• 제목/요약/키워드: Kawasaki syndrome

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Diagnosis of incomplete Kawasaki disease

  • Yu, Jeong-Jin
    • Clinical and Experimental Pediatrics
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    • 제55권3호
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    • pp.83-87
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    • 2012
  • Several authors suggested that the clinical characteristics of incomplete presentation of Kawasaki disease are similar to those of complete presentation and that the 2 forms of presentation are not separate entities. Based on this suggestion, a diagnosis of incomplete Kawasaki disease in analogy to the findings of complete presentation is reasonable. Currently, the diagnosis of incomplete Kawasaki disease might be made in cases with fewer classical diagnostic criteria and with several compatible clinical, laboratory or echocardiographic findings on the exclusion of other febrile illness. Definition of incomplete presentation in which coronary artery abnormalities are included as a necessary condition, is restrictive and specific. The validity of the diagnostic criteria of incomplete presentation by the American Heart Association should be thoroughly tested in the immediate future.

가와사끼병 및 말단 조직 괴저가 동반된 혈구탐식 증후군 1례 (Hemophagocytic Syndrome with Kawasaki Disease and Peripheral Gangrene)

  • 윤화준;전고운;김황민;박석원;어영
    • Clinical and Experimental Pediatrics
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    • 제45권5호
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    • pp.664-668
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    • 2002
  • 저자들은 이에 가와사끼병 및 말단 조직괴저가 동반된 혈구 탐식증 1례를 보고하는 바이며, 가와사끼병 환자가 간비종대, 혈소판 감소를 보이는 경우에는 드물지만 혈구 탐식증의 가능성을 배제할 수 없으므로 확진을 위하여 혈청 중성지방의 측정과 골수 생검을 시행하여야 할 것으로 사료하는 바이다.

가와사키병 치료를 위한 아스피린 사용 후 발생한 라이 증후군 1예 (A Case of Reye Syndrome Following Treatment of Kawasaki Disease with Aspirin)

  • 이준기;강지은;최은화;최정연
    • Pediatric Infection and Vaccine
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    • 제19권2호
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    • pp.79-83
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    • 2012
  • 라이 증후군은 간부전을 동반하며 급속히 진행하는 뇌증으로, 인플루엔자와 수두 바이러스 등에 감염된 어린이에게 발병한다. 아스피린이 주된 원인 인자로 알려진 이후 소아에서 아스피린의 사용을 줄이면서 현재는 매우 드물게 보고 되고 있다. 저자들은 가와사키병 치료를 위해 정맥내 면역글로불린을 투여받고 아스피린을 복용하던 중 보챔, 강직, 활동량 저하, 식욕 저하, 처짐, 구토, 간수치 상승, 혈액 응고 장애와 고암모니아혈증으로 치료받은 5개월 여아를 보고하는 바이다.

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Clinical features, diagnosis, and outcomes of multisystem inflammatory syndrome in children associated with coronavirus disease 2019

  • Kwak, Ji Hee;Lee, Soo-Young;Choi, Jong-Woon;Korean Society of Kawasaki Diseasety of Pediatric Endocrinology (KSPE),
    • Clinical and Experimental Pediatrics
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    • 제64권2호
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    • pp.68-75
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    • 2021
  • The novel coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been spreading worldwide since December 2019. Hundreds of cases of children and adolescents with Kawasaki disease (KD)-like hyperinflammatory illness have been reported in Europe and the United States during the peak of the COVID-19 pandemic with or without shock and cardiac dysfunction. These patients tested positive for the polymerase chain reaction or antibody test for SARS-CoV-2 or had a history of recent exposure to COVID-19. Clinicians managing such patients coined new terms for this new illness, such as COVID-19-associated hyperinflammatory response syndrome, pediatric inflammatory multisystem syndrome temporally associated with COVID-19, or COVID-19-associated multisystem inflammatory syndrome in children (MIS-C). The pathogenesis of MIS-C is unclear; however, it appears similar to that of cytokine storm syndrome. MIS-C shows clinical features similar to KD, but differences between them exist with respect to age, sex, and racial distributions and proportions of patients with shock or cardiac dysfunction. Recommended treatments for MIS-C include intravenous immunoglobulin, corticosteroids, and inotropic or vasopressor support. For refractory patients, monoclonal antibody to interleukin-6 receptor (tocilizumab), interleukin-1 receptor antagonist (anakinra), or monoclonal antibody to tumor necrosis factor (infliximab) may be recommended. Patients with coronary aneurysms require aspirin or anticoagulant therapy. The prognosis of MIS-C seemed favorable without sequelae in most patients despite a reported mortality rate of approximately 1.5%.

독성 쇼크 증후군 증상을 나타낸 비전형적 가와사끼병 1례 (Atypical Kawasaki Disease Presented with Toxic Shock Syndrome)

  • 이경연;박준은;박우성
    • Clinical and Experimental Pediatrics
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    • 제45권8호
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    • pp.1048-1051
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    • 2002
  • 저자들은 8세 남아에서 고열, 저혈압, 부정형 피부 발진, 의식저하, 결막염, 구토, 설사, 핍뇨, 혈소판 저하, 혈액배양 음성과 같은 독성 쇼크 증후군의 증상과 함께 관상동맥류를 포함한 가와사끼병의 임상증상을 보인, 독성 쇼크 증후군 증상으로 나타난 비전형적 가와 사끼병 1례를 경험하였기에 문헌고찰과 함께 보고하며, 독성 쇼크 증후군의 치료 중 발열이 지속될 때 가와사끼병의 비전형적인 증상 발현일 수 있음을 염두해야 할 것으로 사료된다.

