BACKGROUND: Coronary artery thrombosis and myocardial ischemia caused by giant coronary aneurysms are the main causes of death in children with Kawasaki disease. The use of thrombolytic therapy in children with Kawasaki disease who have coronary thrombosis is a controversial topic, especially with respect to the timing of treatment.
METHODS: In this article, we report a case of a child aged two years and nine months with Kawasaki disease whose coronary arteries had no involvement in the acute phase. However, by only one week after discharge, the patient returned because we found giant coronary aneurysms complicated by thrombosis via echocardiography. Despite aggressive thrombolytic therapy, the child developed myocardial ischemia during thrombolytic therapy. Fortunately, because of timely treatment, the child\'s thrombus has dissolved, and the myocardial ischemia has resolved.
CONCLUSIONS: This case suggests that for patients at high risk of coronary artery aneurysms, echocardiography may need to be reviewed earlier. Low-molecular-weight heparin should be added to antagonize the early procoagulant effects of warfarin when warfarin therapy is initiated. In the case of first-detected coronary thrombosis, aggressive thrombolytic therapy may be justified, particularly during the acute and subacute phases of the disease course.

UNASSIGNED: We aimed to explore simple and effective clinical parameters or combinations to predict coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease (KD).
UNASSIGNED: This retrospective cohort study included pediatric patients with KD from January, 2013 to December, 2022. Multiple demographic and clinical data were collected, collated, and calculated from the medical records. Then they were divided into the coronary artery dilation and aneurysm formation group or the non-coronary artery dilation and aneurysm formation group. Lymphocyte-C-reactive protein ratio (LCR) was transformed into its natural logarithm and expressed as lnLCR.
UNASSIGNED: A total of 64 pediatric patients with KD were enrolled in this cohort study after 1:3 propensity score matching (PSM). For each unit increase in lnLCR, the possibility of coronary artery dilation and aneurysm formation decreased to 0.419 times the original value. The areas under the receiver operating characteristic (ROC) curves of lnLCR combined with albumin (ALB), ALB, and lnLCR to classify pediatric patients with KD into the coronary artery dilation and aneurysm formation group were 0.781, 0.692, and 0.743, respectively.
UNASSIGNED: LCR combined with ALB upon admission is a promising predictor of coronary artery dilation and aneurysm formation in pediatric patients with KD.

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.

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BACKGROUND: Kawasaki disease (KD) is a vasculitis of unknown etiology in children aged under 5 years. Coronary arterial aneurysm (CAA) is the major complication of KD. It is no longer though to be a self-limiting disease because its cardiovascular sequelae might persist into adulthood. NLRP3 is a key protein of the NLRP3 inflammasome that participates in sterile inflammatory disease. This study investigated the serum levels of NLRP3 in patients with KD at different stages to explore the relationships between serum NLRP3 and clinical parameters.
METHODS: A total of 247 children enrolled in this study. There were 123 patients in the acute stage of KD, and 93 healthy children made up the healthy control (HC) group. Among the acute KD patients, 52 had coronary arterial aneurysm (KD-CAA) and 71 did not (KD-NCAA). 36 patient samples were collected after IVIG and aspirin treatment. Additionally, 29 patients were in the cardiovascular sequelae stage. Enzyme-linked immunosorbent assay was used to measure serum NLRP3 levels in all subjects.
RESULTS: Serum NLRP3 was elevated in the KD group and was even higher in the KD-CAA subgroup than in the KD-NCAA subgroup of acute-stage patients. Serum NLRP3 declined when the patients were treated with IVIG and aspirin, but during the convalescent (coronary sequelae) stage, serum NLRP3 re-increased. Serum NLRP3 was higher in the ≥ 6-mm-coronary-arterial-diameter group than that the < 6-mm-diameter group. The ROC curve of serum NLRP3 indicated its utility in the prediction of both KD and KD-CAA.
CONCLUSIONS: NLRP3 may be involved in the development of KD and CAA in children with KD. Targeting NLRP3 might mitigate CAA, thereby reducing the risk of cardiovascular events in adulthood.

