Abstract 124: Patient Condition Adaptive Path System of Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Masako Fujiwara ◽  
Tohru Kobayashi ◽  
Satoko Tsuru ◽  
Hiroyuki Ida

Background: In Japan, 2 guidelines are published, the clinical guidelines for medical treatment of acute stage Kawasaki disease(KD)(2012) and guidelines for diagnosis and management of cardiovascular sequelae in KD(2013). Patient Condition Adaptive Path System (PCAPS) is a technique to structure clinical knowledge. It places “patient condition” as a core, to which multiple “target conditions” are linked. On the other hand, patients of KD ware focused the severity of the disease and therapeutic strategy influences the improvement. Purpose: The purpose is confirming the PCAPS KD contents, which complied two Japanese Guidelines and to evaluate adaption of the contents. Methods: PCAPS content is composed of Clinical Process Chart (CPC) and Unit Sheet (US).CPC is an overhead view of clinical path consisting of a chain of units. CPC was made according the guidelines, and coronary evaluation, CHF, cardiac catheterization and ACS unit can activate on time. CPC stratify the patient’s severity. US are composed of specific healthcare tasks in a unit. Results: We confirm PCAPS KD contents on the base of 2 guidelines. We can evaluate diagnostic process and severity of KD by route analysis using CPC (figure). We can visualize relationship between treatments and severity by US. US are effective to support the decision on treatment and examinations. From the analysis, there are no lack of the unit and route, and confirm the advice to decision making. Conclusions: PCAPS can easily analyze the severity and clinical process from CPC route analysis because PCAPS is electrical path which can automatically store the data of each hospital. From US data, there are possibilities to find new severity score.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yizhou Wen ◽  
Xianmin Wang ◽  
Yonghong Guo ◽  
Mei Jin ◽  
Jimei Xi ◽  
...  

AbstractCoronary artery abnormalities (CAAs) are a severe complication of Kawasaki disease (KD) that may lead to cardiovascular events. Given the evidence that brachial artery flow-mediated dilation (FMD) decreases in children after the onset of KD, we hypothesized that it could be an early marker of CAA development in the acute stage and investigated its relationship with variation in the coronary artery diameter. A total of 326 sex- and age-matched children were enrolled, including 120 with KD, 109 febrile children and 97 healthy controls. In this study, FMD was significantly decreased in the KD group compared with the febrile and healthy groups. FMD was lower in the CAA group than in the no coronary artery abnormality group. The comparison of FMD showed an obvious difference among the CAA subgroups. The FMD in the coronary aneurysm (CA) group showed a strong negative correlation with the pretreatment maximum coronary artery Z-score (preZmax). While preZmax was 2.5, the receiver operating characteristic curve indicated an optimal cutoff point of 3.44% for FMD. FMD ≤ 3.44% could be considered as a signal of coronary lesions in acute stage of KD.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
yeo hyang kim ◽  
Chae Ok Shin ◽  
Myung Chul Hyun ◽  
Dong Seok Lee

Purpose: Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a systemic vasculitis, especially involving the coronary arteries. Although, sometimes, subclinical myocarditis is combined in KD, symptomatic myocarditis is extremely uncommon. We report a 7 year old boy who developed hypotension and decreased left ventricular systolic function (EF 40%) in the acute phase of KD. Case: A 7 year old boy (height 115 cm, body weight 20 kg) was admitted because of 2 days of persistent fever and left cervical lymphadenopathy (white blood cell count 17,870 /mm 3 , C reactive protein 23.6 mg/dL). Conjunctiva injection and lip redness developed on the 4th day of illness, and hypotension and tachycardia (SBP 59/DBP 29 mmHg, HR 153/bpm) were combined. The echocardiography revealed a decreased ejection fraction (EF) (40%) without chamber dilatation and normal coronary artery size (LM 1.9mm, z score=-1.3, RCA 2.3mm, z score=0.4). The level of N terminal pro BNP was 28,000 pg/mL. With a diagnosis of KD with myocarditis, he was initially treated with inotropics and intravenous immunoglobulin (2 g/kg). Without clinical improvement in spite of initial treatment, A change of coronary arterial size (LM 2.9mm, z score=1.2, RCA 3.1mm, z score=2.3) was developed and decreased LV systolic function (EF 45%) and fever were persisted. Then, he was given 3 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. He showed prompt clinical recovery after pulse therapy of intravenous methylprednisolone (SBP 95/DBP 49 mmHg, HR 98/bpm). Although EF was improved (59%), coronary arterial dilatation was progressed (LM 3.4mm, z score=2.4 RCA 5.5mm, z score=7.9). Conclusions: The present case serves to highlight the fact that methylprednisolone should be considered as the priority in children with KD who have symptomatic myocarditis during the acute stage.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Yuka Torii ◽  
Kazuhiro Horiba ◽  
Satoshi Hayano ◽  
Taichi Kato ◽  
Takako Suzuki ◽  
...  

