A COVID-19-Related Kawasaki Disease: Our First Multi-system Inflammatory Syndrome in Children Case

A new type of coronavirus disease-2019 (COVID-19) epidemic, which started in the last months of 2019, has spread rapidly all over the world and caused many deaths, especially in adults. Recently, a syndromic condition caused by the hyperinflammatory response that is thought to be due to this new pandemic coronavirus factor has been identified, especially in the childhood age group. It has been stated that this response is particularly similar to Kawasaki disease and can manifest itself with multiorgan involvement. This new clinical condition was defined as pediatric inflammatory multi-system syndrome temporally associated with severe acute respiratory syndrome-coronavirus 2 or multi-system inflammatory syndrome in children associated with COVID-19. In this case presentation, a nine-year-old male patient was admitted to our emergency department with complaints similar to ones in Kawasaki disease. The patient was followed up in our pediatric intensive care unit due to hypotension and myocarditis, as well as likely macrophage activation syndrome and simultaneous COVID-19 positivity. For Kawasaki disease, intravenous immunoglobulin, acetylsalicylic acid, low molecular weight heparin and methylprednisolone; for myocarditis, enalapril and furosemide; and for COVID-19, favipiravir was administered to the patient. We would like to present our case to exemplify clinicians dealing with pediatric patients and to assist in easier recognition, prevention, diagnosis and treatment of COVID-19 in all children worldwide

Evaluation of Clinical Features of Patients Diagnosed with MIS-C

Objective:Evaluation of clinical features and results in multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease-2019.Method:Patients diagnosed with MIS-C between September 1, 2020 and February 25, 2021, followed at University of Health Sciences Turkey, İstanbul Bağcılar Training and Research Hospital, Clinic of Pediatrics were included. Their clinical findings and laboratory results were evaluated retrospectively.Results:The average age of 16 patients diagnosed with MIS-C was found to be 6.5 years; of them, 62.5% were male and 37.5% were female. Our patients had no chronic disease. Fever (100%) and stomachache (81.25%) were the most common symptoms. All patients had high levels of C-reactive protein, procalcitonin, D-dimer and pro-brain natriuretic peptide at the time of diagnosis. The average ejection fraction was found as 63.1% in echocardiography. Intravenous immunoglobulin, corticosteroids and acetylsalicylic acid were administered to all patients whereas enoxaparin and vasopressors were administered to 11 (68.75%) and 3 (18.5%) patients, respectively. Left ventricular ejection fraction was found to be within the normal range at all patients at the time of discharge. The average in patient follow-up was found to be 10 days.Conclusion:It is important to have a long-term follow-up of MIS-C patients, who show similar symptoms to Kawasaki disease and yet have their own particular symptoms and cardiac involvement, in order not to miss the opportunity of early diagnosis as well as cardiac complications

Flekainid Andersen-Tawil sendromlu pediyatrik hastalarda bidireksiyonel ventriküler taşikardiyi tamamen ortadan kaldırabilir mi?

WOS: 000453086300012PubMed ID: 30516532Andersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 60% of all cases of the disorder are caused by mutations in the KCNJ2 gene. A 13-year-old female patient was referred for frequent premature ventricular contractions. Suspicion of ATS due to dysmorphic findings, electrocardiogram changes, and periodic muscle weakness was genetically confirmed. Beta-blocker therapy was initiated as a first-line treatment for bidirectional VT and frequent polymorphic premature ventricular contractions. Despite proper treatment, the VT attacks were not brought under control. Flecainide was added to the treatment regime. The number of premature ventricular contractions was dramatically reduced with flecainide and the VT attacks completely disappeared. This patient is a rare example of ATS in our country. This article provides a description of successful management of rhythm disturbance in a patient with ATS.Andersen-Tawil sendromu (ATS), kas güçsüzlüğü(periyodik paralizi), ritm bozuklukları ve gelişim bozukluklarına neden olan bir hastalıktır. QT uzaması ve bidireksiyonel ventriküler taşikardi (VT) ve polimorfik VT’yide içeren ventriküler aritmiler ortaya çıkabilir. Tüm olguların yaklaşık %60’ı, KCNJ2 genindeki mutasyonlardankaynaklanmaktadır. On üç yaşında kız hasta, sık ventriküler erken atımlar nedeniyle hastanemize sevk edildi.Hasta, morfolojik bozuklukları, EKG değişiklikleri ve periyodik kas güçsüzlüğü nedeniyle ATS olarak düşünüldüve tanı genetik olarak doğrulandı. Bidireksiyonel VT vesık polimorfik erken ventriküler atımları için ilk basamak tedavi olarak beta bloker başlandı. Ancak tedaviyerağmen, hastanın VT atakları kontrol altına alınamadı.Bunun üzerine Flekainid tedaviye eklendi. Flekainid ileprematüre ventriküler atımların sayısı çarpıcı bir şekildeazaldı. Ayrıca VT atakları tamamen kayboldu. Bu hasta, ülkemizde nadir görülen ATS’li hastalardan biridir. Bumakalede ATS’li bir hastada ritm bozukluğunun başarılıyönetimi anlatılmıştır

