Acute Adrenocortical Insufficiency Research Articles

Relative Adrenocortical Insufficiency Following Radioactive Iodine Therapy for Graves’ Disease: A Report of Two Cases

IntroductionIn recent years, radioactive iodine (RAI) therapy has become a main choice for Graves’ disease. The rapid release of thyroid hormones following RAI may on occasion trigger severe events, such as thyroid storm or heart block. This study presents two cases of possible acute adrenocortical insufficiency precipitated by radioiodine therapy.Case PresentationTwo females aged 65 and 19 years with long-standing Graves’ disease underwent radioiodine treatment at our clinical center. Both patients suffered nausea, fatigue, aggravated palpitation, and relative hypotension thereafter. Laboratory data showed severe thyrotoxicosis with free thyroxine higher than three times the upper limit, while basal serum cortisol (8 AM) was below the lower limit (5.08 μg/dL and 2.08 μg/dL respectively) under stress, indicating a potential relative adrenocortical insufficiency. There was then full recovery after adequate hydrocortisone therapy. As thyrotoxicosis resolved, the levels of basal cortisol were subsequently raised to normal.ConclusionPost-RAI adrenocortical insufficiency might occur in patients with severe Graves’ disease. Basal serum cortisol might be a cost-effective parameter for the identification of potential patients.

Novel medications inducing adrenal insufficiency

Emerging evidence demonstrates that an increasing number of novel medications have considerable potential to induce adrenal insufficiency. This condition might lead to acute adrenocortical insufficiency, which is potentially fatal; however, the condition could be avoided if clinicians are more aware of the new findings and their implications.

Clinical characteristics and prognosis analysis of children with idiopathic nephrotic syndrome with different responses to steroid therapy from a single center in 20 years

Objective To analyze the clinical characteristics and prognosis of children diagnosed with idiopathic nephrotic syndrome that showed different responses to steroid treatment. Methods In this study, idiopathic nephrotic syndrome was defined as a 24-hour urinary protein of more than 50 mg/kg body weight as well as a serum albumin concentration of less than 25 g/L.A retrospective study was undertaken to analyze the children who were patients at Peking University First Hospital during Jan.1993 and Dec.2012 and conformed to the above criteria.According to their response to steroid therapy, the children were divided into 3 groups: group that demonstrated remission within 4 weeks after therapy, group that demonstrated remission within 4 to 8 weeks and group that demonstrated resistance to therapy after 8 weeks.Among the children who had more than 1 year of follow up, type of nephrotic syndrome(simple vs nephritic), complications, steroid side effects and serious prognosis(chronic renal failure, renal transplantation or death) were further analyzed. Results Out of total 1 059 children with idiopathic nephrotic syndrome, 841 cases had a documented steroid response and were analyzed.Among them, 71.7%(603/841 cases) showed remission within 4 weeks following steroid treatment, while 4.0%(34/841 cases) showed remission within 4 to 8 weeks and 24.3%(204/841 cases) showed no remission even after 8 weeks.The average remission time after steroid therapy was(13.2±8.9) d with a median of 10.0 d. Of these children, 369 cases had at least 1 year of follow up.In this subset, 74.8%(276/369 cases) went into remission within 4 weeks of steroid therapy, 4.9%(18/369 cases) showed remission within 4 to 8 weeks, and 20.3%(75/369 cases) were resistant to steroid therapy even after 8 weeks.Onset ages were significantly different(P<0.01) among the 3 groups [remission within 4 weeks: (5.2±3.3) years old; remission within 4 to 8 weeks: (5.7±3.3) years old; resistant to therapy after 8 weeks: (6.6±3.8) years old]. Simple type nephrotic syndrome was more common in children that responded to steroid therapy within 4 weeks(95.3%, 263/276 cases) and within 4 to 8 weeks(83.3%, 15/18 cases) than in children who were resistant to therapy after 8 weeks(45.3%, 34/75 cases). The occurrence rates of acute adrenocortical insufficiency showed a significant difference(P=0.04) among the 3 groups(remission within 4 weeks: 4.3%, 12/276 cases; remission within 4 to 8 weeks, 2/18 cases: 11.1%; resistant to therapy after 8 weeks: 0). The occurrence rates of poor prognosis were also significantly different(P<0.01) among the 3 groups(remission within 4 weeks: 1.4%, 4/276 cases; remission within 4 to 8 weeks: 0; resistant to therapy after 8 weeks: 16.0%, 12/75 cases). Conclusions Seventy-one point seven percent of children with idiopathic nephrotic syndrome responded to steroid therapy within 4 weeks.Among children that did not respond within 4 weeks following steroid therapy, 14.3% did respond within 8 weeks, while 85.7% showed no remission 8 weeks after therapy.The children who responded to steroid therapy within 8 weeks mainly presented as a simple type of nephrotic syndrome and had better prognosis, whereas the children that did not respond within 8 weeks mainly presented with nephritic type nephrotic syndrome and had a poorer prognosis.It should be noted that acute adrenocortical insufficiency was more likely to occur in children that responded to steroid therapy within 4 to 8 weeks. Key words: Nephrotic syndrome; Steroid response; Criteria; Child

