scholarly journals MON-LB038 Isolated Hypoaldosteronism Due to Autoimmune Adrenalitis in a Patient With Autoimmune Polyglandular Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Dheera Grover ◽  
Chenxiang Cao ◽  
Sarika Rao

Abstract BACKGROUND At the initial presentation of autoimmune adrenal insufficiency, most patients present with hormonal deficiencies from all three layers of adrenal cortex. However, isolated aldosterone deficiency causing a true partial adrenal insufficiency in the setting of autoimmune adrenalitis remains underrecognized. CASE REPORT A 67-year old female patient with a known history of diabetes mellitus type 1 since the age of 13 and morphea, presented with progressively worsening symptoms of confusion and hallucinations, fatigue, and loss of appetite over the past 5 years. During this time, she has had frequent and recurrent episodes of mild intermittent hyponatremia with hyperkalemia requiring intravenous fluids and ingested salt tablets, especially when she felt more symptomatic. On her initial evaluation here, she presented with hyponatremia (125 mmol/l, n: 135-145 mmol/l), low osmolality (264 mOsm/kg, n: 275-295 mOsm/kg), and normal potassium level (3.6 mmol/l, n: 3.6-5.2 mmol/l). Further investigations drawn at the same time revealed a low aldosterone (<4 ng/dL), normal renin (5.3 ng/mL/hr, ref 2.9-10.8), normal serum cortisol level (and normal response to Cortrosyn stimulation), though all in the setting of positive antibodies against 21-hydroxylase. Pan-imaging revealed no evidence of malignancy that can be causing ectopic SIADH production. Additional testing showed presence of auto antibodies contributing to pernicious anemia and thyroid disease. Treatment was started with fludrocortisone 0.1 mg tablet daily and she was advised to take the salt tablets only if she has any symptoms. The patient’s symptoms have resolved 8 months since this diagnosis, with normalized sodium and potassium levels. CONCLUSION Autoimmune primary adrenal insufficiency usually affects all three layers of the adrenal cortex, where patients present with deficiencies in cortisol and aldosterone. Isolated hypoaldosteronism has rarely been reported, however because it can cause life-threatening hyponatremia, it is an important entity to recognize. It is important to work up in such patients as they may be in the initial stages of autoimmune Addison’s disease and can progress to developing cortisol deficiency, though the time course to this progression is not well known.

Endocrines ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 125-137
Author(s):  
Shogo Akahoshi ◽  
Yukihiro Hasegawa

The present review focuses on steroid-induced adrenal insufficiency (SIAI) in children and discusses the latest findings by surveying recent studies. SIAI is a condition involving adrenocorticotropic hormone (ACTH) and cortisol suppression due to high doses or prolonged administration of glucocorticoids. While its chronic symptoms, such as fatigue and loss of appetite, are nonspecific, exposure to physical stressors, such as infection and surgery, increases the risk of adrenal crisis development accompanied by hypoglycemia, hypotension, or shock. The low-dose ACTH stimulation test is generally used for diagnosis, and the early morning serum cortisol level has also been shown to be useful in screening for the condition. Medical management includes gradually reducing the amount of steroid treatment, continuing administration of hydrocortisone corresponding to the physiological range, and increasing the dosage when physical stressors are present.


2018 ◽  
Vol 4 (4) ◽  
pp. e282-e284
Author(s):  
Lauren M. Maiorini ◽  
Kent Chan ◽  
Lawrence E. Shapiro

2019 ◽  
Vol 47 (5) ◽  
pp. 2288-2295 ◽  
Author(s):  
Yansheng Jin ◽  
Maoxiao Fan

This study aimed to report a unique case of primary adrenal insufficiency that was accompanied by painful gynecomastia, which was resolved by treatment with prednisone. Enlargement of the left breast with continuous weakness and generalized nausea in a male was discovered 3 months before admission. Magnetic resonance imaging of the brain was normal 1 month before presentation. A physical examination revealed that the diameter of the left breast was 5 cm and the height was 3 cm. Laboratory investigations revealed hyponatremia, with a low serum cortisol level and an elevated prolactin level. Hyperprolactinemia was suspected because of adrenal deficiency that was directly or indirectly associated with increased prolactin levels. Thus, a diagnosis of hyperprolactinemia was confirmed. Ultrasonography of the left breast showed glandular tissue hyperplasia. In the present study, treating adrenal insufficiency with prednisone relieved both gynecomastia and hyponatremia. However, gynecomastia regression and hyponatremia resolution were observed when prednisone was stopped. Gynecomastia completely resolved by re-administering prednisone. Therefore, treating the underlying disease is essential so that prednisone can be given in a timely manner.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A147-A147
Author(s):  
Sowjanya Naha ◽  
Joseph Theressa Nehu Parimi ◽  
Rajani Gundluru ◽  
John Chen Liu ◽  
Hasan Syed ◽  
...  

