Outcomes of transcatheter closure for coronary artery fistulas with or without aneurysm: A comparative study.

BACKGROUND: Coronary Artery Fistulas (CAFs) Patients with aneurysm may face severe complications, necessitating prompt treatment. However, data on the outcomes of transcatheter closure in CAFs patients with aneurysm are notably scarce. METHODS: This retrospective study included all consecutive CAFs patients who underwent transcatheter closure at Fuwai Hospital from January 2010 to December 2023. Patients were divided into two groups based on the presence of aneurysm, and baseline characteristics, anatomical features, and transcatheter closure outcomes were further compared. RESULTS: The study ultimately included 104 patients, consisting of 56 in the aneurysm group and 48 in the non-aneurysm group. Patients in the aneurysm group were younger [39.79 (16.35) versus 50.69 (13.31) years, p < 0.001] and more frequently present with heart murmurs (21.43% vs. 6.25%, p = 0.03). Multivariate logistic regression indicated that a larger fistula diameter and the presence of CCFs are independent risk factors for the presence of aneurysm in CAF patients. The procedural success rate (75% vs. 75%, P = 1), fistula recanalization rate (11.11% vs. 16.67%, p = 0.42), and reintervention rate (3.7% vs. 6.25%, p = 0.89) were similar between the aneurysm and non-aneurysm groups. CONCLUSION: A larger fistula diameters and the presence of coronary-cameral fistulas are independent risk factors for the occurrence of aneurysms in patients with CAFs. The outcomes of transcatheter closure are comparable for CAFs patients with and without aneurysm, though post-closure thrombosis within the fistula appears to be more common in patients with aneurysm.

Etiology and prognosis of non-Kawasaki disease induced coronary aneurysms in children: a retrospective case series study.

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. WHAT IS KNOWN: • 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. WHAT IS NEW: • 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.

Advancing Kawasaki Disease Research in the Arab World: Scoping Literature Review Analysis with Emphasis on Giant Coronary Aneurysms.

To evaluate giant aneurysms (GiAn) prevalence in Arab countries and examine contributing factors; and to review Kawasaki disease (KD) publication trends and collaborations among Arab nations. A scoping literature review was conducted to analyze the publications across the Arab world, spanning 16 countries from 1978 to 2023. The collected articles were a combination of database search with a call on Kawasaki Disease Arab Initiative (Kawarabi) members to share non-PubMed publications. Over 45 years, 50 articles originated from the Arab Countries with a 30% average annual growth rate in KD research output. Publications were evenly split between case reports (42%) and institutional series (52%). Research productivity lagged in developing nations with UAE, KSA and Egypt, contributed to 64% of total publications. Among 26 institutional series, 256 coronary artery aneurysms (CAA) from a total of 1264 KD cases were reported. Of those, 25 CAA were GiAn (prevalence 1.43% [range 0-12.5%]). The initial KD misdiagnosis rate was 4%, and incomplete KD (iKD) averaged 10.6%. Series (38.5%) that did not report iKD correlated with a higher prevalence of CAA, but not of GiAn. Longer fever duration emerged as a pivotal factor for GiAn (OR 5.06, 95%CI 1.51-17). This review unveils the research landscape of KD in the Arab world over 45 years. Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of iKD are contributing factors for an underestimated epidemiology, explaining the higher GiAn prevalence. This calls for strategic interventions to enhance KD research in these countries, aligning with Kawarabi's mission.

A successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula in a young patient.

Giant coronary aneurysm with ventricular fistula is rare. Due to the limited data from randomised clinical trials, therapeutic strategies for coronary aneurysms predominantly rely on on case series and anecdotal evidences. Reporting cases that provide practical experience in managing these aneurysms is therefore crucial. In this article, we report a rare case of a successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula, which is larger than any previously reported cases.

Vascular aneurysms in Ehlers-Danlos syndrome subtypes: A systematic review.

