Risk Of Adrenal Crisis Research Articles

8205 Within day Symptoms Of Adrenal Insufficiency Predict Reduced Quality Of Life And Adrenal Crises In 712 MyAI Registry Participants

Abstract Disclosure: J.G. Kallet: None. W. Hover: None. A. Krein: None. M. Lowe: None. M.F. Slovick: None. G. Kinney: None. S. Majka: None. K. Warren: None. S. Watts: None. M.T. McDermott: None. E.A. Regan: None. Multiple studies have shown reduced quality of life in people with adrenal insufficiency based on 1- 4 week recall of symptoms. However, “within-day” symptoms are commonly reported to treating physicians, and there is no clear understanding of what these symptoms represent for quality of life or adequate disease management. Lack of adequate cortisol has been postulated as a factor for both persistent symptoms and reduced quality of life but dose adjustment is not typically prescribed. Within-day symptoms are similar to those reported prior to treatment, and include lack of energy, nausea, vomiting, anorexia, headache, dizziness, shakiness, muscle cramps, and cognitive impairments. Addressing the relationship between “within-day” symptoms and replacement dosing could improve disease management. Further, understanding the association of within-day symptoms to outcomes (quality of life and adrenal crises) provides an impetus to change the standard of care. Individuals enrolled in the MyAI registry provided information about “within-day” symptoms. These included energy to accomplish tasks, cognitive symptoms, and overall symptoms. The questions were scored with increased values representing greater and more frequent symptoms and were summed to create a severity score. Patients also reported adrenal crisis within the past year and completed the AddiQoL as quality of life over the past 4 weeks.Responses were obtained from 712 adult registry participants - 84% female, age range 23-89, mean 51.2 (15.2) years - and 47% reported an adrenal crisis within the past year. Within-day symptom score range was 3-15 points with mean 7.9 and SD of 2.96. Higher “within-day” symptom scores were associated with stepwise reductions in the AddiQoL (worse quality of life). Individuals who reported an adrenal crisis within the past year had significantly increased symptom scores [8.8 (2.9) vs 6.7 (2.5), p<0.0001]. Regression model for the AddiQoL total score (R2 = 0.8) found after adjusting for group, age and sex, that BMI (p = 0.0002) and symptom score (p<0.0001) were highly significant predictors of worse AddiQoL score. ConclusionAdrenal crisis is reported frequently by patients who are being treated for adrenal insufficiency. We found a strong association of “within-day” symptoms to both risk of adrenal crisis and reduced quality of life. Addressing persistent disease-related symptoms in adrenal insufficiency would be an appropriate disease management response to improve care. The initial choice of replacement dosing based on body surface area or other metric may be a starting point for care but should be informed by studies that show large variation in absorption and metabolism between individuals. Adjusting doses of replacement hormones and careful management of adrenal insufficiency symptoms could improve outcomes. Presentation: 6/1/2024

7658 A Case of Recurrent Cardiac Tamponade as Initial Presentation of Primary Adrenal Insufficiency

Abstract Disclosure: A. Farooq: None. A. Ghumatkar: None. J. Snitzer: None. Introduction:Cardiac tamponade, a life-threatening emergency, can be associated with primary adrenal insufficiency (Addison Disease), and accurate diagnosis requires a high degree of clinical suspicion in patients with uncertain etiology and nonspecific signs and symptoms. Case Presentation:A 58-year-old female with a history of Hashimoto thyroiditis was evaluated in the hospital for two episodes of cardiac tamponade requiring emergent pericardiocentesis within one month. The patient had been experiencing symptoms of fatigue, nausea, lightheadedness, dizziness, hiccups, and tanned skin for the past year. She also had a recent flu-like illness with nausea and vomiting four months before her current presentation. Her vitals were notable for hypotension during these episodes. Her lab work showed hyponatremia (128 mEq/L, reference range: 136-145 mEq/L) with normal potassium and glucose levels. Her thyroid function tests were within normal limits on her home levothyroxine dosage (TSH 4.588 IU/ml, reference range: 0.5-8.9 IU/ml). An ACTH stimulation test was performed based on the patient's clinical presentation, consistent with adrenal insufficiency. Her baseline cortisol level was 5.9 mcg/dL (reference range: 6.7-22 mcg/dL), a post-ACTH stimulation level of 6.7 mcg/dL after 30 minutes, and 6.0 mcg/dL after 60 minutes. Her 21 alpha-hydroxylase antibody titer was also positive for Addison Disease (231U/mL, reference range <1 IU/mL). A CT scan of the abdomen with intravenous contrast was done, which revealed no abnormality of the adrenal glands. She was treated with stress doses of hydrocortisone during hospitalization and discharged with maintenance dosage and follow-up with endocrinology. She had another episode of acute adrenal crisis about a month later but had no pericardial effusion this time and responded well to the treatment with stress doses of steroids. She has remained healthy at 14 years of follow-up. Conclusion: This case emphasizes the need to consider adrenal insufficiency in the evaluation of cardiac tamponade or pericardial effusions, especially in patients with existing autoimmune glandular disorders. This patient may have autoimmune polyglandular syndrome type 2 (APS-2), which is a cluster of two or more endocrine glandular disorders primarily diagnosed in adulthood and predominant in females. The risk of adrenal crisis is more than twofold in Addison disease due to APS-2. Lastly, this case highlights that hiccups can be related to adrenal insufficiency, with pathophysiology possibly involving diaphragmatic irritation due to adrenal inflammation that resolves with treatment. Presentation: 6/3/2024

