- Delayed diagnosis of complex glycerol kinase deficiency in a Chinese male infant: a case report. [Case Reports]
- CONCLUSIONS: Overall, CGKD, although rare, cannot be easily excluded in children with persistent vomiting. Extensive blood tests can help to detect abnormal indicators. Adrenal crisis needs to be avoided as much as possible during corticosteroid replacement therapy.
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- Adrenal insufficiency. [Review]
- Adrenal insufficiency (AI), first described by Thomas Addison in 1855, is characterised by inadequate hormonal production by the adrenal gland, which could either be primary, due to destruction of the adrenal cortex, or secondary/tertiary, due to lack of adrenocorticotropic hormone or its stimulation by corticotropin-releasing hormone. This was an invariably fatal condition in Addison's days with…
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- Management of Patients With Glucocorticoid-Related Diseases and COVID-19. [Review]
- The ongoing coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is a global health crisis affecting millions of people worldwide. SARS-CoV-2 enters the host cells by binding to angiotensin-converting enzyme 2 (ACE2) after being cleaved by the transmembrane protease serine 2 (TMPRSS2). In addition to the lung, gastrointestin…
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- Latent Adrenal Insufficiency: From Concept to Diagnosis. [Review]
- 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 zo…
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- A Novel Intronic Splice-Site Mutation of the CYP11A1 Gene Linked to Adrenal Insufficiency with 46,XY Disorder of Sex Development. [Case Reports]
- A novel CYP11A1: c.1236 + 5G > A was identified, expanding the mutation spectrum of the congenital adrenal insufficiency with 46,XY sex reversal. In a now 17-year-old girl delivered full-term (G2P2, parents unrelated), adrenal failure was diagnosed in the first year of life based on clinical picture of acute adrenal crisis with vomiting, dehydration, weight loss, hypotension, and electrolyte dist…
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- Predictors of 30-day readmissions for adrenal insufficiency: A retrospective national database study. [Journal Article]Clin Endocrinol (Oxf). 2021 08; 95(2):269-276.CE
- CONCLUSIONS: The 30-day all-cause readmission rate was 17.3%. AI was the most common reason for readmission among other causes. Readmissions were associated with increased mortality. CCIs of 3 or more, protein-energy malnutrition and obesity were significant predictors of readmission.
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- Venoarterial extracorporeal membrane oxygenation as bridge to effective treatment in a 19-year-old woman with acute adrenal crisis: a case report. [Case Reports]
- CONCLUSIONS: An Addison crisis requires rapid diagnosis and immediate treatment to end a life-threatening condition caused by critical glucocorticoid deficiency. In patients with non-specific symptoms, such as fatigue, hypotension, weight loss, and hyponatraemia, adrenocortical insufficiency should be considered as differential diagnosis. If patients suffer from an Addison crisis, clinical suspicion requires immediate substitution of hydrocortisone as this is essential for patient's survival. Venoarterial extracorporeal membrane oxygenation therapy can serve as a bridge to diagnosis and effective treatment in patients requiring temporary cardiopulmonary support, especially as salvage intervention for patients in cardiogenic shock. To our knowledge, this is the first case of a young patient with acute Addison crisis and cardiogenic shock, who was successfully salvaged by VA-ECMO support.
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- Endotext: Familial or Sporadic Adrenal Hypoplasia Syndromes [BOOK]
- Congenital adrenal hypoplasia is a rare cause of primary adrenocortical failure, which was first described in 1948. During the last two decades, the genetic basis for several forms of familial adrenal insufficiency syndromes has been elucidated. The molecular mechanisms for these disorders involve a broad spectrum of cellular and physiologic processes, including metabolism, nuclear protein import…
- [The 464th case: sudden convulsion and coma in a patient with acute leukemia]. [Case Reports]Zhonghua Nei Ke Za Zhi. 2018 Jul 01; 57(7):539-541.ZN
- A 46-year-old female patient was diagnosed as mixed phenotype acute leukemia with chief complaints of intermittent gingival swelling and bleeding for 1 week. The induction chemotherapy was not effective. During the second course chemotherapy, the patient had sudden convulsion and coma. She was transferred to the intensive care unit with worsened condition after transient improvement. Her final di…
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- Treatment of Addison's disease during pregnancy. [Journal Article]
- CONCLUSIONS: An increase in glucocorticoid replacement dose is expected to be necessary during pregnancy in a woman with Addison's disease.Patient education regarding steroid cover and symptoms of acute adrenal crisis are fundamental.Monitoring in this period is challenging and remains mainly clinical.The increase in hydrocortisone dose often obviates the need to increase fludrocortisone dose.