Aneurysmal lesions are commonly seen in Ehlers-Danlos Syndrome (EDS). To better identify the regional and vessel-specific spectrum of aneurysms in different subtypes of EDS, we performed a systematic review. We searched Medline for relevant studies from 1963 to April 2022. Studies providing a report of any EDS subtype by genetic diagnosis, histologic analysis, or clinical criteria were included. A total of 448 patients from 220 studies were included. 720 vessel-specific aneurysms were reported: 386 in the abdominopelvic area, 165 in the intracranial region, 98 in the thorax, 2 in the extremities, and 6 in the venous system. In 27 out of the 65 patients with ruptured aneurysms, the ruptured aneurysm was the initial presentation. Multiple aneurysms were present in 163 out of 249 patients who had been systematically evaluated for other locations of aneurysms. The head and neck and abdominopelvic regions are two potential foci for aneurysm formation in patients with EDS. The aneurysm development in EDS is not confined to arteries; the venous system and cardiac septa may also be affected. Many patients develop multiple aneurysms, either at the time of the initial presentation or throughout their lifetime and aneurysm formation or rupture may be the first presentation of EDS.

Management of a right coronary artery mycotic coronary aneurysm with percutaneous intervention with covered stents.

Coronary artery aneurysmal dilation is a rare finding with poorly understood mechanism of action that is found in small population of patients undergoing coronary angiography. Mycotic coronary aneurysm is an even rarer cause of coronary aneurysmal dilatation that develops as a potentially fatal complication of bacteremia. We present a case of mycotic right coronary artery aneurysm in a nonsurgical candidate with complex medical comorbidities treated with percutaneous coronary intervention via covered stents.

Delayed coronary perforation four days after percutaneous coronary intervention with subsequent cardiac tamponade: A case report.

Coronary artery perforation (CAP) is a rare but lethal complication of percutaneous coronary interventions (PCIs), and its incidence has been increasing with advances in PCI techniques. Delayed CAP presents a highly challenging complication, as it occurs 30 min-9 days after intervention, making subsequent diagnosis and treatment difficult. We present the case of a 63-year-old male patient who underwent PCI for an obtuse marginalis II because of posterior wall myocardial infarction. Following 4 days of uneventful postoperative stay, the patient developed angina pectoris and hypotension 4 h after reinitiation of anticoagulant therapy with edoxaban. Angiography revealed distal vessel perforation from a side branch of the obtuse marginalis II. The vessel was occluded using autologous fat embolization via a microcatheter, resulting in complete sealing of the perforation. After discharge, 4 weeks after the infarction, the patient started rehabilitation therapy. Distal vessel perforations are typically caused by wire damage. In our case, we also suspected distal wire perforation, which was initially not recognized possibly due to distal occlusion through the thrombotic material. The temporal correlation between the re-initiation of anticoagulant therapy and the occurrence of cardiac tamponade suggests that the thrombotic material was resolved due to the former. The management of delayed CAP does not differ from that of CAP; thus, this rare complication should be considered even days after PCI as it could prove lethal if not recognized early.

Self-navigated coronary MR angiography for coronary aneurysm detection in Kawasaki disease at 3T: comparison with conventional diaphragm-navigated coronary MR angiography.

OBJECTIVES: To assess the scan time, image quality, and diagnostic performance of self-navigated coronary MR angiography (SN-CMRA) for coronary aneurysm (CAA) detection in Kawasaki disease (KD) patients and compare it with diaphragm-navigated CMRA (DN-CMRA). MATERIALS AND METHODS: SN-CMRA and DN-CMRA were performed on 76 pediatric patients with KD (48 males, 6.75 ± 3.59 years). Thirty-three of whom underwent coronary CT angiography (CCTA)/invasive coronary angiography (ICA). The scan time and qualitative and quantitative image quality assessment were compared between the two sequences. The diagnostic performance for CAA detection by the two approaches using CCTA/ICA as the reference standard was compared on per-patient, per-vessel, and per-segment basis. RESULTS: The scan time of SN-CMRA was significantly shorter than that of DN-CMRA (7.49 ± 2.31 min vs. 10.03 ± 4.47 min, p < 0.001). There was no difference in overall and segmental image quality to reach the clinical diagnostic criteria between the two sequences (all p > 0.05). No significant difference in vessel length of the three main coronary arteries was found between the two approaches (all p > 0.05). Moreover, SN-CMRA showed no difference from DN-CMRA in contrast ratio of blood-myocardium (1.25 (interquartile range [IQR], 1.06 to 1.51) vs. 1.18 (IQR, 0.95 to 1.64), p = 0.706). There was no difference in the diagnostic accuracy of SN-CMRA and DN-CMRA for CAA detection on per-patient, per-vessel, or per-segment basis (all p > 0.05). CONCLUSION: SN-CMRA at 3T showed reliable diagnostic performance and application value for CAA detection in children with KD. Compared with DN-CMRA, SN-CMRA can simplify the scanning procedure and shorten the scan time, achieving comparable image quality and diagnostic accuracy. CLINICAL RELEVANCE STATEMENT: Coronary aneurysm in children with Kawasaki disease (KD) can be detected by self-navigated coronary MR angiography (CMRA) non-invasively and without radiation, achieving comparable image quality and diagnostic performance as diaphragm-navigated CMRA while shortening scanning time. It can provide reference for risk stratification and treatment management of KD. KEY POINTS: • Evaluating the size of coronary aneurysm is important for risk stratification and treatment of Kawasaki disease. • Self-navigated coronary MR angiography (SN-CMRA) shortens scan time and achieves comparable image quality and diagnostic performance compared with diaphragm-navigated coronary MR angiography. • SN-CMRA can evaluate coronary aneurysm non-invasively and without radiation, providing information for risk stratification and treatment.