Ketoacidosis and Adrenocortical Insufficiency

We herein report an autopsy case involving a 27-year-old Caucasian woman suffering from chronic adrenocortical insufficiency with a background of a polyendocrine disorder. Postmortem biochemistry revealed pathologically decreased aldosterone, cortisol, and dehydroepiandrosterone levels in postmortem serum from femoral blood as well as decreased cortisol and 17-hydroxycorticosteroid in urine. Decreased vitreous sodium and increased 3-beta-hydroxybutyrate and C-reactive protein concentrations were observed. The cause of death was determined to be acute adrenocortical insufficiency. Fasting ketoacidosis was postulated to have precipitated the Addisonian crisis. Traumatic causes of death and third-party involvement were excluded. The case highlights the importance of systematically performing exhaustive postmortem biochemical investigations to formulate appropriate hypothesis regarding the pathophysiological mechanisms involved in the death process.

Adrenocortical Insufficiency due to Spontaneous Bilateral Adrenal Hemorrhage Presented as Acute Abdominal Pain

Adrenocortical insufficiency is the clinical manifestation of deficient production or action of glucocorticoids. It is a life-threatening disorder that can result from primary adrenal failure or secondary adrenal failure due to impairment of the hypothalamicpituitary axis. Primary adrenocortical insufficiency can be caused by autoimmune adrenalitis, infection (especially, tuberculosis), metastatic cancer, lymphoma, adrenal hemorrhage, infarction or drugs. Among these, adrenal hemorrhage may be caused by anticoagulant drug or heparin therapy, thromboembolic disease, hypercoagulable states such as antiphospholipid syndrome, physical trauma, postoperative state, sepsis and severe stress from any cause. However, even fewer reports exist of adrenocortical insufficiency due to spontaneous bilateral adrenal hemorrhage. We report a rare case of acute adrenocortical insufficiency due to spontaneous bilateral adrenal hemorrhage presenting as acute abdominal pain. (Ewha Med J 2014;37(Suppl):S10-S14)

Acute Adrenal Insufficiency After a Single Dose of Etomidate

Acute adrenocortical insufficiency is a critical care emergency characterized by hemodynamic instability, lethargy, and cardiovascular collapse. Acute adrenal insufficiency has many etiologies, from rapid withdrawal of exogenous glucocorticoids to adrenocortical destruction to poor adrenal reserve after administration of steroid synthesis inhibitors. Etomidate, a parenteral hypnotic agent, is a steroid synthesis inhibitor. Although the use of continuous etomidate infusion in the intensive care unit fell from favor secondary to reports of adrenal crisis, single-dose etomidate for induction of anesthesia is common for the hemodynamically unstable patient or in patients who may not tolerate wide variance in heart rate or blood pressure. A case is presented of acute adrenocortical insufficiency and crisis after a standard induction dose of etomidate. Acute adrenal insufficiency should be suspected in intensive care unit patients who have undergone general anesthesia with etomidate induction and present with hypotension refractory to standard vasopressor administration.

