A Ruptured Coronary Artery Aneurysm Secondary to Kawasaki Disease
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ABSTRACT:
SUBMITTER: Suzuki H
PROVIDER: S-EPMC9270623 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:ObjectiveKawasaki disease (KD) is a vasculitis of unknown aetiology with a high risk of coronary aneurysms if untreated. Timely treatment with intravenous immunoglobulin decreases the risk for coronary artery aneurysms (CAA). In this study, we set out to elucidate the factors associated with the risk of developing CAA.MethodsRecords of all KD-diagnosed children in Skåne between 2004 and 2014 were collected and clinical and demographic data were compiled. KD is defined according to the revised American Heart Association diagnostic criteria and classified as either complete KD (cKD) or incomplete KD (iKD).ResultsKD was diagnosed in 77 children and CAA was found in 31% (n = 24). Children with CAA were younger compared with children without (median; 20 vs 34 months) and intravenous immunoglobulin treatment within 10 days was less likely to be received (75% vs 91%). In children presenting with iKD, 47% developed CAA compared with 21% in cKD patients. Using multivariate analysis, an association between the risk of CAA with low age in children with iKD was observed.ConclusionThe risk of CAA development is disturbingly high in young children with iKD. This highlights the importance of rapid intense treatment and vigilance in infants, who are the most difficult to diagnose, in order to reduce the frequency of CAA.
| S-EPMC8023999 | biostudies-literature
Project description: Not available
| S-EPMC10899712 | biostudies-literature
Project description:Aneurysms of the vascular wall represent a final common pathway for a number of inflammatory processes, including atherosclerosis and idiopathic vasculitis syndromes. Kawasaki disease (KD) is an acute, self-limited vasculitis in children and the leading cause of acquired coronary artery aneurysms. We sought to identify shared molecular mechanisms of aneurysm formation by genotyping eight polymorphisms in matrix metalloproteinase (MMP)-1, 3, 7, 12 and 13 in the gene cluster on Chr.11q22, whose gene products have been implicated in aneurysm formation or are known to have elastase activity. We genotyped 482 US-UK KD patients (aneurysm+: n=111, aneurysm-: n=371) and tested our findings in an independent cohort of 200 Japanese KD patients (aneurysm+: n=58, aneurysm-: n=142). Analysis of the five MMP genes identified modest trends in allele and genotype frequencies for MMP-3 rs3025058 (-/T) and haplotypes containing MMP-3 rs3025058 (-/T) and MMP-12 rs2276109 (A/G) (nominal P=2 to 4 × 10(-5)) that conferred increased risk of aneurysm formation in US-UK subjects. This finding was validated in Japanese subjects and suggests the importance of this locus in aneurysm formation in children with KD. The region encompassing these risk haplotypes is a prime candidate for resequencing to look for rare genetic variation that may influence aneurysm formation.
| S-EPMC3005773 | biostudies-literature
Project description:BACKGROUND:The sorting nexin (SNX) family is involved in endocytosis and protein trafficking and plays multiple roles in various diseases. The role of SNX proteins in Kawasaki disease (KD) is not known. We attempted to test whether genetic SNX variation associates with the risk of coronary artery aneurysm (CAA) formation in KD. METHODS AND RESULTS:Chi-square tests were used to identify SNX24 genetic variants associated with KD susceptibility and CAA formation in KD; models were adjusted for fever duration and time of first administration of intravenous immunoglobulin. We obtained clinical characteristics and genotypes from KD patients (76 with CAA and 186 without CAA) in a population-based retrospective KD cohort study (n?=?262). Clinical and genetic factors were associated with CAA formation in KD. In addition, endothelial cell inflammation was evaluated. Significant correlation was observed between KD with CAA complications and the rs28891 single-nucleotide polymorphism in SNX24. Patients with CC?+?CT genotypes had lesser CAA complications. In lipopolysaccharide-treated human umbilical vein endothelial cells, siRNA knockdown of SNX24 significantly decreased gene expression of the proinflammatory cytokines IL-1 beta, IL-6, and IL-8. CONCLUSIONS:Polymorphisms in SNX24 may be used as genetic markers for the diagnosis and prognosis of CAA formation in KD.
| S-EPMC4176999 | biostudies-literature
Project description:A form of systemic vasculitis that affects mostly small and medium-sized vessels, Kawasaki disease (KD) is most commonly found in children under the age of 5 years old. Though its etiology is unknown, KD has been the most frequent acquired heart disease in developing countries. Its incidence has increased over recent decades in many centuries, including Japan, Korea, and China. The most severe complications of KD are coronary artery lesions (CAL), including dilation, fistula, aneurysm, arterial remodeling, stenosis, and occlusion. Aneurysm formation has been observed in 20-25% of KD patients that do not receive intravenous immunoglobulin (IVIG) treatment, and in 3-5% that do receive it. Coronary artery dilation has been found in about 30% of KD patients in the acute stage, although mostly in the transient form. Diminishing the occurrence and regression of CAL is a vital part of treating KD. In this review article, I demonstrate the clinical method to prevent CAL formation used at the Kawasaki Disease Center in Taiwan.