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- Thyrotoxicosis leading to adrenal crises reveals primary bilateral adrenal lymphoma. [Journal Article]
- CONCLUSIONS: Primary adrenal lymphoma is a rare cause of adrenal insufficiency, but progression can be fast and fatal.Hyperpigmentation is frequently absent.The presenting symptoms are nonspecific and might mimic infection. Disproportion of the general state with signs of specific organ symptomatology is a diagnostic clue.Infection may precipitate adrenal crisis and worsen thyroid function with further adrenal insufficiency exacerbation.In the context of thyrotoxicosis, there may be little clinical response to a therapeutic trial with standard dose glucocorticoids.High-dose glucocorticoid substitution may be required to achieve clinical stability in thyrotoxic patients.
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- Adrenal crisis secondary to bilateral adrenal haemorrhage after hemicolectomy. [Journal Article]
- CONCLUSIONS: Adrenal haemorrhage is a rare cause of hypoadrenalism, and thus requires prompt diagnosis and management to prevent death from primary adrenocortical insufficiency.Mechanisms of adrenal haemorrhage include reduced adrenal vascular bed capillary resistance, adrenal vein thrombosis, catecholamine-related increased adrenal blood flow and adrenal vein spasm.Standard diagnostic assessment is a non-contrast CT abdomen.Intravenous hydrocortisone and intravenous substitution of fluids are the initial management.A formal diagnosis of primary adrenal insufficiency should never delay treatment, but should be made afterwards.
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- Acute psychosis in the course of treatment of acute adrenal crisis with hydrocortisone in the patient with secondary adrenal insufficiency - a case study. [Case Reports]
- CONCLUSIONS: The case focuses attention on the risk of psychosis connected with the treatment of the adrenal crisis with high doses of Hydrocortisone. Because of the risk of psychiatric complications, the patients treated with high doses of corticosteroids, require an evaluation of risk factors for mental disturbances, and safety precautions in cooperation of endocrinologist and psychiatrist.
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- An endocrinological emergency masquerading as an overdose. [Case Reports]
- Primary adrenocortical insufficiency (Addison's disease) is a potentially life-threatening endocrinological disorder caused by destruction of the adrenal cortex with consequent reduction in glucocorticoid and mineralocorticoid function. Although this is a rare condition, misdiagnosis can result in fatal consequences. Previous case reports have described the neuropsychiatric manifestations of Addi…
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- [Perioperative Addisonian crisis]. [Case Reports]
- An Addisonian crisis marks an acute adrenocortical failure which can be caused by decompensation of a chronic insufficiency due to stress, an infarct or bleeding of the adrenal cortex and also abrupt termination of a long-term glucocorticoid medication. This article reports the case of a 25-year-old patient with Crohn's disease who suffered an Addisonian crisis with hypotension, hyponatriemia and…
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- Autoimmune adrenal insufficiency: recognition and management. [Journal Article]
- The main cause of Addison's disease is an autoimmune organ-specific destruction of the cells in the adrenal cortex by an autoreactive process of activated immune cells directed against the steroid-synthesising enzyme 21-hydroxylase. The diagnosis of Addison's disease is suspected in a patient presenting with symptoms of fatigue, bodyweight loss, anorexia, salt craving, and signs of low blood pres…
- Treatment of acute adrenal insufficiency. [Review]
- Hypoadrenocorticism is caused by a lack of endogenous glucocorticoid and mineralocorticoid. These deficiencies can cause a myriad of clinical signs. This disease is uncommon and its clinical presentation is similar to many much more commonly recognized diseases such as renal failure and various gastrointestinal disorders. Severely affected patients may present in a life-threatening adrenocortical…
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- Acute adrenal insufficiency after a single dose of etomidate. [Case Reports]
- 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 s…
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- Acute adrenal crisis together with unilateral adrenal mass caused by isolated tuberculosis of adrenal gland. [Case Reports]
- CONCLUSIONS: Our case indicates that acute or chronic adrenocortical failure can occur as a result of tuberculosis of the adrenal gland, despite the absence of clinical and laboratory evidence of tuberculosis.