Mycoplasma pneumoniae (MP) infection serves as a substantial cofactor in Kawasaki disease (KD) among patients. Although the dominant issue triggering KD has recently focused on MP infection, the complete demonstration of the relationship between MP infection and KD remains elusive. This study endeavors to scrutinize and compare the clinical manifestations and cardiac involvement between MP-triggered KD and non-infection-associated KD.
This retrospective study (2023-039, approved by the Institutional Review Board of West China Second University Hospital of Sichuan University) encompassed 247 consecutive patients diagnosed with KD between June 2017 and December 2022. Patients were categorized into two groups: the MP group (n = 38) and the non-MP group (n = 209). Univariable analysis was utilized to discern differences in clinical features, severity of inflammation, and initial or persistent cardiac complications between the two groups.
The MP group exhibited a more intricate clinical profile compared with the non-MP group, characterized by prolonged hospital stays, a higher incidence of incomplete KD, and elevated comorbidities. In addition, MP infection correlated with severe hematological disorders, coagulation dysfunction, and myocardial injuries. Our findings revealed that MP infection led to prolonged inflammation after initial treatment with intravenous immunoglobulin. Although initial cardiac assessments failed to discern disparities between the two groups, MP infection notably exacerbated coronary artery aneurysms (CAAs), resulting in sustained dilation.
Recognizing MP infection as a significant infectious factor associated with KD is imperative. In patients with KD, MP infection significantly prolongs inflammation and causes hematological disturbances during the initial treatment phase. Moreover, the presence of MP infection exacerbates the progression of CAAs and myocardial injuries during the subacute phase of KD, consequently contributing to the persistence of CAAs.

BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects infants and young children but is extremely rare in neonates, especially afebrile KD. We present a case of KD without fever in a neonate and review the literature on KD in neonates.
METHODS: A newborn female was hospitalized because her peripheral blood leukocytes increased for half a day. The admission diagnosis was considered neonatal sepsis and bacterial meningitis. She had no fever since the admission, but a rash appeared on her face by the 7th day. On day 11 after admission, there was a desquamation on the distal extremities. On day 15 after admission, ultrasound showed non-suppurative cervical lymphadenopathy. Echocardiogram revealed coronary artery aneurysms in both sides. Finally, the patient was diagnosed with incomplete KD (IKD). The follow-up echocardiogram showed that the internal diameter of both coronary arteries returned to normal three months after birth.
CONCLUSIONS: Fever, rash, and distal extremity desquamation during the recovery phase are the most common symptoms of IKD. When newborns present with clinical manifestations such as rash, distal extremity desquamation and cervical lymph adenitis and with an increased peripheral blood leukocyte count and progressive increase in platelets simultaneously, the medical staff should be highly alert to the possibility of KD even without fever. The echocardiogram needs to be performed promptly. The incidence of coronary artery lesions is significantly higher if neonatal KD patients miss timely diagnosis and treatment.

