摘要
目的探讨胎传梅毒致继发先天性肾病综合征的诊断、治疗和预后,以提高临床医生对该病的认识。方法报告我院1例胎传梅毒继发先天性肾病综合征临床诊治过程、随访情况,对该患儿行超声引导下负压肾脏穿活检术,肾活检病理进行分析,并复习相关文献。结果患儿男,2个月起病,临床表现为肾病综合征、贫血、肺部感染、皮疹,尿常规:尿蛋白4+,潜血+。本例患儿其梅毒螺旋体定性滴度4倍于其母,肾活检病理结果回报,排除了由基因致的原发性肾病综合征,明确胎传梅毒继发先天性肾病综合征的诊断。停用糖皮质激素,经青霉素驱梅毒治疗14天,患儿全身无水肿,双侧腹股沟区少量结痂,尿常规:蛋白+-,潜血-。以"congenital syphilis"为关键词在万方数据库检索报道胎传梅毒致先天性肾病综合征,选取7篇国内文中报道典型18例梅毒性肾损害及临床资料完整者,仅有2例报道肾穿病理为非典型模性肾病,放弃3例,死亡2例,余13例临床给予驱梅治疗,经随访效果良好。结论婴儿期起病为主,临床以肾病综合征现象为突出表现,伴肝脾大、皮疹、贫血感染现象等,应考虑到梅毒肾病可能,需对患儿父母进行梅毒血清学检查,明确病理诊断后给予驱梅疗法,而非激素治疗。本例患儿早期经足量足疗程驱梅治疗疗效佳,预后良好。
Objective： To investigate the clinical features of congenital nephritic syndrome caused by congenital syphilis and improve the awareness of this kind of diseases. Method： We report the clinical process and the follow-up of a case of congenital nephritic syndrome caused by congenital syphilis. We analyzed the pathological features of kidney by ultrasonography-guided percutaneous renal biopsy. Result： A 2-month male baby was clinically diagnosed of nephritic syndrome, anemia, pulmonary infection and erythra with urinal positive protein 4＋ and positive occult blood. The result of his syphilis qualitative test was 4 times of his mother. And the outcome of the renal biopsy showed that the nephritic syndrome could not be caused by the gene mutation so we could make a definite diagnosis that the congenital nephritic syndrome of this baby was caused by the congenital syphilis. After the clear diagnosis, we shut down the use of glucocoticoids and treated the baby by application of 14-day penicillin. The edema of this baby was cured on his whole body after the treatment and only leaved a little scab on his inguinal fold area and the urinal routine showed that the protein 2＋ and no occult blood. We retrieved in the database system of wanfang by using the ＂congenital syphilis＂ as the key word and chose 6, reports about 18 typical renal damages caused by the syphilis. Among these reports, only 5 patients in the 2 papers have received renal biopsy and 3 of them were abandoned while 2 patients died. The other 13 patients receiving treatment with penicillin grow up healthily. Conclusion： When clinically attacked at the infancy stage, the nephritic syndrome with hepatomegaly, splenomegaly, erythra, anemia and infection, we need to aware the possibility of nephrapathy due to syphilis and then make the serum syphilis test of the parents and renal biopsy. If so, we should use penicillin as anti-syphilis therapy but not the glucocoticoids.
出处
《中国优生与遗传杂志》
2016年第1期108-110,F0003,共4页
Chinese Journal of Birth Health & Heredity
关键词
梅毒
先天性
肾病综合征
病理诊断
治疗
Syphilis
Congenital
Nephritic syndrome
Pathological diagnosis
Treatment
