Treatment of acute multiorgan dysfunction occurring in congenital adrenal hyperplasia

Excerpt

A 23-year-old female was admitted to the hospital with a 2-day history of vomiting, diarrhea, and fever. The patient had a history of clitoral hypertrophy at birth, which was surgically corrected in her childhood. She presented with primary amenorrhea, and her chromosome karyotype was 46, XX. Approximately 12 months before admission, her steroid hormone profile showed elevated levels of testosterone (T 3.31 ng/ml, 0.15–0.51 ng/ml), dehydroepiandrosterone (DHEA 618.9 μg/dl, 19.00–391.00 μg/dl), progesterone (P 9.79 ng/ml, 0.2–0.9 ng/ml), and 17-hydroxyprogesterone (17OHP 10.99 ng/ml, 0.23–1.36 ng/ml), while the levels of follicle-stimulating hormone (FSH 5.83 mIU/ml, 21–104 mIU/ml) and luteinizing hormone (LH 3.58 mIU/ml, 0.9–58.6 mIU/ml) were decreased. A pelvic ultrasound indicated the presence of a uterus and ovaries. The patient was diagnosed with congenital adrenal hyperplasia (CAH) and 21-hydroxylase deficiency (21OHD). Then, she was treated with cortisone acetate (25 mg qd). Her levels of 17OHP (6.83 ng/ml) and T (0.15 ng/ml) decreased 6 months after treatment. Nine months after the treatment, she experienced menstruation and withdrew herself from the drug. …