先天性梅毒致先天性肾病综合征一例并文献复习

Case report of congenital nephrotic syndrome caused by congenital syphilis and literature review

目的探讨先天性梅毒致先天性肾病综合征(CNS)的临床特征及治疗措施。方法先天性梅毒致先天性肾病综合征患儿1例.患儿,女,2月余,因呕吐腹泻5d,腹胀,水肿1d入院。回顾性分析该患儿临床资料,检索国内外文献,进行总结分析。结果患儿急性起病,主要表现为腹胀,水肿,呕吐腹泻,尿隐血3+,蛋白3+。生化蛋白降低,血脂升高,梅毒特异性抗体(TPHA)阳性,其母TPHA阳性.经青霉素治疗2周后,尿蛋白,血清蛋白基本恢复正常。检索国内外文献发现,先天性梅毒致先天性肾病综合征患儿均有梅毒接触史,临床以腹胀、水肿为主要表现,符合先天性肾病综合症的主要诊断标准。青霉素足量治疗,疗效较佳,不需激素治疗,预后良好。结论对于CNS伴腹胀,肝脾大患儿应考虑到先天性梅毒致肾病可能,应对患儿父母进行梅毒血清学检查,以期早期确诊.及时足量青霉素治疗是关键。

Objective： To explore the clinical characteristics and therapy of congenital nephrotic syndrome caused by congenital syphilis. Methods： One case with congenital nephrotic syndrome caused by congenital syphilis was reported and relevant literatures were reviewed . A 2 months old girl was admitted to our hospital with a history of emesis and diarrhea for 5 days, abdominal distention and edema for a day. Results： The onset of the disease of this reported child was acute. Clinical manifestations included abdominal distention, edema, emesis and diarrhea. Occult blood and protein in urine was 3 ＋ , serum albumin was decreased, serum cholester- ol and glycerine increased, specific antibody of syphilis - TPHA in the case and her mother were positive. After the treatment of penicillin for 2 weeks, protein in urine and serum become normal. The domestic and foreign documents showed that all children with congenital nephrotic syndrome caused by congenital syphilis had syphilis contact history. Abdominal distention and edema were the main symptoms, which was in line with CNS major diagnostic criteria. Adequate penicillin therapy got better effects without steroid treatment, and the prognosis were good. Conclusions： Children with congenital nephrotic syndrome accompanied by abdominal distention and hepatosplenomegaly should be taken into account the possibility of congenital nephrotic syndrome caused by congenital syphilis. Their parents should be check relevant serologic examination to get the earlier diagnosis. In time therapy with full dose penicillin is critical.