Faculty Opinions recommendation of ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms.

2008 ◽  
Author(s):  
Dan Lipsker
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Disease Susceptibility ◽  
Functional Polymorphism ◽  
Coronary Artery Aneurysms

scholarly journals ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms

2007 ◽  
Vol 40 (1) ◽  
pp. 35-42 ◽  
Author(s):  
Yoshihiro Onouchi ◽  
Tomohiko Gunji ◽  
Jane C Burns ◽  
Chisato Shimizu ◽  
Jane W Newburger ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Disease Susceptibility ◽  
Functional Polymorphism ◽  
Coronary Artery Aneurysms

Abstract 221: Use of Statins in Patients with Coronary Artery Aneurysms: A Pilot Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Michael Khoury ◽  
Michael A Portman ◽  
Cedric Manlhiot ◽  
Anne Fournier ◽  
Rejane F Dillenburg ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Pilot Study ◽  
Kawasaki Disease ◽  
Linear Regression ◽  
North American ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
The North ◽  
Significant Difference

Background: Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation. Methods: The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions. Results: Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41). Conclusions: This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.

scholarly journals Genetic variants of glutamate receptor gene family in Taiwanese Kawasaki disease children with coronary artery aneurysms

2014 ◽  
Vol 4 (1) ◽  
pp. 67 ◽  
Author(s):  
Ying-Ju Lin ◽  
Jeng-Sheng Chang ◽  
Xiang Liu ◽  
Hsinyi Tsang ◽  
Ting-Hsu Lin ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Gene Family ◽  
Glutamate Receptor ◽  
Genetic Variants ◽  
Receptor Gene ◽  
Coronary Artery Aneurysms ◽  
Receptor Gene Family

Aerobic exercise function of patients with persistent coronary artery aneurysms secondary to Kawasaki disease

1996 ◽  
Vol 17 (4) ◽  
pp. 226-230 ◽  
Author(s):  
J. Rhodes ◽  
Z. M. Hijazi ◽  
G. R. Marx ◽  
D. R. Fulton
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Aerobic Exercise ◽  
Coronary Artery Aneurysms

Atypical coronary artery aneurysms due to Kawasaki disease in Noonan syndrome with a novel PTPN11 mutation

2018 ◽  
Vol 29 (2) ◽  
pp. 228-230
Author(s):  
Shiori Takai ◽  
Kei Takasawa ◽  
Shozaburo Doi
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Therapeutic Intervention ◽  
Noonan Syndrome ◽  
Coronary Artery Aneurysms ◽  
Ptpn11 Mutation

AbstractWe report a 3-year-old boy with giant and atypical coronary artery aneurysms in the acute phase of Kawasaki disease, despite appropriate therapeutic intervention, in Noonan syndrome with a novel heterozygous PTPN11 mutation, c. 907 G>A (p.Asp303Asn). We hypothesised that this PTPN11 mutation might affect both hyperinflammation caused by Kawasaki disease and vascular fragility in the coronary artery, resulting in coronary artery aneurysms.

scholarly journals Management of sequelae of Kawasaki disease in adults

2018 ◽  
Vol 2017 (3) ◽  
Author(s):  
John B Gordon ◽  
Jane C Burns
Keyword(s):  
Young Adults ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Management Strategies ◽  
Collaborative Study ◽  
Calcium Score ◽  
Calcium Deposition ◽  
Coronary Artery Aneurysms ◽  
Study Results ◽  
Growing Population

Background: A growing population of young adults is presenting to cardiologists with late manifestations of Kawasaki disease (KD) that include cardiomyopathy, ischemia, and infarction. The management of these conditions differs in important ways from atherosclerotic heart disease, and yet there is little awareness in the adult cardiology community regarding the special challenges posed by the cardiovascular sequelae of KD. Methods: Observations were made on a population of 140 adult KD patients enrolled in the San Diego Adult KD Collaborative Study. Results: Coronary artery aneurysms resulting from KD in childhood are associated with a high risk of thrombosis and stenosis at the inlet or outlet of the aneurysm. These aneurysms are often highly calcified and may contain a large thrombus burden that may obscure the true size of the aneurysm. Pitfalls in the management of these patients stem largely from failure to recognize the nature of the lesions, which leads to attempts to dilate highly calcified stenotic segments and undersizing of stents. Intravascular ultrasound is helpful in appreciating the true dimensions of the aneurysm, which may be filled with thrombus. Thrombolysis and use of anti-platelet agents followed by systemic anti-coagulation are appropriate management strategies for patients presenting with acute infarction. Bypass grafting with the internal thoracic arteries can be a successful strategy, but care must be taken to avoid competitive flow through the native vessel leading to graft failure. In contrast to the individuals who developed coronary artery aneurysms, young adults who had documented normal echocardiograms associated with their acute KD in childhood and who have no evidence of calcium deposition in the arterial wall as assessed by computed tomography (CT) calcium score appear to have no increased cardiovascular risk in the medium term. Long-term outcomes for adults post-KD in childhood are still being defined. Conclusions: KD poses special management challenges for the adult cardiologist who must recognize the unique features of the cardiovascular lesions in this growing population of patients. 

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