J Endocrinol Metab

Journal of Endocrinology and Metabolism, ISSN 1923-2861 print, 1923-287X online, Open Access

Article copyright, the authors; Journal compilation copyright, J Endocrinol Metab and Elmer Press Inc

Journal website http://www.jofem.org

Case Report

Volume 5, Number 4, August 2015, pages 261-266

Cushing Syndrome Due to Primary Pigmented Nodular Adrenal Disease in Three Related Adolescent Girls With Carney Complex

Figures

Figure 1. Genealogic tree of the family. Arrow indicates the index case. Circles identify female members; squares indicate males; black symbols show patient with PPNAD. Age of the subjects is shown below the symbols. Family members are numbered by generation above symbol. Examined members who are normal (suppressed cortisol in response to 1 mg of dexamethasone) are identified with (n) inside the symbol.

Figure 2. Centrofacial lentigines including the vermilion borders of the lips.

Figure 3. (A) Macroscopic appearance of transverse slices of the left adrenal gland removed from patient IV-17. (B) Adrenal nodules surrounded by atrophic cortex. (C) Large cortical cells with eosinophilic cytoplasm rich in lipofuscin.

Tables

**Table 1.** Clinical Features Before Bilateral Adrenalectomy
Case	CNC features	Menarche age (years + months)	Chief complaint	Height (cm)	Weight (kg)	BMI (kg/m²)	Blood pressure (mm Hg)	Age at bilateral adrenalectomy
CNC: Carney complex; BMI: body mass index; Age: years + months.
1	Lentigines	11 + 9	Amenorrhea	158	55	22.4	148/83	14
2	Lentigines	11 + 7	Weight gain	156	58	22.9	132/87	15
3	None	12 +1	Menorrhagia	163	61	23.1	122/78	14

**Table 2.** Evolution of Adrenal Function in Affected Patients
	Age (years)					Liddle’s test % increase
	8	9	10	11	12	Liddle’s test % increase
Cortisol: morning 4.3 - 22.4 µg/dL; afternoon: 2.9 - 12.3 µg/dL. ACTH: adrenocorticotropin (7.2 - 63.3 pg/mL). UFC: urinary free cortisol (4.3 - 176 µg/24 h). Liddle’s test: dexamethasone 0.5 mg each 6 h for 48 h; % increase in UFC and not percent. DHEA-S: dehydroepiandrosterone sulfate (44 - 362 µg/dL).
Case 1
ACTH			17.3	12.7	2.3
Cortisol
9 h	10.6	16.6	14.3	13.5	10.1
16 h	5.5	7.6	6.6	5.9	11.1
24 h					10.6
UFC	68	99	138	106	171	50
DHEA-S	98	178	186	197	192
Case 2
ACTH			16.2	8.9	1.5
Cortisol
9 h	8.9	17.9	13.5	14.7	12.1
16 h	7.6	5.3	4.3	6.2	12.7
24 h					13.3
UFC	64.1	91.6	91	98	184	61
DHEA-S	87	107	162	219	223
Case 3
ACTH			14.8	13.1	6.3
Cortisol
9 h	12.2		16.6	12.7	9.3
16 h	4.3		6.3	5.4	9.7
24 h					10.3
UFC	78		69	91	151	157
DHEA-S	89		145	158	167