Diagnostic puzzler: Hypertension in teen

Don’t try to lower BP through usual means
Hypertension, a hallmark finding in approximately 80% of adults and 47% of children with Cushing’s syndrome,⁵ stems from hypercortisol-driven pathologic changes in the mechanisms controlling plasma volume, peripheral vascular resistance, and cardiac output. In addition, these cortisol-driven changes have a direct effect on mineralocorticoid and glucocorticoid receptors within the central nervous system. Secondary effects such as insulin resistance and the development of sleep apnea further complicate the management of this generally treatment-resistant hypertension. Lastly, specific mechanisms such as the cross-reactivity of excess glucocorticoids with mineralocorticoid receptors acting on targets within the kidney, can also lead to metabolic derangements, such as profound hypokalemia and metabolic alkalosis.

Thus, controlling hypertension and the metabolic changes seen in Cushing’s syndrome often requires addressing the underlying hypercortisolism rather than achieving normotension and normal serum electrolytes through the usual means.⁵

Treatment puts our patient back on track
Our patient was transferred to a tertiary care hospital for further management and consultation with endocrinology and neurosurgery. He was started on high-dose ketoconazole, an imidazole-derivative antifungal medication that acts to inhibit adrenal steroidogenesis and has been used successfully in patients with Cushing’s syndrome.^6,7 (Ketoconazole is typically dosed at 400-1200 mg/d⁷ and can be used for >6 months to 1 year, or temporarily in advance of surgery.)

Our patient underwent successful transsphenoidal adenectomy by neurosurgery, and his blood pressure, serum electrolytes, and serum glucose returned to normal levels. He is about to begin his senior year in high school.

CORRESPONDENCE Michael Barna, MD, Department of Family Medicine, Naval Hospital Camp Pendleton, Box 555191, Camp Pendleton, CA 92055; michael.barna@med.navy.mil