on various domains as compared to the lower dose of HC. The fact that a higher dose of HC may improve patient well-being should be taken into consideration when individualizing the HC substitution dose.

Much to be desired in self-management of patients with adrenal insufficiency.

We assessed self-management in patients with adrenal insufficiency and patient-related factors that affect self-management. A self-report questionnaire was developed to assess self-management. The questionnaire contained three main topics, including: (i) medication adherence; (ii) anticipated measures; and (iii) dose adaptation during medical emergencies. Sixty per cent of the patients (n = 116) completed the questionnaire. The score for the medication adherence was 3.5 out of 4. The score for anticipated measures was 3.4 out of 5, and dose adaptation during medical emergencies was 1.9 out of 3. Older age was a positive predictor for all three self-management topics. The female sex was a positive predictor for anticipated measures and dose adaptation during medical emergencies. High education level was associated with higher scores on dose adaptation during medical emergencies in women, not in men. Education level did not affect other self-management aspects. There seems to be a need to improve self-management in these patients. Self-management might be improved by continuous education, and involvement of endocrine nurses and nurse practitioners is likely to be a key factor in the effectiveness of patient education.

Modified-release hydrocortisone decreases BMI and HbA1c in patients with primary and secondary adrenal insufficiency.

OBJECTIVE: Patients with adrenal insufficiency (AI) have impaired health-related quality of life (HRQoL), which is thought to be in part due to unphysiological glucocorticoid replacement therapy. The aim was to compare once-daily hydrocortisone (HC) dual-release tablet (modified-release) with conventional HC therapy regarding clinical data and HRQoL. DESIGN AND METHODS: We conducted an open, prospective trial at one endocrine center. There were 15 of 26 patients with primary AI, nine of 18 patients with secondary AI, and six congenital adrenal hyperplasia patients switched to modified-release HC therapy by their own decision. We evaluated clinical outcome and disease-specific HRQoL by using AddiQoL questionnaire at baseline and at follow-up (median 202 days (85-498)). RESULTS: Patients on modified-release HC (n=30) showed significant decreases in BMI (26.0±0.75-25.6±0.71, P for change=0.006) and HbA1c (6.04±0.29-5.86±0.28, P for change=0.005), whereas patients remaining on conventional HC (n=20) showed no change in these parameters (P for interaction=0.029 and 0.017 respectively). No significant change in AddiQoL score were found in the modified-release HC group (83.8 baseline and 84.9 at follow-up; P for change=0.629). In the conventional HC group, there was a significant decrease in scores (84.0 baseline and 80.9 at follow-up; P for change=0.016), with a between-treatment P for interaction of 0.066. The fatigue subscore of AddiQoL showed the same pattern with a significant decrease (P for change=0.024) in patients on conventional HC therapy (P for interaction=0.116). CONCLUSIONS: Modified-release HC decreases BMI and HbA1c compared with conventional HC treatment. In addition, it seems to stabilize HRQoL over time.

The effects of two different doses of hydrocortisone on cognition in patients with secondary adrenal insufficiency--results from a randomized controlled trial.

CONTEXT: A wide variety in hydrocortisone (HC) substitution dose-regimens are considered physiological for patients with secondary adrenal insufficiency (SAI). However, it is likely that cognition is negatively influenced by higher cortisol exposure to the brain. OBJECTIVE: To examine the effects of a high physiological HC dose in comparison to a low physiological HC dose on cognition. DESIGN AND SETTING: This study was a randomized double blind cross-over study at the University Medical Center Groningen. This study is registered with ClinicalTrials.gov, number NCT01546922. PATIENTS: Forty-seven patients (29 males, 18 females; mean [SD] age, 51 [14] years, range 19-73) with SAI participated. INTERVENTION(S): Patients randomly received first a low dose of HC (0.2-0.3 mg/kg body weight/day) during 10 weeks followed by a high dose (0.4-0.6 mg/kg body weight/day) for another 10 weeks, or vice versa. HC substitution was given in three divided doses with the highest dose in the morning. MAIN OUTCOME MEASURE(S): Cognitive performance (memory, attention, executive functioning and social cognition) of patients was measured at baseline and after each treatment period using a battery of 12 standardized cognitive tests. RESULTS: The higher dose of HC resulted in significantly higher systemic cortisol exposure for example measured at 1h after first dose ingestion (mean [SD], low dose: 653 [281] nmol/L; high dose: 930 [148] nmol/L; P<0.001). No differences in cognitive performance were found between the two dose regimens. CONCLUSIONS: No negative influence on memory, attention, executive functioning and social cognition was observed after 10 weeks of treatment with a higher physiological dose of HC in patients with SAI when compared to a lower dose.

