BACKGROUND Children with rare bone diseases (RBDs), whether medically complex or not, raise multiple issues in emergency situations. The healthcare burden of children with RBD in emergency structures remains unknown. The objective of this study was to describe the place of the pediatric emergency department (PED) in the healthcare of children with RBD. METHODS We performed a retrospective single-center cohort study at a French university hospital. We included all children under the age of 18 years with RBD who visited the PED in 2017. By cross-checking data from the hospital clinical data warehouse, we were able to trace the healthcare trajectories of the patients. The main outcome of interest was the incidence (IR) of a second healthcare visit (HCV) within 30 days of the index visit to the PED. The secondary outcomes were the IR of planned and unplanned second HCVs and the proportion of patients classified as having chronic medically complex (CMC) disease at the PED visit. RESULTS The 141 visits to the PED were followed by 84 s HCVs, giving an IR of 0.60 [95% CI: 0.48-0.74]. These second HCVs were planned in 60 cases (IR = 0.43 [95% CI: 0.33-0.55]) and unplanned in 24 (IR = 0.17 [95% CI: 0.11-0.25]). Patients with CMC diseases accounted for 59 index visits (42%) and 43 s HCVs (51%). Multivariate analysis including CMC status as an independent variable, with adjustment for age, yielded an incidence rate ratio (IRR) of second HCVs of 1.51 [95% CI: 0.98-2.32]. The IRR of planned second HCVs was 1.20 [95% CI: 0.76-1.90] and that of unplanned second HCVs was 2.81 [95% CI: 1.20-6.58]. CONCLUSION An index PED visit is often associated with further HCVs in patients with RBD. The IRR of unplanned second HCVs was high, highlighting the major burden of HCVs for patients with chronic and severe disease.

中文翻译：

---

就诊于儿科急诊科的罕见骨病儿童的医疗保健轨迹。

背景技术患有罕见骨病（RBD）的儿童，无论是否在医学上很复杂，在紧急情况下都会引发多个问题。RBD儿童在急救机构中的医疗保健负担仍然未知。这项研究的目的是描述儿科急诊科（PED）在RBD儿童的医疗保健中的地位。方法我们在法国大学医院进行了一项回顾性单中心队列研究。我们纳入了2017年访问PED的所有18岁以下RBD患儿。通过交叉检查医院临床数据仓库中的数据，我们能够追踪患者的医疗保健轨迹。感兴趣的主要结果是在对PED进行索引访问后30天内第二次就诊（HCV）的发生率（IR）。次要结果是有计划和无计划的第二次HCV的IR，以及在PED访视时被分类为患有慢性药物复杂性（CMC）疾病的患者比例。结果对PED进行了141次随访，之后是84 s HCV，IR为0.60 [95％CI：0.48-0.74]。这些第二批HCV计划使用60例（IR = 0.43 [95％CI：0.33-0.55]），而未计划使用24种（IR = 0.17 [95％CI：0.11-0.25]）。CMC疾病患者占59次指数访视（42％）和43 s HCV（51％）。多变量分析包括CMC状态作为独立变量，并根据年龄进行了调整，得出第二批HCV的发生率比（IRR）为1.51 [95％CI：0.98-2.32]。计划中的第二批HCV的IRR为1.20 [95％CI：0.76-1.90]，未计划中的第二批HCV的IRR为2.81 [95％CI：1.20-6.58]。结论PED访视通常与RBD患者进一步的HCV相关。计划外的第二批HCV的IRR很高，突出了HCV对慢性和重症患者的主要负担。