An Adolescent with Pulmonary Hypertension Secondary to Idiopathic Fibrosing Mediastinitis: Case Report

Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Fibrosing mediastinitis is a rare disorder caused by proliferation of acellular collagen. In fibrosing mediastinitis, clinical presentation is determined by compression to the vital mediastinal organs. Fibrosing mediastinitis may rarely lead to pulmonary veno-occlusive disease. In this case report, a 16 year old boy with pulmonary arterial hypertension was presented. His right pulmonary artery was completely occluded secondary to idiopathic fibrosing mediastinitis. The patient died after mediastinal biopsy. Herein, we discussed pulmonary arterial hypertension secondary to idiopathic fibrosing mediastinitis in children. © 2012 by Erciyes University School of Medicine

An adolescent with pulmonary hypertension secondary to idiopathic fibrosing mediastinitis: Case report İdiyopatik fibröz mediastinite sekonder pulmoner hipertansiyonlu bir adolesan: Olgu sunumu

Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Fibrosing mediastinitis is a rare disorder caused by proliferation of acellular collagen. In fibrosing mediastinitis, clinical presentation is determined by compression to the vital mediastinal organs. Fibrosing mediastinitis may rarely lead to pulmonary veno-occlusive disease. In this case report, a 16 year old boy with pulmonary arterial hypertension was presented. His right pulmonary artery was completely occluded secondary to idiopathic fibrosing mediastinitis. The patient died after mediastinal biopsy. Herein, we discussed pulmonary arterial hypertension secondary to idiopathic fibrosing mediastinitis in children. © 2012 by Erciyes University School of Medicine

Retrospective evaluation of 31 cases with Williams-Beuren syndrome Williams-Beuren sendromlu 31 olgunun retrospektif deǧerlendirilmesi

Purpose: Williams Beuren syndrome, is a genetic disorder which gets its characteristic features by aging. The aim of this study is to evaluate patients diagnosed WBS, and to share our experience. Material and Methods: Patients who diagnosed WBS were evaluated retrospectively. Systemic examination, echocardiographic and renal doppler findings, thyroid functions and genetic analysis results were collected from patient file, and statistical analyses were done. Results: 1996 to 2009, 31 patients (20 male), who were 1 month to 13 years old diagnosed WBS. Main reason for admission was murmur evaluation in 71%, and growth retardation in 16.1%. Genetic analyses were done on 23 patients and 7q11.23LSI.ELN deletion was positive in 73.9% patients. Analyses of cardiovascular examinations revealed; isolated peripheral pulmonary stenosis in 38.7%, isolated supravalvular aortic stenosis in 35.5% and both in 25.8%. Symptomatic seven patients with pulmonary stenosis and three patients with aortic stenosis applied balloon angioplasty. But three of them, which were not benefit from angioplasty, treated by surgically. In rest of the patients, stenosis did not worsen. Idiopathic hypercalcemia was evaluated in 10 patients, but not detected. Retinal arterial vascular tortiosity ratio was 82.6%. Two patients (8.7%) diagnosed hypothyroidism. Conclusion: In this study patients diagnosed WBS were evaluated, and results were discussed with literature