7203 A case of undifferentiated shock found to be due to voriconazole-related primary adrenal insufficiency

Abstract Disclosure: K.X. Zhang: None. C. Bergwitz: None. Primary adrenal insufficiency can be a rare effect observed with azole antifungal therapy, classically ketoconazole, via inhibition of cortisol and aldosterone synthesis. Whether primary AI can be observed with newer triazole drugs used for neutropenic prophylaxis and treating invasive fungal infections is far less understood. We describe a case of a 65-year-old man with medical history notable for COPD and gout, who was on voriconazole for Aspergillus pneumonia, and admitted to the medical ICU with confusion and undifferentiated shock. His potassium was 6.5 mmol/L, sodium 133 mmol/L, bicarbonate 18 mmol/L, and blood pressure 72/63 requiring pressor support. Voriconazole was withdrawn. He also had a fever to 101.7F that was attributed to a gout flare, and he received a course of high dose prednisone. His shock and electrolyte abnormalities quickly resolved, and he was discharged on isavuconazole. At the 1 week mark, he was found to have a morning cortisol 2.3 ug/dL, potassium 5.4 mmol/L, and sodium 134 mmol/L, along with fatigue, nausea, and hypotension, which prompted resumption of steroid and mineralocorticoid replacement. One month after completing his course of isavuconazole, morning cortisol normalized to 11.0 ug/dL and ACTH 22.3 pg/mL. We present a convincing clinical story supporting the etiology of this patient’s initial shock as primary adrenal insufficiency related to a voriconazole effect. This case illustrates how iatrogenic AI can be a missed diagnosis in the emergency room setting. Primary AI has classically been reported following ketoconazole therapy, but there are only 4 reports of primary AI involving the newer triazole posaconazole, and none for voriconazole or isavuconazole. Due to the rarity of this effect, AI due to voriconazole was not on the initial shock differential and timely endocrine labs to seal the diagnosis (morning cortisol, ACTH, and renin/aldosterone levels) were not obtained. If our patient had not received steroids serendipitously for gout, he may have been at risk for adrenal crisis. Whereas central AI is more common and induced by chronic use of systemic steroids, it is important to also think about AI caused by antifungal therapy with newer azoles. Presentation: 6/3/2024

