Adrenal Morphology As An Indicator Of Long-Term Disease Control in Adults With Classic 21-Hydroxylase Deficiency Ⅱ

RESULTS

Patient characteristics 

Detailed information on the study group is summarized in Table 1. Forty-four (48.9%) of the 90 patients with 21OHD were men. Forty-six patients (51.1%) had the salt-wasting type of 21OHD, while 44 patients (48.9%) had the simple virilizing type. There were no significant differences in the biochemical parameters and glucocorticoid doses between the salt-wasting and simple virilizing types (Table 2). All 90 patients were treated with prednisolone, two patients with hydrocortisone, and 55 with fludrocortisone. The median values of daily glucocorticoid dose/BSA and fludrocortisone dose/BSA were 17.5 mg/m2 (range, 5.2 to 26.7) and 0.06 mg/m2 (range, 0.03 to 0.40), respectively. The ACTH level and androstenedione/T ratio were significantly higher in male than in female patients (P=0.02 and P<0.001, respectively), while 11β-OHT/T was significantly higher in female than in male patients (P<0.001). Fifty-six out of 90 patients (62.2%) had at least one metabolic morbidity, and the male patients had a significantly higher incidence of metabolic morbidity than female patients (75% vs. 47.8%, P=0.01). The biochemically well-controlled group comprised 26 patients (28.9%, 11 men and 15 women), while the poorly-controlled group contained the remaining 64 patients.

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Radiologic findings of Classical 21OHD patients 

Among the 90 patients, 41 (45.6%) had adrenal hypertrophy, 38 (42.2%) had a normal appearance, and 11 (12.2%) had adrenal hypotrophy. In the well-controlled group, one patient showed hypertrophy, while 15 and 10 patients had a normal appearance and hypotrophy of the adrenal glands, respectively. In the poorly controlled group, 40, 23, and 1 patients showed hypertrophy, normal appearance, and hypotrophy of the adrenal glands, respectively. Twelve patients (13.3%) had adrenal tumors, which were unilateral in nine patients and bilateral in three patients. Among them, 11 patients showed gross fat in CT images, confirming the presence of myelolipoma. The other patient presented with adrenal adenoma. Detailed information on the radiologic findings of 21 OHD patients is summarized in Table 3.

Volumetric and linear evaluation of adrenal glands in 21OHD patients and comparison with healthy controls 

The mean and standard deviation of the right, left, and total adrenal volumes of 21 OHD patients were 8.5±5.5, 9.8±7.3, and 18.2±12.2 mL, respectively. These values were all significantly larger than in healthy controls (3.5±1.0, 3.6±1.1, 7.1±2.0 mL, P<0.001 for all). The right, left, and average adrenal width were also significantly higher in 21OHD patients than in normal controls (4.7±1.8 mm vs. 3.3±0.5 mm, 4.6±2.0 mm vs. 3.3±0.5 mm, 4.7±1.9 mm vs. 3.3±0.5 mm, P<0.001 for all). All limb width and volume parameters were significantly higher in men than in women among the healthy controls (P<0.001 for all); however, there was no statistically significant difference between men and women with 21OHD (P>0.05 for all). We obtained an area under the curve (AUC) of 0.85 for classifying patients with 21OHD (P<0.001) with a sensitivity of 81.1% and specificity of 90% at a cut-off value of 9.1 mL. Table 4 presents detailed information on linear and volumetric parameters.

Subgroup analysis of adrenal volume in 21OHD patients 

The adrenal volume between patients with salt-wasting 21OHD and those with the simple virilizing type was not significantly different (17.9±13.9 mL vs. 18.4±8.9 mL, P=0.31). Patients with irregular menstruation showed significantly larger adrenal volumes than those with regular menstruation (21.0±12.9 mL vs. 12.7±8.5 mL, P=0.009). In addition, patients with adrenal tumors had significantly larger adrenal volumes than those without any masses (31.8±16.1 mL vs. 15.7±9.5 mL, P=0.025).