피부 감염과 연관된 비월경성 독성 쇽 증후군 1례 (A Case of Nonmenstrual Toxic Shock Syndrome Associated with Skin Infection)

  • 장지현;김종현;허재균;강진한;고대균
    • Pediatric Infection and Vaccine
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    • 제4권1호
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    • pp.160-166
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    • 1997
  • Toxic shock syndrome(TSS) is a multisystemic disease presenting with high fever, sunburn like rash that subsequently desquamates, and hypotension mainly caused by toxin producing strains of Staphylococcus aureus. It was first reported in 1978 by Todd et al, thereafter many patients have been reported. In children, TSS is rare and must be differentiated from other erythematous febrile diseases such as Kawasaki disease, scarlet fever, drug eruption etc. We experienced a case of TSS associated with staphylococcal cellulitis in 26-month old boy, who was presenting similar symptoms to Kawasaki disease at initial stage of illness. As time passed, the patient represented more typical symptoms of TSS and Staphylococcus aureus was isolated from cellulitis of the right elbow area. Therefore, we report this case with a brief review of related literatures.

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A Boy With Blau Syndrome Misdiagnosed as Refractory Kawasaki Disease

  • Kyungwon Cho;Yoonsun Yoon;Joon-sik Choi;Sang Jin Kim;Hirokazu Kanegane;Yae-Jean Kim
    • Pediatric Infection and Vaccine
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    • 제29권3호
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    • pp.166-172
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    • 2022
  • 블라우증후군(Blau syndrome)은 nucleotide-binding oligomerization domain protein 2 (NOD2) 유전자의변이에 의해 발생하는 질환으로 육아종성 피부염 및 만성 포도막염, 관절염을 특징으로 한다. 증상이 비특이적이고 동시다발적으로 발생하지 않아 진단이 어려운 경우가 많다. 반복되는 피부 발진 및 발열에 대해 두 차례 가와사키병으로 오진되어 면역글로불린과 전신 스테로이드로 치료받은 바 있는 13개월 남자 환자에서 블라우 증후군을 진단한 증례를 보고하고자 한다.

Etiological and pathophysiological enigmas of severe coronavirus disease 2019, multisystem inflammatory syndrome in children, and Kawasaki disease

  • Rhim, Jung-Woo;Kang, Jin-Han;Lee, Kyung-Yil
    • Clinical and Experimental Pediatrics
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    • 제65권4호
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    • pp.153-166
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    • 2022
  • During the coronavirus disease 2019 (COVID-19) pandemic, a novel multisystem inflammatory syndrome in children (MIS-C) has been reported worldwide since the first cases were reported in Europe in April 2020. MIS-C is temporally associated with severe acute respiratory syndrome coronavirus 2 infection and shows Kawasaki disease (KD)-like features. The epidemiological and clinical characteristics in COVID-19, KD, and MIS-C differ, but severe cases of each disease share similar clinical and laboratory findings such as a protracted clinical course, multiorgan involvement, and similar activated biomarkers. These findings suggest that a common control system of the host may act against severe disease insult. To solve the enigmas, we proposed the protein-homeostasis-system hypothesis in that every disease involves etiological substances and the host's immune system controls them by their size and biochemical properties. Also, it is proposed that the etiological agents of KD and MIS-C might be certain strains in the microbiota of human species and etiological substances in severe COVID-19, KD, and MIS-C originate from pathogen-infected cells. Since disease severity depends on the amounts of inflammation-inducing substances and corresponding immune activation in the early stage of the disease, an early proper dose of corticosteroids and/or intravenous immunoglobulin (IVIG) may help reduce morbidity and possibly mortality among patients with these diseases. Corticosteroids are low cost and an analogue of host-origin cortisol among immune modulators. This study's findings will help clinicians treating severe COVID-19, KD, and MIS-C, especially in developing countries, where IVIG and biologics supplies are insufficient.

치료불응 가와사끼병의 임상양상을 보인 대식세포활성 증후군 (Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease)

  • 박현진;조윤정;배이영;최의윤;이수영;정대철;이경일;강진한
    • Pediatric Infection and Vaccine
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    • 제17권2호
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    • pp.177-181
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    • 2010
  • KD의 급성 열성기에 드물지만 MAS가 발생할 수 있다. 저자들은 19개월 여아의 KD 치료 중에 발생된 MAS를 경험하였다. 환아는 IVIG과 steroid 치료를 받았지만, 발열과 간비종대는 지속되었고 골수생검에서 혈구탐식구증이 관찰되었다. KD에 동반된 MAS를 인지하여 적기에 치료하기 위해서는 임상양상과 치료반응을 토대로, MAS의 가능성을 고려하는 것이 필요하다.