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

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

Arcus aorta, aortic and mitral valve calcifications in a patient with gaucher's disease homozygous for the D409H mutation; case report Homozigot D409H mutasyonlu gaucher hastasinda arkus aorta, aort ve mitral kapak kalsifikasyonu; olgu sunumu

Introduction: Cardiovascular involvement in Gaucher disease is infrequent. D409H point mutation has been defined in patients with aortic and mitral valve calcifications. Case Report: In this case report, a 17 year-old girl with Gaucher disease, having homozygous D409H point mutation is presented, who has mitral-aortic valve and aortic calcifications with corneal involvement. Her two siblings also had died according to valvular heart disease. Because of having high cardiovascular surgery risk, she is under enzyme replacement therapy. Conclusion: We aimed to point out the rare but important and vital prognostic cardiovascular involvement associated with corneal opacity and D409H mutation in Gaucher disease. © The Journal of Current Pediatrics