Objective: To evaluate the intermediate and long-term outcomes and technical aspects of transcatheter closure (TCC) of coronary cameral fistulas (CCF) in pediatric patients. Methods: This was a case-control study. All pediatric patients with CCF who underwent TCC between January 2005 and December 2019 were retrospectively reviewed. Data was collected from medical records, including demographic characteristics, procedural details, intraoperative and postoperative serious adverse events, follow-up results and prognosis. Patients with serious adverse events and without serious adverse events were compared regarding their clinical features and CCF characteristics. Comparisons between groups were performed with independent sample t test, chi-square test or Fisher exact test. Results: A total of 66 CCF patients (34 boys, 32 girls, 3.9 (1.9, 6.2) years old, 15 (11, 20) kg) underwent attempted TCC. All of the CCF were all medium or large fistulas including 55 proximal fistulas (83%) and 11 distal fistulas (17%). The CCF originated more frequently from the right coronary artery (38 cases (58%)), followed by the left coronary artery (28 cases (42%)). The incidence of coronary artery aneurysms (CAA) was 61% (40/66).Procedural treatment was achieved in 64 patients and procedural success was achieved in 59 patients (92%). Six (9%) serious adverse events occurred in 5 patients during the perioperative period. Acute complications included procedure-related death in one patient and acute myocardial infarction in one patient. Periprocedural complications occurred in 3 patients at one day postoperatively including acute myocardial infarction (2 cases), occluder detachment (1 case), and tricuspid chordae tendinae rupture (1 case). Clinical follow-up data were available in 58 of the 62 patients who underwent initial successful TCC with a follow-up period of 9.3 (6.5, 13.4) years. Ten adverse events occurred in 9 patients including 5 complications consisted of aortic valve perforation (1 case), coronary thrombosis (1 case), progressive aneurysmal dilation after reintervention (1 case), and new-onset tricuspid valve prolapse with significant regurgitation (2 cases) and large residual shunts due to fistula recanalization (5 cases). Therefore, the incidence of intermediate and long-term adverse events was 17% (10/58). During the periprocedural and follow-up period, 16 adverse events occurred in 13 patients, whereas no adverse events occurred in 51 patients. Patients with seriovs adverse events presented with larger proportion of large CCF (11/13 vs. 39% (20/51), P=0.005), giant CAA (10/13 vs.14% (7/51), P=0.030), and higher mean pulmonary artery pressure ((20±9) vs.(16±6) mmHg, 1 mmHg=0.133 kPa, t=2.02, P=0.048) compared to patients without serious adverse events. Conclusions: TCC in CCF children appears to be effective with favorable intermediate and long-term outcomes. Strict indication of TCC is mandatory.
目的： 总结经导管介入治疗儿童先天性冠状动脉-心腔瘘（CCF）的经验及中远期随访结果。 方法： 病例对照研究。选择2005年1月至2019年12月在广东省人民医院接受经导管介入治疗的66例先天性CCF患儿作为研究对象，收集并分析其临床基线资料、手术效果、术中及术后严重不良事件、随访结果及预后等资料。根据是否发生严重不良事件将患儿进行分组，比较严重不良事件组和无严重不良事件组一般情况及CCF特征的差异。组间比较采用独立样本t检验、χ2检验或Fisher确切概率法。 结果： 66例先天性CCF的患儿中男34例、女32例，年龄3.9（1.9，6.2）岁，体重15（11，20）kg。66例CCF均为中型或大型瘘管，其中近端型瘘管55例（83%），远端型瘘管11例（17%）；瘘管起源于右冠状动脉38例（58%），起源于左冠状动脉28例（42%）；合并冠状动脉瘤（CAA）40例（61%）。66例患儿中64例患儿完成封堵治疗，5例患儿发生6例次围手术期严重不良事件。围手术期手术成功率为92%（59/64），围手术期不良事件发生率为9%（6/64），其中2例患儿术中发生了2例次严重不良事件，包括手术相关死亡1例，急性心肌梗死1例；3例患儿术后随访1 d发生了4例次严重不良事件，包括急性心肌梗死2例、三尖瓣腱索断裂1例、封堵器脱落1例。共58例患儿完成中远期随访，随访时间9.3（6.5，13.4）年。9例患儿发生10例次严重不良事件，中远期严重不良事件发生率为17%（10/58），其中5例次并发症（1例次新发CAA、1例次主动脉瓣穿孔、1例次冠状动脉血栓形成、2例次新发三尖瓣脱垂伴重度反流）以及5例次大量残余分流（瘘管再通）。围手术期及随访期间，13例患儿发生16例次严重不良事件，51例患儿未发生严重不良事件。发生严重不良事件组患儿的大型瘘管［11/13比39%（20/51），P=0.005］、巨大CAA［10/13比14%（7/51），P=0.030］所占比例均明显高于无不良事件组患儿，肺动脉平均压明显高于无不良事件组患儿［（20±9）比（16±6）mmHg，1 mmHg=0.133 kPa，t=2.02，P=0.048］。 结论： 经导管介入治疗儿童先天性CCF即刻疗效确切，中远期有效性和安全性尚可，但需严格把握CCF介入治疗适应证。.

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