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Calcium channel blockers are the first-choice drugs for angina, but their intravenous administration is contraindicated in newborns and infants [ 17 ]. The use of all the above-mentioned drugs is off-label in KD. Table 6 Long-term therapy for patients with coronary artery aneurysms related to Kawasaki disease and anginal symptoms. I - C Thrombolytic drugs The most commonly administered thrombolytic therapy is intravenous tissue plasminogen activator tPA, alteplase [ 66 — 68 ]. Tissue plasminogen activator Mechanism of action Plasminogen activators are enzymes activating the conversion of plasminogen into plasmin, which can promote clot lysis.

Therapeutic indications KD patients with acute heart attack or intra-aneurysmal thrombi, and KD patients with sudden extension of a thrombus in the coronary artery. Dosage For alteplase: Recommendation 9. I - C Recommendation Invasive cardiologic procedures 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. Short-term follow-up 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.

VI - B 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. Long-term follow-up 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. The occurrence of thrombosis or stenosis associated with CAA, in fact, increases the risk of myocardial ischemia. Furthermore, the optimal follow-up of patients with regressed aneurysms remains still controversial since, even with the normalization of vessel diameter, morphological and functional alterations might persist.

Indeed, the normal ultrasound coronary artery framework does not necessarily coincide with the normal endothelial function. This justifies continuing seriated controls, although less frequent, to trace the natural history of the disease, also regarding the possible risk of atherosclerosis.

It is reasonable to use echocardiographic coronary artery luminal dimensions converted to body surface area-adjusted z scores to determine risk stratification of patients [ 81 , 82 ]. Different therapies and follow-up modalities are recommended for each cardiovascular risk class. Table 7 Cardiovascular risk classes in patients with Kawasaki disease.

Reference personnel for patients with a previous KD who want to perform agonistic sports competitive sports or high-intensity activities includes doctors specialized in sports medicine who work in the health systems or in authorized private structures; certification for non-agonistic sports can be provided by the family pediatrician and practitioner In KD patients the evaluation for fitness certification is complicated by several reasons: - there are non-subjective functional limitations patients do not feel sick ; - cardiovascular risks depending on the coronary artery damage is difficult to quantify, and patients affected by giant aneurysms may show normal stress test when myocardial perfusion is evaluated; - few data are available to define exactly risks caused by sport activity; - it is difficult to correlate size of aneurysms to any certification for fitness, though giant or multiple aneurysms should be very carefully evaluated.

Each patient on antiplatelet treatment must avoid contact and collision sports, while patients on anticoagulant therapy must also avoid limited contact sports [ 89 ]. Table 8 Sport classification according to the American Academy of Pediatrics.

Acknowledgements We would like to thank prof. Funding No person who participated in the drafting of these guidelines has been sponsored. Availability of data and materials No datasets were generated or analyzed during the current study. Ethics approval and consent to participate Not applicable.

Consent for publication Not applicable. Competing interests The authors declare that they have no competing interests. Onofrio n. Eugenio, Rome, Italy.

Postsplenectomy Kawasaki disease in a 17-year‑old male

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    The decrease in body temperature was only relative and short as a result of antibiotic treatments. Within 36 hours of his discharge from the hospital, he once again presented with fever, generalized maculopapular erythrodermic rash, oral lesions, sore throat, swollen neck, malaise, and myalgia. He had severe conjunctivitis and edema of the periorbital areas and the eyelids. Cracking and edema of the lips was also evident Figure 1B. There was multiple, painful lymphadenopathy in the anterior and posterior cervical and axillary regions and maximum diameter of 17 mm was reported to be present among the cervical lymph nodes.

    With the exception of tachycardia, the physical examination of the heart, lungs, and abdomen were all unremarkable. No clinical signs of infection in the surgical site were present. There were no clinical signs and symptoms of meningeal irritation.

    [Full text] Postsplenectomy Kawasaki disease in a year‑old male | IMCRJ

    Neurological examination only revealed the peripheral paralysis of the seventh right cranial nerve. Figure 1 The physical examination of patient showed diffused maculopapular erythrodermic rash, and scaling in the distal extremities and around the nails A and severe conjunctivitis of the periorbital and the eyelids and also cracking and edema of the lips B.

    Table 1 Obtained tests during the first 48 hours of hospitalization. Ultrasonography of the abdomen and neck 2 days after hospitalization showed bilateral neck and axillary lymphadenopathy in addition to mild ascites. The size of the liver and pancreas were normal. No pathology in the para-aortic lymph nodes was detected. Echocardiography that was carried out during his second day of hospital stay demonstrated only traces of pericardial fluid, and did not exhibit any signs of cardiac contusion or valvular abnormalities.

    The patient received supportive care, which included IV normal saline, IV antibiotics vancomycin and imipenem , antihistamine loratadine , and acetaminophen. In addition, his erythroderma and scaling were further intensified Figure 1A. Prior to IVIG administration and despite the treatment with acetaminophen and antibiotics, the patient remained pyretic. After 2 days of IVIG treatment, the fever as well as the pain in the lymph nodes subsided. After 4 days and a total received dosage of g, IVIG administration was discontinued.

    Given his remarkable clinical improvement, after 4 days, his IVIG treatment was discontinued. His echocardiogram and coronary angiography that were carried out on the 14th and 20th day of his admission, respectively, reported normal and showed no signs of coronary artery aneurysm Figure 3. Figure 2 The physical examination of patient was normal 10 days after admission and his lymphadenopathies resolved. Figure 3 Echocardiogram and coronary angiography of patient reported normal and showed no signs of coronary artery aneurysm.

    Written informed consent of the patient was obtained to publish this case report. Simultaneous occurrence of fever and skin rash is a common and challenging clinical presentation in both pediatric and adult patients. Although the differential diagnosis is very broad, adequate history and physical examination may help the clinician to narrow down a potential list of probable etiologies. Since the spectrum of diagnosis can include mild self-limiting condition to life-threatening disease, clinical discretion remains imperative.

    In our patient, the presentation of fever and mucocutaneous rash, postsplenectomy, led to clinical suspicion for the diagnosis of sepsis and toxic shock syndrome. Yet, the negative result of blood cultures and lack of response to antibiotic treatments as well as clinical features not consistent with infectious etiologies led to the consideration of other alternative diagnoses. Moreover, the use of phenytoin in the patient, led to the consideration of drug reaction as an alternative diagnosis, which could have presented in a clinical spectrum from mild-to-severe reaction, such as that seen in Steven—Johnson syndrome SJS.

    Case Report ARTICLE

    However, the presence of thrombocytosis and diffuse desquamation was against this diagnosis. The diagnosis of SJS or toxic epidermal necrolysis is not plausible. Fever is not a primary feature of these diseases and systemic reactions, such as increased white blood cell count, elevated ESR, positive CRP levels, and thrombocytosis are not expected to occur with this degree and severity; edema of the extremities, scaling, and the absence of target lesions are supportive of the diagnoses other than SJS.

    Thus, by considering all of the signs and symptoms in the patient and fulfillment of the diagnostic criteria for the Kawasaki syndrome, clinical diagnosis was made, and patient was started on appropriate therapy, which led to an uneventful course of recovery. Given that prevalence of Kawasaki syndrome remains low, high index of clinical suspicion and application of the diagnostic criteria for this potentially treatable disease remain invaluable. Early diagnosis and effective therapy with IVIG have been shown to significantly reduce the morbidity and mortality from this disease.

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