A Chinese Woman with Congenital Adrenal Hyperplasiadue to 21-Hydroxylase Deficiency after Adrenalectomy: What Did We Ignore of?

Congenital adrenal hyperplasia (CAH) is not a rare disease, but the possibility of misdiagnosis or late diagnosis is high especially in primary hospitals in China. Here, we report a case of a 53-year-old Chinese woman with CAH due to 21-hydroxylase deficiency after adrenalectomy. The primary physicians gave no diagnosis of CAH and directly performed the surgery for the possibility of compression caused by the large adrenal mass. Postoperatively, the patient experienced repeated hyperkalemia and cutaneous and gingival hyper pigmentation. Meantime, physicians ignored the patient with a history of noncompliance and postoperatively the patient was in danger many times. This study rein forces the close association between CAH and adrenal adenoma. CAH should be considered and baseline follicular-phase early-morning17- hydroxyprogesterone levels should be measured when discovered adrenal mass or nodule incidentally. Meanwhile, risk for noncompliance should be considered before surgery.