12573 Management Challenges In A Young Male With Bilateral Macronodular Adrenal Hyperplasia

Abstract Disclosure: C. Nguyen: None. V. Ghernautan: None. L.Y. Melendez-Ramirez: None. M.C. Chiang: None. D. Lovre: None. Bilateral adrenal hyperplasia (BAH) may present in isolation or as part of a syndrome. It is classified according to nodule size as Macronodular: primary bilateral macronodular adrenal hyperplasia (PBMAH), or Micronodular: primary pigmented micronodular adrenal hyperplasia (PPNAD) and isolated micronodular adrenal hyperplasia (iMAD). High variability in cortisol secretion and disease progression is seen in both forms subclinical and overt Cushing’s Syndrome (CS). In PBMAH, bilateral adrenalectomy is advised for remission of CS, but benefits must be weighed against risks of lifelong adrenal insufficiency. A 45-year-old male with hypertension, depression, prediabetes, obesity presented to clinic for second opinion regarding incidentally discovered adrenal nodules five years prior with negative initial hormonal workup. Over the previous year he had gained 20 lbs. and developed proximal muscle weakness and sleep apnea. Physical exam showed uncontrolled hypertension and increased abdominal and supraclavicular fat. Hormonal testing revealed hypercortisolism. Adrenal CT confirmed bilateral adrenal nodules up to 4.9 cm with absolute washout ≥60% consistent with benign etiology. CS secondary to PBMAH was diagnosed. Prior to surgical evaluation. Ketoconazole 200 mg twice daily was started and titrated to 400 mg twice daily over the next three months. ACTH became detectable and symptoms of hypercortisolism improved. Right adrenalectomy was performed. Surgical pathology showed golden yellow-orange nodules with minimal grey adrenal parenchyma without necrosis or hemorrhage. The gland was 9.5 x 5.8 x 2.6 cm and weighed 85 g. Microscopic examination showed macronodular hyperplasia. Following surgery, he experienced a 17-pounds weight loss, improvement in blood pressure and quality of life. Three months later, hypercortisolism recurred. He elected to be monitored clinically and contemplating option of left adrenalectomy.Our case highlights the challenges in the care of patients with PBMAH. Bilateral adrenalectomy (BA) is the classical treatment. Its disadvantages include life-long hormone replacement with risk for adrenal crisis, increased morbidity, and mortality. Surgical alternatives, such as unilateral adrenalectomy (UA) and UA with subtotal contralateral adrenal resection, have shown promising results with up to 90% remission rates. The role of adjuvant novel medical therapy is unknown. Studies to compare cost, quality of life, cardiovascular risk, comorbidities, and life span between BA and UA are lacking. New biomarkers are needed to allow closer monitoring of disease progression. Additional research is necessary on medical management of hypercortisolism recurrence after UA and its long-term safety. Finally, given the presence of germline defects associated with adrenal hyperplasia, genetic testing should be offered to all patients with BAH. Presentation: 6/1/2024

Course of COVID-19 infection in patients with congenital adrenal hyperplasia.

Patients with primary adrenal insufficiency due to congenital adrenal hyperplasia (CAH) are at risk for adrenal crisis during infectious illnesses. Increased risk of infection including COVID-19 has been variably reported. To evaluate COVID-19 illness outcomes and stress dose practices in a large cohort of patients with CAH during the first two years of the pandemic and compare observations of COVID-19 infection in patients with CAH to the general USA population. Between March 2020 and November 2022, patients with CAH followed at the National Institutes of Health Clinical Center were queried about COVID-19 infection during their routine visits. Cases of COVID-19 were compared to controls. COVID-19 infection rates and symptoms were compared to general USA population data from the Centers for Disease Control and Prevention. Of 168 patient visits, there were 54 (32%) cases of COVID-19 infection, and 15 (28%) were pediatric. Overall an association was found between acquiring COVID-19 and obesity (p=0.018), and adults acquiring COVID-19 were on lower doses of fludrocortisone (p=0.008). Fewer cases of COVID-19 infection were reported in those receiving hydrocortisone or modified-release hydrocortisone compared to longer acting glucocorticoids (p=0.0018). In our CAH population, the pattern of COVID-19 infection rates and COVID-related symptomatology were similar to those observed in the general USA population. Most patients with the presumed alpha variant reported anosmia and ageusia, while gastrointestinal symptoms were commonly reported during the delta and omicron waves. Stress dosing occurred in 30/54 cases, and 7 received parenteral hydrocortisone. Two hospitalizations occurred; one pediatric and one adult, both with co-morbidities. There were 5 emergency room visits and no reported deaths. Patients with CAH with close follow-up do not appear to be at increased risk of acquiring COVID-19 or to have a more severe course of COVID-19 compared to the general USA population. Obesity may increase risk of acquiring COVID-19 in patients with CAH, and overall infection risk may be lower in those receiving short-acting and circadian glucocorticoid replacement therapy. Established age-appropriate guidelines for stress dosing during infectious illnesses should be used for patients with CAH and COVID-19. COVID-19 specific guidelines are not indicated. Clinical Trial Registration: ClinicalTrials.gov, identifier NCT00250159.

