Table 2.

Biochemical and dynamic testing results of multiple endocrine neoplasia type 1 patients with Cushing’s syndrome.

Patient ID	UFC (nmol/d) or [17OHCS (µmol/d)] (a)	Plasma ACTH (pmol/l) (b)	CRH Stim Test ( c )	HD Dex Suppr Test (d)	IPSS Result (e)
Cushing's Disease Group
1	381, 488	5	Pos	ND	Pos
2	361,604	8	Pos	Pos	Pos
3	596, 579	13, 11	Pos	Pos	Pos
4	1020, 1098	NA	NA	NA	NA
5	NA	NA	NA	NA	NA
6	1076, 1214	4	Pos	Pos	Pos
7	1145, 1424	2,3	Pos	Pos	Pos
8	544, 999	5	Pos	Pos	Pos
9	836	<1	Pos	Pos	Pos
10	731, 1244	4, 10	Pos	Pos	Pos
11	1021	15	ND	Pos	Pos
ACTH-independent Cushing's, Adrenal Tumor Group
12	552, 720	2	Neg	Neg	ND
13	684, 781	2	Pos	Neg	Pos
14	5518	ND	ND	ND	ND
Cushing's Syndrome from Unknown Etiology Group
15	[ 52 ]	ND	ND	Neg	ND
16	546, 646	ND	ND	Neg	ND
17	4701	2	Pos	Pos	ND
18	7035, 7932	29	Neg	Neg	Pos
19	883	3	Pos	ND	Pos

^(a)baseline urinary free cortisol or 17-hydroxy corticosteroid excretion at the time of initial presentation with Cushing’s syndrome; two representative values are provided when available, otherwise a single value is shown; normal range for urinary free cortisol 70–300 nmol/d; normal range for urinary 17-hydroxy corticosteroid 6–28 µmol/d.

^(b)baseline plasma ACTH at the time of initial presentation with Cushing’s syndrome; two representative values are provided when available, otherwise a single value is shown; normal range for ACTH 2–11 pmol/l.

^(c)CRH Stim Test positive result (Pos)= >35% increase in ACTH (measured at 15' and 30') above baseline; Neg = negative.

^(d)High dose dexamethasone suppression test result positive (Pos) if UFCs suppressed > 90% or urinary 17OHCS suppressed > 64%; Neg= negative.

^(e)Positive (Pos) indicates central-to-peripheral ACTH gradient of ≥ 3:1 observed with CRH stimulation during inferior petrosal sinus sampling.

Abbreviations: UFC, urinary free cortisol; 17OHCS, 17-hydroxy corticosteroids; HD, high dose; NA, result or data not available; ND, not determined.