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目的 探讨肾上腺性征异常症的临床特点。 方法 总结 1981～ 1998年收治 2 0例该病患者的临床资料 ,其中先天性肾上腺皮质增生 (CAH) 14例 ;肾上腺腺瘤 3例 ,肾上腺皮质癌 3例。 结果 12例CAH随访 1～ 11年。口服强的松替代治疗后 ,5例青春前期身体发育正常 ,1例无效停药 ,6例青春期后月经来潮 ,其中 3例怀孕分娩。 6例肾上腺肿瘤者行切除术 ,术后异常性征消失 ,3例腺瘤随访 2～ 14年无复发 ,3例皮质癌术后 1年复发并死亡。 结论 CAH行皮质激素治疗效果满意 ,关键在于早期、坚持、调节用药 ,性征异常的肾上腺皮质肿瘤常为网束状带混合性肿瘤。
Objective To investigate the clinical features of adrenal abnormalities. Methods The clinical data of 20 patients with this disease from 1981 to 1998 were summarized, including 14 cases of congenital adrenal hyperplasia (CAH), 3 cases of adrenal adenoma and 3 cases of adrenocortical carcinoma. Results 12 cases of CAH were followed up for 1 to 11 years. Oral prednisone replacement therapy, 5 cases of early pre-normal physical development, 1 patient discontinued, 6 cases of menstrual cramps after puberty, of which 3 cases of childbirth. 6 cases of adrenal tumor resection, abnormal signs disappeared, 3 cases of adenomas were followed up for 2 to 14 years without recurrence, 3 cases of cortical cancer recurrence and died 1 year after surgery. Conclusion CAH corticosteroids satisfactory results, the key lies in the early, adhere to, regulation of medication, abnormal sexual adrenal cortical tumors often bundles of mesh tumor.