Growing Coronary Aneurysm Secondary to Coronary Fistula Despite Decreased Pulmonary Blood Flow/Systemic Blood Flow Ratio in a Child: A Case Report.

Coronary aneurysm secondary to coronary fistula is a rare condition, with no existing report on its pathological examination. We report the case of a patient diagnosed with a right coronary artery fistula with coronary aneurysm during the fetal period. During follow-up after delivery, the aneurysm became larger, even though the shunt size decreased. We were afraid the aneurysm would rupture and therefore, planned elective catheter embolization. At the age of 4 years, the patient underwent surgery, which involved closing the fistula and making the lumen of the aneurysm smaller. However, the surgery was not catheter embolization as planned because segment 3 branched off from the largest aneurysm where we planned to embolize. Pathologically, the structure of the coronary artery differed from that of a healthy one, with thickened intima and media, fewer scattered smooth muscle cells, widely distributed elastic fibers, and mucoid degeneration in the media. The structure of the coronary artery suggested that the vessel wall was weak and that the aneurysm would rupture if not treated. Postoperative coronary angiography showed that segment 2 was obstructed, while the collaterals from the left coronary artery perfused the area. We could have treated the fistula with a catheter as scheduled.

Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum.

Polyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab presented with persistent rise in inflammatory markers and gastrointestinal bleeding. Digital subtraction angiography (DSA) revealed celiac artery branches stenosis and beading suggestive of PAN. Another 2-year-old girl presented with persistent fever, abdominal pain, and distension. She had hypertension, hepatomegaly, and splenomegaly on examination. Echocardiography revealed multiple coronary aneurysms and DSA revealed numerous renal artery aneurysms. Coronary aneurysm although is a rare presentation of childhood PAN, and can mimic Kawasaki disease. Although both are medium-vessel vasculitis differentiation between these two entities is pivotal, as there are differences in treatment modalities, duration of immunomodulatory therapy, and the outcome. This manuscript describes the salient differences which can help differentiate PAN masquerading as Kawasaki disease at initial presentation.

Prednisolone versus cyclosporine as initial treatment for Kawasaki disease.

BACKGROUND: Several studies have demonstrated the efficacy of prednisolone and cyclosporine as initial combination treatments for the prevention of coronary artery abnormalities (CAA) in patients with Kawasaki disease. However, whether prednisolone or cyclosporine results in superior clinical outcomes is unknown. Thus, this study aimed to compare the outcomes of these two treatments. METHODS: Using the Japanese Diagnosis Procedure Combination database, we identified patients with Kawasaki disease who had received prednisolone or cyclosporine in addition to initial intravenous immunoglobulin treatment between April 2014 and March 2021. The primary outcome was the proportion of CAA; secondary outcomes included intravenous immunoglobulin resistance, medical costs, and length of hospital stay. Propensity score matching was conducted to compare outcomes between the two groups. RESULTS: We identified 6288 patients with Kawasaki disease who had received prednisolone (n = 6147) or cyclosporine (n = 141) as an initial treatment in combination with intravenous immunoglobulin. Four-to-one propensity score-matched analysis demonstrated no significant difference in the proportion of CAA (0.7% vs. 2.8%; p = 0.098), intravenous immunoglobulin resistance, or medical costs between the treatment groups. The length of hospital stay was significantly longer in the prednisolone group (14 vs. 11 days, p < 0.001). CONCLUSIONS: Prednisolone and cyclosporine used in the initial combination treatment for Kawasaki disease showed similar clinical outcomes regarding the risk of CAA, intravenous immunoglobulin resistance, and medical costs, whereas the length of hospital stay was longer in the prednisolone group than in the cyclosporine group.