Correlation analysis of adrenal volume and width with hormonal status 

Both the plasma levels of 17-OHP and androstenedione were significantly different among groups according to adrenal morphology (hypertrophy, normal appearance, and hypotrophy; P<0.001 and P<0.02, respectively). Patients with adrenal hyhypertrophy showed the highest 17-OHP and androstenedione levels (median, 49.9 ng/mL [interquartile range (IQR), 22.2 to 108.7] and 2.6 ng/mL [IQR, 1.0 to 5.0], respectively), followed by those with normal adrenal glands (32.1 ng/mL [IQR, 5.1 to 58.3] and 1.1 ng/mL [IQR, 0.5 to 1.6]) and those with adrenal hypotrophy (3.6 ng/mL [IQR, 2.8 to 8.9] and 0.4 ng/mL [IQR, 0.2 to 1.1]) (Fig. 2).

The total adrenal volume correlated positively with ACTH, 17-OHP, DHEA-S, androstenedione, 11β-OHT, 11β-OHA, and Preg-S in both male and female patients (r=0.43–0.94, P<0.05 for all). Positive correlations were found for 11β-OHT/T and plasma renin activity in male patients (r=0.49–0.70, P<0.05 for both) and for testosterone in female patients (r=0.70, P<0.001) (Fig. 3). Adrenal width correlated positively with ACTH, 17- OHP, 11β-OHT, 11β-OHT/T, Preg-S, DHEA-S in both male and female patients (r=0.31–0.95, P<0.05 for all). The adrenal width showed positive correlations with androstenedione, 11β-OHA, and plasma renin activity only in males (r=0.43–0.81, P<0.05 for all), and showed a positive correlation with testosterone only in females (r=0.44, P=0.03). Table 5 presents detailed information on the correlation analysis.

The total adrenal volume correlated positively with ACTH, 17-OHP, DHEA-S, androstenedione, 11β-OHT, 11β-OHA, and Preg-S in both male and female patients (r=0.43–0.94, P<0.05 for all). Positive correlations were found for 11β-OHT/T and plasma renin activity in male patients (r=0.49–0.70, P<0.05 for both) and for testosterone in female patients (r=0.70, P<0.001) (Fig. 3). Adrenal width correlated positively with ACTH, 17- OHP, 11β-OHT, 11β-OHT/T, Preg-S, DHEA-S in both male and female patients (r=0.31–0.95, P<0.05 for all). The adrenal width showed positive correlations with androstenedione, 11β-OHA, and plasma renin activity only in males (r=0.43–0.81, P<0.05 for all), and showed a positive correlation with testosterone only in females (r=0.44, P=0.03). Table 5 presents detailed information on the correlation analysis.

The daily glucocorticoid dose/BSA correlated negatively with adrenal volume (r=−0.24, P=0.02), but showed no significant correlation with adrenal width (r=−0.14, P=0.19). The daily fludrocortisone dose/BSA did not show significant correlations 

Fig. 2. Plasma levels of (A) 17α-hydroxyprogesterone (17-OHP) and (B) androstenedione according to the adrenal morphology. Patients with adrenal hypertrophy showed the highest 17-OHP and androstenedione levels, followed by those with normal adrenal glands and adrenal hypotrophy.

Diagnostic performance of adrenal volume and width for the identifying 21OHD patients with well-controlled status and metabolic morbidity 

The total adrenal volume and width yielded AUCs of 0.87 (95% confidence interval [CI], 0.78 to 0.93) and 0.82 (95% CI, 0.72 to 0.89), respectively, for predicting the biochemically well-controlled group (P<0.001 for both) (Fig. 4). We could achieve a sensitivity of 73.1% and specificity of 90.6% using a cut-off value of 10.7 mL of adrenal volume. Using a cutoff value of 4.0 mm of adrenal width, a sensitivity of 80.8% and specificity of 79.7% were obtained. The AUCs of adrenal volume and width showed no significant differences (P=0.24).

The total adrenal volume resulted in an AUC of 0.67 (95% CI, 0.56 to 0.76) for predicting patients with one or more metabolic morbidity (P=0.004). Using a cut-off value of 17 mL, a sensitivity of 55.4% and specificity of 76.5% were obtained. Adrenal width showed no significant diagnostic value for predicting the patients with metabolic morbidity (AUC, 0.57; 95% CI, 0.46 to 0.67; P=0.29). The AUC of adrenal volume was significantly higher than that of adrenal width (P=0.03).

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