SAT301 Patient-centered Outcomes Measures To Assess Quality Of Life In Adrenal Insufficiency Treatment

Abstract Disclosure: W.J. Hover: None. K.E. Lowe: None. M.E. Lowe: None. J.G. Kallet: None. M.F. Slovick: None. S.L. Majka: None. M.T. McDermott: None. E.A. Regan: None. Individuals with adrenal insufficiency (AI) have been shown to have reduced quality of life in spite of hormone replacement. Although late-stage insufficiency and adrenal crisis may show signs including hypotension and electrolyte abnormalities, patient-reported symptoms may predict outcomes in AI and can be a key factor in understanding poor quality of life in adrenal insufficiency. The SF-36, a generic quality of life (QoL) survey has been reported in adrenal insufficiency but may lack disease-specific domains to define QoL deficits in AI. An AI disease-specific QoL survey, the AddiQoL has also been validated. We evaluated the AddiQol and SF-36 for distinguishing the health status of AI patients in terms of diagnosis, medication use and risk of crisis using a patient registry. Methods: We identified 344 individuals from the MyAI patient registry who had provided responses to both the AddiQoL and SF-36. We compared the AddiQoL overall score with three domains of the SF-36: physical function, role physical, vitality, and the physical and mental component scores (PCS and MCS). Results: Respondents were from all 50 US states with 83% female and mean (SD) age of 50 (15) years. AI diagnosis was Primary 63%, Central 32% and congenital adrenal hyperplasia 5% based on self-report and confirmation with medical records. Both the SF-36 and AddiQoL identified reduced QoL in the AI participants and were significantly correlated: PCS to AddiQoL 0.73 and MCS to AddiQoL 0.63. Role Physical, Vitality and General Health were the most reduced domains with mean values of 38.4 (43.5), 35.6 (24.5) and 40.8 (24.7) respectively. Primary AI showed significantly better QoL than Central AI by both SF-36 and AddiQol [80.3(15.1) vs 69.9 (14.2), p<0.001]. Respondents who reported an adrenal crisis within the past year had significantly lower AddiQol [72.8(15.4) vs 82.3 (15.4), p<0.001] and SF-36 scores. Respondents who were currently treated with hydrocortisone (HC) had better QoL than those on Prednisone [HC 78.1 (15.2), Prednisone 74.1 (15.9), p=0.02 adjusted for diagnosis group]. Conclusions: In a geographically broad cohort of US AI patients, we confirm significant reductions in QoL in spite of treatment. Both the SF-36 and AddiQoL were able to distinguish differences in QoL by diagnosis, risk of adrenal crisis and medication. Further work to define specific patterns of high-risk symptoms may improve early diagnosis of impending crisis. Presentation: Saturday, June 17, 2023

S2793 Adrenal Crisis in a Patient With Crohn’s Disease: A Legacy of the Corticosteroid Treatment Era

Introduction: Before the era of biologic agents, corticosteroids were the most effective therapy available for inflammatory bowel disease (IBD) as well as other inflammatory and immune-mediated disorders, and treatment often was prolonged. As a result, many patients developed secondary adrenal insufficiency, in which the normal circadian rhythm of cortisol secretion was maintained but a defect in the cortisol response to stress persisted, as was the risk of adrenal crisis in response to severe physiologic stress. As corticosteroids are no longer used chronically in IBD, adrenal crisis has become a rare complication, and many gastroenterologists have no personal experience with it. We recently saw such a case which we are presenting as a reminder of its continued existence. Case Description/Methods: A 73-year-old female with Crohn’s disease diagnosed at the age of 18, complicated by short bowel syndrome and esophageal involvement, who had been steroid dependent for 15 years though stable on ustekinumab and no steroids for more than 8 years, presented to the emergency department with an acute onset of abdominal pain, diarrhea with incontinence, profound weakness, disorientation, and possible syncope, one hour after IV iron sucrose therapy. Initial physical exam was notable for pallor and edema of hands and feet. Laboratory exams showed hemoconcentration, leukocytosis, hyponatremia, plus elevated serum lactate concentration and anion gap. Because of the severity of this response, the possibility of adrenal crisis was raised: serum cortisol was 15.5 ug/dL (nl 6.2-29) despite the stressful situation and the patient responded clinically to 100 mg of hydrocortisone IV. She was discharged within 24 hours and all laboratory abnormalities reversed within 72 hours. Discussion: Adrenal crisis can present with a wide variety of nonspecific symptoms and should be in the differential diagnosis when a patient presents after a significant physiologic stress and has a history of chronic oral corticosteroid use. Electrolyte disturbances are less common in secondary adrenal insufficiency as the zona glomerulosa of the adrenal gland is not affected. Secondary adrenal insufficiency may persist for many years after chronic steroid use and may be permanent. In patients with secondary adrenal insufficiency. Important hints are the patient’s age and GI history, which includes extent of exposure to corticosteroids. Effective diagnosis requires maintaining a high index of suspicion.

