• Clinical and Experimental Pediatrics
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• v.55 no.3
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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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• v.58 no.10
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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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• v.56 no.9
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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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• v.61 no.5
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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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• v.22 no.1
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• pp.40-44
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• 2015

Cases of incomplete Kawasaki disease (KD), wherein the patient does not fulfill the full diagnostic criteria for KD, are often detected in infants younger than 6 months of age. The clinical manifestations in infants with incomplete KD may resemble other infectious diseases, including meningitis. For this reason, clinicians may have difficulty differentiating incomplete KD from other infectious diseases in this population. Various neurological features are associated with KD, including aseptic meningitis, subdural effusion, facial nerve palsy, cerebral infarction, encephalopathy, and reversible corpus callosum splenial lesions on magnetic resonance imaging. We report a case of a 5-month-old girl with incomplete KD, associated with cerebrospinal fluid pleocytosis and an epidural fluid collection. Echocardiography indicated dilatation of the main coronary arteries. The girl made a complete recovery, with resolution of both the epidural fluid collection and coronary artery aneurysms. In this case, the child is well, and showed normal developmental milestones at the 7-month follow-up.

• Clinical and Experimental Pediatrics
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• v.56 no.9
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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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• v.58 no.3
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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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• v.22 no.3
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• pp.206-209
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• 2015

We report the case of a 7-year-old boy who showed treatment-nonresponsive hypotension (59/29 mmHg) and decreased left ventricular systolic function (fractional shortening 22%) in the acute stage of Kawasaki disease (KD). The present case serves to highlight that methylprednisolone pulse therapy should be considered in patients with intravenous immunoglobulin nonresponsive symptomatic myocarditis during the acute stage of KD.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.292-297
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• 2007

Purpose : Kawasaki disease (KD) rarely occurs in school-aged children. We clarified the characteristics of KD in this age group to provide tips for a high index of suspicion. Methods : Features of 38 patients with KD who were 7 years of age or older were retrospectively reviewed. Results : The incidence of the KD patients ${\geq}7years$ was 4.9 percent. The ratio of male to female was 2.5:1. Of the 38 patients, nine patients (24.0 percent) were diagnosed with typical KD and 29 patients (76.0 percent) with incomplete KD. In incomplete KD patients, cervical lymphadenopathy (69.0 percent) occurred most frequently, followed by conjunctival injection (62.0 percent) and polymorphous rash (45.0 percent). These patients occasionally presented with other additional symptoms including abdominal pain, headache, vomiting and arthralgia. Incomplete KD was initially diagnosed as cervical lymphadenitis (34.0 percent), viral infection (14.0 percent), scarlet fever (7.0 percent), meningitis (7.0 percent), and Kikuchi disease (7.0 percent). Coronary complications were noted in 15 patients (39.0 percent). Of the 37 patients treated with intravenous immunoglobulin, five (14.0 percent) were resistant to the therapy and all had coronary abnormalities. Conclusion : Most patients with KD ${\geq}7years$ of age have incomplete presentations. They tend to have a higher incidence of initial presentations of unilateral neck mass and coronary artery involvement. In school-aged children, fever and cervical lymphadenitis or suspected neck infection unresponsive to intravenous antibiotics should signal the possibility of KD. A high index of suspicion and prompt treatment is essential in this age group of patients.