Occurrence of Kawasaki disease and neoplasms in temporal proximity-single-center experience and systematic review of literature.

Various factors (e.g., infections) have been postulated to trigger Kawasaki disease (KD) in genetically predisposed individuals. Whether neoplasms can trigger KD is largely unknown due to paucity of data. Herein, we provide a detailed account of KD occurring in temporal proximity (within 6 months) to neoplasms ('neoplasm-KD'). Patients with 'neoplasm-KD' diagnosed/treated at our center from January 1994 to May 2021 were included. Additionally, we performed a systematic literature review (as per PRISMA 2020 guidelines) utilizing PubMed, Web of Science and Scopus databases to retrieve details of all patients with 'neoplasm-KD' reported till June 2021. Patients with multisystem inflammatory syndrome in children were excluded. As all reports pertained to case description(s), risk of bias assessment was not performed. The details of patients with 'neoplasm-KD' were analyzed using SPSS software. Primary and secondary outcomes were occurrence of coronary artery abnormalities (CAAs) and clinical characteristics of 'neoplasm-KD', respectively. A total of 25 patients (data from 18 reports) were included in the 'neoplasm-KD' dataset. The most frequently diagnosed neoplasm was acute lymphoblastic leukemia followed by neuroblastoma and acute myeloblastic leukemia. Overall, CAAs were noted in 48% of patients. Interval between diagnoses of KD and neoplasm was shorter in patients with CAAs as compared to patients with normal coronary arteries (p-value = 0.03). Besides providing a comprehensive description of 'neoplasm-KD', this study raises a possibility that neoplasms might trigger KD. Also, 'neoplasm-KD' may be associated with a higher risk of development of CAAs. However, the small size of 'neoplasm-KD' dataset precludes definitive conclusions regarding this association. Funding: nil. Registration: PROSPERO (CRD42021270458).

This study is the first exhaustive description of cancers and Kawasaki disease (KD) occurring in close temporal proximity. Nearly half of these patients develop coronary artery abnormalities. In KD, persistent lymphadenopathy, enlargement of liver/spleen and development of low blood cell counts should trigger evaluation for cancer. Our study also raises a possibility that cancers might occasionally trigger KD.

Circulating miRNA-451a and miRNA-328-3p as Potential Markers of Coronary Artery Aneurysmal Disease.

MicroRNAs (miRNAs) are currently investigated as crucial regulatory factors which may serve as a potential therapeutic target. Reports on the role of miRNA in patients with coronary artery aneurysmal disease (CAAD) are limited. The present analysis aims to confirm the differences in the expression of previously preselected miRNAs in larger study groups and evaluate their usefulness as potential markers of CAAD. The study cohort included 35 consecutive patients with CAAD (Group 1), and two groups of 35 patients matched Group 1 regarding sex and age from the overall cohort of 250 patients (Group 2 and Group 3). Group 2 included patients with angiographically documented coronary artery disease (CAD), while Group 3 enrolled patients with normal coronary arteries (NCA) assessed during coronary angiography. We applied the RT-qPCR method using the custom plates for the RT-qPCR array. We confirmed that the level of five preselected circulating miRNAs was different in patients with CAAD compared to Group 2 and Group 3. We found that miR-451a and miR-328 significantly improved the CAAD prediction. In conclusion, miR-451a is a significant marker of CAAD compared to patients with CAD. In turn, miR-328-3p is a significant marker of CAAD compared to patients with NCA.

A Case of Giant Left Circumflex Coronary Fistula Aneurysm with Unidentified Fistula.

