Therefore, a screening strategy with family history, interferon-gamma release assays, and also chest X-ray might be useful to rule out tuberculosis. IFX should also be avoided in carriers of hepatitis B or C virus patients because of a risk of activation and acute exacerbation of chronic hepatitis. There are no data about the development of malignant tumors.
Non-invasive imaging of the coronary arteries | European Heart Journal | Oxford Academic
Anakinra is a recombinant IL-1 receptor antagonist blocking the natural biological activity of IL-1, by competitively inhibiting binding of IL-1 and its receptor, and down-regulating many ILmediated inflammatory reactions [ 53 ]. Based on a hypothetical model of KD as an autoinflammatory disease, it was assumed that anakinra might exert an antinflammatory effect on systemic and coronary artery inflammation in these patients [ 54 ]. There are only anecdotal reports about using anakinra in children with KD.
They might involve the gastrointestinal tract nausea, diarrhea, abdominal pain, hypertransaminasemia , respiratory system upper respiratory infections, sinusitis, influenza-like illness, rarely pneumonia , or skin bruising, urticarial-like lesions, local infections. Rare are allergic reactions, including anaphylaxis. Frequent is the report of neutropenia, moderate eosinophilia, or moderate thrombocytopenia. Clinical trials are currently in phase I to test canakinumab as a potential treatment for different disorders, such as chronic obstructive pulmonary disease, gout, and coronary heart disease.
They can be of minor relevance: rhinitis, nasopharyngitis, nausea, vomiting, diarrhea, dizziness, headache, or a mild redness with swelling, warmth, or itching at the injection site. Sometimes they can be severe: allergic reactions rash, hives, difficulty in breathing or swallowing, asthma, oral allergy syndrome , hemoptysis, and infections.
Ulinastatin [ 93 , 94 ]. Cyclosporine A [ 95 — 97 ]. Methotrexate [ 98 ]. Optimal dosing in not yet determined in children, though in many studies. Anaphylactic shock, liver dysfunction, leukopenia, rash, itching, diarrhea, pain at the injection site. Gastrointestinal signs, alopecia, risk of myelosuppression, anaphylaxis, infections, liver dysfunction, acute kidney failure. In Onouchi et al. Plasmapheresis removes cytokines and chemokines activated in KD directly from the bloodstream. There are no prospective randomized studies to evaluate the efficacy of plasmapheresis in KD, but few retrospective ones comparing plasmapheresis to IVIG [ 58 ].
Hypotension, hypovolemic shock, urticaria, anaphylactic reactions, hypocalcemia, nausea, vomiting, or bleeding disorders.
Regression of CAA depends on the initial size of the vascular dilation: about half of CAA resolves within a few years. Rarely giant aneurysms show a tendency to regress.
Because of the repair process, however, a stenosis can occur in the incoming or outgoing aneurysmal area, secondary to intimal hyperplasia or thrombotic occlusion, with risk of myocardial ischemia, heart attack, and unexpected death [ 59 ]. Such therapy depends on the severity and extension of coronary artery involvement. Mechanism of action of clopidogrel is based on the inhibition of platelet aggregation induced by ADP, and antiplatelet action is usually achieved with low doses of clopidogrel 0.
In case of ASA allergy or varicella either wild disease or vaccination against varicella clopidogrel might be an alternative to ASA [ 63 ]. Side effects of clopidogrel include malaise, myalgia, headache, dizziness, gastrointestinal symptoms, rash, itching, thrombotic thrombocytopenic purpura, and bleeding tendency.
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Clopidogrel is off-label in KD. Medium to giant aneurysms, history of heart attack, history of intra-aneurysm thrombosis. Aneurysms with high thrombotic risk, before starting therapy with warfarin. Initial dose of 0. Bleeding epistaxis, gum bleeding, intracranial and intra-abdominal hemorrhage , embryopathies dysostosis, dyschondroplasia, microcephaly. Hemorrhage, thrombocytopenia, hepatic dysfunction, rash, diarrhea, hair loss, osteoporosis. Reduced efficacy with chlorophyll contained in green and yellow vegetables with high contents of vitamin K , vitamin K-enriched milk, phenobarbital, carbamazepine, rifampicin; increased efficacy if breastfeeding, use of erythromycin, fluconazole, corticosteroids, amiodarone.
The new oral anticoagulants e. Recommendation 4. Recommendation 5. Recommendation 6. Recommendation 7. Long-term therapy for patients with coronary artery aneurysms related to Kawasaki disease and anginal symptoms. Hydroxymethylglutaryl coenzyme-A reductase inhibitors statins are a cornerstone of therapy for the primary and secondary prevention of atherosclerotic cardiovascular events in adults.
They reduce low-density lipoprotein cholesterol and have potentially beneficial pleiotropic effects on inflammation, endothelial function, oxidative stress, platelet aggregation, coagulation, and fibrinolysis [ 1 ]. Although controversy continues concerning whether the vascular pathology of KD may lead to early atherosclerosis, statins could have a role in the long-term management of KD and an empirical low-dose treatment may be considered for patients with past or current aneurysms, regardless of age or sex [ 1 ].
Patients with medium and giant aneurysms of coronary arteries and those in whom the aneurysm sizes rapidly increase over time are at greater risk of developing thrombosis and acute coronary syndrome. It is therefore recommended to check frequently echocardiography during this period. A heart attack in childhood and in young children may be clinically silent or it may be associated with sneaky and nonspecific symptoms, such as unusual restlessness, vomiting, or shock.
