虽然川崎病(KD)引起的儿童冠状动脉瘤(KDCAAs)得到了很好的研究,在儿科人群中,非KD诱导的CAAs(非KDCAAs)的特征和预后记录不甚清楚.本病例系列研究旨在分析儿童非KDCAAs的病因和预后,并比较非KDCAAs和KDCAAs的特征。回顾性收集了我们部门2022年1月至2023年12月的非KDCAA和KDCAA病例。分析非KDCAAs的病因及预后。此外,人口统计数据,对非KDCAAs患儿和KDCAAs患儿的生化指标和结局进行了比较研究。本研究包括15名非KDCAAs儿童,中位年龄为6岁,117名KDCAAs儿童,中位年龄为2.0岁(p=0.022)。非KDCAAs的病因包括:病因不明(2例),冠状动脉结构异常(4),大动脉炎(2),病毒感染(2),心肌病(2),再生障碍性贫血与粒细胞缺乏(1),ANCA相关性血管炎(1),和粘多糖贮积症(1)。在非KDCAA组中,总共有19个CAA,其中3个是巨型的,5个中等,11例;4例患者的CAA完全消退;一名婴儿在右冠状动脉和冠状静脉窦之间有瘘管,并伴有心脏扩大,死于心力衰竭。KD组CRP水平明显增高,白细胞计数和ESR,死亡率为零。非KDCAA病例的消退率显着低于KD-CAA病例(26.7%vs66.7%,p=0.004),非KD患者的CAA消退概率为KD患者的0.341(p=0.006,OR=0.341,95%CI:0.179-0.647)。
结论:确定了非KDCAAs的各种病因。与KDCAAs患者相比,非KDCAAs患者的炎症指标较低,但恢复较差。非KDCAAs可能需要不同于KD的治疗策略。
背景:•儿童冠状动脉瘤(CAA)最常见于川崎病(KDCAA),在1~2年内有50~70%的回归率。•儿童中由KD(非KDCAA)以外的疾病引起的CAA很少见,其预后仍不清楚。
背景:•大多数非KDCAA病例是由冠状动脉结构畸形引起的。•与KDCAA相比,儿童非KDCAA的预后较差,消退率较低。•除了指南指导的抗血小板和抗凝治疗,针对致病因素的治疗对于非KDCAA是必要的。
While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179-0.647).
CONCLUSIONS: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs.
BACKGROUND: • Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years. • CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown.
BACKGROUND: • Most non-KD CAA cases are caused by coronary artery structural malformations. • Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA. • In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA.