Table 1 A comparison of clinical characteristics of patients with ACTH-secreting PAs with or without USP8 mutations

From: Recurrent gain-of-function USP8 mutations in Cushing's disease

Characteristic

 

WT (n = 45)

Mutant (n = 75)

P value

Age at diagnosis (Year)

 

Median

40

36

0.08†

 

Interquartile range

29.5-46.0

26.0-42.0

 

Body Mass Index (BMI)

 

Median

25.4

24.2

0.36†

 

Interquartile range

21.6-27.8

22.4-26.7

 

Plasma corticotropin (ACTH) (pg/ml)

 

Median

94.0

68.8

0.054†

 

Interquartile range

60.6-163.0

51.4-105.5

 

ACTH (pg/ml)/Size (cm)

 

Median

51.0

103.0

< 0.001†

 

Interquartile range

31.5-104.6

76.3-201.7

 

Midnight serum cortisol (μg/dl)

 

Median

21.82

22.86

0.79†

 

Interquartile range

16.18-31.20

18.35-29.41

 

Urinary free cortisol (μg/24 h)

 

Median

650.24

552.64

0.39†

 

Interquartile range

412.13-1168.25

262.75-977.47

 

Immediate postoperative biochemical remission (%)Φ

 

Yes

33(73.3)

63(84.0)

0.16‡

 

No

12(26.7)

12(16.0)

 

Postoperative recurrence (%)я

 

Yes

8(25.8)

12(20.7)

0.58‡

 

No

23(74.2)

46(79.3)

 

Average recurrence period (month)

 

Median

48

29

0.28†

 

Interquartile range

15.0-48.0

16.3-38.3

 
  1. †Values were compared by Mann-Whitney U-test.
  2. ‡Values were compared by Pearson's χ2 test.
  3. ΦImmediate postoperative biochemical remission was defined on the basis of the biochemical evidence of adrenal cortical insufficiency as witnessed by the drop in the immediate postoperative serum cortisol (range, < 3 μg/dL).
  4. яPostoperative recurrence was defined when a patient with initial biochemical and clinical evidence for remission after operation developed symptomatic recurrence of Cushing disease with biochemical reconfirmation of pituitary-dependent endogenous hypercortisolism.
  5. Summary of patient number for each parameter:
  6. Age: Data were available for 45 patients with no mutation and 75 patients with mutation.
  7. BMI: Data were available for 45 patients with no mutation and 75 patients with mutation.
  8. ACTH: Data were available for 43 patients with no mutation and 65 patients with mutation.
  9. ACTH/Size: Data were available for 32 patients with no mutation and 51 patients with mutation.
  10. Midnight serum cortisol: Data were available for 29 patients with no mutation and 45 patients with mutation.
  11. Urinary free cortisol: Data were available for 28 patients with no mutation and 41 patients with mutation.
  12. Immediate postoperative biochemical remission: Data were available for 45 patients with no mutation and 75 patients with mutation.
  13. Postoperative recurrence: Among patients who reached immediate postoperative biochemical remission, long-term follow-up was performed in 33 cases with no mutation (2 were lost) and 63 cases with mutation (5 were lost).
  14. Average recurrence period: In no mutation group, data were available for 8 recurrent patients, while in mutation group, data were available for 12 recurrent patients.
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