| S-EPMC6136281 | biostudies-other
Project description:ObjectivesTo determine the safety, tolerability, pharmacokinetics, and immunomodulatory effects of a 6-week course of atorvastatin in patients with acute Kawasaki disease with coronary artery (CA) aneurysm (CAA).Study designThis was a Phase I/IIa 2-center dose-escalation study of atorvastatin (0.125-0.75 mg/kg/day) in 34 patients with Kawasaki disease (aged 2-17 years) with echocardiographic evidence of CAA. We measured levels of the brain metabolite 24(S)-hydroxycholesterol (24-OHC), serum lipids, acute-phase reactants, liver enzymes, and creatine phosphokinase; peripheral blood mononuclear cell populations; and CA internal diameter normalized for body surface area before atorvastatin treatment and at 2 and 6 weeks after initiation of atorvastatin treatment.ResultsA 6-week course of up to 0.75 mg/kg/day of atorvastatin was well tolerated by the 34 subjects (median age, 5.3 years; IQR, 2.6-6.4 years), with no serious adverse events attributable to the study drug. The areas under the curve for atorvastatin and its metabolite were larger in the study subjects compared with those reported in adults, suggesting a slower rate of metabolism in children. The 24-OHC levels were similar between the atorvastatin-treated subjects and matched controls.ConclusionsAtorvastatin was safe and well tolerated in our cohort of children with acute Kawasaki disease and CAA. A Phase III efficacy trial is warranted in this patient population, which may benefit from the known anti-inflammatory and immunomodulatory effects of this drug.
| S-EPMC6878161 | biostudies-literature
Project description:Background:Coronary artery fistulas are rare and most commonly asymptomatic; however, they can become enlarged and rupture in some cases. Case summary:We report a case of a 51-year-old woman who was brought to our hospital unconscious in an ambulance. Cardiac tamponade caused by the rupture of an aneurismal coronary-pulmonary artery fistula (CPAF) was detected by contrast-enhanced computed tomography and confirmed by invasive coronary angiography. Due to prompt diagnosis and subsequent surgical intervention, the patient's condition was rapidly improved, and she was discharged from the hospital. Discussion:Coronary-pulmonary artery fistula aneurysm rupture requires rapid diagnosis and treatment, and thus, in cases with cardiac tamponade and coronary aneurysm, CPAF aneurysm rupture should be considered.
| S-EPMC7319849 | biostudies-literature
Project description:Giant coronary artery aneurysm (CAA) is a relatively uncommon disease that is defined by a focal dilation of at least 20 mm and characterized by various clinical symptoms. However, cases presenting primarily with hemoptysis have not been reported. A man in his late 20 s suffering from persistent chest pain for over 2 months was transferred to our emergency department for intermittent hemoptysis lasting for 12 h. Bronchoscopy detected fresh blood in the left upper lobe bronchus without a definite bleeding source. Magnetic resonance imaging (MRI) demonstrated a heterogeneous mass and the high-intensity signals suggested active bleeding. coronary computed tomography (CT) angiography demonstrated a giant ruptured CAA wrapped in a large mediastinal mass Coronary angiography confirmed the CAA originating from the left anterior descending artery. The patient underwent an emergency sternotomy and an enormous hematoma arising from a ruptured CAA densely adhering to the left lung was identified. The patient recovered uneventfully and was discharged on the 7th day later. The ruptured CAA masquerading as hemoptysis highlights the indispensability of multimodality imaging for accurate diagnosis. Urgent surgical intervention is desirable in such life-threatening conditions.
| S-EPMC10244565 | biostudies-literature
Project description:We present a case of a ruptured mycotic coronary aneurysm effectively treated with covered stents and phased surgery. The covered stent, however, became occluded two years later. Because of the low invasiveness, a covered stent treatment may be advantageous over conventional surgery but trade off long-term vascular patency.Learning objectiveTo recognize the presence of a ruptured infectious coronary aneurysm after a primary coronary stenting for ST-elevation myocardial infarction.To discuss the treatment strategies for a ruptured infectious coronary aneurysm with a covered stent.
| S-EPMC10562108 | biostudies-literature
Project description:We report a 7-year-old boy with Menkes disease complicated by rupture of a large splenic artery aneurysm. The aneurysm was successfully embolized with microcoils and n-butyl cyanoacrylate. Further angiographic evaluation revealed marked tortuosity of mesenteric and lower extremity vasculature, including the femoral arteries bilaterally, without aneurysm formation. The patient has since been evaluated annually with computed tomography angiography and there have been no additional vascular complications of his disease during 3-year follow up.
| S-EPMC6158999 | biostudies-literature