Diagnosis and Management of Adrenal Insufficiency and Adrenal Crisis in the Emergency Department

BackgroundAdrenal insufficiency can result in significant patient morbidity and mortality, but due to the range of symptoms and variable clinical course and etiologies, it can be a challenging condition to diagnose and manage. ObjectiveThis narrative review will discuss the evaluation of an adult patient at risk for a new diagnosis of adrenal insufficiency and the management of a patient with known or suspected adrenal insufficiency. DiscussionA new presentation of adrenal insufficiency can range from nonspecific, minor symptoms including fatigue, to a life-threatening adrenal crisis with hemodynamic instability. Due to the variety of signs and symptoms, the diagnosis is often missed. Those with known adrenal insufficiency are at risk for adrenal crisis, which may occur due to a variety of triggers. Initial evaluation includes assessment for the underlying etiology or concomitant condition, laboratory analysis, and imaging, when clinically indicated. Although not necessary for evaluation in the emergency department setting, the diagnosis is confirmed by specific testing such as the cosyntropin stimulation test. The mainstay of treatment in adrenal crisis is hydrocortisone, intravenous fluid, glucose repletion, and treatment of the underlying acute trigger. ConclusionsEmergency clinicians must be prepared to recognize, evaluate, and manage those with known or suspected adrenal insufficiency or adrenal crisis.

The effects of selected inhibitors on human fetal adrenal steroidogenesis differs under basal and ACTH-stimulated conditions

BackgroundDisordered fetal adrenal steroidogenesis can cause marked clinical effects including virilization of female fetuses. In postnatal life, adrenal disorders can be life-threatening due to the risk of adrenal crisis and must be carefully managed. However, testing explicit adrenal steroidogenic inhibitory effects of therapeutic drugs is challenging due to species-specific characteristics, and particularly the impact of adrenocorticotropic hormone (ACTH) stimulation on drugs targeting steroidogenesis has not previously been examined in human adrenal tissue. Therefore, this study aimed to examine the effects of selected steroidogenic inhibitors on human fetal adrenal (HFA) steroid hormone production under basal and ACTH-stimulated conditions.MethodsThis study used an established HFA ex vivo culture model to examine treatment effects in 78 adrenals from 50 human fetuses (gestational weeks 8–12). Inhibitors were selected to affect enzymes critical for different steps in classic adrenal steroidogenic pathways, including CYP17A1 (Abiraterone acetate), CYP11B1/2 (Osilodrostat), and a suggested CYP21A2 inhibitor (Efavirenz). Treatment effects were examined under basal and ACTH-stimulated conditions in tissue from the same fetus and determined by quantifying the secretion of adrenal steroids in the culture media using liquid chromatography-tandem mass spectrometry. Statistical analysis was performed on ln-transformed data using one-way ANOVA for repeated measures followed by Tukey’s multiple comparisons test.ResultsTreatment with Abiraterone acetate and Osilodrostat resulted in potent inhibition of CYP17A1 and CYP11B1/2, respectively, while treatment with Efavirenz reduced testosterone secretion under basal conditions. ACTH-stimulation affected the inhibitory effects of all investigated drugs. Thus, treatment effects of Abiraterone acetate were more pronounced under stimulated conditions, while Efavirenz treatment caused a non-specific inhibition on steroidogenesis. ACTH-stimulation prevented the Osilodrostat-mediated CYP11B1 inhibition observed under basal conditions.ConclusionsOur results show that the effects of steroidogenic inhibitors differ under basal and ACTH-stimulated conditions in the HFA ex vivo culture model. This could suggest that in vivo effects of therapeutic drugs targeting steroidogenesis may vary in conditions where patients have suppressed or high ACTH levels, respectively. This study further demonstrates that ex vivo cultured HFAs can be used to evaluate steroidogenic inhibitors and thereby provide novel information about the local effects of existing and emerging drugs that targets steroidogenesis.

Peri-operative steroid management in the paediatric population.