Abstract Background: Pregnancy is characterized by physiologic immunosuppression and the postpartum period is often associated with rebound autoimmunity leading to unmasking of latent autoimmune disorders. Clinical Case: A thirty-one-year-old Caucasian female presented with generalized fatigue, myalgias and arthralgias that started three months after the birth of her first child. She was seen at a local hospital on multiple occasions and was treated symptomatically. She was noted to have orthostatic hypotension during these visits and was initiated on midodrine and fludrocortisone for presumed postural orthostatic tachycardia syndrome. When her symptoms did not abate, she was referred to our center for further care. At the time that we saw her, she reported nausea and vomiting in addition to the aforementioned symptoms. On careful questioning she also admitted to salt craving and unintentional weight loss of about 20 lbs over the past three months. She did not have a past history of autoimmune or endocrine disorders, but family history was positive for Grave’s disease and celiac disease in her aunt and mother respectively. Physical examination revealed hyperpigmentation of the face, knuckles, toes and abdominal scars. Random serum cortisol level was low (1.06 mcg/dL) with no perceptible response to ACTH stimulation (1.10 mcg/dL and 1.04 mcg/dL at 30 and 60 mins respectively). Serum aldosterone was undetectable (<4 ng/dL) and dehydroepiandrosterone sulfate level was low (19 mcg/dL, n: 45–295 mcg/dL). The patient was immediately initiated on corticosteroid replacement therapy. Her symptoms rapidly resolved and she was discharged on maintenance dosing of hydrocortisone and fludrocortisone. ACTH was grossly elevated (577 pg/mL) and 21-Hydroxylase antibodies were positive, confirming a diagnosis of autoimmune adrenalitis. Conclusion: Symptoms of adrenal insufficiency are often non-specific and can overlap with physiological symptoms of pregnancy. Persistence of these symptoms after delivery should raise concerns for autoimmune adrenalitis precipitated by immune reconstitution in the postpartum period.


2019 ◽  
Vol 42 (4) ◽  
pp. 78-82
Author(s):  
Lickhasit Saenglootong ◽  
Somchai Insiripong

The majority of cases with dyspepsia usually have normal gastroscopic finding, so some unusual causes may be easily neglected under the umbrella of nonulcer dyspepsia as in this case. We report a 64-year-old Thai patient who suffered from dyspepsia and fatigue for many years. Her latest physical examination was unremarkable. Although she did not have any warning signs, many abdominal investigations including the ultrasonography, computerized tomography, barium enema with air contrast, esophagogastroduodenoscopy, and colonoscopy were repeatedly performed in different hospitals and all revealed unremarkable. The last esophagogastroduodenoscopy showed only mild acute gastritis that was not expected to account for the many-year dyspepsia and fatigue. The unusual and rare causes of long term dyspepsia were extensively investigated. Finally, she was found to have serum cortisol less than 0.8 , normal serum calcium, phosphorus, and electrolytes but minimally abnormal thyroid function test. Therefore, she was diagnosed as having adrenal insufficiency and concurrent subclinical hyperthyroidism. She was fully treated with many dyspeptic drugs such as pantoprazole, sucralfate, simethicone, ondansetron, and intravenous hydrocortisone infusion 300 mg a day. She responded well to therapy in 2 days and was discharged home with oral prednisolone 15 mg a day with many dyspeptic drugs. Adrenal insufficiency is a very rare entity and most cases have no specific signs and symptoms, so it could be easily overlooked as the very rare and unusual cause of chronic dyspepsia. In this report, the association between the adrenal insufficiency and the gastrointestinal upset is discussed.  


2018 ◽  
Vol 31 (2) ◽  
pp. 107-115 ◽  
Author(s):  
Kate C. Verbeeten ◽  
Alexandra H. Ahmet

AbstractCortisol is a hydrophobic molecule that is largely bound to corticosteroid-binding globulin (CBG) in the circulation. In the assessment of adrenal insufficiency, many clinicians measure a total serum cortisol level, which assumes that CBG is present in normal concentrations and with a normal binding affinity for cortisol. CBG concentration and affinity are affected by a number of common factors including oral contraceptive pills (OCPs), fever and infection, as well as rare mutations in the serine protease inhibitor A6 (SERPINA6) gene, and as such, total cortisol levels might not be the ideal way to assess adrenal function in all clinical circumstances. This paper reviews the limitations of immunoassay and liquid chromatography-tandem mass spectrometry (LC-MS/MS) in the measurement of total cortisol, the challenges of measuring free serum cortisol directly as well as the difficulties in calculating an estimated free cortisol from total cortisol, CBG and albumin concentrations. Newer approaches to the evaluation of adrenal insufficiency, including the measurement of cortisol and cortisone in the saliva, are discussed and a possible future role for these tests is proposed.