Selective Use of Steroid Replacement After Adrenalectomy

Only selected patients require steroid replacement therapy following adrenalectomy. Retrospective review. University tertiary care center and veterans' hospital. A total of 331 patients who underwent adrenalectomy by 1 surgeon (Q.-Y.D.) between April 1, 1993, and August 31, 2005. Laparoscopic, open, and hand-assisted adrenalectomy. Steroid replacement therapy was administered using a standardized hydrocortisone taper protocol. Indications for adrenalectomy, operative approach, requirement for postoperative steroid replacement, and episodes of acute adrenocortical insufficiency. Of the 331 adrenalectomies, 304 were laparoscopic, 23 were open, and 4 were hand assisted. There were 299 unilateral adrenalectomies and 32 bilateral adrenalectomies performed. Fifty-seven (17%) of the 331 patients required steroid replacement after adrenalectomy. Of the 57 patients requiring steroid replacement, 52 had Cushing syndrome and 5 had bilateral pheochromocytomas. The 52 patients with Cushing syndrome included 16 with pituitary tumors who had failed pituitary resection and/or medical therapy, 14 with unilateral adrenal adenomas, 9 with ectopic corticotropin-secreting tumors who had failed resection and/or medical therapy, 7 with incidentalomas and subclinical Cushing syndrome, 4 with macronodular hyperplasia, and 2 with adrenocortical carcinoma. No patients undergoing unilateral adrenalectomy for non-Cushing adrenal disease required steroid replacement. Four (7%) of the 57 patients receiving steroid replacement had episodes of acute adrenocortical insufficiency following operation and required increased steroid supplementation. There were no cases of acute adrenocortical insufficiency in the 274 patients who did not receive steroid replacement. Steroid replacement therapy after adrenalectomy should be reserved for patients with Cushing syndrome (overt or subclinical) and patients undergoing bilateral adrenalectomy. Patients undergoing adrenalectomy for unilateral non-Cushing adrenal tumors do not require postoperative steroid replacement.

Prise en charge des hématomes post-traumatiques isolés de la surrénale

We report three cases of isolated adrenal haematoma diagnosed at admission to the Emergency Department. Post-traumatic adrenal haematoma have been observed in 2% of patients with abdominal injury and can be life threatening in cases of bilateral haemorrhage with risk of acute adrenocortical insufficiency. Diagnosis remains difficult due to absence of clinical and biological specific parameters. Therefore, abdominal CT examination should be the gold standard in management of abdominal trauma patients.

Endocrine emergencies

Endocrine emergencies are uncommon and require a high index of suspicion if they are to be managed appropriately. This is especially pertinent to physicians accepting emergency admissions to hospital. Treatment needs to be started prior to diagnostic confirmation. We outline the management of the following conditions that may present acutely: Thyroid emergencies – Myxoedema coma – Thyroid Storm Pituitary apoplexy Phaeochromocytoma crisis Acute adrenocortical insufficiency.

Perioperative stress dose steroids: do they make a difference?

