Abstract：
BACKGROUND: Dystrophic epidermolysis bullosa (DEB) describes a rare genetic blistering disorder characterized by fragile skin. This study aimed to classify the frequency, demographics, cost, and comorbidities associated with emergency department (ED) visits due to DEB.
METHODS: The Nationwide Emergency Department Sample (NEDS) was analyzed for pediatric (age <18) ED visits from 2015 to 2019. DEB was identified with ICD-10-CM code Q81.2. Weighted frequency, prevalence, and 95% confidence intervals (CIs) of comorbidities were determined among ED visits with and without a DEB diagnosis.
RESULTS: From 2015 to 2019, 53 (weighted 242) cases of DEB among 27,223,220 pediatric ED visits were captured. Patients with DEB were more likely to visit the ED in summer compared with those without a diagnosis of DEB (35.7% vs. 21.4%, P < .05). More than half of patients with DEB were admitted to the hospital (56.2%, 95% CI: 39.3-72.5, P < .001) versus only 3.4% (95% CI: 3.1-3.7) of other patients. For ED visits with a secondary DEB diagnosis, the top three primary diagnoses were fever, constipation, and bone marrow transplant aftercare. Patients with DEB had higher rates of hypertension, cellulitis, sepsis, acute and chronic kidney injury, esophageal obstruction, gastroesophageal reflux disease, cardiomyopathy, and anxiety, compared to patients without DEB (all P < .001).
CONCLUSIONS: DEB is a complex blistering disorder with multisystemic manifestations. Patients with DEB have significantly higher admission rates and commonly present with infectious or gastrointestinal complications. Understanding the features of ED visits due to DEB can better prepare healthcare teams and improve patient outcomes.
METHODS: The Nationwide Emergency Department Sample (NEDS) was analyzed for pediatric (age <18) ED visits from 2015 to 2019. DEB was identified with ICD-10-CM code Q81.2. Weighted frequency, prevalence, and 95% confidence intervals (CIs) of comorbidities were determined among ED visits with and without a DEB diagnosis.
RESULTS: From 2015 to 2019, 53 (weighted 242) cases of DEB among 27,223,220 pediatric ED visits were captured. Patients with DEB were more likely to visit the ED in summer compared with those without a diagnosis of DEB (35.7% vs. 21.4%, P < .05). More than half of patients with DEB were admitted to the hospital (56.2%, 95% CI: 39.3-72.5, P < .001) versus only 3.4% (95% CI: 3.1-3.7) of other patients. For ED visits with a secondary DEB diagnosis, the top three primary diagnoses were fever, constipation, and bone marrow transplant aftercare. Patients with DEB had higher rates of hypertension, cellulitis, sepsis, acute and chronic kidney injury, esophageal obstruction, gastroesophageal reflux disease, cardiomyopathy, and anxiety, compared to patients without DEB (all P < .001).
CONCLUSIONS: DEB is a complex blistering disorder with multisystemic manifestations. Patients with DEB have significantly higher admission rates and commonly present with infectious or gastrointestinal complications. Understanding the features of ED visits due to DEB can better prepare healthcare teams and improve patient outcomes.
摘要：
背景：营养不良性大疱性表皮松解症（DEB）描述了一种罕见的以皮肤脆弱为特征的遗传性起泡疾病。本研究旨在对频率进行分类，人口统计，成本,以及由于DEB而与急诊科（ED）就诊相关的合并症。
方法：分析了2015年至2019年全国急诊科样本（NEDS）的儿科（年龄<18岁）ED就诊情况。DEB被识别为ICD-10-CM代码Q81.2。加权频率,患病率,在有和没有DEB诊断的ED访视中确定合并症的95%置信区间(CIs).
结果：从2015年到2019年，在27,223,220例儿科ED访视中捕获了53例（加权242例）DEB。与未诊断为DEB的患者相比，DEB患者在夏季更有可能去ED（35.7％vs.21.4%,P<0.05)。超过一半的DEB患者入院(56.2%,95%CI：39.3-72.5，P<.001），而其他患者仅为3.4%（95%CI：3.1-3.7）。对于二次DEB诊断的ED就诊，前三名的主要诊断是发烧，便秘,和骨髓移植后护理。DEB患者的高血压发病率更高，蜂窝织炎,脓毒症,急性和慢性肾损伤，食管梗阻,胃食管反流病,心肌病,和焦虑，与无DEB的患者相比（所有P<.001）。
结论：DEB是一种多系统表现的复杂的起泡障碍。DEB患者的入院率明显较高，并且通常存在感染或胃肠道并发症。了解由于DEB导致的ED访问的特征可以更好地准备医疗团队并改善患者结果。
方法：分析了2015年至2019年全国急诊科样本（NEDS）的儿科（年龄<18岁）ED就诊情况。DEB被识别为ICD-10-CM代码Q81.2。加权频率,患病率,在有和没有DEB诊断的ED访视中确定合并症的95%置信区间(CIs).
结果：从2015年到2019年，在27,223,220例儿科ED访视中捕获了53例（加权242例）DEB。与未诊断为DEB的患者相比，DEB患者在夏季更有可能去ED（35.7％vs.21.4%,P<0.05)。超过一半的DEB患者入院(56.2%,95%CI：39.3-72.5，P<.001），而其他患者仅为3.4%（95%CI：3.1-3.7）。对于二次DEB诊断的ED就诊，前三名的主要诊断是发烧，便秘,和骨髓移植后护理。DEB患者的高血压发病率更高，蜂窝织炎,脓毒症,急性和慢性肾损伤，食管梗阻,胃食管反流病,心肌病,和焦虑，与无DEB的患者相比（所有P<.001）。
结论：DEB是一种多系统表现的复杂的起泡障碍。DEB患者的入院率明显较高，并且通常存在感染或胃肠道并发症。了解由于DEB导致的ED访问的特征可以更好地准备医疗团队并改善患者结果。
