- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Adrenal crisis is an acute life-threatening condition precipitated by an internal or external process in the setting of known or unknown adrenal insufficiency and corticosteroid deficiency. It is imp...
Adrenal crisis is an acute life-threatening condition precipitated by an internal or external process in the setting of known or unknown adrenal insufficiency and corticosteroid deficiency. It is important to note that there is a difference between adrenal insufficiency and adrenal crisis. Addison's disease, among other causes, is characterized by long-term adrenal insufficiency, whereas adrenal crisis is an acute process that can present with symptoms such as abdominal pain, vomiting, fever, weakness, among others. However, it is most notably accompanied by cardiovascular collapse with patients found to be in acute distress. While this disease is well described, it is often difficult to recognize, and treatment initiation may be delayed leading to notable morbidity and mortality.
- Acute adrenal crisis and mortality in adrenal insufficiency: Still a concern in 2018! [Journal Article]
- AEAnn Endocrinol (Paris) 2018; 79(3):164-166
- Despite established replacement therapy, mortality in patients suffering from chronic adrenal insufficiency is increasing. This may be partly explained by the fact that lack of adrenal stress hormone...
Despite established replacement therapy, mortality in patients suffering from chronic adrenal insufficiency is increasing. This may be partly explained by the fact that lack of adrenal stress hormones impairs the body's capacity to deal adequately with stress situations, resulting in life-threatening adrenal crises. Since many such situations are of rapid onset, concepts that allow for quick response to emergencies are particularly important. Optimal education for patients and relatives, improved awareness on the part of health professionals and the development of new easy-to-use drugs for acute therapy are of prime importance.
- Mortality data from the European Adrenal Insufficiency Registry-Patient characterization and associations. [Journal Article]
- CEClin Endocrinol (Oxf) 2018 Apr 22
- CONCLUSIONS: This is the first study to provide detailed characteristics of deceased patients with AI. Older, male patients with SAI and frequent AC had a high mortality risk.
- Treatment of Addison's disease during pregnancy. [Journal Article]
- EDEndocrinol Diabetes Metab Case Rep 2018; 2018
- Addison's disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant p...
Addison's disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison's disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms. Hydrocortisone dose was adjusted, and the patient maintained regular follow-up. No complications were reported for the remainder of gestation and delivery. Pregnant patients with Addison's disease should be monitored during gestation and in the peripartum period by multidisciplinary teams. Adjustments in glucocorticoid and mineralocorticoid replacement therapy are often necessary, and monitoring should be based mainly on clinical findings, which becomes increasingly difficult during pregnancy. Patient education and specialized monitoring are key to avoiding complications from under- or over-replacement therapy in this period.
- Hyporeninemic hypoaldosteronism in a patient with diabetes mellitus: an unforgettable case report. [Journal Article]
- IMInt Med Case Rep J 2018; 11:69-72
- A 58-year-old man presented with a 3-year history of chronic and intermittent hyperkalemia requiring recurrent attendances to the emergency department for urgent treatment. His medical history includ...
A 58-year-old man presented with a 3-year history of chronic and intermittent hyperkalemia requiring recurrent attendances to the emergency department for urgent treatment. His medical history included secondary diabetes mellitus following a bout of acute pancreatitis and a previous splenectomy for a spontaneous splenic rupture. He also had a history of prolonged use of non-steroidal anti-inflammatory drugs for back pain and painful neuropathy. He was not on any medication or diet that would cause a raised serum potassium level and his renal function was normal. He was on a basal-bolus insulin regimen but his diabetes control had been poor for several years. As the hyperkalemia had gone on for so long in the presence of normal renal function, he went on to have further tests. Adrenal insufficiency had been ruled out following a short Synacthen test. Further investigations revealed low serum aldosterone levels and inappropriately low serum renin levels in the presence of hyperkalemia. This was suggestive of hyporeninemic hypoaldosteronism (HH). He was then treated with fludrocortisone and furosemide and his serum potassium levels remained normal. Additionally, he did not require any more emergency admissions to treat hyperkalemia thereafter. It was concluded that the HH-induced hyperkalemia was caused by diabetes mellitus or due to a combination of diabetes and prolonged use of non-steroidal anti-inflammatory drugs. The absence of renal impairment may have contributed to the delay in diagnosis. HH is a commonly overlooked cause of hyperkalemia. This case highlights the fact that it should always be suspected when unexplained hyperkalemia is found in patients with only mild-moderately impaired renal function, especially in the presence of diabetes mellitus.
- News about the genetics of congenital primary adrenal insufficiency. [Journal Article]
- AEAnn Endocrinol (Paris) 2018; 79(3):174-181
- Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-...
Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-threatening. Today, 80% of pediatric forms of PAI have a genetic origin but 5% have no clear genetic support. Recently discovered mutations in genes relating to oxidative stress have opened the way to research on genes unrelated to the adrenal gland. Identification of causal mutations in a gene responsible for PAI allows genetic counseling, guidance of follow-up and prevention of complications. This is particularly true for stress oxidative anomalies, as extra-adrenal manifestations may occur due to the sensitivity to oxidative stress of other organs such as the heart, thyroid, liver, kidney and pancreas.
