Hamilton Gavin M, Fish Joel
From the*Faculty of Medicine, University of Toronto, Ontario, Canada; and †Sick Children's Hospital, Burn Unit, Plastic Surgery Division, Toronto, Ontario, Canada.
J Burn Care Res. 2013 Nov-Dec;34(6):e351-8. doi: 10.1097/BCR.0b013e31827a2ac3.
Stevens Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare debilitating mucocutaneuous diseases most commonly associated with severe drug reactions. A review of SJS/TEN cases was undertaken to describe the etiology, patient characteristics, treatment, and outcome in children affected at a large tertiary pediatric hospital. A retrospective chart review examined SJS/TEN cases admitted to a pediatric intensive care unit (PICU) between 2001 and 2011. Data concerning total body surface area (TBSA) involvement, causative agents, length of stay, and treatment were reviewed. PICU mortality predictors including Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN), Pediatric Index of Mortality 2 scores, and Pediatric Logistic Organ Dysfunction scores were also reviewed. Nutritional feeding information was collected and compared with calculated and measured (calorimetry) requirements. There were 10 SJS/TEN pediatric patients (mean age = 6.6 years) with significant skin involvement (mean TBSA = 42.2%) requiring PICU admission. Prescription drugs were the most common causative factor (n = 7). Intravenous immunoglobulin and corticosteroids were used for treatment in eight and two of the cases, respectively. The mean length of stay in the PICU was 10.2 days. There were no mortalities, and all mortality scores predicted high likelihood of survival. Actual feeds (n = 9) were uniformly lower than the calculated feeding requirements (mean = 1059 kcal/day vs mean = 2027 kcal/day). This data will help to provide insight into the management of SJS/TEN in an intensive care setting, and will help form the best treatment approach for future cases in a pediatric population.
史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是罕见的、使人虚弱的皮肤黏膜疾病,最常与严重的药物反应相关。对SJS/TEN病例进行了回顾,以描述一家大型三级儿科医院中受影响儿童的病因、患者特征、治疗及结果。一项回顾性病历审查研究了2001年至2011年间入住儿科重症监护病房(PICU)的SJS/TEN病例。审查了有关全身表面积(TBSA)受累情况、致病因素、住院时间和治疗的数据。还审查了PICU死亡率预测指标,包括中毒性表皮坏死松解症疾病严重程度评分(SCORTEN)、小儿死亡率指数2评分和小儿逻辑器官功能障碍评分。收集了营养喂养信息,并与计算得出的和测量的(量热法)需求进行比较。有10例SJS/TEN儿科患者(平均年龄 = 6.6岁),皮肤受累严重(平均TBSA = 42.2%),需要入住PICU。处方药是最常见的致病因素(n = 7)。8例和2例患者分别使用静脉注射免疫球蛋白和皮质类固醇进行治疗。在PICU的平均住院时间为10.2天。无死亡病例,所有死亡率评分均预测生存可能性高。实际喂养量(n = 9)均低于计算得出的喂养需求(平均 = 1059千卡/天对平均 = 2027千卡/天)。这些数据将有助于深入了解重症监护环境中SJS/TEN的管理,并有助于为未来儿科患者的病例形成最佳治疗方法。