Cushing syndrome (CS), a fatal disease, is suspected in many thousands of patients each year, but confirmed in only a fraction of these. This project seeks to identify accurately which patients have Cushing syndrome, to define the etiology of their disease and to treat it optimally. A major initiative in the past year has been to determine whether octreotide scintigraphy (using a 6mCi dose of 111Indium pentetreotide) is a useful imaging modality for the localization of ectopic ACTH-secreting tumors. 18 consecutive patients with presumed ectopic ACTH secretion received octreotide scintigraphy as well as CT and MRI. Tumor was detected initially in 7/18 patients, and in 3/18 during follow-up. No ACTH-secreting tumor was detected by octreotide scintigraphy when CT/MRI were negative, although octreotide confirmed the presence of tumor in 6/10 patients. 17 of 40 scintigrams were abnormal. Of these, 10 represented tumor, 6 were falsely positive, and one non-ACTH-secreting hepatic lesion was detected by CT also. We conclude that octreotide may be a useful adjunctive modality, but is not a significant advance over conventional imaging, and should not be used alone for the localization of these tumors.An additional initiative was to evaluate the utility of salivary cortisol as a screening test for Cushings syndrome. Salivary cortisol has a high diagnostic accuracy for the identification of Cushings syndrome in adults. - Human Subjects

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Intramural Research (Z01)
Project #
1Z01HD000638-06
Application #
6290187
Study Section
Special Emphasis Panel (DEB)
Project Start
Project End
Budget Start
Budget End
Support Year
6
Fiscal Year
1999
Total Cost
Indirect Cost
City
State
Country
United States
Zip Code
Raff, Hershel; Sharma, Susmeeta T; Nieman, Lynnette K (2014) Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing's syndrome, adrenal insufficiency, and congenital adrenal hyperplasia. Compr Physiol 4:739-69
Sharma, S T; Nieman, L K (2013) Is prolactin measurement of value during inferior petrosal sinus sampling in patients with adrenocorticotropic hormone-dependent Cushing's Syndrome? J Endocrinol Invest 36:1112-6
Sharma, S T; Nieman, L K (2012) Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion. Eur J Endocrinol 166:531-6
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Nieman, Lynnette K; Biller, Beverly M K; Findling, James W et al. (2008) The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93:1526-40
Nieman, Lynnette (2007) Editorial: The dexamethasone-suppressed corticotropin-releasing hormone test for the diagnosis of Cushing's syndrome: what have we learned in 14 years? J Clin Endocrinol Metab 92:2876-8
Baid, Smita K; Sinaii, Ninet; Wade, Matt et al. (2007) Radioimmunoassay and tandem mass spectrometry measurement of bedtime salivary cortisol levels: a comparison of assays to establish hypercortisolism. J Clin Endocrinol Metab 92:3102-7
Nieman, Lynnette K (2007) Screening for reversible osteoporosis: is cortisol a culprit? Ann Intern Med 147:582-4
Newell-Price, John; Bertagna, Xavier; Grossman, Ashley B et al. (2006) Cushing's syndrome. Lancet 367:1605-17
Weil, Robert J; Vortmeyer, Alexander O; Nieman, Lynnette K et al. (2006) Surgical remission of pituitary adenomas confined to the neurohypophysis in Cushing's disease. J Clin Endocrinol Metab 91:2656-64

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