Self-perceived health status of patients with adrenal insufficiency receiving glucocorticoid replacement therapy - French data from a worldwide patient survey.

An international survey was undertaken to investigate current practices in glucocorticoid replacement therapy and self-perceived health status of patients with adrenal insufficiency, using a 39-item questionnaire. Results were published in 2012. We analyzed data from French patients, extracted from the database. Participants were recruited via a patient advocacy group to respond anonymously to a questionnaire developed by clinical experts. Ninety-four patients participated (primary adrenal insufficiency 79% and secondary adrenal insufficiency 16%). They were treated mainly with hydrocortisone (97.5%). Dosing regimens were once daily (8%), twice daily (38%), thrice daily (30%) or other (24%). Nearly 80% of the participants considered their affection to have an impact on their physical activity, work, family or social life and 38% reported absence from work/school in the last 3 months. Fatigue in the morning or during the day was a problem for 57% and 69% of respondents respectively. Eighty percent were concerned about long-term side effects of therapy, mainly osteoporosis (79%), fatigue (57%) and obesity (43%). Despite obvious biases in the survey, we should be alerted by the high number of patients' complaints and the clear signs of quality of life impairment in this population, and think about strategies to improve their management.

Health-related quality of life in primary and secondary adrenal insufficiency.

Adrenal insufficiency (AI) is characterized by a deficient production of glucocorticoids with or without associated mineral corticoid and/or adrenal androgen deficiencies. Despite the low prevalence of AI, its impact on the affected patient is very high, and can be life-threatening disease if not adequately treated. Several glucocorticoid treatment regimens are available, but none is capable of perfectly imitating the cortisol circadian rhythm. Cortisol rhythmicity and treatment of other possible concomitant conditions often associated (e.g., autoimmune disorders and panhypopituitarism) are essential to improve outcome of AI. Morbidity often present in treated AI include an unhealthy metabolic profile, bad quality of sleep, infertility, sexual dysfunction and worse health-related quality of life. This review focuses on psychological morbidity and impaired quality of life in patients with primary or secondary AI of any origin, including a special section devoted to congenital adrenal hyperplasia.

[Treatment of adrenal deficiency: To what issues? By which therapeutic means?]. / Traiter l'insuffisance surrénale : pour quels enjeux ? Par quels moyens thérapeutiques ?

Quality of life remains altered in the adrenal insufficient patient treated by hydrocortisone and fludrocortisone. Due to its pharmacokinetics properties, hydrocortisone requires 2 to 3 daily doses - including a morning intake of 2/3 or half of the daily dose respectively - while fludrocortisone requires a once morning daily dose. Titration of hydrocortisone and fludrocortisone relies on the clinical symptoms of over- or under-replacement together with biochemical indexes such as plasma sodium, potassium and renin. DHEA replacement may improve quality of life and mood and may increase lean body mass and bone mineral density, especially in women with adrenal insufficiency. The new slow release hydrocortisone formulations may help reproduce a more physiological cortisol plasma profile and thus reduce the impact of over-replacement on cardiovascular risk factors and glucose metabolism. Acute adrenal insufficiency is a life-threatening complication of glucocorticoid and mineralocorticoid under-replacement, which may be prevented by educational programs towards the adrenal insufficient patient.

Acute mental status change as the presenting feature of adrenal insufficiency in a patient with autoimmune polyglandular syndrome type II and stroke.

Primary adrenal insufficiency (AI) in children usually presents with non-specific symptoms such as fatigue, nausea, vomiting, and anorexia. Here, we report an unusual case of a 15 year old girl who presented with acute mental status change and was ultimately diagnosed with AI due to autoimmune polyglandular syndrome type II (APS2). Central nervous system imaging revealed a cerebral infarction. To our knowledge, the constellation of APS2, stroke and acute mental status change has not been previously reported. We review the literature with regard to the presentation of AI as well as the association between vasculitis and APS2.

Early intervention and management of adrenal insufficiency in children.

The endocrine disorder adrenal insufficiency includes inadequate production of the steroid hormone cortisol. This results in poor physiological responses to illness, trauma or other stressors and risk of adrenal crisis. Management is based on administration of hydrocortisone. It is important to avoid under- or over-treatment and increase the dosage during times of physiological stress. To reduce morbidity, hospital admissions and mortality, the education and empowerment of parents and carers, and prompt intervention when necessary are essential. A steroid therapy card for adrenal insufficiency containing personal information on a patient's condition was developed for use by families and their specialist centres.