Patients with adrenal insufficiency are at risk of adrenal crisis, a potentially life-threatening emergency in the peri-operative period due to their attenuated ability to mount a cortisol response. There is a lack of standardization regarding peri-operative stress-dose glucocorticoids in paediatric clinical practice with the absence of agreed protocols. For the individual patient, the risk of adrenal crisis must be weighed against the potential adverse clinical outcomes associated with unnecessary or supra-physiologic glucocorticoid dosing in susceptible patients. Specific clinical concerns in the paediatric population include osteopenia, growth restriction and increased risk of cardiovascular disease in adulthood. This review aimed to identify and evaluate available literature in the field of peri-operative stress-dose glucocorticoids. A comprehensive literature search was conducted to construct a narrative review. The outcome of this review identified that paediatric patients, unlike adults, do not show a graded response to surgical stress with implications for glucocorticoid stress dose regimens for general anaesthesia and less invasive surgical procedures. The studies highlight a lack of information on physiological steroid responses to stress situations and differences in the approach to glucocorticoid replacement strategies in the paediatric population. The review identified there is a lack of high-quality paediatric-specific studies evaluating appropriate stress-dose glucocorticoid regimens in paediatric patients with or at risk of adrenal insufficiency. Further research is needed to establish clear evidence-based clinical guidelines for paediatric peri-operative practice regarding steroid stress dosing in adrenal insufficiency. Current knowledge would suggest that a balanced view of risks and benefits should be taken appropriate to the clinical context, to dictate peri-operative stress-dose glucocorticoids use that permits safe perioperative management.

SUN-369 Challenges in Diagnosing and Managing PPNAD in a Patient with Carney Complex

Background Carney complex (CNC) is a rare disorder associated with endocrine, cardiac, cutaneous and neural tumors. Primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine manifestation in patients with CNC and can be challenging to diagnose and manage. Case A 35 yo female with CNC diagnosed at the age of 3 yrs presented with a 20 lb. weight gain over last 1 year associated with easy bruising, anxiety, dorsocervical and supraclavicular fat pad. She had no other comorbidities and had normal bone density. Her morning cortisol after 8 mg dexamethasone was not suppressed (cortisol 14.9 mcg/dl, dexamethasone 1920 ng/dl); however, urine cortisol values were high normal to only mildly elevated (42.7, 48.4; n-3.5-45 mcg/24hr). Loss of circadian rhythm was noted on comparing midnight and morning cortisol level (8.4, 8.3 mcg/dl respectively), ACTH was suppressed (<5 pg/ml) and DHEAS was low (0.26; n-0.35-4.30 mcg/ml). Her Liddle’s test was not consistent with PPNAD. Adrenal CT was suggestive of possible focal enlargement of medial limb of left adrenal. She was offered bilateral adrenalectomy, but has deferred it at this time. Discussion CNC was first described by Dr. Carney in 1985 as ‘the complex of myxomas, spotting pigmentation and endocrine over-reactivity’. It is a rare disorder with 750 known cases. It has autosomal dominant inheritance with almost 100% penetrance. PPNAD is seen clinically in 25-60% of patients with CNC, however, it has been found in almost every individual with CNC who underwent autopsy, suggesting significant variability in presentation. Symptoms may evolve over a span of many years, making it difficult to determine the appropriate timing of treatment. Diagnosing PPNAD can be challenging as cortisol production can be cyclical, periodic or subclinical. The paradoxical rise in glucocorticoids in response to dexamethasone (Liddle’s test) is reported in 69% of patients but is not pathognomonic. Radiological diagnosis is also challenging as most nodules are less than 4 mm in size, so the adrenals may appear normal on CT scan. Recommended treatment for PPNAD is bilateral adrenalectomy, but this introduces a lifelong burden of steroid replacement and risk of adrenal crisis. Recently, Xu et al performed unilateral adrenalectomy in patients with PPNAD with remission of disease in 12/13 patients with mean follow-up of 3.9 yrs. Longer follow up is needed to determine if unilateral adrenalectomy is an acceptable treatment in PPNAD. Conclusion Diagnosing PPNAD and deciding appropriate time of surgery can be challenging. Unilateral adrenalectomy as a treatment modality needs to be further studied. References Correa et al. Carney Complex: An Update. Eur J Endocrinol 2015. Xu et al. The Role of Unilateral Adrenalectomy in Corticotropin-Independent Bilateral Adrenocortical Hyperplasias. World J Surg 2013.

