Kim, Taek Min Kim, Jung Hee Jang, Han Na Choi, Man Ho Cho, Jeong Yeon Kim, Sang Youn 2024-01-19T12:33:53Z 2024-01-19T12:33:53Z 2022-04-05 2022-02 2093-596X https://pubs.kist.re.kr/handle/201004/115668 Background: Monitoring adults with classical 21-hydroxylase deficiency (21OHD) is challenging due to variation in clinical and laboratory settings. Moreover, guidelines for adrenal imaging in 21OHD are not yet available. We evaluated the relationship between adrenal morphology and disease control status in classical 21OHD. Methods: This retrospective, cross-sectional study included 90 adult 21OHD patients and 270 age- and sex-matched healthy controls. We assessed adrenal volume, width, and tumor presence using abdominal computed tomography and evaluated correlations of adrenal volume and width with hormonal status. We investigated the diagnostic performance of adrenal volume and width for identifying well-controlled status in 21OHD patients (17 alpha-hydroxyprogesterone [17-OHP] <10 ng/mL). Results: The adrenal morphology of 21OHD patients showed hypertrophy (45.6%), normal size (42.2%), and hypotrophy (12.2%). Adrenal tumors were detected in 12 patients (13.3%). The adrenal volume and width of 21OHD patients were significantly larger than those of controls (18.2 +/- 12.2 mL vs. 7.1 +/- 2.0 mL, 4.7 +/- 1.9 mm vs. 3.3 +/- 0.5 mm, P<0.001 for both). The 17-OHP and androstenedione levels were highest in patients with adrenal hypertrophy, followed by those with normal adrenal glands and adrenal hypotrophy (P<0.05 for both). Adrenal volume and width correlated positively with adrenocorticotropic hormone, 17-OHP, 11 beta-hydroxytestosterone, progesterone sulfate, and dehydroepiandrosterone sulfate in both sexes (r=0.33-0.95, P<0.05 for all). For identifying well-controlled patients, the optimal cut-off values of adrenal volume and width were 10.7 mL and 4 mm, respectively (area under the curve, 0.82-0.88; P<0.001 for both). Conclusion: Adrenal volume and width may be reliable quantitative parameters for monitoring patients with classical 21OHD. English 대한내분비학회 Adrenal Morphology as an Indicator of Long-Term Disease Control in Adults with Classic 21-Hydroxylase Deficiency Article 10.3803/EnM.2021.1278 1 Endocrinology and Metabolism, v.37, no.1, pp.124 - 137 Endocrinology and Metabolism 37 1 124 137 N scie scopus kci ART002815031 000764890400012 Endocrinology & Metabolism Endocrinology & Metabolism Article BODY-COMPOSITION HYPERPLASIA VOLUME GLANDS SIZE Adrenal hyperplasia, congenital Adrenal cortex Adrenal volume Androgens