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Am J Emerg Med 2021 Jun 8;49:148-152. Epub 2021 Jun 8.

Department of Emergency Medicine, Brooke Army Medical Center, USA.

Background: Multisystem inflammatory syndrome in children (MIS-C) is a dangerous pediatric complication of COVID-19.

Objective: The purpose of this review article is to provide a summary of the diagnosis and management of MIS-C with a focus on management in the acute care setting.

Discussion: MIS-C is an inflammatory syndrome which can affect nearly any organ system. Read More

Front Pediatr 2021 25;9:673554. Epub 2021 May 25.

Department of Pediatrics, Research Centre of the Sainte Justine University Hospital, Sainte Justine University Hospital, University of Montreal, Montreal, QC, Canada.

To review major epidemiological aspects of Kawasaki disease (KD) in Europe, describing demographic characteristics, revising its incidence along with time trends and geographic variations, and describing migration studies to provide clues about its etiology. The annual incidence of KD in Europe is about 10-15 per 100,000 children under 5 years old and seems to be relatively stable over time and space. Demographic characteristics are in line with those in other countries of the world, with a higher incidence in children from Asia and possibly North African origin. Read More

Keio J Med 2021 Jun 8. Epub 2021 Jun 8.

Department of Pediatrics, National Hospital Organization Saitama Hospital, Saitama, Japan.

Infection with Yersinia pseudotuberculosis, a known causal pathogen of human bacterial gastroenteritis, causes various symptoms and complications. A previously healthy 7-year-old girl was admitted because of fever and gastrointestinal symptoms. She was initially diagnosed with intussusception by abdominal ultrasonography. Read More

Cardiol Young 2021 Jun 10:1-5. Epub 2021 Jun 10.

Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan.

Background: Several studies have reported treatment options for patients with Kawasaki disease refractory to standard immunoglobulin therapy; however, no studies have reported low-dose immunoglobulin therapy for patients with a low risk of Kawasaki disease.

Methods: A total of 277 patients with Kawasaki disease were included in this study. We used Kobayashi score and our Less high-risk score to divide the patients into three groups. Read More

Crit Care 2021 06 9;25(1):199. Epub 2021 Jun 9.

Critical Care Research Group, The Prince Charles Hospital, Chermside, Australia.

Background: Heterogeneous respiratory system static compliance (C) values and levels of hypoxemia in patients with novel coronavirus disease (COVID-19) requiring mechanical ventilation have been reported in previous small-case series or studies conducted at a national level.

Methods: We designed a retrospective observational cohort study with rapid data gathering from the international COVID-19 Critical Care Consortium study to comprehensively describe C-calculated as: tidal volume/[airway plateau pressure-positive end-expiratory pressure (PEEP)]-and its association with ventilatory management and outcomes of COVID-19 patients on mechanical ventilation (MV), admitted to intensive care units (ICU) worldwide.

Results: We studied 745 patients from 22 countries, who required admission to the ICU and MV from January 14 to December 31, 2020, and presented at least one value of C within the first seven days of MV. Read More

Int J Clin Pract 2021 Jun 9:e14471. Epub 2021 Jun 9.

Ankara Yıldırım Beyazıt University, Ankara City Hospital, Department of Pediatric Surgery, Ankara.

Objective: Although the initial reports of coronavirus disease (COVID-19) cases in children described that children were largely protected from severe manifestations, clusters of pediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease (KD), toxic shock syndrome (TSS), and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (MAS). Our objective was to develop a protocol for the evaluation, treatment, and follow-up of patients with MIS-C. Read More

Int J Clin Pract 2021 Jun 9:e14450. Epub 2021 Jun 9.

Faculty of Medicine, University of Porto, Porto, Portugal, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal.

Objective: Analyze current literature and reported cases of MISC, concerning its clinical spectrum, complications associated, therapeutic strategies and distinguishing features of other clinical syndromes.

Methods: Extensive literature research was performed in MEDLINE (trough PubMed), Scopus and Web of Science from December 2019 to December 2020. First analysis included all article titles and abstracts screening to identify relevant studies and second analysis included a full text screening of previous selected studies. Read More

Turk Arch Pediatr 2021 1;56(3):192-199. Epub 2021 May 1.

