Hypotension case 1

Hypotension case 1

A 32-year-old woman presents to an emergency department and loses consciousness while going through the financial screening process.
According to her husband, she has been having severe vomiting and diarrhea. He took her to the emergency room when she also began to
have severe pain in her back, abdomen, and legs. Physical examination demonstrates marked hypotension, dry mucus membranes, Ioss of
skin turgor, and a generalized hyperpigmentation of the skin that is most noticeable on scars, skin folds, knees, knuckles, and lips.

Q 1
Which of the following features of this woman's presentation is most suggestive of the likely diagnosis?
/ A. Abdominal pain
/ B. Hyperpigmentation
/ C. Hypotension
/ D. Loss of consciousness
/ E. Vomiting

Q 2
Which of the following is the most likely diagnosis?
/ A. Addison disease
/ B. Diabetes mellitus type 2
/ C. Hyperthyroidism
/ D. Hypoparathyroidism
/ E. Pheochromocytoma

Q 3
This patient's severe hypotension is most likely related to abnormal serum levels of which of the following hormones?
/ A. AIdosterone
/ B. Epinephrine
/ C. Insulin
/ D. Parathyroid hormone
/ E. Thyroxine

Q 4
Which of the following is the most common cause in the United States of this patient's probable condition?
/ A. Amyloidosis
/ B. Autoimmune disease
/ C. Metastatic cancer
/ D. Surgical removal
/ E. Tuberculosis

Q 5
If this patient had been a child who had a familial form of this disease, which of the following would most likely also be affected?
/ A. Exocrine pancreas
/ B. Pancreatic islets
/ C. Parathyroid gland
/ D. Salivary glands
/ E. Thyroid gland

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Hypotension case 1 answers

A1
The correct answer is B. One of the tricks of physical diagnosis is to have a sense of which features of a patient's presentation are most suggestive of specific illnesses. This woman is obviously severely ill, but the majority of her signs and symptoms (including those listed in choices A, C, D, and E) are very nonspecific and may be seen in many different diseases affecting a variety of organ systems. The most specific finding is the unusual pattern of hyperpigmentation.


A2
The correct answer is A. While roughly 75% of patients with Addison disease have been previously diagnosed when they develop a crisis, the remaining 25% have not. Most patients' symptoms are nonspecific (although usually severe) and many physicians have never seen a case (Addison disease is rare, with an incidence of 1 in 100,000). These facts together mean that this potentially fatal crisis is very often misdiagnosed, and vital time is wasted while working the patient up as a probable sepsis, surgical abdomen, gastrointestinal illness, or back pain case. An alert physician who notices the hyperpigmentation of both sun-exposed and unexposed skin and also knows to associate this with Addison disease could save this patient's life. (A helpful clue in patients with naturally pigmented skin is to look for the specific areas of hyperpigmentation mentioned in the case history.) Those patients who do not present with a crisis may come to medical attention because of chronic fatigue and muscle weakness, loss of appetite with weight loss, chronic nausea and vomiting, hyperkalemia picked up on routine serum chemistry screens, hypotension with fainting spells, or irritability and depression. Other features can include craving of salty foods, hypoglycemia, and irregular menstrual periods. The symptoms usually develop slowly, and may be ignored until exacerbated by illness or accident, thereby producing an Addisonian crisis. Once Addison disease is suspected, the diagnosis can be confirmed with an ACTH stimulation test, in which cortisol secretion is measured after exogenous ACTH is administered.
A patient with undiagnosed type 2 diabetes mellitus 2 (choice B) might also lose consciousness (most likely due to hyperosmolar coma) in the emergency room, but would not have the skin pigmentation, pain, or nausea and vomiting seen in this case. Hyperthyroidism (choice C) causes heat intolerance, nervousness, and can occasionally produce an arrhythmia severe enough to cause loss of consciousness, but would not cause the skin pigmentation, pain, or nausea and vomiting seen in this case.
Hypoparathyroidism (choice D) causes hypocalcemia with tetany, but would not cause this patient's symptom pattern.
Pheochromocytoma (choice E) could cause nausea and vomiting, abdominal pain, and loss of consciousness secondary to arrhythmia, but would not cause hyperpigmentation.


A3
The correct answer is A. The hormones that are deficient in Addison disease are cortisol (always) and aldosterone (sometimes). Aldosterone is a mineralocorticoid produced by the adrenal cortex that normally helps the body maintain blood pressure and water and salt balance. It acts by stimulating the kidney to retain sodium and excrete potassium. Hypotension and hyperkalemia can develop when aldosterone levels are too low. Cortisol also helps to maintain blood pressure and cardiovascular function, and has additional actions, including modulating the inflammatory response and opposing insulin's actions in the regulation of protein, carbohydrate, and fat metabolism. Treatment of patients with Addison disease usually includes glucocorticoid replacement (often with oral hydrocortisone) and may include mineralocorticoid replacement (typically with oral fludrocortisone).
Epinephrine (choice B) stimulates the adrenergic system and tends to increase blood pressure secondary to cardiotropic and vasoactive effects.
Insulin (choice C) affects the metabolism of carbohydrate, fat, and protein, but does not directly alter blood pressure.
Parathyroid hormone (choice D) affects serum calcium levels.
Thyroxine (choice E) tends to increase metabolic rate, and, when deficient, may be associated with low blood pressure, but would not produce the clinical presentation seen with this patient.



A4
The correct answer is B. Primary adrenal insufficiency, or Addison disease, only develops when at least 90% of the adrenal cortex has been destroyed. While, at the time that Addison initially described the disease, tuberculosis (choice E) was the most common cause (and still causes approximately 20% of cases), medical control of tuberculosis has made this cause much less likely, and a gradual autoimmune destruction of the adrenal cortex now causes approximately 70% of all cases of Addison disease in developed countries. Less common causes of Addison disease include chronic infections (usually fungal), metastatic cancer (choice C), amyloidosis (choice A), and surgical removal of the adrenal glands (choice D). Secondary adrenal insufficiency differs from primary adrenal insufficiency by the lack of skin pigmentation, and is usually due to a lack of pituitary-secreted ACTH, most commonly either following cessation of glucocorticoid therapy for other diseases, or removal of an ACTH-producing tumor of the pituitary gland. Secondary adrenal insufficiency often eventually resolves spontaneously, although interim hormonal support is usually required.

A5
The correct answer is C. The autoimmune form of Addison disease can occur either as an isolated condition or as part of a polyendocrine deficiency syndrome, which is possibly inherited, since multiple family members may develop endocrine deficiencies. These associations are important because patients with autoimmune Addison disease should be initially evaluated, and then periodically screened for development of other endocrine deficiencies. Type I polyendocrine deficiency develops in childhood, and may include adrenal insufficiency, hypoparathyroidism (choice C), pernicious anemia, chronic active hepatitis, chronic Candida infections, and slow sexual development. Type II polyendocrine deficiency (Schmidt syndrome) develops in young adults and may include adrenal insufficiency, hypothyroidism (choice E), diabetes mellitus type I (affecting the pancreatic islets, choice B), vitiligo, and slow sexual development. The exocrine pancreas (choice A) and the salivary glands (choice D) are not affected in either type