A coronary aneurysm is a rare type of cardiovascular disease. We report a case of a 53-year-old male patient who presented to our hospital with a giant left circumflex coronary fistula aneurysm (LCCA) (75 mm × 70 mm). Since coronary angiography and coronary computed tomography angiography failed to detect the fistula of the coronary aneurysm, interventional occlusion surgery could not be performed. We discovered the fistula in the right atrium by anterograde perfusion with blood-containing myocardial protective fluid after switching to intraoperative exploration during cardiac surgery. The coronary aneurysm's fistula and inlet were then sutured, and the aneurysm was resected. The patient recovered successfully after the operation. This case was instructive in managing LCCA, especially with an unidentified fistula.

Retrospective analysis of children diagnosed with Kawasaki disease.

BACKGROUND: The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course. METHODS: Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement. Patients in group 1 had no echocardiographic findings, while the ones in group 2 had coronary artery dilatation and ones in group 3 had coronary artery aneurysm (CAA). RESULTS: Among 104 patients, the median age was 9.15 (3.0-22.0) years, and 61 of the patients were male while 43 of the patients were female. With a wide range of 1.50-16.50 years of follow-up time, the median diagnosis age of our patients was 31 months (3.0-164.0). Fever duration (median day 10 (5-21), p = 0.025) was statistically significantly higher in group 3. Blood C-reactive protein (CRP) levels, white blood cell (WBC) counts, and neutrophil counts were significantly higher in group 3. There was a statistically significant difference between patients in group 3 and group 2 in which the lowest strain deformation values were in the patients of group 3. In contrast to group 1, the time for initiation of IVIG therapy is significantly prolonged both in group 2 (median: 9.5 days, p = 0.028) and group 3 (median: 10 days, p = 0.036). DISCUSSION: In our study, serum CRP levels, WBC count, and neutrophil count were higher in patients with coronary artery abnormalities, in agreement with the previous studies. In the light of our results, we consider that the most important determining factor for the development of coronary artery aneurysm is the time of intravenous immunoglobulin (IVIG) administration.

Coronary Artery Changes in Patients with Multisystem Inflammatory Syndrome in Children: Los Angeles Experience.

We compared cardiac findings in patients with multisystem inflammatory syndrome in children and Kawasaki disease in the first 6 months of the 2020 coronavirus disease pandemic to patients with Kawasaki disease during 2016-2019. We saw a high rate of coronary aneurysms in 2020, with a similar rate of coronary involvement but greater volume and incidence of cardiac dysfunction compared with previous years.

Kawasaki disease in children younger than 6 months of age: characteristics of a Spanish cohort.

A retrospective study that compared children younger than 6 months versus older children of a Spanish cohort of patients diagnosed with Kawasaki disease between 2011 and 2016 (Kawa-Race study). From the 598 patients recruited, 42 patients were younger than 6 months (7%) and presented more frequently with an incomplete diagnosis of Kawasaki disease (52.4 vs 27.9%, p = 0.001). Cardiac abnormalities detected by echocardiography were more common in younger patients (52.4 vs 30%, p = 0.002). These younger patients presented with a higher proportion of coronary aneurysms as well (19 vs 8.6%, p < 0.001). Shock at diagnosis (9.5 vs 1.9%, p = 0.016) and admission to intensive care units (17.7 vs 4.1%, p = 0.003) were more frequent in patients younger than 6 months. There were no statistically significant differences in relation to infections, non-response to IVIG, or mid- or long-term outcomes.Conclusion: Data of the Spanish cohort are consistent with other American and Asian studies, although Spanish children younger than 6 months had a lower rate of non-response to IVIG and better clinical outcomes. A high index of suspicion should be considered for this population due to a higher risk of coronary abnormalities, presentation of shock, and admission to the intensive care unit. What is Known: •Children below 6 months of age with Kawasaki disease (KD) have different features compared to older. •Younger patients usually have an incomplete form of KD and coronary artery abnormalities. What is New: •Younger than 6 months with KD presented with shock and required admission to PICU more frequently compared to older. •Infections play a similar role in KD despite the age of the patients.

Sivelestat's effect on Candida albicans water-soluble fraction-induced vasculitis.

