부산대학교 도서관

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is an autosomal recessive disorder characterized by multiple hormonal imbalances . Symptoms are caused by disease-related glucocorticoid and mineralocorticoid deficiencies as [...]
Context: Patients with congenital adrenal hyperplasia (CAH) are exposed to hyperandrogenism and supraphysiologic glucocorticoids, both of which can increase risk of metabolic morbidity. Objective: Our aim was to evaluate cardiovascular and metabolic morbidity risk in a longitudinal study of patients with CAH spanning both childhood and adulthood. Design and Setting: Patients with classic CAH followed for a minimum of 5 years during both childhood and adulthood (n = 57) at the National Institutes of Health were included and compared with the US general population using NHANES data. Main outcome measures: Obesity, hypertension, insulin resistance, fasting hypergly-cemia, and dyslipidemia. Results: Compared to the US population, patients with CAH had higher (P< 0.001) prevalence of obesity, hypertension, insulin resistance, fasting hyperglycemia, and low high-density lipoprotein (HDL) during childhood and obesity (P = 0.024), hypertension (P Conclusion: Patients with CAH develop metabolic morbidity at a young age associated with treatment-related and familial factors. Judicious use of glucocorticoid and min-eralocorticoid is warranted. Key Words: metabolic syndrome, congenital adrenal hyperplasia, androgen, glucocorticoid, mineralocorticoid