Wilson病患者角膜K-F环的临床研究

摘要

Objective To investigate the relationships between the corneal Kayser-Fleischer(K-F) ring for patients with Wilson's disease (WD) and their clinical classification types and the age of onset.Methods Test results of corneal K-F ring of 1039 WD patients using by slit lamp examination were summarized,and the positive rates of K-F ring of these WD patients with different clinical types and the age of onset were compared.Results The positive rates of WD patients with liver type,brain and brain-visceral were significantly higher than subclinical patients(P=-0.00),and the positive rates of brain and brain-visceral were significantly higher than liver patients(P=0.00).The positive rates of the group of range in age from 5 to 9,10 to 19,20 to 29,and more than 30 years were significantly higher than the patients under 5 years (P=0.00),and the positive rates of patients range in age from 10 to 19,20 to 29,were significantly higher than the patients whose age ranging from 5 to 9 (P=0.00).Conclusion The test results of Corneal K-F ring have high diagnostic value for patients who are suspicious for WD with brain damage symptoms(or with multiple organ damage symptoms which include liver,brain and so on) and their onset age is over 10 years.On the other hand,in order to avoid misdiagnose or missed diagnosis,it is necessary for patients who are under 10 years,or their symptoms are only liver damage to check their copper metabolism and other examinations,even if their Corneal K-F ring test results are negative.%目的 探讨Wilson病(WD)患者角膜Kayser-Fleischer(K-F)环与临床分型以及发病年龄之间的关系.方法 总结1039例WD患者裂隙灯检查角膜K-F环的检测结果,比较不同临床分型方法和发病年龄段的患者的阳性率. 结果 肝型、脑型和脑-内脏型患者的K-F环阳性率显著高于亚临床型患者(P=0.00),脑型和脑-内脏型患者的K-F环阳性率显著高于肝型患者(P=0.00).不同年龄组中,5～9岁、10～19岁、20～29岁和≥30岁组患者的K-F环阳性率显著高于＜5岁组患者(P=0.00),10～19岁、20～29岁、≥30岁组患者的K-F环阳性率显著高于5～9岁患者(P=0.00).结论 对于发病年龄在10岁以上且有脑损害症状(或有肝、脑等多脏器损害症状)而怀疑为本病的患者,角膜K-F环检查具有较高的诊断价值.而对于10岁以下或以肝损伤为主的疑诊患者,即使角膜K-F环检查结果阴性仍不能排除本病,需行铜代谢检查等检查明确诊断,以避免误诊或漏诊的发生.