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. 2015 Apr 8;100(6):E926–E935. doi: 10.1210/jc.2014-4204

Table 1.

Clinical and Hormonal Characteristics of PBMAH Patients

Characteristics Total Cohorta,b Mutateda,b Wild-Typea,b P Valuec
Number of patients 98 24 68
Age, y 53 [30–75] 49 [30–73] 55 [32–75] .046
Sex (F/M), % 64.3/35.7 50/50 69.1/30.9 .137
CS, % .007
    Clinical 43 71 35
    Subclinical 47 29 53
    Absent 10 0 11.8
Hypertension, %d 69.3 94.7 63.5 .009
Diabetes, %d 33.7 52.9 30.2 .093
Hypokalemia, %d 12.9 17.6 11.3 .441
ACTH, %e .003
    <10 pg/mL 60 92 53
    10 to 15 pg/mL 30 8 34
    >15 pg/mL 9 0 12
UFC (% of increase above upper limit of normal)e 204 [11–2433] 355 [11–1626] 167 [16–2433] .006
Plasma cortisol after 1 mg dexamethasone overnight test, μg/dLe 9.9 [0.3–36.7] 18.6 [3.6–36.7] 6.3 [0.3–2.7] <.001
Plasma cortisol at midnight, μg/dLe 12.1 [1.4–71.7] 20 [4.6–71.7] 9.4 [1.4–42.8] .019
Morning plasma cortisol, μg/dLe 14.1 [4.7–57.1] 16.9 [5.5–34] 13.8 [4.7–57.1] .245
Plasma cortisol after ACTH 1–24 250 μg, μg/dLe 39.2 [11.1–123] 48.9 [19.8–81.3] 37 [11.1–123] 0.058
Basal 17OHP, ng/dLe 99 [0–726] 132 [0–594] 99 [0–726] .28
17OHP after ACTH 1–24 250 μg, ng/dLe 990 [132–7128] 1089 [264–5578] 990 [132–7128] .79
Aldosterone/direct renin ratio, pmol/L/μIU/mLe 22 [0–331] 33 [0–331] 19 [0–133] .45
Food response, %d 17.6 0 28.1 <.001
Upright response, %d 52.9 68.8 46.9 .221

Abbreviations: F, female; M, male.

a

Quantitative variables are presented as means [minimum-maximum]; qualitative variables are presented as relative counts percentage.

b

Of the 98 patients, 24 were classified as ARMC5-mutated, 68 as ARMC5-wild type, and six were excluded for the comparison, presenting variants of uncertain significance.

c

Comparison of mutated patients vs wild-type patients.

d

Data about hypertension were available for 88 patients (19 mutated, 63 wild-type), diabetes for 86 (17 mutated, 63 wild-type), hypokalemia for 85 (17 mutated, 62 wild-type), and abnormal response for 51 (16 mutated, 32 wild-type).

e

ACTH was available for 97 patients, UFC for 94, morning plasma cortisol for 74, cortisol after suppression test for 77, cortisol at midnight for 67, aldosterone/renin ratio for 85, basal 17OHP for 80, cortisol after ACTH for 74, and 17OHP after ACTH for 68.