摘要
目的获取我国着色性干皮病(XP)患者更多的临床定量描述数据。方法回顾分析了我国39例XP患者的临床资料。结果3岁以内发病者占2/3。28.2%的XP患者出现眼部病变,20.5%的患者出现神经病变。恶性肿瘤的平均发病年龄为19.3岁。患者父母为近亲结婚者占61.3%,1/3患者中有兄弟姊妹同患本病。兄弟姊妹同患本病者,父母近亲结婚达72.7%。结论本组XP患者发生恶性肿瘤的平均年龄(19.3岁)大于国外平均发病年龄(8岁)。本组XP患者的存活年龄比国外患者大。近亲结婚者后代出现XP患者,兄弟姊妹同患本病比非近亲结婚者可能性大。
Objective To obtain more quantitative descriptive data of Chinese patients with xeroderma pigmentosum （XP）. Methods Retrospective Study of the clinical materials of 39 cases with XP was performed. Results 2/3 of the patients with XP presented cutaneous symptoms at the age within 3 years. Ocular abnormalities were reported in 28.2% of the patients and neurologic abnormalities were fimnd in 20.5% of the patients with XP. Malignant skin tumors had a median age of onset of 19.3 years. Consanguineous marriage of the patient＇s parents occurred in 61.3% of the patients in the entire series. 1/3 of the patients had. siblings with XP. Of the patients of siblings with XP,consanguineous marriage of their parents was noted for 72.7%. Conclusion The median age of onset of malignant skin tumors reported in Chinese patients with XP was 19, 3 years,much older than that of 8 years reported in other countries （ most patients are white ） and that the life-span of Chinese patients with XP was longer than that of the XP patients of other countries. The possibility of siblings with XP of parents in consanguineous marriage was larger than that of siblings with XP of parents in none consanguineous marriage.
出处
《中国皮肤性病学杂志》
CAS
北大核心
2005年第10期606-607,共2页
The Chinese Journal of Dermatovenereology
关键词
着色性干皮病
恶性皮肤肿瘤
近亲结婚
Xeroderma pignlentosum
Malignant skin tumor
Consanguineous marriage
