bluuebunny/crossref_metadata_2025 huggingface dataset:
Abstract 150: A Case Of Kawasaki Disease Complicated By Autoimmune Hemolytic Anemia In A Boy
Authors: Yu Seung-Taek , Kim Dong-Sup , Kim Jong-Duck , Oh Yeon-Kyun , Lee Chang-Woo , Choi Du-Young
Published: July 2021

Introduction:
Kawasaki disease is a systematic disease, usually affecting infants and toddlers. It can be characterized by rare complications or by unusual associations. One of these is autoimmune hemolytic anemia that has been observed seldom up to now. In this report, we present a case with this rare association.

Case:
A previous healthy 7-year-old boy was transferred form a primary clinic due to fever and neck mass. Neck CT showed acute pharyngitis with focal abscess formation and multiple enlarged lymph nodes at both retropharyngeal space. He was treated intravenous antibiotics for 3 days. On the 4th hospital days, the patient sustained high fever and presented diffuse maculopapular rash, conjunctival injection without exudate, erythema and edema of the hands and feet. Laboratory finding showed Hb 11.6 g/dL; hematocrit (Hct) 33.9 %; WBC count 15,350 μL; platelets count 308,000 μL; AST 123 IU/L; ALT 237 IU/L; CRP 103.14 mg/L; creatinine kinase (CK) 28 IU/L; CK-MB 0.6 ng/mL; pro-BNP 783.2 pg/mL. Mild dilatation of right coronary artery (4.14mm) was noted in echocardiogram. The patient was diagnosed Kawasaki disease and treated intravenous immunoglobulin (IVIG) (2g/kg) and aspirin. Retreatment with IVIG was administered for persistence of fever 48 hours after the first infusion. Thereafter, his symptoms gradually improved. On the 12th hospital day, his follow-up laboratory finding showed decreased Hb 7.1 g/dL and Hct 20.8 %; reticulocyte 10.71 %; peripheral blood smear: normocytic normochromic anemia with anisopoikilocytosis, immune hemolytic anemia; Direct polyspecific antiglobulin test positive. There was no hemodynamic instability. Prednisolone 2mg/kg/day P.O. was given for hemolytic anemia and hemolysis resolved following treatment. On follow-up, hemoglobin and hematocrit increased gradually. He made a good clinical recovery without cardiovascular complication.

Conclusion:
A 7-year-old boy presented with the fever, skin rash, conjunctival injection and lymphadenopathy. He was diagnosed Kawasaki disease. Autoimmune hemolytic anemia occur on the way of improving state after treatment. The authors report a case of autoimmune hemolytic anemia during Kawasaki disease
Abstract 153: Automated Angiographic Assessment Of Coronary Artery Vasomotion In Kawasaki Disease Patients
Authors: Benovoy Mitchel , Cheriet Farida , Maurice Roch L , Dahdah Nagib
Published: July 2021

Background:
Mechanical properties of coronary arteries (CA) hold clues to vascular health and viability. Traditionally assessed with intracoronary imaging, we present an angiography-based system to assess CA vasomotion using automatic vessel segmentation and spatio-temporal tracking. Elastic moduli computed from dynamic CA calibers are compared between non-KD patients (CTL), KD patients with no CA aneurysms (KDAN-), and those with aneurysms (KDAN+).

Methods:
Proximal CA angiograms are automatically segmented and tracked over a cardiac cycle. CA centerline is extracted and the mean caliber is computed from diameters along its length. The resulting caliber variation reflects the CA vasomotion (Figure 1a). We then calculated the Vasomotion Standard Deviation (VSD) and CA recoil with the mean constriction velocity (MCV). Finally, Elastic Pressure moduli were computed using trans-myocardium pressure gradients.

Results:
We analyzed 51 left CA segments from 23 patients (5 CTL, 5 KDAN-, 13 KDAN+). Data are mean ± SD normalized pixels (npx). VSD was significantly reduced (
p
<0.01) in KDAN+ (0.25±0.05) and KDAN- (0.27±0.04) vs CTL (0.38±0.07 npx). Coronary recoil was significantly reduced (p<0.05) in KDAN+ vs CTL, with MCV 3.50±0.67 vs 4.59±1.94 npx/sec. Pressure-dependent stiffness characteristics were equally atypical (Figure 1b).

Conclusion:
The proposed angiography-based stiffness assessment system shows abnormal CA vascular physiology in our cohort of KD patients. These results concur with previous invasive studies. The potential usability of this system for vascular health assessment could be applied to previously recorded CA angiograms for risk stratification.
Abstract 173: Coronary artery aneurysm measurement and z-score variability in Kawasaki Disease
Authors: Ronai Christina , Hamaoka-Okamoto Akiko , Stopp Christian , Newburger Jane W , Friedman Kevin
Published: July 2021

Background:
Coronary artery (CA) z-scores are commonly used for clinical decisions in Kawasaki disease (KD). We evaluated reliability in CA measurement, reproducibility of z-score calculation, and frequency with which different z-score formulas lead to divergent management strategies.