Since the Nobel prize-winning description of the use of synthetic glucocorticoids in the treatment of rheumatoid arthritis by Hench and colleagues in 1949, steroid use has become ubiquitous among patients with inflammatory conditions. Although the benefits of steroids are clear for these patients, it has become apparent that there are very serious potential side effects of the treatment. The potential dangers of chronic steroid use in surgical patients were first illustrated by Fraser and colleagues in 1952. They describe a steroid-dependent patient undergoing major orthopaedic surgery who died of intractable hypotension postoperatively. An autopsy revealed adrenal atrophy, and the authors conclude that the patient died from adrenocortical insufficiency secondary to adrenal suppression. Since the publication of this singlecase report, supraphysiologic “stress dose” steroids have become a regular part of the perioperative management of patients treated longterm with steroids. In individuals with an intact hypothalamic-pituitaryadrenal (HPA) axis undergoing the stress of an operation, the adrenal glands increase their baseline secretion of cortisol. Although the details are not clear, it is thought that increased circulating cortisol helps maintain hemodynamic stability in the face of stress. Patients treated longterm with exogenous steroids have a blunted HPA axis response to surgical stress when compared with normal controls, resulting in lower levels of circulating cortisol. Based on this physiologic data and a handful of case reports like that of Fraser and colleagues, several reviews have been published over the past 50 years recommending supraphysiologic corticosteroid regimens for such patients undergoing operations. Although it is known that endogenous steroid levels become elevated during surgical stress, it is uncertain whether a reduced level of circulating cortisol results in hypotensive crisis. Kehlet and Binder followed 31 patients treated with exogenous steroids through major surgical procedures. Each patient was shown to have HPA axis suppression by a failed response to an adrenocorticotropic hormone (ACTH) stimulation test. None of these patients was treated with steroids perioperatively, and none of the patients showed any symptoms of acute adrenocortical insufficiency. Nine of these patients had subnormal plasma cortisol levels throughout the operation. Although surrogate biochemical markers of adrenal function were shown to be suppressed, there was no correlation between these data and patients’ clinical outcomes when subjected to the stress of a surgical procedure. In 1976, Kehlet reviewed the world literature and found 57 case reports of perioperative hypotension attributed to adrenal insufficiency. His investigation revealed that only three of these reports had sufficient clinical and biochemical data to support this diagnosis. Kehlet’s work began to undermine the tenets of perioperative stress dose steroids over 20 years ago. Studying an animal model, Udelsman and colleagues have also challenged the necessity of stress dose steroids in the perioperative period. First, they surgically removed the adrenal glands of a cohort of primates. They then randomized the monkeys to receive supraphysiologic (10 times normal dose), physiologic, and subphysiologic (one-tenth normal dose) perioperative steroid coverage for an open cholecystectomy. The animals treated with subphysiologic doses of steroids demonstrated a markedly decreased mean blood pressure, increased incidence of electrolyte abnormalities, and increased mortality when compared with normal controls, but there was no difference in these outcomes when the monkeys treated with physiologic doses of steroids were compared with monkeys with supraphysiologic treatment or nonadrenalectomized controls. Udelsman and No competing interests declared.

The Treatment of Acute Adrenocortical Insufficiency in the Dog

SummaryAcute hypoadrenocorticism in the dog is a true medical emergency that requires a prompt and accurate diagnosis and appropriate treatment in order to provide for the most optimal outcome. Because the signs of adrenal insufficiency mimic those caused by other more common clinical conditions such as renal failure, intoxications, and various gastrointestinal disorders, it behooves the emergency clinician to always include this condition in the list of differential diagnoses. This paper will review the main elements of this hypoendocrinopathy in order to provide the emergency clinician with the essentials that will be required for appropriate diagnosis and treatment.J Vet emerg Crit Care 2001; 11(1):7–14

Adrenoleukodystrophy presenting with Addisonian crisis

AbstractThe case of a young boy who presented with Addisonian crisis as a manifestation of adrenoleukodystrophy (ALD) is presented. Adrenoleukodystrophy is an uncommon X‐linked genetic disease that may present with acute adrenocortical insufficiency. The pathogenesis, clinical presentation and management of adrenoleukodystrophy are presented.

Adrenalectomy for treatment of hyperadrenocorticism in cats: 10 cases (1988-1992)