BACKGROUND: We investigated the efficacy of sivelestat sodium hydrate (SSH) as a treatment for Kawasaki disease, and its pharmacological action sites, in mice with Candida albicans water-soluble fraction-induced vasculitis. METHODS: Sivelestat sodium hydrate was administered intraperitoneally to Candida albicans water-soluble fraction-induced vasculitis model mice to assess its efficacy in preventing the development of coronary artery lesions based on the degree of inflammatory cell infiltration in the aortic root and coronary arteries (vasculitis score). The pharmacological sites of action were investigated based on changes in neutrophil elastase (NE) and intercellular adhesion molecule 1 (ICAM-1) positive areas, ICAM-1 and tumor necrosis factor-α mRNA expression levels in the upper heart, and the proportion of monocytes in the peripheral blood. RESULTS: The vasculitis score decreased below the lower limit of the 95% confidence interval of untreated mice in 69% of the SSH-treated mice. The NE- and ICAM-1-positive regions, and the mRNA expression of ICAM-1 and tumor necrosis factor-α were lower in the SSH-treated mice than in the untreated mice. The proportion of monocytes in the peripheral blood was higher in the SSH-treated mice than in the untreated mice, whereas monocyte migration to inflammation areas was suppressed in the SSH-treated mice. CONCLUSIONS: Our results showed that SSH might prevent the development of coronary artery lesions and ameliorate disease activity. In addition to its NE-inhibitory effect, SSH sites of action may also include monocytes.

The impact of vitamin D on the onset and progress of Kawasaki disease.

BACKGROUND: The seasonal epidemic of Kawasaki disease (KD) in winter in Japan suggests that low vitamin D status may affect KD through the immune system. We aimed to evaluate the effect of vitamin D on the onset and clinical course of KD. METHODS: We conducted a case-control study to compare 25-hydroxyvitamin D (25(OH)D) levels in KD patients admitted to our hospital between March 2018 and June 2021, with those in healthy controls from published Japanese data. In patients with KD, we evaluated the association of 25(OH)D levels with intravenous immunoglobulin resistance and coronary artery lesions. RESULTS: We compared 290 controls and 86 age-group-adjusted patients with KD. The 25(OH)D levels in KD patients were lower than those in the controls (median: 17 vs. 29 ng/mL, P < 0.001). In winter, 25(OH)D levels in KD patients were lower than those in summer (median: 13 vs. 19 ng/mL). The adjusted odds ratios for the onset of KD were 4.9 (95% CI: 2.5-9.6) for vitamin D insufficiency (25(OH)D: 12-20 ng/mL) and 29.4 (95% CI: 12.5-78.2) for vitamin D deficiency (25(OH)D < 12 ng/mL). Among 110 KD patients, 25(OH)D levels at diagnosis of KD were not associated with intravenous immunoglobulin resistance or coronary artery lesions. CONCLUSIONS: The 25(OH)D levels in patients with KD were lower than those in the controls, especially in winter. Lower 25(OH)D levels in winter were associated with an increased risk of KD onset. It remains to be elucidated whether the observed association has a causal relationship.

Delayed diagnosis of Kawasaki disease in Malaysia: Who is at risk and what is the outcome.

BACKGROUND: Data on the delayed diagnosis of (KD) is limited. This study aimed to determine the prevalence and trend of delayed diagnosis over time and identify the associated risk factors in Malaysia. METHODS: This retrospective, observational, population-based study involved all children with KD registered in Johor Kawasaki Clinical Registry over 10 years (January 2010 and December 2019). The prevalence of delayed diagnosis and its trend over time was calculated. Multivariable binary logistic regression was used to identify the independent risk factors for delayed diagnosis. RESULTS: There were 556 cases of KD, with 28% having incomplete criteria, 11% atypical presentation, while 10% developed a coronary aneurysm. The overall prevalence of delayed diagnosis was 9.9% (95% confidence interval (CI): 7.6-12.7%). There was a statistically significant decrease in delayed diagnosis over time (P = 0.008), with the latest rate of 4.6%. The majority of delayed diagnoses were due to failure to diagnose the disease during the initial consultation. Independent risk factors for delayed diagnosis were children older than 1 year, diagnosis before 2015, atypical presentation, and incomplete KD, with adjusted odds ratios (ORs) of 2.7, 2.3, 4.3, and 3.6, respectively. Compared to early diagnosis of KD, delayed diagnosis was significantly associated with coronary aneurysms (27.3% vs. 8.2%, P < 0.001, OR 4.2, [95% CI: 2.1-8.3]). CONCLUSIONS: One-tenth of cases of KD were diagnosed late, but it has improved over time. Children > 1 year, presenting with atypical presentation, and incomplete criteria are associated with late diagnosis.