- [Acute adrenocortical insufficiency]. [Review]Medicina (Kaunas). 2002; 38(7):769-75; quiz 776.M
- Severe acute adrenocortical insufficiency or adrenal crisis are often elusive diagnoses that may result in severe morbidity and mortality when undiagnosed or ineffectively treated. Although more than 50 steroids are produced within the adrenal cortex, cortisol and aldosterone are far the most abundant and physiologically active. In primary adrenocortical insufficiency, glucocorticoid and mineral-…
- Acute adrenocortical crisis: three different presentations. [Case Reports]Int J Clin Pract. 2001 Mar; 55(2):141-4.IJ
- The adrenal cortex normally produces three principal steroid hormones: the glucocorticoid cortisol, the mineralocorticoid aldosterone, and a small quantity of sex steroids. In primary adrenocortical insufficiency, there is a deficiency of both cortisol and aldosterone with characteristic clinical and laboratory findings. In contrast, with a pituitary disorder there is isolated hypocortisolism, be…
- Autoimmune polyendocrine syndrome type 1 (APS I) in Norway. [Journal Article]
- CONCLUSIONS: Norwegian patients with APS I clinically resemble patients from Finland and other European countries. The diagnosis APS I must be considered in children and adolescents with chronic mucocutaneous candidiasis, autoimmune adrenocortical failure or hypoparathyroidism in order to avoid fatal complications. Analysis of autoantibodies and mutational analysis of the AIRE gene are valuable diagnostic tools, especially in the early stages of the disease.
- Isolated adrenocorticotropic hormone deficiency: an autopsy case of adrenal crisis. A case report. [Case Reports]
- We present a case of fatal adrenal crisis due to isolated adrenocorticotropic hormone (ACTH) deficiency. Autopsy revealed each adrenal gland weighed 0.9 g and the adrenal cortexes were very thin and atrophic. Additionally, cortisol could not be observed in the adrenal cortex by immunohistochemical staining. Furthermore, urine cortisol and 17-OHCS concentration had decreased to a very low level, 2…
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- Addison disease in patients treated with glucocorticoid therapy. [Case Reports]
- Acute adrenal crisis in patients with unrecognized chronic adrenocortical failure is difficult to diagnose and potentially fatal. We describe 2 patients with acute adrenal crisis whose diagnoses were hindered because of concomitant glucocorticoid treatment. Acute adrenal insufficiency is primarily a state of mineralocorticoid deficiency. Prednisolone and prednisone, the most frequently prescribed…
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- Addisonian crisis following pericardiectomy. [Case Reports]Cardiovasc Surg. 1994 Oct; 2(5):646-8.CS
- 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 hypotens…
- Acute adrenal insufficiency after unilateral adrenalectomy in Cushing's syndrome: precipitation by lithium-induced thyrotoxicosis during cortisol replacement. [Case Reports]
- We present a patient with Cushing's syndrome due to adrenocortical adenoma who developed acute adrenal insufficiency one month after unilateral adrenalectomy. She had received lithium carbonate for five years for manic-depressive psychosis. Drug administration was interrupted for 2 weeks postoperatively and was resumed thereafter. At the adrenal crisis, her serum free T4 and T3 levels were both h…
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- [Glucocorticoid therapy in endocrine disease]. [Review]Nihon Rinsho. 1994 Mar; 52(3):734-9.NR
- Natural and synthetic glucocorticoids have been used not merely to manage chronic and acute adrenocortical insufficiency as replacement therapy, but to manage a wide variety of conditions as pharmacological agents. In endocrine disease, they are also used to achieve pharmacological effects, not necessarily related to normal physiological roles. In general, the desired effects is suppression of im…
- Severe gastrointestinal tract hemorrhage in three dogs with hypoadrenocorticism. [Case Reports]
- Three dogs with gastrointestinal tract bleeding of sufficient severity to necessitate blood transfusion were determined to have hypoadrenocorticism on the basis of adrenocortical response to exogenous adrenocorticotropic hormone. All dogs survived the acute crisis and are being managed with fludrocortisone acetate. Hypoadrenocorticism should be considered in the differential diagnosis of acute se…
- [Perioperative steroid substitution in patients with adrenal cortex diseases]. [Review]Infusionsther Transfusionsmed. 1993 Apr; 20(1-2):47-53.IT
- CONCLUSIONS: The perioperative management of patients with diseases of the adrenal cortex is a continuing challenge to the interdisciplinary cooperation of surgeon, neurosurgeon, gynecologist, anesthesiologist and internist.
- Adrenal crisis in the setting of high-dose ketoconazole therapy. [Case Reports]
- We describe a patient with type I diabetes mellitus and hypothyroidism who developed frank adrenocortical insufficiency while receiving a high-dose ketoconazole therapy for keratitis caused by Acanthamoeba species. While impaired cortisol responses to corticotropin and mildly symptomatic hypoadrenalism have been described previously with ketoconazole therapy, to our knowledge, this case represent…