Division of Cardiology, Department of Pediatrics, Kocaeli University, Kocaeli, Turkey.

Objective: Multisystem inflammatory syndrome in children (MIS-C) associated with the coronavirus disease 2019 (COVID-19) is a new concern emerging as a severe presentation of COVID-19 in children. We aimed to describe the characteristics and short-term outcomes of children diagnosed with MIS-C.

Material And Methods: A retrospective study was conducted on 24 patients who were diagnosed with MIS-C between June 1, 2020 and December 1, 2020. Read More

Ann Pediatr Cardiol 2021 Apr-Jun;14(2):187-191. Epub 2021 Feb 16.

Department of Cardiac Sciences, Division of Pediatric Cardiology, Aster CMI Hospital, Bengaluru, Karnataka, India.

Background: Kawasaki disease (KD) is the most common cause of acquired heart disease in developed countries. KD is increasingly being reported from India; however, studies involving the large number of patients are few.

Methods: All children presenting to the center from January 2017 to December 2019, diagnosed to have KD, were retrospectively included in the study. Read More

Ann Pediatr Cardiol 2021 Apr-Jun;14(2):146-151. Epub 2021 Apr 16.

Social Determinants of Health Research Center, Institute for Futures Studies in Health, Kerman University of Medical Sciences, Kerman, Iran.

Background: Standard first-step therapy for Kawasaki disease consists of Intravenous immunoglobulin and high dose Aspirin (80-100 mg/kg/day). The standard dose of Intravenous immunoglobulin (2gr/kg) is strongly effective in reducing the risk of coronary arteries abnormalities. So, the proper dose and efficacy of Aspirin to decrease the risk of coronary arteries abnormalities is a controversial issue. Read More

J Am Heart Assoc 2021 Jun 5:e020505. Epub 2021 Jun 5.

Kawasaki Disease Center and Department of Pediatrics Kaohsiung Chang Gung Memorial HospitalChang Gung University College of Medicine Kaohsiung Taiwan.

J Cardiol Cases 2021 Jun 11;23(6):281-284. Epub 2021 Feb 11.

Department of Pediatrics, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan.

A 33-year-old pregnant woman with a history of a giant coronary artery aneurysm (CAA) of the right coronary artery owing to Kawasaki disease (KD) was referred to our hospital for the management of pregnancy and delivery. The CAA was detected when she was 10 months old on the 24th day from the onset of KD and showed transient regression followed by progressive dilation and reached a size of 25 mm when she was 30 years old. The baby was delivered at 38 weeks of gestational age. Read More

Front Immunol 2021 19;12:660506. Epub 2021 May 19.

Department of Pediatrics, Dalhousie University, Halifax, NS, Canada.

Intravenous immunoglobulin (IVIG) is an effective immunomodulatory treatment for immune dysregulation diseases. However, the mechanisms by which it reduces systemic inflammation are not well understood. NK cell cytotoxicity is decreased by IVIG in women with reduced fertility, but IVIG effects on NK cells in immune dysregulation are less clear. Read More

Circulation 2021 Jun 7. Epub 2021 Jun 7.

Department of Pediatrics, Severance Children's Hospital and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, South Korea.

Can J Cardiol 2021 Jun 3. Epub 2021 Jun 3.

Division of Pediatric Cardiology, CHU Ste-Justine, Université de Montréal, Montréal, QC, Canada. Electronic address:

Background: The emergence of increasing reports worldwide of a severe inflammatory process and shock in pediatric patients resembling Kawasaki disease (KD) and more specifically Kawasaki disease shock syndrome (KDSS), prompted us to explore KDSS in a preamble of a systematic comparison between the two conditions.

Methods: We completed a systematic review of KDSS and performed a meta-analysis comparison between reported KDSS cases and KD controls.

Results: A total of ten case-control series were included in the meta-analysis. Read More

Mol Genet Metab 2021 May 12. Epub 2021 May 12.

