儿童中毒性表皮坏死松解症11例临床研究

Clinical study on toxic epidermal necrolysis in 11 children

目的探讨儿童中毒性表皮坏死松解症(TEN)的临床特点及治疗策略。方法收集西安交通大学附属儿童医院PICU 2018年1月至2022年6月救治的11例TEN患儿的临床资料、实验室检查、诊治经过及治疗转归,并进行回顾性分析。结果11例患儿中,男5例,女6例,年龄1岁4个月~11岁4个月,9例由药物引起,其中6例由抗癫痫药物引起,2例由中成药引起,1例由抗生素引起,余2例由感染引起。除大面积皮肤剥脱外,1例患儿并发角膜穿孔,1例患儿并发指(趾)甲脱落,2例患儿并发上气道梗阻,所有患儿均合并有不同程度心、肝、肾等多脏器损害。8例患儿首诊科室为免疫科,入院后均给予甲泼尼龙及静脉注射人免疫球蛋白治疗,3例患儿皮疹无变化,5例患儿皮损范围较入院时扩大,全部转入PICU。3例患儿首诊科室为PICU,入院后均未使用甲泼尼龙及静脉注射人免疫球蛋白治疗。11例患儿均给予血浆置换治疗。首诊科室为PICU的患儿,PICU平均住院时间为(8.00±3.00)d,总的平均住院时间为(33.66±20.10)d,平均住院费用为(7.39±3.05)万元。首诊科室为免疫科的患儿,PICU平均住院时间为(21.62±16.18)d,总的平均住院时间为(41.87±16.97)d,平均住院费用为(13.08±5.22)万元。1例患儿因合并角膜穿孔,皮疹好转后家属因经济原因要求出院,其余患儿均治愈出院。结论TEN临床罕见,常合并多器官功能障碍,病死率高,血浆置换可能作为该病的有效治疗方法之一。

Objective To investigate the clinical characteristics and treatment strategies of toxic epidermal necrolysis(TEN)in children.Methods The clinical data,laboratory examination,diagnosis and treatment process and treatment outcomes of 11 children with TEN treated in PICU at Children′s Hospital of Xi′an Jiaotong University from January 2018 to June 2022 were collected and analyzed retrospectively.Results There were five males and six females,aged from one year to 11 years old.Nine cases were caused by drugs,among which six cases were caused by antiepileptic drugs,two cases were caused by Chinese patent medicine,one case was caused by antibiotics,and the remaining two cases were caused by infection.In addition to large area of skin exfoliation,one case was complicated with corneal perforation,one case was complicated with finger(toe)nail fall off,and two cases were complicated with upper airway obstruction.All children were complicated with various degrees of heart,liver,kidney and other organ damage.Eight cases who were admitted to the department of immunology were treated with methylprednisolone and intravenous immune globulin.Three cases had no change in rash,and five cases had an enlarged range of skin lesions compared with admission,and finally all of them were transferred to PICU.Three cases were first diagnosed in PICU and were not treated with methylprednisolone and intravenous immune globulin after admission.All 11 children were given therapeutic plasma exchange in PICU.For children whose first department was PICU,the average hospitalization time of PICU was(8.00±3.00)days,the total average hospitalization time was(33.66±20.10)days,and the average hospitalization cost was(73.9±30.5)thousand yuan.For children whose first department was the immunology department,the average hospitalization time of PICU was(21.62±16.18)days,the total average hospitalization time was(41.87±16.97)days,and the average hospitalization cost was(130.8±52.2)thousand yuan.One case,because of corneal perforation,the family members asked to leave the hospital for economic reasons after the rash improved,and the rest of the children were cured and discharged.Conclusion TEN is rare,often complicated with multiple organ dysfunction,and has a high mortality.Early administration of therapeutic plasma exchange may alleviate multiple system damage and shorten the duration of disease.