Abstract Background Kawasaki disease (KD) is an idiopathic systemic vasculitis that predominantly damages coronary arteries in children. Various pathogens have been investigated as triggers for KD, but no definitive causative pathogen has been determined. As KD is diagnosed by symptoms, several days are needed for diagnosis. Therefore, at the time of diagnosis of KD, the pathogen of the trigger may already be diminished. The aim of this study was to explore comprehensive pathogens in the sera at the acute stage of KD using high-throughput sequencing (HTS). Methods Sera of 12 patients at an extremely early stage of KD and 12 controls were investigated. DNA and RNA sequences were read separately using HTS. Sequence data were imported into the home-brew meta-genomic analysis pipeline, PATHDET, to identify the pathogen sequences. Results No RNA virus reads were detected in any KD case except for that of equine infectious anemia, which is known as a contaminant of commercial reverse transcriptase. Concerning DNA viruses, human herpesvirus 6B (HHV-6B, two cases) and Anelloviridae (eight cases) were detected among KD cases as well as controls. Multiple bacterial reads were obtained from KD and controls. Bacteria of the genera Acinetobacter, Pseudomonas, Delfita, Roseomonas, and Rhodocyclaceae appeared to be more common in KD sera than in the controls. Conclusion No single pathogen was identified in serum samples of patients at the acute phase of KD. With multiple bacteria detected in the serum samples, it is difficult to exclude the possibility of contamination; however, it is possible that these bacteria might stimulate the immune system and induce KD.


2013 ◽  
Vol 35 ◽  
pp. 505-511 ◽  
Author(s):  
Hwa Jin Cho ◽  
Young Earl Choi ◽  
Eun Song Song ◽  
Young Kuk Cho ◽  
Jae Sook Ma

Incomplete Kawasaki disease (iKD) is considered to be a less complete form of Kawasaki disease (cKD), and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group). Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study. We obtained four measurements of serum procalcitonin levels and those of other inflammatory markers from each patient. Samples were taken for analysis on the day of diagnosis (thus before treatment commenced; D0) and 2 (D2), 14 (D14), and 56 days (D56) after intravenous immunoglobulin infusion. We obtained control group data at D0. The mean D0 serum procalcitonin levels of cKD patients (0.71±1.36 ng/mL) and controls (0.67±1.06 ng/mL) were significantly higher than those of iKD patients (0.26±0.26 ng/mL) (P=0.014andP=0.041, resp.). No significant difference in mean procalcitonin level was evident among groups at any subsequent time. In conclusion, the serum procalcitonin level of patients with acute-stage cKD was significantly higher than that of iKD patients.


2003 ◽  
Vol 53 (1) ◽  
pp. 161-161
Author(s):  
Tomoyo Matsubara ◽  
Motoki Fujiwara ◽  
Mayumi Koga ◽  
Takashi Ichiyama ◽  
Susumu Furukawa
Keyword(s):  
T Cells ◽  

2017 ◽  
Vol 28 (3) ◽  
pp. 386-390 ◽  
Author(s):  
Luis M. Garrido-García ◽  
Edna Morán-Villaseñor ◽  
Marco A. Yamazaki-Nakashimada ◽  
Patricia Cravioto ◽  
Fernando Galván

AbstractBackgroundOne of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear.MethodsA retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of p<0.05 was considered statistically significant. Odds ratios and their 95% confidence intervals were calculated to define risk factors.ResultsDuring the study period, 416 patients were diagnosed with Kawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms.ConclusionsOne of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Y Huang

Abstract Kawasaki disease (KD) is the most common acute coronary vasculitis disease occurring in children. Its occurrence has been attributed to the combined effects of infection, genetics, and immunity. While the etiopathogenesis of KD remains unknown, we have performed a survey of global genetic DNA methylation status and transcripts expression in KD patients to address how they contribute to the pathogenesis of KD. Methods We recruited 148 participants for this study. The chip studies consisted of 18 KD patients that were analyzed prior to administering intravenous immunoglobulin (IVIG) treatment and at least 3 weeks afterward, as well as 36 non-KD control subjects. We performed a separate cohort of 94 subjects to validate real-time quantitative PCR. Results According to the microarray study, CD177, a neutrophil surface molecule, appeared to be most significantly upregulated in KD patients compared to controls with epigenetic hypomethylation. After patients received IVIG treatment, CD177 mRNA levels decreased significantly. PCR validation indicated that the expression of CD177 is consistent with the Transcriptome Array 2.0 results. Furthermore, the area under the curve values of CD177 between KD patients and controls is 0.937. We also observed significantly higher CD177 levels in typical KD than incomplete presentation, which was associated with IVIG resistance in KD patients. Conclusion The present study is the first to highlight the epigenetic hypomethylation of an increased CD177 transcript during the acute stage of KD. Furthermore, the higher expression of CD177 in a typical presentation of KD patients and was related to IVIG resistance.


2010 ◽  
Vol 74 (5) ◽  
pp. 983-989 ◽  
Author(s):  
Ken-Pen Weng ◽  
Kai-Sheng Hsieh ◽  
Yu-Tung Hwang ◽  
Shih-Hui Huang ◽  
Tsung-Jen Lai ◽  
...  

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