- Adrenal hemorrhage: A single center experience and literature review. [Journal Article]
- ACAdv Clin Exp Med 2018 Mar 27
- CONCLUSIONS: It seems that there is a need to distinguish patients with AH who do not require surgical intervention. Follow-up radiological examination is necessary to reassess the lesion. The patients in whom shrinkage of the tumor can be observed are likely not to require surgical treatment.
- Therapeutic patient education in adrenal insufficiency. [Journal Article]
- AEAnn Endocrinol (Paris) 2018; 79(3):167-173
- It is essential to encourage patient autonomy in the management of their illness, and notably their participation in treatment education programs; specific programs target avoidance or early preventi...
It is essential to encourage patient autonomy in the management of their illness, and notably their participation in treatment education programs; specific programs target avoidance or early preventive treatment of acute adrenal insufficiency, which is a life-threatening complication. Therapeutic patient education is recommended by the two international consensus statements on the management of primary adrenal insufficiency and the French consensus on adrenal insufficiency. Although there is no common international reference framework to date, the objective of the French consensus was to provide a frame of reference to facilitate the development of therapeutic education for patients with adrenal insufficiency. The principal educational objectives were: for the patient to always carry the necessary emergency equipment; be able to identify situations of increased risk and the early signs of adrenal crisis; know how to adjust oral glucocorticoid treatment; be capable of administering hydrocortisone by subcutaneous injection; be able to adjust treatment to different situations (heat, physical exercise, travel); and be able to appropriately use the resources of the healthcare services. Other programs could also be developed to respond to patients' needs and expectations, notably concerning hydrocortisone dose adjustment to avoid overdose in the context of chronic fatigue syndrome.
- A rare cause of primary adrenal insufficiency due to a homozygous Arg188Cys mutation in theSTARgene. [Journal Article]
- EDEndocrinol Diabetes Metab Case Rep 2018; 2018
- Steroidogenic acute regulatory protein (STAR) is a key protein for the intracellular transport of cholesterol to the mitochondrium in endocrine organs (e.g. adrenal gland, ovaries, testes) and essent...
Steroidogenic acute regulatory protein (STAR) is a key protein for the intracellular transport of cholesterol to the mitochondrium in endocrine organs (e.g. adrenal gland, ovaries, testes) and essential for the synthesis of all steroid hormones. Several mutations have been described and the clinical phenotype varies strongly and may be grouped into classic lipoid congenital adrenal hyperplasia (LCAH), in which all steroidogenesis is disrupted, and non-classic LCAH, which resembles familial glucocorticoid deficiency (FGD), which affects predominantly adrenal functions. Classic LCAH is characterized by early and potentially life-threatening manifestation of primary adrenal insufficiency (PAI) with electrolyte disturbances and 46,XY disorder of sex development (DSD) in males as well as lack of pubertal development in both sexes. Non-classic LCAH manifests usually later in life with PAI. Nevertheless, life-long follow-up of gonadal function is warranted. We describe a 26-year-old female patient who was diagnosed with PAI early in life without detailed diagnostic work-up. At the age of 14 months, she presented with hyperpigmentation, elevated ACTH and low cortisol levels. As her older brother was diagnosed with PAI two years earlier, she was put on hydrocortisone and fludrocortisone replacement therapy before an Addisonian crisis occurred. Upon review of her case in adulthood, consanguinity was noted in the family. Genetic analysis for PAI revealed a homozygous mutation in theSTARgene (c.562C>T, p.Arg188Cys) in both siblings. This mutation has been previously described in non-classic LCAH. This case illustrates that early onset, familial PAI is likely due to autosomal recessive genetic mutations in known genes causing PAI.
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- Impairment of Thyroid Function in Critically Ill Patients in the Intensive Care Units. [Journal Article]
- AJAm J Med Sci 2018; 355(3):281-285
- Unexplained hypotension in the intensive care unit is commonly attributed to volume depletion, cardiorespiratory failure, sepsis, or relative adrenal insufficiency. In these acute conditions, thyroid...
Unexplained hypotension in the intensive care unit is commonly attributed to volume depletion, cardiorespiratory failure, sepsis, or relative adrenal insufficiency. In these acute conditions, thyroid hormone levels measured in blood, serum or plasma are often altered and solely attributed to critical illness. We report a series of 3 critically ill patients with prolonged respiratory failure, suppressed mental status and unexplained hypotension. Thyroid stimulating hormone levels ranged from normal to mildly elevated (2.36-7.65IU/mL; normal: 0.27-4.20), but free thyroxin was markedly suppressed (0.239-0.66ng/dL; normal: 0.93-1.70). After initiation of intravenous levothyroxine (75-100μg/day), the patients could be weaned off vasopressors and were successfully extubated shortly thereafter. These cases demonstrate that hypothyroid intensive care unit patients may exhibit even seemingly normal or mildly abnormal thyroid stimulating hormone values. Early recognition and treatment of a hypothyroid state superimposed on critical illness may contribute to recovery from hypotension or the need for mechanical ventilation.