Cushing disease is caused by excessive
adrenocorticotropic hormone (
ACTH) production by the
pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of
Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male
Cushing disease patient who presented with rapid
weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free
cortisol and lack of overnight
cortisol suppression by low-dose
dexamethasone test. The serum
cortisol and 24-hour urine free
cortisol, by high-dose
dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of
ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free
cortisol levels were persistently high. Thus the patient underwent a total
hypophysectomy, since which time he has been treated with
hydrocortisone,
levothyroxine, recombinant
human growth hormone, and
testosterone enanthate. Intravenous
bisphosphonate for
osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a
Cushing disease patient according to growth profile, pubertal status, and responses to
hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of
Cushing disease patients who have undergone transsphenoidal surgery.