Summary Outcome of and complications associated with bilateral adrenalectomy in 8 cats with pituitary-dependent hyperadrenocorticism and bilateral adrenocortical hyperplasia and outcome of and complications associated with unilateral adrenalectomy in 2 cats with adrenocortical tumor (adrenocortical adenoma, 1 cat; adrenocortical carcinoma, 1 cat) and unilateral adrenomegaly were determined. Glucocorticoids were administered to all cats at the time of surgery, and mineralocorticoids were administered to the 8 cats that underwent bilateral adrenalectomy. A ventral midline celiotomy was performed in all cats. Intraoperative complications did not develop in any cat. Postoperative complications developed in all cats and included abnormal serum electrolyte concentrations (n = 8), skin lacerations (n = 5), pancreatitis (n = 3), hypoglycemia (n = 2), pneumonia (n = 1), and venous thrombosis (n = 1). Three cats died within 5 weeks after surgery of complications associated with sepsis (n = 2) or thromboembolism (n = 1). Clinical signs and physical abnormalities caused by hyperadrenocorticism resolved in the remaining 7 cats 2 to 4 months after adrenalectomy. Insulin treatment was discontinued in 4 of 6 cats with diabetes mellitus. Median survival time for these 7 cats was 12 months (range, 3 to &gt; 30 months). Two cats died of acute adrenocortical insufficiency 3 and 6 months after bilateral adrenalectomy, 2 cats were euthanatized because of chronic renal failure 3 and 12 months after bilateral (n = 1) or unilateral (n = 1) adrenalectomy, and 2 cats were alive 9 and 14 months after bilateral adrenalectomy. In the remaining cat, clinical signs recurred 10 months after the cat had undergone unilateral adrenalectomy. The remaining adrenal gland was found to contain an adrenocortical adenoma and was removed. The cat was doing well when it was lost to follow-up 15 months after the second surgery.

Addisonian Crisis following Pericardiectomy

In developing countries tuberculosis is a common cause of constrictive pericarditis and its Involvement of other organs may sometimes complicate the surgical management of such patients. A case is reported of constrictive pericarditis in which the patient developed protracted hypotension which was unresponsive to inotropic therapy, following pericardiectomy. On investigation the cause of hypotension was diagnosed as acute adrenocortical insufficiency, secondary to adrenal tuberculosis. The patient was successfully treated with corticosteroids. Low cardiac output after pericardiectomy, which is not uncommon, should not necessarily be attributed to a ‘myocardial factor’. Patients with tuberculous pericarditis may have co-existent Addison's disease.

Hemodynamic changes in acute adrenal insufficiency.

Acute adrenocortical insufficiency is an unusual cause of isolated shock. The purpose of this study is to describe the cardiovascular changes in 6 patients with acute adrenal insufficiency presenting with hemodynamic instability. Retrospective and prospective study. Medical intensive care unit in a university hospital. 6 patients studied by right cardiac catheterization. Results before glucocorticoid treatment show two possible hemodynamic states: 1) myocardial depression with hypovolemia in 3 patients, and 2) hyperdynamic shock with high cardiac output and diminished systemic arterial resistance in 3 other patients. The 3 patients presenting hyperdynamic shock were all given intravenous fluid therapy of over 20 ml/kg before the first hemodynamic measurement. For 2 other patients with low cardiac index and high systemic arterial resistance studied prospectively, 20 ml/kg intravenous fluid therapy transformed the hemodynamic state to hyperdynamic shock. The cardiovascular effect of glucocorticoid treatment studied in 4 patients was resulted in an improvement in the left ventricular systolic work index. Diagnosis of acute adrenocortical insufficiency must be considered if clinical manifestations are present suggesting septic shock without any obvious infectious cause in patients having undergone considerable intravenous fluid therapy as an initial course of treatment.

Pitfalls in the Management of Acute Adrenocortical Insufficiency: Discussion Paper

In patients with acute adrenocortical insufficiency prompt recognition and treatment may be life-saving. Treatment should be initiated immediately before confirmation of the diagnosis. As shown by these case reports, junior staff on acute medical and surgical services, to whom these patients usually first present, may not appreciate that (a) hyponatraemia and hyperkalaemia, in the absence of renal failure, should immediately suggest the diagnosis of adrenal insufficiency and (b) treatment should precede confirmation of the diagnosis. Attempts to correct hyperkalaemia due to adrenocortical insufficiency with insulin and infusions of dextrose is inappropriate and potentially dangerous but seems to be a not unusual mistake.

