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SBA Answers and Explanations
Published in Vivian A. Elwell, Jonathan M. Fishman, Rajat Chowdhury, SBAs for the MRCS Part A, 2018
Vivian A. Elwell, Jonathan M. Fishman, Rajat Chowdhury
Adrenal insufficiency (also known as Addison’s disease) results in decreased production of glucocorticoids and mineralocorticoids from the adrenal cortex. It is most commonly a result of destruction of the adrenal cortex by autoimmune adrenalitis. Decreased mineralocorticoid activity results in sodium loss and decreased potassium excretion, with consequent hyperkalaemia, hyponatraemia, volume depletion, and hypotension. Hypoglycaemia may occasionally occur as a result of glucocorticoid deficiency and impaired gluconeogenesis. Stresses such as infections, trauma, or surgery may precipitate a life-threatening adrenal crisis, which may prove fatal unless corticosteroid therapy is begun immediately.
Hormone disorders
Published in Steve Hannigan, Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
Symptoms commonly present in newborns with ambiguous genitalia. Girls often present with an enlarged clitoris and fused labia, whereas boys can have undescended testes and hypospadia, where the opening ofthe uretha is on the underside ofthe penis. In addition, ‘adrenal crises’ occur due to stress or illness. These episodes are characterised by dehydration, low blood pressure, vomiting, low levels of salt and sugar in the blood and raised potassium levels. They require prompt treatment with steroid hormones, otherwise unconsciousness and death can occur. This form of CAH can be either mild or severe, and may be detected in early infancy, in adolescence or not until adulthood. In childhood, very early development of secondary sexual characteristics, such as changes in body shape, acne and the development of pubic hair, may occur. Afected individuals may also grow too fast and too early in childhood, and consequently they stop growing too soon, resulting in a shortened adult stature.
Conditions
Published in Sarah Bekaert, Women's Health, 2018
There are many different grades of severity of CAH depending on the degree of impairment of production of cortisol and aldosterone. In the most severe type of CAH, aldosterone is completely lacking and loss of salt from the body is the most prominent problem – salt-losing CAH accounts for 80% of children with CAH. The loss of salt in the urine is uncontrolled and can cause acute dehydration, very low blood pressure and vomiting. The levels of salt and glucose in the blood fall, and the level of potassium rises. This is known as an adrenal crisis, and requires very urgent treatment as it is a potentially life-threatening condition.
Ten-year trends in adrenal insufficiency admissions
Published in Baylor University Medical Center Proceedings, 2022
Hafeez Shaka, Shannon Manz, Zain El-amir, Farah Wani, Michael Salim, Asim Kichloo
Adrenal insufficiency (AI) is a condition that presents as a lack of endogenously produced glucocorticoids. This could be due to a deficiency of steroids produced in the adrenal cortex, known as primary AI (PAI), or to an adrenocorticotropic hormone deficiency, known as secondary AI (SAI).1 Approximately 144 million individuals in the developed world have Addison disease, a chronic form of autoimmune PAI found most often in women in their fourth decade of life.2,3 Adrenal crisis, an acute form of AI, is the main cause of hospitalization in patients with PAI. This condition may present with hypotension, hypovolemic shock, weakness, anorexia, fever, vomiting, electrolyte abnormalities, confusion, or coma.4 Metabolic stressors may precipitate adrenal crises, most commonly in the form of gastrointestinal illnesses, infections, and febrile illnesses.5 Patients with PAI or SAI have a higher risk of hospitalization than the general population.6 The study utilized nationally representative longitudinal data to estimate epidemiologic trends in AI hospitalizations and outcomes over the past decade.
Bilateral adrenal haemorrhage after a high energetic trauma: a case report and review of current literature
Published in Acta Chirurgica Belgica, 2020
N. Jimidar, D. Ysebaert, M. Twickler, M. Spinhoven, K. Dams, P. G. Jorens
The therapy in case of acute adrenal insufficiency consists of high dose (intravenously) hydrocortisone replacement. Due to the introduction of cortisol in the circulation, endothelial nitric oxide is generated with a subsequent arterial vasodilation with a subsequent need of aggressive fluid resuscitation. Initial electrolyte disturbances will often be reversible after the start of hydrocortisone treatment and subsequently they need no additional correction. As high dose of hydrocortisone may initiate psychiatric symptoms, patients with artificial ventilation and/or inability to express should be carefully monitored. Longer term use of high dose steroids could also result in a higher incidence of venous thrombosis indicating optimal prophylaxis. In selected cases such as ongoing active bleeding, embolization could be an option in the management of adrenal haemorrhage [2,6,7]. In our case, corticosteroids were adequately given before any disastrous adrenal crisis could occur stressing the importance of rapid diagnosis.
Prolonged adrenal insufficiency after high-dose glucocorticoid in infants with leukemia
Published in Pediatric Hematology and Oncology, 2018
Kyoko Moritani, Hisamichi Tauchi, Fumihiro Ochi, Sachiko Yonezawa, Koji Takemoto, Minenori Eguchi-Ishimae, Mariko Eguchi, Eiichi Ishii, Kozo Nagai
Each suffered from prolonged adrenal insufficiency during or after treatment. Adrenal insufficiency was evaluated by dose-modified ITT; dysfunction of HPA was defined as a low peak value of intrinsic cortisol with <18 μg/dL. Symptoms of adrenal crisis in these patients are summarized in Table 1. In Patient 1, after methylprednisone pulse for the treatment of chronic GVHD, oral corticosteroid was gradually tapered for 6 months and hydrocortisone replacement therapy was started due to a high cumulative dose of steroids. However, two days after onset of viral enteritis, the patient was somnolent and had hypoglycemia (blood sugar, less than 30 mg/dL). Drip infusion of glucose and hydrocortisone immediately relieved these symptoms. Hydrocortisone dose was increased in order to avoid a further adrenal crisis event, but nausea and hypoglycemia appeared again after respiratory infection. Insulin tolerance test (insulin, 0.05 U/kg) revealed significant adrenal suppression: intrinsic cortisol, 0.6 μg/dL and ACTH, 5.2 pg/mL at peak value (Figure 1). The patient has been in remission, and intrinsic cortisol levels have remained low with daily hydrocortisone for more than 18 months after initiation of replacement therapy.