Risk of adrenal crisis with hydrocortisone buccal tablets

Risk of adrenal crisis with hydrocortisone buccal tablets

A228 FIRST REPORT OF ADRENAL CRISIS IN TWO PATIENTS WITH EOSINOPHILIC ESOPHAGITIS

Eosinophilic Esophagitis (EoE) is an immune mediated allergic condition affecting the esophagus. Risk factors include male sex and atopy. A treatment option is swallowed corticosteroids (cs). Patients with EoE are at risk for adrenal insufficiency (AI). We screened 14 patients on Oral Viscous Budesonide (OVB) for ≥ 3 months using the low dose ACTH stimulation test and found 6 (43%) had evidence of AI. This was found in patients with and without inhaled cs. Golekoh found 10% of their patients on Fluticasone to have AI. We present 2 cases of adrenal crisis requiring major intervention from the ‘at risk’ screened group. Case 1 17 y.o with atopy and food allergies, diagnosed with EoE in 2011 presenting with vomiting. He was on baseline inhaled cs for asthma and cs nasal spray. OVB was successful at achieving remission and he maintained 0.5 mg po daily. Through routine screening in 2013 he was shown to be at risk of AI with an abnormal ACTH stim test result of 403 nmol/L @ 30 mins. Stress-dose cs management was taught for illnesses and stress. Despite the patient deciding to stop the OVB, he had an abnormal ACTH stim test ≥ 3 months later. Limited follow-up occurred. In June 2016, patient underwent a GA for elective surgery at an outlying hospital. No pre-operative cs were received. In recovery he had hypotension and apnea requiring a Code Blue and ICU admission. At the time, a low cortisol level was documented (44 nmol/L). He responded well to IV Hydrocortisone and was discharged the next day. A subsequent ACTH stim test was abnormal (peak 417 nmol/L). Case 2 17 y.o. ex prem with chronic lung disease, asthma, laryngeal cleft, rhinitis and food allergies. Pre-diagnosis he was on inhaled cs for asthma. He was diagnosed with EoE in 2008 presenting with dysphagia. Initially he was on Fluticasone but in 2012 was switched to 1 mg bid OVB with incomplete histological remission (some symptom improvement). He routinely underwent ACTH stim testing in 2013 and was found to have AI with a peak level of 459 nmol/L. Education was provided for episodes of moderate and severe stress. He remained on the OVB. In the interim he received stress coverage for viral meningitis in 2015 and was weaned off. He switched to Fluticasone and did not have improvement in his ACTH stim testing. In 2016 he had a URTI and took 3 po doses of cs for moderate stress. Despite the oral cs, he continued to evolve with headache, aches and weakness. Upon presentation to emergency he was hypotensive and required IV steroids and fluids. Recently, he has had difficulty weaning the cs and has shown to have low baseline cortisol production necessitating daily cs. Identifying EoE patients with AI is clinically relevant as they are at risk for adrenal crisis as exemplified by these two cases. The risk for AI can be enhanced by the swallowed cs, and can persist post-discontinuation. None

Massive adrenal incidentalomas and late diagnosis of congenital adrenal hyperplasia in prostate cancer.

In a 61-year-old Caucasian male with prostate cancer, leuprolide and bicalutamide failed to suppress the androgens. He presented to endocrinology with persistently normal testosterone and incidental massive (up to 18 cm) bilateral adrenal myelolipomas on CT scan. Blood test did not reveal metanephrine excess. The patient was noted to have short stature (151 cm) and primary infertility. Elementary school photographs demonstrated precocious puberty. Physical examination revealed palpable abdominal (adrenal) masses. Abiraterone and glucocorticoid treatment was commenced with excellent suppression of testosterone. Genetic testing revealed a mutation in CYP21A2 confirming 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH). Association of large myelolipomas with CAH has been reported in the literature. Our case highlights the importance of considering CAH in patients with non-suppressed testosterone despite androgen deprivation therapy. Large myelolipomas should raise the suspicion of congenital adrenal hyperplasia.Learning points:Adrenal myelolipomas are rare benign lesions that are more common in patients with longstanding untreated congenital adrenal hyperplasia thought to be due to ACTH stimulation.Consider undiagnosed congenital adrenal hyperplasia in patients with adrenal myelolipoma.Glucocorticoid replacement may be an efficacious treatment for patients with prostate cancer and CAH. Abiraterone therapy has a risk of adrenal crisis if glucocorticoids are not replaced.