2021 ◽  
Author(s):  
Valentina Gasco ◽  
Chiara Bima ◽  
Alice Geranzani ◽  
Jacopo Giannelli ◽  
Lorenzo Marinelli ◽  
...  

Introduction According to guidelines, a morning serum cortisol level <83 nmol/L is diagnostic for central adrenal insufficiency (CAI), a value >414 nmol/L excludes CAI, while values between 83 and 414 nmol/L require stimulation tests. However, there are no currently reliable data on morning serum cortisol for prediction of cortisol response to insulin tolerance test (ITT). Objective Using the receiver operating characteristic curve analysis, the purpose of this study was to detect the morning serum cortisol cut-off with a specificity (SP) or a sensitivity (SE) above 95% that identify those patients who should not be tested with ITT. Methods We included 141 adult patients (83 males) aged 42.7+/-12.3 (mean +/- SD) years old. Based on serum cortisol response to ITT, patients have been divided in two groups: subjects with CAI (peak serum cortisol <500 nmol/L; 65 patients) and subjects with preserved adrenocortical function (peak cortisol >500 nmol/L; 76 patients). Results The best morning cortisol cut-off, in terms of SE (87.7%) and SP (46.1%), was ≤323.3 nmol/L. The cut-off of morning serum cortisol concentration that best predicted a deficient response to ITT was ≤126.4 nmol/L (SE 13.8%, SP 98.7%). The cut-off of morning serum cortisol concentration that best predicted a normal response to ITT was >444.7 nmol/L (SE 96.9%, SP 14.5%). Conclusions This is the first study that identifies a morning serum cortisol cut-off that best predict the response to ITT in order to simplify the diagnostic process in patients with suspected CAI. A new diagnostic flow chart for CAI is proposed.


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.


2006 ◽  
Vol 72 (6) ◽  
pp. 552-554 ◽  
Author(s):  
G.D. Rushing ◽  
R.C. Britt ◽  
J.N. Collins ◽  
F.J. Cole ◽  
L.J. Weireter ◽  
...  

Adrenal insufficiency during sepsis is well documented. The association between hemorrhagic shock and adrenal insufficiency is unclear and may be related to ischemia, necrosis, or resuscitation. This study was designed to determine the incidence of relative adrenal insufficiency in hemorrhagic shock. A retrospective review of a prospectively gathered database for patients admitted to the trauma intensive care unit with hemorrhagic shock was undertaken. A random serum cortisol of <25 mcg/dL defined relative adrenal insufficiency. All of the cortisol levels were drawn within the first 24 hours of admission. Data analyzed included demographics, length of stay, injury mechanism, infections, and mortality. Fifteen patients presented with hemorrhagic shock, with 14 of 15 meeting the criteria for relative adrenal insufficiency. The average serum cortisol level was 15.8 (9–26.8). The average APACHE II score was 18.3 (4–33), and the average Injury Severity Score was 22.5 (8–41). The mechanism was blunt trauma in 10 patients and penetrating trauma in 5. The average intensive care unit and hospital length of stay were 13.2 and 27.4 days, respectively. There were five urinary tract infections, four blood stream infections, and two wound infections. Two of the 15 patients died. Relative adrenal insufficiency appears to be common in hemorrhagic shock. Future research is warranted to elucidate the pathophysiology, as well as to prospectively determine which patients may benefit from steroid replacement.


2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
David A. Baran ◽  
Cheryl Rosenfeld ◽  
Mark J. Zucker

Background. Despite earlier studies describing the feasibility of steroid weaning in heart transplant patients, the majority of patients are maintained on steroid therapy for life. We examined a strategy based on a single morning serum cortisol measurement. Methods. We assigned stable posttransplant patients, who were maintained on tacrolimus, mycophenolate mofetil, and corticosteroids, into one of two groups based on a screening morning cortisol level. Patients with a cortisol < 8 micrograms/deciliter were assigned to a “maintenance” group and the others were assigned to the weaning group and steroids were tapered off over 4–6 weeks. Patients were monitored on subsequent office visits for adrenal insufficiency and allograft rejection. Results. Thirty-one patients were enrolled (6 patients in the maintenance group and 25 in the steroid-weaning group). Mean follow-up was 10.2±4 years for the weaning group and 9.0±4.9 years in the maintenance group (p=0.6). No cases of rejection were noted, nor did any patient resume steroid treatment following discontinuation. Conclusion. Steroids can be safely discontinued in stable heart transplant patients with an AM serum cortisol ≥ 8 micrograms/deciliter with appropriate outpatient follow-up. In this study, no patient suffered late rejection or clinically noted adrenal insufficiency.


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