Primary Insufficiency

Loss of cortex

••Congential adrenal hypoplasia

••••X-linked adrenal hypoplasia (DAX-1 gene on Xp21)

••••"Miniature" type adrenal hypoplasia (unknown cause)

••Adrenoleukodystrophy (ALD gene on Xq28)

••Autoimmune adrenal insufficiency

••••Autoimmune polyendocrinopathy syndrome type 1 (AIRE-1 gene on 21q22)

••••Autoimmune polyendocrinopathy syndrome type 2 (polygenic)

••••Isolated autoimmune adrenalitis (polygenic)

••Infection

••••Acquired immune deficiency syndrome

••••Tuberculosis

••••Fungi

••••Acute hemorrhagic necrosis (Waterhouse-Friderichsen syndrome)

••Amyloidosis, sarcoidosis, hemochromatosis

••Metastatic carcinoma

Metabolic failure in hormone production

••Congenital adrenal hyperplasia (cortisol and aldosterone deficiency with virlization)

••Drug- and steroid-induced inhibition of adrenocorticotropic hormone or cortical cell function

Secondary Insufficiency

Hypothalamic pituitary disease

••Neoplasm, inflammation (sarcoidosis, tuberculosis, pyogens, fungi)

Hypothalamic pituitary suppression

••Long-term steroid administration

••Steroid-producing neoplasms

Waterhouse-Friderichsen Syndrome

This uncommon but catastrophic syndrome is characterized by the following:

• An overwhelming bacterial infection, which is classically associated with Neisseria meningitidis septicemia but occasionally is caused by other highly virulent organisms, such

as Pseudomonas species, pneumococci, Haemophilus influenzae, or staphylococci

• Rapidly progressive hypotension leading to shock

• Disseminated intravascular coagulation with widespread purpura, particularly of the skin

• Rapidly developing adrenocortical insufficiency associated with massive bilateral adrenal hemorrhage

Figure 24-49Waterhouse-Friderichsen syndrome in a child. The dark, hemorrhagic adrenal glands are distended with blood.

Figure 24-50Waterhouse-Friderichsen syndrome. At autopsy, the adrenals were grossly hemorrhagic and shrunken; microscopically, little residual cortical architecture is discernible.

Figure 24-51Autoimmune adrenalitis. In addition to loss of all but a subcapsular rim of cortical cells, there is an extensive mononuclear cell infiltrate.

Figure 24-52Adrenal carcinoma. The hemorrhagic and necrotic tumor dwarfs the kidney and compresses the upper pole.

Figure 24-53Adrenal carcinoma (A) revealing marked anaplasia, contrasted with normal cortical cells (B).

Figure 24-54The paraganglion system. This schematic representation of the paraganglion system demonstrates sites of paraganglion cell nests, in which neoplasms may form. The extraadrenal

portion of the paraganglion system is grouped into three families based on anatomic distribution, innervation, and microscopic structure: (1) branchiomeric, (2) intravagal, and

(3) aorticosympathetic. (From Whalen RK, et al: Extra-adrenal pheochromocytoma. J Urol 147:1–10, 1992; copyright Williams & Wilkins, 1992.)

TABLE 24-11-- Familial Syndromes Associated with Pheochromocytoma

Date: 2016-04-22; view: 701