Pediatric Examination and Board Review (72 page)

(A) serum sodium 127, potassium 2.5 mEq/L; urine potassium 200 mEq/day

(B) serum sodium 127, potassium 3.5 mEq/L; urine potassium 15 mEq/day

(C) serum sodium 127, potassium 7.5 mEq/L; urine potassium 100 mEq/day

(D) serum sodium 147, potassium 7.5 mEq/L; urine potassium 5 mEq/day

(E) serum sodium 127, potassium 7.5 mEq/L; urine potassium 5 mEq/day

3.
What would you expect this patient’s adrenocorticotropic hormone (ACTH) and cortisol level to be before treatment?

(A) low ACTH, low cortisol

(B) low ACTH, high cortisol

(C) high ACTH, low cortisol

(D) high ACTH, high cortisol

(E) normal ACTH, low cortisol

4.
All of the following signs and symptoms except which one should clue you in to a diagnosis of primary adrenal insufficiency?

(A) hyperpigmentation

(B) anorexia

(C) weight loss

(D) hepatosplenomegaly

(E) vomiting

5.
Which of the following is not a clinical manifestation of Addison disease?

(A) unexplained hypoglycemia

(B) vitiligo

(C) neutropenia

(D) nausea

(E) weakness

6.
Which of the following statements regarding adrenal insufficiency is (are) true?

(A) adrenal crisis is rare in patients with secondary adrenal insufficiency

(B) hyperpigmentation is common in secondary adrenal insufficiency

(C) hyperkalemia is commonly seen at presentation in secondary adrenal insufficiency

(D) the major cause of adrenal crisis is glucocorticoid deficiency

(E) all of the above

7.
Which of the following would be the preferable treatment for a child in adrenal crisis?

(A) IV methylprednisolone

(B) IV hydrocortisone

(C) IV dexamethasone

(D) A or B

(E) any of the above

8.
On physical examination, the patient has candidiasis in the mouth and noticeable vitiligo on the face and trunk. Which of the following tests should you check to help with your diagnosis?

(A) magnesium

(B) CBC

(C) immunoglobulin levels

(D) urinalysis

(E) calcium

9.
Your patient also describes neurologic symptoms. What would be at the top of the differential?

(A) adrenoleukodystrophy

(B) infectious adrenalitis

(C) drug-induced adrenal failure

(D) autoimmune adrenalitis

(E) tuberculosis

10.
Which of the following has been associated with adrenal hemorrhage in children?

(A)
Pseudomonas aeruginosa
sepsis

(B)
Escherichia coli
sepsis

(C) meningococcemia

(D) neonatal asphyxia

(E) all of the above

11.
Addison disease is most commonly caused by which of the following?

(A) infectious adrenalitis

(B) autoimmune adrenalitis

(C) adrenal hemorrhage

(D) metastatic cancer

(E) trauma

12.
Which of the following statements regarding serum cortisol concentrations is true?

(A) serum cortisol concentrations are highest in the early morning

(B) serum cortisol concentrations are highest in the early afternoon

(C) serum cortisol concentrations are highest in the early evening

(D) serum cortisol concentrations are highest in the late evening

(E) serum cortisol concentrations do not vary during the day

13.
You diagnose your patient with Addison disease. Which of the following is true regarding outpatient treatment?

(A) he should be discharged on fludrocortisone but only needs glucocorticoid during times of stress

(B) he should be discharged on an oral glucocorticoid but only needs fludrocortisone during times of stress

(C) he only needs glucocorticoid at discharge

(D) he only needs glucocorticoids and fludrocortisones during times of stress

(E) he should be discharged on glucocorticoid and fludrocortisone

14.
Your patient is doing well at home on his medications and his skin pigmentation has faded. His mother calls because he has developed a low-grade fever with diarrhea. Which of the following would be the most appropriate initial management?

(A) he should double his fludrocortisone dose

(B) he should come directly to your clinic

(C) he should double his hydrocortisone dose

(D) he should make no changes in his medications

(E) he should come directly to the emergency department

15.
One year after discharge, the patient notes that his skin pigmentation is beginning to darken again. What would be your major concern?