Advanced Clinical Research Center, Southern Tohoku Research Center for Neuroscience, Kanagawa, Japan.

Introduction: Lysosomal storage disorders and peroxisomal disorders are rare diseases caused by the accumulation of substrates of the metabolic pathway within lysosomes and peroxisomes, respectively. Owing to the rarity of these diseases, the prevalence of lysosomal storage disorders and peroxisomal disorders in Japan is unknown. Therefore, we conducted a nationwide survey to estimate the number of patients with lysosomal storage disorders and peroxisomal disorders in Japan. Read More

Pediatr Rheumatol Online J 2021 Jun 5;19(1):82. Epub 2021 Jun 5.

Department of Pathology and Matrix Biology, Mie University Graduate School of Medicine, Tsu, Mie, Japan.

Background: Tenascin-C (TN-C) is an extracellular matrix glycoprotein related to tissue inflammation. Our previous retrospective study conducted in 2016 revealed that the serum tenascin-C level was higher in patients with Kawasaki disease (KD) who were resistant to intravenous immunoglobulin (IVIG) and developed coronary artery lesions (CALs). The present study is a prospective cohort study to assess if the serum level of tenascin-C could be used as a novel biomarker to predict the risk of resistance to initial treatment for high-risk patients. Read More

BMC Cardiovasc Disord 2021 Jun 5;21(1):278. Epub 2021 Jun 5.

Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama, 641-0012, Japan.

Background: Coronary artery aneurysm (CAA) is an important complication of Kawasaki disease (KD) that is associated with arterial structure damage. However, few studies have examined structural changes in coronary arteries that are not associated with CAA.

Methods: We examined coronary arteries in KD patients with CAAs who underwent follow-up coronary angiography (CAG) and optical coherence tomography (OCT). Read More

Eur J Pediatr 2021 Jun 4. Epub 2021 Jun 4.

Department of Pediatrics, Hospital Sultanah Aminah, Ministry of Health Malaysia, Persiaran Abu Bakar Sultan, 80100, Johor Bahru, Johor DT, Malaysia.

Data on Kawasaki disease from tropical countries are scarce. Hence, this population-based study aims to determine the epidemiology, clinical characteristics, and outcome of Kawasaki disease in children enrolled in the Kawasaki disease registry between 2006 and 2019 in Southern Malaysia. Diagnosis of Kawasaki disease was made using standard criteria. Read More

Circ Cardiovasc Imaging 2021 Jun 4:CIRCIMAGING120012153. Epub 2021 Jun 4.

Kawasaki Disease Center and Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Taiwan. (I.-H.T., H.-C.K.).

BMJ Open 2021 Jun 3;11(6):e042996. Epub 2021 Jun 3.

Department of Pediatrics and Kawasaki Disease Center, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan

Objective: Kawasaki disease (KD) is an acute form of febrile vasculitis that occurs in early childhood. The multisystemic vasculitis common in patients with KD may influence blood perfusion in the brain, and thus caregivers of children with KD may feel stress with regard to caring for them. Intravenous immunoglobulin (IVIG) infusion is the standard treatment for acute KD, and the most serious complication of KD is coronary artery aneurysms (coronary artery lesion (CAL)). Read More

Front Immunol 2021 17;12:640315. Epub 2021 May 17.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Innate immune activity plays an essential role in the development of Kawasaki disease (KD) vasculitis. Extracellular release of high mobility group box-1 (HMGB-1), an endogenous damage-associated molecular pattern protein that can activate the innate immune system and drive host inflammatory responses, may contribute to the development of coronary artery abnormalities in KD. Prednisolone (PSL) added to intravenous immunoglobulin treatment for acute KD may reduce such abnormalities. Read More

Ital J Pediatr 2021 Jun 2;47(1):120. Epub 2021 Jun 2.

Department of Pediatrics, Sahloul University Hospital, 4054, Sousse, Tunisia.

Background: Children affected by Coronavirus disease 2019 (COVID-19) showed various manifestations. Some of them were severe cases presenting with multi-system inflammatory syndrome (MIS-C) causing multiple organ dysfunction.

