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Cushing's Syndrome Diagnosis

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Diagnosis ➤ In individuals with at least one abnormal test result (for whom the results could be falsely positive or indicate Cushing's syndrome), ES recommends further evaluation by an endocrinologist to confirm or exclude the diagnosis (1|⊕ ). Subsequent Evaluation ➤ For the subsequent evaluation of abnormal initial test results, ES recommends performing another recommended test (Fig. 1, 1|⊕ ). ➤ ES suggests the additional use of the dexamethasone-suppressed corticotropin-releasing hormone (Dex-CRH) test or the midnight serum cortisol test in specific situations (Fig. 1, 1|⊕ ). ➤ ES suggests against the use of the desmopressin test, except in research studies, until additional data validate its utility (2|⊕ ). ➤ ES recommends against any further testing for Cushing's syndrome in individuals with concordantly negative results on two different tests (except in patients suspected of having the very rare case of cyclical disease) (1|⊕ ). ➤ ES recommends tests to establish the cause of Cushing's syndrome in patients with concordantly positive results from two different tests, provided there is no concern regarding possible non-Cushing's hypercortisolism (Table 2) (1|⊕⊕ ). ➤ ES suggests further evaluation and follow-up for the few patients with concordantly negative results who are suspected of having cyclical disease and also for patients with discordant results, especially if the pretest probability of Cushing's syndrome is high (2|⊕ ). Special Populations/Considerations ➤ Pregnancy: ES recommends the use of UFC and against the use of dexamethasone testing in the initial evaluation of pregnant women (1|⊕⊕⊕ ). ➤ Epilepsy: ES recommends against the use of dexamethasone testing in patients receiving antiepileptic drugs known to enhance dexamethasone clearance and recommends instead measurements of nonsuppressed cortisol in blood, saliva, or urine (1|⊕⊕⊕ ). ➤ Renal failure: ES suggests using the 1-mg overnight DST rather than UFC for initial testing for Cushing's syndrome in patients with severe renal failure (2|⊕ ). ➤ Cyclic Cushing's syndrome: ES suggests use of UFC or midnight salivary cortisol tests rather than DSTs in patients suspected of having cyclic Cushing's syndrome (2|⊕ ). ➤ Adrenal incidentaloma: ES suggests use of the 1-mg DST or late-night cortisol test, rather than UFC, in patients suspected of having mild Cushing's syndrome (2|⊕⊕ ).

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