• 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.

• Clinical and Experimental Pediatrics
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• 제58권10호
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• pp.369-373
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• 2015

Purpose: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. Results: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001). Conclusion: Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.

• Clinical and Experimental Pediatrics
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• 제56권9호
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• pp.377-382
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• 2013

Kawasaki disease (KD) is an acute febrile illness that is the predominant cause of pediatric acquired heart disease in infants and young children. Because the diagnosis of KD depends on clinical manifestations, incomplete cases are difficult to diagnose, especially in infants younger than 1 year. Incomplete clinical manifestations in infants are related with the development of KD-associated coronary artery abnormalities. Because the diagnosis of infantile KD is difficult and complications are numerous, early suspicion and evaluation are necessary.

• Clinical and Experimental Pediatrics
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• 제61권5호
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• pp.167-173
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• 2018

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. Methods: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. Results: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5-1792.0) pg/mL vs. 233.0 (IQR, 107.0-544.0) pg/mL. Conclusion: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

• Pediatric Infection and Vaccine
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• 제22권1호
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• pp.40-44
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• 2015

비정형 가와사키병은 가와사키병의 진단기준을 충족하지 않는 경우를 말하며, 주로 6개월 미만의 영아에게서 발현하는 경우가 많다. 비정형 가와사키병의 임상소견은 뇌수막염과 같은 감염질환과 비슷할 때가 있어서, 이러한 경우 임상적으로 비정형 가와사키병을 감염질환과 감별하기 어려울 때가 많다. 또한 가와사키병과 연관되어 보고된 신경계 이상은 무균수막염, 경막하삼출, 안면신경마비, 뇌경색증, 뇌병증, 뇌자기공명영상의 가역적 뇌량팽대 변화 등이 있다. 본 저자들은 뇌척수액세포증가증과 경막외삼출액이 동반된 비정형 가와사키병으로 진단된 5개월 여아에 대해 보고하는 바이다. 환자의 심장초음파검사에서 관상동맥이 늘어나 있었고, 추적관찰에서 경막외 삼출액과 관상동맥 합병증이 모두 회복되었으며 생후 12개월에 발달이정표는 정상이었다.

• Clinical and Experimental Pediatrics
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• 제56권9호
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• pp.389-395
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• 2013

Purpose: This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. Methods: This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. Results: The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. Conclusion: The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions.

• Clinical and Experimental Pediatrics
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• 제58권3호
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• pp.84-88
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• 2015

Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.

• Pediatric Infection and Vaccine
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• 제22권3호
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• pp.206-209
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• 2015

저자들은 가와사키병 급성기에 치료에 반응하지 않은 저혈압(수축기 혈압 59 mmHg와 이완기 혈압 29 mmHg)과 수축기 심실 기능 부전(단축분획 22%)이 있던 7세 남아를 치료한 경험을 보고한다. 이 증례를 통해 가와사키병 급성기에 동반될 수 있는 유증상 심근염은 면역 글로블린 치료로 증상이 호전되지 않는 경우 스테로이드 펄스 요법이 필요함을 알 수 있다.

• Childhood Kidney Diseases
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• 제18권1호
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• pp.42-46
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• 2014

가와사끼병은 전신성 혈관염을 일으키는 질환중의 하나로 여러 장기들을 침범할 수 있다. 신장증세로는 농뇨, 혈뇨, 단백뇨, 간질성 신염, 급성 신부전증, 용혈성 요독 증후군, 신반흔 등이 있다. 가와사끼병의 신장침범에 대한 병리기전은 아직 알려져 있지 않지만, 자가면역질환으로 인한 것으로 사려된다. 가와사끼병이 요로감염 이 후에 발병한다는 몇몇 보고들이 있었다. 하지만, 이미 보고된 논문들에 포함된 많은 요로감염 환자들은 신장방광 초음파, DMSA 스캔이나 배뇨중 요도방광조영술 등을 모두 받은 경우는 없었다. 이에 저자들은 급성 신우신염이 재발한 후 불완전 가와사끼병이 발생한 고도의 방광요관역류가 있는 8개월 남아를 보고하는 바이다. 급성 신우신염은 가와사끼병의 초기 증세일 수 있다. 그런 경우, 환아가 가와사끼병으로 확진되더라도 요로감염 진료지침에 따라 요로기형에 대한 이미지 검사를 시행할 필요가 있다고 생각한다.

• Clinical and Experimental Pediatrics
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• 제50권3호
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• pp.292-297
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• 2007

목 적 : 학동기 아동에서의 가와사끼병은 드물게 나타나며 대부분 불완전한 양상을 보이므로 진단이 늦어지고 심혈관계 합병증의 발생이 증가한다. 본 연구에서는 이들의 임상 특징을 조사하여 이 연령군에서 가와사끼병의 조기 진단에 도움이 되고자 하였다. 방 법 : 1995년 6월부터 2006년 5월까지 가와사끼병으로 입원하여 치료받은 7세 이상의 소아 38명을 대상으로 임상 특징을 후향적으로 조사하였다. 결 과 : 연구 기간 중 7세 이상의 환아는 4.9%이었고, 남녀 비는 2.5:1이었고, 연령 분포는 7-12세였다. 76%가 불완전형 가와사끼병으로 이 때에는 발열을 제외하고 경부 림프절 비대가 가장 흔히 나타났고(69%), 다음으로 양측성 결막 충혈(62%), 부정형 발진(45%)의 순이었다. 또한 복통, 두통, 구토 및 관절통 등 다른 증상을 동반하는 경우가 있었다. 불완전형 가와사끼병의 초기 진단명은 경부 림프절염이 10명(34%)으로 가장 많았고, 그 외에 바이러스 감염 4명(14%), 성홍열 2명(7%), 뇌수막염 2명(7 %), Kikuchi 병(Kikuchi disease) 2명(7%)의 순이었다. 관상동맥 합병증은 15명(39%)에서 나타났고, 첫번째 IVIG 치료에 반응하지 않은 경우는 5명(14%)이었으며 이들에서 모두 관상동맥 병변이 발생하였다. 결 론 : 학동기 아동의 가와사끼병은 대부분 불완전형으로, 경부 림프절 비대가 흔하게 나타나며 심혈관계 합병증의 발생률이 높고 비특이적인 증상들을 동반하는 경우가 많아 다른 감염성 질환으로 오진되기 쉽다. 따라서, 항생제 치료에 반응하지 않고 발열이 지속될 경우 가와사끼병의 가능성을 항상 염두에 두어야 하며 조기 진단 및 치료를 함으로써 심혈관계 합병증의 위험성을 감소시켜야 한다.