L’insuffisance surrénalienne chez l’adulte

Adrenal insufficiency is a rare but life-threatening disorder. Clinical manifestations include fatigue, weight loss, gastrointestinal manifestations and skin hyperpigmentation, the latter being specific of primary adrenal failure. Because of non-specific clinical features of this rare disorder, diagnosis can be delayed and adrenal failure be revealed by an acute crisis. Adrenal insufficiency can be primary (Addison disease), most frequently autoimmune, or secondary, resulting from long term administration of exogenous glucocorticoids or more rarely from pituitary disorders. Monitoring of substitutive treatment is now well codified. Patient education is very important in this chronic disease that remains associated with a persistent high risk of adrenal crisis.

An overview of the nursing issues involved in caring for a child with adrenal insufficiency

Adrenal insufficiency is an endocrine condition defined as the inadequate production or action of glucocorticoids, principally a steroid hormone called cortisol. While rare in childhood, it carries the risk of adrenal crisis in the event of a child becoming unwell as a result of intercurrent illness, injury or surgery. Children's nurses must be vigilant in caring for a child with adrenal insufficiency and have a clear understanding and awareness of the principles of emergency management at home and in hospital.

Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

Background/Aims: Factors in congenital adrenal hyperplasia (CAH) that may affect quality of life (QOL) include the need for lifelong medication, the risk of adrenal crisis, and hyperandrogenic symptoms. The objectives were to evaluate health-related QOL (HRQOL) in children with CAH, and whether CAH poses an additional burden compared to other endocrine disorders. Methods: The validated PedsQL 4.0 generic core scales were administered to subjects (8-18 years) with CAH and hypothyroidism and their parents. The minimal clinically important difference (MCID) was determined for each scale score, allowing a comparison with the healthy population. A score of >1 standard deviation below the population mean was considered at risk for impaired HRQOL. Results: In CAH, the mean total HRQOL scores were >1 MCID below the population mean, and a higher percentage than expected had scores considered at risk. Conclusion: Compared to subjects with hypothyroidism, subjects with CAH self-reported lower school domain scores. CAH subjects more frequently reported peers not wanting to be friends.

Modern Hydrocortisone Replacement Regimens in Adrenal Insufficiency Patients and the Risk of Adrenal Crisis.

The aim of this study was to examine the incidence of adrenal crises (AC) and the prescription of short-acting glucocorticoids (GC) in different geographic areas. To do this we conducted a descriptive study of AC hospitalisations and prescriptions for two GCs (hydrocortisone (HC) and cortisone acetate (CA)), and fludrocortisone acetate (FA), in different geographic areas of Australia between 1999/2000 and 2011/2012, using government databases.There were 2,584 hospital admissions for AC in Australia between 1999/00 and 2011/12 and the corresponding admission rates increased significantly from 7.4 to 11.1/10(6)/year (p<0.001). AC admission rates increased in 5 out of 6 geographic areas. Prescription rates for the combined GCs (HC/CA) increased at an annual rate of between 0.2-2.0% in all areas. All areas had significant (p<0.01) increases in HC prescription rates (4.5% to 13.7% annually) and CA prescription rates decreased in 5 out of the 6 regions (3.5% annual decrease to a 0.5% annual increase). When the geographic areas were combined, there was a significant correlation between the AC admission rates and HC/CA prescription rates (r=0.30, p<0.01). Admissions for AC and GC prescriptions increased significantly in Australia after 1999 and these varied significantly by geographic area. These results suggest that modern recommendations for lower dose, short-acting GC replacement may be of concern and further investigation is warranted.

Bilateral adrenalectomy for Cushing's disease.

Review the indications, outcomes, and consequences of bilateral adrenalectomy (BLA) in patients with Cushing's disease. A literature review was performed. The primary therapy for Cushing's disease is surgery, with medical therapy and radiation therapy relegated to an adjuvant role. BLA is indicated in cases of persistent disease following pituitary surgery or in situations where rapid normalization of hypercortisolism is required. When performed via the laparoscopic approach, BLA is associated with a significantly reduced morbidity compared to the traditional, open approach. Following BLA, patients are at risk for adrenal crisis and the concern of Nelson's syndrome. However, BLA leads to a rapid resolution of the signs and symptoms of CS and leads to an improved long-term quality of life. BLA should be considered in the treatment algorithm for patients with persistent CD after failed pituitary surgery, especially in patients who have severe consequences of hypercortisolism or desire pregnancy.

Cushing syndrome.

Cushing syndrome.