Primary Adrenal Insufficiency (PAI)

 Al-Jurayyan NA
Background: Primary adrenal insufficiency (PAI) in children is an uncommon, but potentially fatal. The current symptoms include weakness, fatigue, anorexia, abdominal pain, weight loss, orthostatic hypotension, salt craving and characterized by hyperpigmentation.
Material and Methods: This is a retrospective, hospital based-study, conducted at King Khalid University Hospital (KKUH), during the period January 1989 and December 2014. Review of medical record of patient diagnosed with primary adrenal insufficiency. The diagnosis was based on medical history, physical examination and low levels of glucocorticoids and raised adrenocorticotropic hormone (ACTH). Appropriate laboratory and radiological investigations were also reviewed.
Results: During the period under review, January 1989 and December 2014, a total of 125 patients with the diagnosis of primary adrenal insufficiency were seen. Inherited disorders like congenital adrenal hyperplasia and hypoplasia were common, 85.5%. However, variable autoimmune mediated etiologic diagnosis accounted for, 13%, were also seen. The appropriate various laboratory and radiological investigations should be planned.
Conclusion: Although, congenital adrenal hyperplasia was the commonest etiology, however, congenital adrenal hypoplasia should not be over looked. The diagnosis of PAI can be challenging in some patients, and therefore appropriate serological and radiological investigations should be done.

Given Adrenal Symptoms, Blood Test Recommended

adrenal-glands

 

Q: My husband’s recent CT scan of his stomach and digestive system revealed that he has nodules on both adrenal glands. It was suggested that he undergo a blood test to determine whether the nodules are producing hormones.

For 21 months, he has experienced high blood pressure, nausea, diarrhea, anxiety and abdominal pain. Could this be the source of his problems? If so, what course of action would you recommend?

A: The adrenal gland is responsible for the production of several essential hormones.

Tumors, or nodules, of the adrenal glands are common. They can be categorized into those that make hormones and those that don’t, and also by whether the tumors are benign or malignant.

The most common, by far, are benign, nonfunctioning tumors. These are usually discovered on an ultrasound or a CT scan obtained for some other reason.

More than 4 percent of people have an adrenal mass, and 85 percent are nonfunctional.

The symptoms that your husband has, however, raise a concern that he might have a hormone-producing tumor.

Four types of hormones are commonly produced by adrenal tumors: cortisone, aldosterone, sex hormones (estrogen or androgens) and catecholamines (epinephrine and norepinephrine).

A cortisone-producing adrenal tumor causes Cushing’s syndrome. It usually causes weight gain, especially in the abdomen; skin changes, including striae, or “stretch marks”; high blood pressure; and a predisposition to diabetes. Anxiety and abdominal pain are uncommon.

Aldosterone raises blood pressure, so a person with a functioning adrenal tumor making aldosterone usually has high blood pressure, but the other symptoms you mention for your husband aren’t common for this type of tumor.

Adrenal tumors that make epinephrine and the related norepinephrine are called pheochromocytomas. Hypertension is almost universal with this condition, and anxiety is frequently reported.

Tumors that produce sex hormones are rare, and they are present in men with androgen excess or feminization, in the case of estrogen-secreting tumors.

Although your husband’s symptoms aren’t specific for any one condition, the combination of his symptoms and adrenal nodules concerns me.

I agree with the recommendation to look for excess amounts of hormones in the blood. This can often be achieved with a simple blood test; however, a catheter is occasionally placed in the adrenal vein to sample blood coming from the gland (and its nodule) directly.

By comparing one side against the other, doctors can determine which side might be producing excess hormones.

An endocrinologist is the expert most likely to be familiar with these conditions.

Dr. Roach answers letters only in his North America Syndicate column but provides an order form of available health newsletters at http://www.rbmamail.com. Write him at 628 Virginia Dr., Orlando, FL 32853-6475; or ToYour GoodHealth@med. cornell.edu.

From http://www.dispatch.com/content/stories/life_and_entertainment/2015/07/27/given-adrenal-symptoms-blood-test-recommended.html

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