Hypothalamic-Pituitary-Adrenocortical Suppression and Recovery in Renal Transplant Patients Returning to Maintenance Dialysis

Sixty-six per cent of a group of 21 renal transplant recipients with chronic renal failure were shown to have adrenal suppression due to glucocorticoid treatment. Gradual withdrawal of steroids in these patients returning to maintenance dialysis therapy was achieved with few symptoms of hypoadrenalism. Adrenal recovery occurred in 52 per cent of patients after three months and 71 per cent after six months. However, the plasma cortisol response to insulin-induced hypoglycaemia, studied in patients in whom adrenal recovery had been demonstrated, was impaired in 46 per cent of cases. These results indicate that corticosteroids in renal transplant recipients induce profound hypothalamic-pituitary-adrenal suppression which is slow to recover. Such patients returning to maintenance dialysis are at risk of acute adrenocortical insufficiency for several months. Although withdrawal of steroids can be achieved safely, cover during periods of stress should be given until the hypothalamic-pituitary-adrenal axis has been shown to respond normally.

HYPOTENSION DURING AND AFTER OPERATION IN GLUCOCORTICOID-TREATED PATIENTS

The frequency and likely aetiology of systemic arterial hypotension occurring either during or following operation, in patients receiving steroids was analysed retrospectively. Hypotension occurred during surgery in 29 of 250 operations. Although patients receiving steroids until shortly before operation appeared more likely to develop hypotension during operation the differences were eliminated when allowance was made for the severity of the disease. In almost half of the instances of hypotension during surgery there was no adequate explanation. Hypotension occurred after surgery on eight occasions; there was one case of acute adrenocortical insufficiency, six of various types of circulatory failure and in one the cause as uncertain.

ADRENAL HEMORRHAGE IN THE ADULT

Bilateral adrenal gland hemorrhage was found in 22 of 2,000 (1.1%) consecutive general hospital autopsies. Clinical features of these 22 patients with diffuse or focal bilateral adrenal hemorrhage have been analyzed and compared with previous series. In our experience, patients with this postmortem finding rarely manifest features of adrenocortical insufficiency and appear instead to die as a consequence of concomitant overwhelming illness, such as septicemia, body surface burns or cardiovascular catastrophe. Two-thirds of the current series had impaired renal function at the time of adrenal hemorrhage and three patients had pituitary gland necrosis. Experimental and clinical observations indicate that the "stressed" adrenal gland--under substantive endogenous or exogenous ACTH stimulation--is unusually susceptible to hemorrhage. While our own experience indicates that bilateral adrenal hemorrhage can infrequently be implicated as a factor contributing to patients' death, the possibility of adrenocortical insufficiency must be considered in patients at risk for adrenal hemorrhage. Such patients are those who are azotemic and have bacteremia, burns, or recent cardiovascular catastrophe, particularly when the latter is managed with anticoagulant administration. In these patients, unexplained clinical deterioration or the appearance of findings consistent with adrenocortical insufficiency mandate measurement of serum cortisol concentration and institution of stress-level corticosteroid replacement therapy until a diagnosis of acute adrenocortical insufficiency can be established or refuted.

ADRENOCORTICAL FUNCTION AND CLINICAL COURSE DURING AND AFTER SURGERY IN UNSUPPLEMENTED GLUCOCORTICOID-TREATED PATIENTS

Adrcnocortical function, as expressed by repeated plasma corticosteroid measurements and the clinical course, were studied in 104 glucocorticoid-treated patients undergoing surgery without supplementary glucocorticoid administration. Eight patients developed otherwise unaccountable hypotension during surgery but only 1 patient showed concomitant low plasma corticosteroid values. Eighteen patients had minimal adrenocortical function without accompanying signs of adrenocortical insufficiency. Postoperatively, 4 patients developed hypotension, which could not be correlated to cortisol deficiency. It is concluded that cortisol is not the prime determinant of blood pressure during and after surgery in glucocorticoid-treated patients, that stress-induced acute adrenocortical insufficiency must be infrequent in these patients, and that an absence of an adrenocortical response to minor surgery cannot be taken as the sole indication of pathological adrenocortical function.