Long Term Post Operative Follow-Up of Cushing Syndrome
Long Term Post Operative Follow-Up of Cushing Syndrome
Although most patients with hypercortisolism can be diagnosed and treated the long-term
 effects of hypercortisolism and its treatment are unknown. This study will attempt to answer
 the following questions:

 1. What is the rate of perioperative complications? Patients with Cushing syndrome often
 undergo transsphenoidal surgery of the pituitary gland as treatment for the disease.
 During this surgical procedure the pituitary gland is reached by passing through the
 sphenoid bone. The risk for patients to develop hypopituitarism in the immediate
 postoperative period is unknown. Patients with Cushing syndrome have abnormal levels of
 hormones circulating in the blood and affects of the surgery are often not apparent
 until long after the procedure.

 2. What is the recurrence rate? The recurrence rate of the disease has been estimated
 between 5 - 10%. However, these figures have not been confirmed. If the actual rate of
 recurrence is higher than estimated many patients may elect to undergo radiation therapy
 which has a lower rate of recurrence.

 3. Do any factors in the immediate postoperative period predict who will experience a
 recurrence of Cushing syndrome?

 4. What are the long-term complications of hypercortisolism? Studies have shown that
 patients with hypercortisolism have a four times greater risk of death than people of
 the same age without hypercortisolism. Researchers tend to believe this figure is too
 high. However, it is well established that hypercortisolism weakens bones (decreased
 bone density), causes secondary hypogonadism, increases levels of fat in the blood
 (hyperlipidemia), and decreases thyroid function (hypothyroidism). The potential for
 these conditions to be reversed is not known.

 These questions will be addressed by blood and urine sampling in the postoperative period,
 and by outpatient follow-up and periodic questionnaires in the first 10 years after curative
 surgery for Cushing syndrome performed at the NIH.
Although most patients with hypercortisolism now can be diagnosed correctly and successfully
 treated, the long-term sequelae of hypercortisolism and its treatment are unknown. This study
 addresses the following questions: 1) What is the recurrence rate after successful treatment
 of Cushing's syndrome? 2) Do any factors in the immediate postoperative period predict
 recurrence of Cushing's syndrome? 3) What are the long-term complications of hypercortisolism
 in terms of mortality, morbidity, return of endocrine function and bone density? and 4) What
 is the quality of life for patients after surgical treatment of Cushing's syndrome? While
 most of these questions relate specifically to the care of patients with Cushing syndrome,
 the final question has relevance to the many patients rendered Cushingoid by the therapeutic
 use of glucocorticoids. These questions will be addressed by blood and urine sampling in the
 post-operative period, and by outpatient follow-up and periodic questionnaires in the first
 20 years after curative surgery for Cushing syndrome performed at the NIH.
INCLUSION CRITERIA:

 Patients with Cushing syndrome documented at the NIH will be recruited into this protocol
 prior to surgery intended to cure Cushing syndrome selectively. Thus, patients undergoing
 transsphenoidal exploration for resection of an adenoma, or unilateral adrenalectomy for an
 adenoma, or resection of a tumor producing ACTH ectopically will be candidates for this
 study. We will also recruit as many of the 500 patients treated since 1983 as possible, so
 as to have a group of "late follow-up" patients. These individuals will participate
 primarily by questionnaire.

 Hematocrit about 30%. A CBC will be obtained prior to entering the study. Patients with
 hematocrit above 30% will be accepted into the study; iron replacement will be given to
 those patients with low TIBC.

 Ages 18 - 85. Children less than 18 are being studied under other protocols and the
 questionnaire has not been validated for younger individuals.

 For the questionnaire portion of the protocol there is an additional inclusion criterion:

 Patients must be able to read and write in English.

 EXCLUSION CRITERIA:

 There are no formal exclusion criteria, if the patient meets the inclusion criteria above.
 All ethnic groups and both genders will be recruited. However, patients may be withdrawn
 from the study by the PI if they are unable to meet study requirments, such as mailing
 questionnaires.