梁贻俊教授治疗小儿原发性血小板减少性紫癜伴Klinefelter综合征1例

Professor Liang Yijun in the Treatment of Idiopathic Thrombocytopenic Purpura and Klinefelter Syndrome in Children for One Case

原发性血小板减少性紫癜,是与免疫相关的出血性疾病,其主症表现为皮肤、黏膜出现瘀点瘀斑,伴有鼻衄、齿衄等,与中医古代医籍中所描述肌衄、葡萄疫、鼻衄等相符合。Klinefelter综合征又称先天性睾丸发育不全症,是男性最常见的性染色体疾病。目前尚未有性染色体异常与原发性血小板减少性紫癜相关性的报道。然而梁贻俊教授从中医病因病机出发,认为二者均与先天不足,肾精亏虚有关,存在内在关联性。治疗以益肾养血,健脾益气,清虚热为主,药用六味地黄丸之“三补”合“犀角地黄汤”加减应用6个月余,患儿复查血小板正常,未再出现瘀点瘀斑,身疲乏力,睡眠不安等症已解,可正常完成学业。停药后随访3年未复发,治疗过程未使用激素,单纯使用中药治疗,获得满意疗效。

Idiopathic thrombocytopenic purpura is an immune related hemorrhagic disease.Its main symptoms are ecchymosis and ecchymosis on the skin and mucous membrane,accompanied by epistaxis,gingival bleecling,etc.,which is consistent with the description of epistaxis,grape disease and epistaxis in ancient Chinese medical books.Klinefelter syndrome,also known as congenital testicular hypoplasia,is the most common sex chromosome disease in men.There is no report about the relationship between sex chromosome abnormality and primary thrombocytopenic purpura.Professor Liang Yijun,from the perspective of etiology and pathogenesis of traditional Chinese medicine,thinks that both of them are related to congenital deficiency and deficiency of kidney essence.The treatment is mainly to tonify the kidney and blood,invigorate the spleen and qi,and clear away deficiency and heat.The addition and subtraction of"three kinds of tonifying method"and"Xijiao Dihuang decoction"of Liuwei Dihuang pill has been used for more than 6 months.There was no recurrence in the follow-up of 3 years after drug withdrawal.No hormone was used in the treatment process,and only traditional Chinese medicine was used for treatment,and the curative effect is satisfactory.