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      Clinical features of Kawasaki disease initially mimicking retropharyngeal abscess: a retrospective analysis

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          Abstract

          Objective

          Incomplete Kawasaki disease (IKD) initially presenting as retropharyngeal abnormality is very rare and is prone to misdiagnosis and missed diagnosis, often leading to poor prognosis. Most patients were misdiagnosed with retropharyngeal abscesses. Here, we describe and compare IKD patients initially presenting with retropharyngeal abnormalities, typical KD patients without retropharyngeal abnormalities and retropharyngeal abscess patients.

          Methods

          We performed a retrospective case–control study comparing IKD patients initially presenting with retropharyngeal abnormalities to both KD patients without retropharyngeal abnormalities and retropharyngeal abscess patients admitted to Shenzhen Children’s Hospital between January 2016 and December 2021.

          Results

          We evaluated data from 10 IKD patients initially presenting with retropharyngeal abnormalities (Group A), 20 typical KD patients (Group B) and 16 surgical drainage confirmed retropharyngeal abscess patients (Group C). Compared to Group B, we observed that Group A was older and had a more intense inflammatory response. On the day of admission, Groups A and C had similar early clinical presentations, and there were no significant differences in any major signs or symptoms. Close observation for the development of new KD signs and symptoms and unresponsiveness to empirical antibiotic therapy after 3 days is extremely important. The CRP ( p = 0.011), AST ( p = 0.002) and ALT ( p = 0.013) levels were significantly higher and the WBC ( P = 0.040) levels were significantly lower in Group A than in Group C. Neck radiological findings, such as the presence of ring enhancement ( p = 0.001) and mass effects on the airway, are also useful tools for distinguishing these two diseases.

          Conclusion

          The careful observation of the signs and symptoms of this disease and the comprehensive analysis of the laboratory tests and neck radiological findings may help clinicians become aware of retropharyngeal abnormality as an atypical presentation of KD. Then, unnecessary treatments could be reduced, and the occurrence of serious complications can be avoided.

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          Most cited references32

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          Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

          Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.
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            The Epidemiology and Pathogenesis of Kawasaki Disease

            Epidemiologic and clinical features of Kawasaki Disease (KD) strongly support an infectious etiology. KD is worldwide, most prominently in Japan, Korea, and Taiwan, reflecting increased genetic susceptibility among Asian populations. In Hawaii, KD rates are 20-fold higher in Japanese ethnics than in Caucasians, intermediate in other ethnicities. The age distribution of KD, highest in children < 2 yo, lower in those < 6 months, is compatible with infection by a ubiquitous agent resulting in increasing immunity with age and with transplacental immunity, as with some classic viruses. The primarily winter-spring KD seasonality and well-documented Japanese epidemics with wave-like spread also support an infectious trigger. We hypothesize KD pathogenesis involves an RNA virus that usually causes asymptomatic infection but KD in a subset of genetically predisposed children. CD8 T cells, oligoclonal IgA, and upregulation of cytotoxic T cell and interferon pathway genes in the coronaries in fatal KD also support a viral etiology. Cytoplasmic inclusion bodies in ciliated bronchial epithelium identified by monoclonal antibodies made from oligoclonal IgA heavy chains also supports a viral etiology. Recent availability of “second generation” antibodies from KD peripheral blood plasmablasts may identify a specific viral antigen. Thus, we propose an unidentified (“new”) RNA virus infects bronchial epithelium usually causing asymptomatic infection but KD in a subset of genetically predisposed children. The agent persists in inclusion bodies, with intermittent respiratory shedding, entering the bloodstream via macrophages targeting coronaries. Antigen-specific IgA plasma cells and CD8 T cells respond but coronaries can be damaged. IVIG may include antibody against the agent. Post infection, 97–99% of KD patients are immune to the agent, protected against recurrence. The agent can spread either from those with asymptomatic primary infection in winter-spring or from a previously infected contact who intermittently sheds the agent.
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              Coronary artery lesions of incomplete Kawasaki disease: a nationwide survey in Japan.

              Incomplete Kawasaki disease (KD) is associated with delayed diagnosis and treatment, which in turn can lead to the development of coronary artery lesions (CALs). The aim of this study was to determine the epidemiological features of incomplete KD compared with complete KD and to identify risk factors for CALs from incomplete KD patients using data from a nationwide survey of 2007-2008 in Japan. A total of 23,263 patients were classified according to the number of principal clinical signs: 80% (n = 18,620) had complete forms of KD, 14.2% had four principal signs, 4.6% had three signs, and 1.2% had only one or two signs. In comparison with complete KD cases, the prevalence of CAL development tended to be larger and the proportion receiving initial intravenous immunoglobulin (IVIG) treatment were significantly smaller in patients with incomplete forms. In addition, hospital attendance after 7 days of illness or later was significantly associated with CAL development in all incomplete groups (OR: 2.52 in total patients with incomplete KD, 3.26 in those with one or two principal signs, 2.94 in those with three signs, 2.35 in those with four signs). The higher prevalence of CALs in incomplete KD reflects difficulties in diagnosis and delays in treatment. More timely diagnosis and treatment of incomplete KD patients could further prevent the development of cardiac lesions.
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                Author and article information

                Contributors
                tys118@163.com
                Journal
                Pediatr Rheumatol Online J
                Pediatr Rheumatol Online J
                Pediatric Rheumatology Online Journal
                BioMed Central (London )
                1546-0096
                13 December 2022
                13 December 2022
                2022
                : 20
                : 115
                Affiliations
                [1 ]GRID grid.452787.b, ISNI 0000 0004 1806 5224, Department of Otorhinolaryngology, , Shenzhen Children’s Hospital, ; 7019 Yitian Road, Futian District, Shenzhen, Guangdong China
                [2 ]GRID grid.412449.e, ISNI 0000 0000 9678 1884, Department of Otorhinolaryngology, Shenzhen Children’s Hospital, , China Medical University, ; Shenzhen, Guangdong China
                Article
                778
                10.1186/s12969-022-00778-4
                9749284
                36514104
                61d3909b-d7b8-471b-bf2d-d1763896772f
                © The Author(s) 2022

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 18 July 2022
                : 6 September 2022
                Funding
                Funded by: Shenzhen Municipal Science and Technology Innovation Committee
                Award ID: JCYJ20210324143008022
                Award Recipient :
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2022

                Pediatrics
                children,kawasaki disease,retropharyngeal abnormality,retropharyngeal abscess,retropharyngeal cellulitis

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