Case Presentation: We report the case of a 12-year-old girl with recent COVID-19 infection who presented with persistent fever, abdominal pain and other symptoms that meet the definition of MIS-C. Read More

Pediatr Rheumatol Online J 2021 Jun 2;19(1):78. Epub 2021 Jun 2.

Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.

Background: This study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels.

Methods: This retrospective analysis was based on the nationwide survey of KD conducted in the Republic of Korea between 2015 and 2017. We enrolled 9131 patients and defined low (< 3 mg/dL) and high (≥3 mg/dL) CRP groups. Read More

Pediatr Cardiol 2021 Jun 2. Epub 2021 Jun 2.

Department of Cardiology, Boston Children's Hospital, Harvard School of Medicine, Boston, MA, USA.

With growing number of pediatric cases of COVID-19, a unique hyper-inflammatory syndrome, linked to SARS-CoV-2 infection, has emerged in children referred to as multisystem inflammatory syndrome in children (MIS-C). This Kawasaki Disease (KD)-like illness has been described across the world. This syndrome shares features of KD, toxic shock syndrome, and macrophage activation syndrome and is associated with significantly elevated inflammatory markers. Read More

Int J Mol Sci 2021 May 26;22(11). Epub 2021 May 26.

Faculty of Medicine, Imperial College London, London SW7 2AZ, UK.

The aetiology of Kawasaki disease (KD), an acute inflammatory disorder of childhood, remains unknown despite various triggers of KD having been proposed. Host 'omic profiles offer insights into the host response to infection and inflammation, with the interrogation of multiple 'omic levels in parallel providing a more comprehensive picture. We used differential abundance analysis, pathway analysis, clustering, and classification techniques to explore whether the host response in KD is more similar to the response to bacterial or viral infections at the transcriptomic and proteomic levels through comparison of 'omic profiles from children with KD to those with bacterial and viral infections. Read More

J Clin Med 2021 May 27;10(11). Epub 2021 May 27.

Department of Pediatrics and Kawasaki Disease Center, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung 83301, Taiwan.

The Fc gamma receptor family contains several activating receptors and the only inhibitory receptor, . In this study, we investigated the dynamic methylation change of in different stages of Kawasaki disease (KD). We enrolled a total of 116 participants, which included patients with febrile diseases as controls and KD patients. Read More

Children (Basel) 2021 May 21;8(6). Epub 2021 May 21.

Department of Pediatrics, Kansai Medical University, Osaka, 2-5-1 Shin-machi, Hirakata-shi, Osaka 573-1010, Japan.

Kawasaki disease (KD) is a systemic vasculitis with an unknown etiology affecting young children. Although intravenous immunoglobulin (IVIG) plus acetylsalicylic acid is effective in most cases, approximately 10-20% of patients do not respond to this therapy. An 8-month-old boy was admitted to a local hospital with the presumptive diagnosis of KD. Read More

Hum Vaccin Immunother 2021 Jun 1:1-11. Epub 2021 Jun 1.

Medical Affairs, MSD K.K., Kitanomaru Square, Chiyoda, Tokyo, Japan.

Vaccine confidence reflects social, individual, and political factors indicating confidence in vaccines and associated health systems. In Japan, the government ceased proactive recommendation of the human papillomavirus (HPV) vaccine in June 2013, only several months after the recommendation had begun. Seven years later, as of October 2020, the suspension persists and vaccine coverage has precipitously declined, resulting in many young women being continually exposed to the risk of preventable HPV-related diseases. Read More

J Paediatr Child Health 2021 Jun 1. Epub 2021 Jun 1.

Faculty of Health and Medical Sciences, Department of Paediatrics Adelaide, The University of Adelaide, Adelaide, South Australia, Australia.

Aim: Kawasaki disease (KD) is one of the most common causes of acquired cardiac disease in children in high-income countries. The incidence of coronary artery disease (CAD), despite treatment with intravenous immunoglobulin, ranges from 5 to 20%. Determining risk factors for CAD may assist with management and reduce long-term complications. Read More