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肾上腺性征异常症(附20例报告) 被引量:5

Adreno-genital syndrome (report of 20 cases)
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摘要 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 study the clinical features of adreno-genital syndrome. Methods 20 cases of adreno-genital syndrome treated from 1981 to 1998 were reviewed.14 cases being congenital adrenal hyperplasia,3 adrenal adenoma and 3 adrenocortical carcinoma. Results 12 of the 14 cases of congenital adrenal hyperplasia have been followed up for 1-11 years and were all treated by hormonal replacement after surgery with oral deltasone.5 were before adolescence and the physical built have been normal.The oral deltasone has been noneffective in 1 cases.6 were adult females,3 with normal menstruation and the other 3 have had normal child birth.No recurrence has been noted after resection of the adrenal adenoma in 3 cases.3 cases of adrenocortical carcinoma had recurred and died after 1 year. Conclusions Adrenal cortical hormone replacement therapy is effective to congenital adrenal hyperplasia with adreno-genital syndrome.The Key point is early diagnosis with persistant drug administration and the dose well adjusted.Adrenal tumors causing adreno-genital syndrome are often mixed tumors consisting of zona reticularis and fasciculata.
出处 《中华泌尿外科杂志》 CAS CSCD 北大核心 2000年第11期650-652,共3页 Chinese Journal of Urology
关键词 肾上腺疾病 性征异常 临床特点 肾上腺肿瘤 Adrenal gland diseases Sex characteristics
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