(A) poor compliance with the glucocorticoid

(B) poor compliance with fludrocortisone

(C) development of ACTH resistance

(D) development of insulin resistance

(E) development of cortisol resistance

16.
Which of the following is (are) not a cause of secondary adrenal insufficiency?

(A) panhypopituitarism

(B) autoimmune adrenalitis

(C) isolated ACTH deficiency

(D) megestrol acetate ingestion

(E) A and D

17.
Which of the following statements regarding secondary adrenal insufficiency is (are) not true?

(A) hyponatremia can occur

(B) hyperkalemia can occur

(C) skin hyperpigmentation does not occur

(D) weakness and fatigue are common symptoms

(E) A and B

18.
Which of the following is the most common cause of tertiary adrenal insufficiency?

(A) cranial radiation

(B) hypothalamic tumors

(C) head trauma

(D) pituitary tumor

(E) chronic administration of high doses of glucocorticoids

ANSWERS

1.
(A)
IV fluids and stress dose glucocorticoids. This adolescent can have many clinical manifestations of Addison disease and is likely to manifest an adrenal crisis. Thus it is imperative to treat him with IV fluids and stress doses of glucocorticoids before any other management. Normal saline with additional glucose should be used as the IV fluids to replace sodium loss and correct hypoglycemia. An emergency exploratory laparotomy without pretreatment with stress dose glucocorticoids could lead to a catastrophic outcome.

2.
(E)
Serum sodium 127, potassium 7.5 mEq/L; urine potassium 5 mEq/day. Hyponatremia is a common feature of primary adrenal insufficiency secondary to mineralocorticoid deficiency and inappropriate vasopressin secretion caused by glucocorticoid deficiency. Mild hyponatremia can also occur in secondary or tertiary adrenal insufficiency because of inappropriate vasopressin secretion. Hyperkalemia is only seen in primary adrenal insufficiency.

3.
(C)
High ACTH, low cortisol. In adrenal insufficiency of any cause, the serum cortisol will be low. The ACTH level will help distinguish between primary adrenal insufficiency (in which ACTH levels will be high) and secondary or tertiary adrenal insufficiency (in which ACTH levels will be low).

4.
(D)
Hepatosplenomegaly. The onset of adrenal insufficiency is often insidious. The presenting signs and symptoms depend on how quickly adrenal function is diminished and whether mineralocorticoid production is affected along with glucocorticoid production. Adrenal insufficiency is often first detected when a stress precipitates an adrenal crisis. Hyperpigmentation in areas exposed to sunlight, areas such, as the palmar creases, axilla, areola, and areas exposed to friction such as the elbows, knees, belt line, and knuckles, is the most characteristic finding of Addison disease and is present in most patients. Anorexia and weight loss are also seen in primary adrenal insufficiency along with other GI symptoms such as vomiting, abdominal pain, diarrhea, and constipation. Dehydration caused by vomiting and diarrhea can often precipitate an adrenal crisis. Although splenomegaly can be seen in primary adrenal insufficiency, hepatomegaly is not a common finding.

5.
(C)
Neutropenia. Unexplained hypoglycemia is found in Addison disease but tends to be more common in younger patients. In adults, it can be precipitated by fever or infection. Although hyperpigmentation is the major manifestation of Addison disease, vitiligo can be seen in patients with autoimmune causes of adrenal insufficiency because of autoimmune destruction of dermal melanocytes. Patients can have eosinophilia. Neutropenia is not a typical clinical manifestation of Addison disease. Other clinical manifestations include generalized weakness, fatigue, postural dizziness, diffuse myalgia, behavioral changes, and splenomegaly.

6.
(A)
Adrenal crisis is rare in patients with secondary adrenal insufficiency. The major cause of adrenal crisis is mineralocorticoid deficiency and not glucocorticoid deficiency. Secondary adrenal insufficiency occurs because of ACTH deficiency, which affects only the glucocorticoid production. Patients with secondary or tertiary adrenal insufficiency typically have normal aldosterone production, which is under the control of the renin-angiotensin system. Thus they have normal serum potassium and rarely present in adrenal crisis. The hyperpigmentation is found only in primary adrenal insufficiency and occurs because of chronic ACTH hypersecretion.

7.
(B)
IV hydrocortisone. In a patient in adrenal crisis, it is important to replace both the deficient glucocorticoid as well as the deficient mineralocorticoid. Of the above, only hydrocortisone has significant mineralocorticoid activity if given at stress doses IV. Unfortunately, currently no IV form of mineralocorticoid is available. Fludrocortisone is a potent oral synthetic mineralocorticoid that can be used once the patient is stable and ready to discontinue IV saline.

8.
(E)
Calcium. The presence of vitiligo with primary adrenal insufficiency suggests an autoimmune etiology. Autoimmune polyglandular syndrome type 1 is a rare autosomal recessive disorder in which primary adrenal insufficiency is associated with chronic mucocutaneous candidiasis and hypoparathyroidism. The candidiasis and hypoparathyroidism typically appear first in early to mid-childhood, and adrenal insufficiency usually develops in mid to late adolescence. Thus it would be important to screen this patient for hypocalcemia. Other common associated manifestations include primary hypogonadism and malabsorption syndromes. Patients rarely develop diabetes mellitus and autoimmune thyroiditis. In contrast, in autoimmune polyglandular syndrome type 2, adrenal insufficiency is typically the initial manifestation. Hypoparathyroidism does not occur in this disorder, and diabetes mellitus and autoimmune thyroiditis are common.