Clinical Presentation

CS is a multisystem disorder (8). The typical clinical presentation of CS results primarily from excess glucocorticoids (hypercortisolism) and, to a lesser extent, from excess mineralocorticoids (hypermineralocorticoidism) and/or adrenal androgens (hyperandrogenism).

One of the earliest signs in most all patients with CS (including CD) is obesity; in growing children this is combined with growth deceleration or arrest (9-12). The accumulation of visceral fat in patients with CS, a result of excess cortisol and insulin secretion, is associated with the full expression of the metabolic syndrome X (hypertension, hyperlipidemia, hypercoagulation, insulin resistance) and its long-term (cardiovascular) sequelae. Typical signs of glucocorticoid excess are fat accumulation in the face (especially temple area and cheeks), neck (buffalo hump, supraclavicular fossae), trunk, abdomen, and epidural fat, with sparing of the limbs (8,13,14). Connective tissue atrophy leads to thin fragile skin, violaceous striae, and easy bruising. Hirsutism and a variable degree of acne (depending on the individual sensitivity to glucocortioids and androgens) can also be seen. Although mild to moderate hypertension often are present in patients with CD, hypokalemia and hyperpigmentation occur less frequently, because the ACTH and cortisol levels are usually less elevated than in patients with ectopic ACTH-secreting tumors.

Hypercalciuria with subsequent renal calculi, glucocorticoid-induced osteopenia/osteoporosis, menstrual irregularities (e.g., amenorrhea), loss of libido in both genders (hypogonadism secondary to hypercortisolism), and muscle weakness in association with proximal muscle atrophy are common features of CD/CS. Avascular necrosis of the hip can be the presenting manifestation of an ACTH-secreting pituitary adenoma and requires immediate attention to save the femoral head and avoid subsequent disability (15). Cataracts (classically posterior subcapsular) and glaucoma frequently occur in patients with exogenous CS but not in those with endogenous hypercortisolism (16). Psychiatric manifestations in CD include cognitive deficits with memory loss and poor concentration, anxiety with insomnia, irritability, atypical depression, acute psychosis, and mania (17-19).

Some patients with CS may experience alternating episodes of mania and depression, pointing to the diagnosis of so-called periodic or cyclic glucocorticoid excess with intermittent hypersecretion of cortisol (20-32). Children with CS often are often compulsive and high performers in school.

Increased awareness of the possible diagnosis of CS, for instance, by improved contemporary imaging modalities ("incidentaloma") and biochemical testing, led to the term "subclinical" CS, a state of glucocorticoid excess without the fullblown clinical features (33).

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