A sudden deterioration of cardiac function or a change in ECG findings should prompt the suspicion of coronary thrombosis. Actually, more and more patients with KD-related coronary aneurysms reach adulthood, and this increases the risk of heart attacks caused by thrombosis of the aneurysm or progressive arterial stenosis caused by vascular remodeling. In the absence of randomized controlled trials recommendations about therapy of coronary thrombosis are derived from evidences in adult population-related studies, despite the etiology of stenosis is different, and from small series of pediatric cases.
Recommendation 8. Coronary artery thrombosis with actual or imminent occlusion of the lumen should be treated with thrombolytic therapy or with interventional cardiac catheterization. The most commonly administered thrombolytic therapy is intravenous tissue plasminogen activator tPA, alteplase [ 66 — 68 ]. Plasminogen activators are enzymes activating the conversion of plasminogen into plasmin, which can promote clot lysis.
KD patients with acute heart attack or intra-aneurysmal thrombi, and KD patients with sudden extension of a thrombus in the coronary artery. Their use in KD patients is off-label. For alteplase: Thrombolytic drugs have to be associated with low-dose ASA and low-dose heparin with careful monitoring for the bleeding risk.
Less frequently, in the case of limited venous access, subcutaneously injected LMWH might be used. Considering the high risk of bleeding, unfractionated heparin should be a second-choice drug. In the case of significant thrombosis with high risk of occlusion, a combination of low-dose thrombolytic therapy may be also associated with a platelet aggregation inhibitor, i. Recommendation Interventional catheterization procedures are limited by the large delivery systems for small patients, high risk of complications, and low efficacy, followed by high risk of reintervention.
Coronary artery reperfusion both by invasive cardiologic interventional procedures and cardiac surgery can be considered after an initial unsuccessful pharmacological thrombolysis. Cardiologic procedures include percutaneous thrombolysis, percutaneous angioplasty, and rotational ablation [ 72 — 74 ]. A rotablator may be required to modify the shape of the lesion. First-choice cardiologic interventional treatment in patients with KD should be chosen on the experience of the center and its technical feasibility in the shortest possible time.
Percutaneous coronary angioplasty is associated with the risk of restenosis or occlusion in patients with KD, requiring often the use of coronary stents or alternative procedures, such as coronary artery bypass grafting CABG or rotational ablation. For children with persistent or progressive CAA who develop ischemic heart disease in early childhood a reliable option is represented by CABG using grafts from the internal mammary artery [ 76 , 77 ].
A dozen of KD cases has been reported worldwide for heart transplantation, with an average age of 8. Patients with KD must undergo a careful clinical monitoring of blood and instrumental exams in the long-term, but cardiological evaluations with ECG and echocardiography are crucial also in the short-term follow-up. Cardiological evaluation performed on the second month since disease onset allows to subdivide KD patients according to the cardiovascular impairment in different risk classes to establish a personalized follow-up.
It is important an accurate measurement of coronary artery diameters by the evaluation of the relative z-scores. Follow-up of KD patients must continue over time, especially for those who have presented CAA, considering that it is not possible to exclude remote complications even in the non-complicated cases.
Such regression may be due to myointimal proliferation or thrombus organization and recanalization. Therefore, it is extremely important a careful long-term follow-up in KD patients with both persistent and regressed aneurysms with different timing and mode. The class stratification risk, related to the risk of myocardial ischemia, established by the American Heart Association AHA , is a useful tool for a standardized management of KD patients with regard to timing of controls, diagnostic tests, and therapeutic indications [ 6 , 8 ].
No abnormality of coronary arteries in the various phases of the disease. Cardiovascular sequelae caused by KD differ substantially from the classic atherosclerosis from a pathological point of view. The endothelial dysfunction and chronic inflammatory reactions may occur even late in KD patients with giant or medium aneurysms, including those which have regressed, causing intimal hyperplasia and calcification if there is a localized stenosis. Although endothelial dysfunction is a precursor of atherosclerosis and autopsy studies seem to suggest that KD patients may have more severe atherosclerotic lesions, it is still unclear whether all KD patients have a higher risk for a clear progression to atherosclerosis [ 83 — 85 ].
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It is reasonable to consider KD patients worthy of close monitoring for cardiovascular risks, evaluating blood pressure, body mass index, cholesterol, LDL, HDL, triglycerides, and promoting correct lifestyles for instance, avoid smoking and a regular physical activity with healthy eating [ 86 , 87 ]. The use of statins is still debated and their role in KD is yet to clarify [ 88 ]. Monitoring cardiovascular risk factors in KD patient requires body mass index evaluation, blood pressure control, and evaluation of the lipid profile total cholesterol, LDL, HDL, triglycerides.
For the reasons above, specialized centres must certify physical fitness through multi-disciplinary evaluations. Activity restrictions depend on ongoing therapy antiplatelet or anticoagulant drugs.
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Pediatricians can assist families in choosing which is the most appropriate sport. Sport classification according to the American Academy of Pediatrics. The following steps should be performed to certificate fitness: family and personal history, clinical examination and blood pressure control, resting lead ECG, mono- and 2D-colour Doppler echocardiogram, tapis roulant stress test to determine stress tolerance, rhythm and heart frequency, and possible myocardial ischemia.
If all these examinations are normal, annual fitness certification can be produced for non-agonistic activities [ 90 — 92 ]. There are different indications for physical activity in children who have presented KD, which can be stratified according to various risk levels. In selected cases the evaluation of myocardial perfusion might be indicated. A coronarography must be performed if there is evidence of myocardial ischemia.
Related Complete Medical Guide and Prevention for Heart Disease Volume XVII; Kawasaki Disease
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