先天性梅毒致继发性先天性肾病综合征5例

目的探讨先天性梅毒致继发性先天性肾病综合征（NS）的临床特点、诊断、治疗及预后。方法对本院2000～2006年收治5例先天性梅毒致继发性先天性NS患儿的临床表现、诊断及治疗、预后进行综合分析,并结合文献进行回顾性研究。结果发病年龄21d～5个月,平均2.8个月。均有梅毒接触史,临床以腹胀、水肿为主要表现,符合先天性NS的主要诊断标准。患者均伴肝脾大,4例有皮疹、脱皮及贫血。3例青霉素足量治疗,疗效较佳,不需激素治疗,预后良好;并肺部感染死亡、自动出院失访各1例。结论对于早期发生NS伴肝脾大、皮疹患儿应考虑到梅毒肾病可能,并对患儿父母进行梅毒血清学检查,注意鉴别诊断。治疗关键在于驱梅疗法,而非激素治疗。多数患儿早期经足量足疗程驱梅治疗症状消失,预后良好。

Congenital Syphilis Presenting Caused Congenital Nephrotic Syndrome in 5 Children

Objective To study clinical features,diagnosis,therapy and prognosis of congenital nephrotic syndrome(CNS)caused by congenital syphilis.Method Five cases diagnosed as congenital syphilis with CNS in Beijing children's hospital from 2000 to 2006 were identified and retrospectively analyzed,including the clinical manifestation,diagnosis,treatment and prognosis.Results The ages of children ranged from 21 days to 5 months,averaging 2.8 months.All the 5 children had syphilis contact history,abdominal distention,edema as the main symptoms,which was in line with CNS major diagnostic criteria.All of the patients were associated with hepatosplenomegaly,and 80% with varying degrees of skin rash,peeling and anemia.Three cases of adequate penicillin therapy got better effects without hormone treatment,and the prognosis was good;1 case with lung infection died,and 1 case was out of contact after discharge.Conclusions For children with early nephropathy syndrome accompanied by rash,hepatosplenomegaly,we should take into account the possibility of syphilis nephropathy.Treatment of secondary CNS caused by congenital syphilis lies in flooding-therapy instead of hormone therapy.For most children with enough doses of antibiotics treatment,the prognosis is good.