Endocrinol Metab: Adrenal morphology as an indicator of long-term disease control in adults with classic 21-hydroxylase deficiency

Background: Congenital adrenal hyperplasia is an autosomal recessive disorder caused by impaired steroidogenesis, with 21-hydroxylase deficiency (21OHD) accounting for approximately 95% of cases

Congenital adrenal hyperplasia is an autosomal recessive disorder caused by impaired steroidogenesis, with 21-hydroxylase deficiency (21OHD) accounting for approximately 95% of cases

 Adrenal plethysmography has been used to assess normal adrenal size and to characterize specific diseases that may affect the adrenal parenchyma

Methods: This retrospective, cross-sectional study included 90 adult patients with 21OHD and 270 age- and sex-matched healthy controls

Results: The adrenal glands of 21 patients with OHD showed hypertrophy (45.

Figure 1 Image analysis of a patient with 21-hydroxylase deficiency (21OHD)

Figure 1 Image analysis of a patient with 21-hydroxylase deficiency (21OHD)

Table 1 Comparison of patients with pure virilizing type and salt-consuming type

Table 1 Comparison of patients with pure virilizing type and salt-consuming type

Table 2 Linear and volumetric parameters between 21 hypertensive patients and control patients

Table 2 Linear and volumetric parameters between 21 hypertensive patients and control patients

Figure 2 Determination of plasma levels of (A) 17α-hydroxyprogesterone (17-OHP) and (B) androstenedione according to adrenal morphology

Figure 2 Determination of plasma levels of (A) 17α-hydroxyprogesterone (17-OHP) and (B) androstenedione according to adrenal morphology

Adrenal volume and width serve as reliable quantitative parameters for monitoring patients with classic 21-OHD

Adrenal Morphology as an Indicator of Long-Term Disease Control in Adults with Classic 21-Hydroxylase Deficiency

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