Methods:
We randomly selected 21 KD patients (pts) with ≥1 CA z-score 1.5-3 and all KD pts with ≥ 1 CA z-score 7-14 (n=20). Two echocardiographers measured LMCA, LAD and RCA. Inter- and intraobserver reliability were calculated. T-tests were used to compare CA z-score using 3 commonly used formulas (Boston, DC and Montreal).

Results:
Median age at KD echo was 1.2 y (0.2-11.5 y). Interobserver reliability was high for LAD (intraclass correlation [ICC] 0.970) and RCA (ICC 0.943) and lower for LMCA (ICC 0.725). Intraobserver reliability was also high for LAD and RCA (ICC 0.991 and 0.999) and lower for LMCA (ICC 0.946). Z-scores for the 3 formulas were similar at smaller CA size, i.e., z < 3, but varied markedly at larger CA dimensions (Figure). Z-scores for the same CA dimension calculated by each of the 3 formulas resulted in disparate classification of normal vs. mild dilation in 7/21 (22%) pts, and different guidance for anticoagulation based on CA z ≥10 in 10/20 (50%) pts.

Conclusion:
Although CA measurements have high inter- and intraobserver agreement, CA z-scores vary dramatically based on the z-score formula, particularly at larger CA dimensions. Discrepancies in CA z-score between calculators impacts not only the distinction between normal and mild dilatation, but most importantly, the recommendation of anticoagulation for pts with larger CA dimensions.
Abstract 179: Evaluation Of Coronary Artery Lesion In 9 Infantile Kawasaki Disease Patients Using 320row Area Detector Ct
Authors: Tsuzuki Yoshimitsu , Mizuno Masanori , Yousuke Osada , Nakano Marie , Sakurai Kenzo , Masumori Chikako , Arima Masaki , Gotou Kenzirou , Aso Kentarou
Published: July 2021

Introduction:
Recent advances of CT technology have made it possible to obtain clear coronary artery lesion (CAL) image. However, high dose radiation exposure is inevitable. Also it becomes difficult to obtain adequate images when patient’s heart rate is high. Recently 320row area detector CT (ADCT) has been introduced. Using this device, clear coronary artery images are obtainable with low dose radiation exposure. Also it provides adequate image for high heart rate patients. However, there is few report which describe its utility for infantile patients.

Aim:
To evaluate the feasibility of 320row area detector CT for evaluation of CAL for infantile KD patients.

Subjects and methods:
Nine infants who have CAL due to KD have been evaluated. Mean age at examination was 30.5+/-22.0 (mean+/-SD) months, weight was 13.6+/-6.5 kg and mean time elapse from diagnosis of KD was 18.7+/-16.0 months. Patients were evaluated using ADCT and findings obtained were compared with either coronary angiography (CAG) or echocardiography. Radiation exposure dose were also explored.

Results:
All patients have accomplished examination and adequate images were obtained. Mean heart rate at examination was 105+/-27 bpm. Examinations were conducted to two patients with breath holding and others with spontaneous breathing. Compared with CAG and echocardiography, similar results were obtained. Mean effective radiation dose was 6.14+/-2.22 mSv.

Conclusion:
Infantile KD patient’s CALs were able to be evaluated by ADCT with relatively low dose radiation exposure, without breath holding. ADCT have contributed to reduce the need for repeated invasive CAG.
Abstract 18: Seasonal Characteristic Of Kawasaki Disease With The Onset At The Different Seasons
Authors: Ozeki Yukie , Yamada Fumiya , Kishimoto Tsuyoshi , Nakamura Yosikazu
Published: July 2021

Background:
Kawasaki disease (KD) has been diagnosed in more than 60 countries. Seasonal trend of the patients with KD is observed in many countries. Little is known about the difference between the patient groups in the term of high incidence.

Objective:
The aim of this study was to characterize the two patient groups during the winter and summer seasons in Japan.

Methods:
Epidemiologic characteristics were compared between January and July, through the 22th nationwide survey included patients who visited hospitals during 2011-2012. The proportions of survey items were observed by age-adjustment.

Results:
The total number of patients during 2011-12 was 26,691, the number of patients was 2812 in January and 2302 in July, and the male/female ratio was 1.4, respectively. The patients <3 years account for 66% of each group (1854/2812, 1530/2302). The proportion of <8 months of age was 13.0% (365/2812) in January, 17.8% (409/2302) in July, that of 15 months to 3 years was 38.8% (1092/2812), and 33.5% (771/2302). Concurrence with the diagnostic guidelines was examined, the typical cases in July were 1.6% lower than in January, and the suspected cases were 1.3% higher than in January. The suspected cases of less than 4 principle symptoms had 5.3% higher than in January. The treatment cases with γ-globulin started within 5 days of illness were 2.2% less than in July. The cases of cardiac sequelae (giant aneurysm, aneurysm, dilatation, and stenosis) were 0.3% higher than in July. The cases of serum albumin concentration less than 3.2g/dL were 3.6% higher than in July. The percentage of occurrence cardiac sequelae in this class was 9.5% higher than in July.

Discussion:
The difference in age distribution in the two patient groups diagnosed in January and July was observed. The symptom had a slight difference, too. These result do not contradict a hypothesis that onset of KD is concerned with plural infections with different seasonal trend and targeting for the specific age. The association between low levels of serum albumin and cardiac sequelae has been known. The association between this class and the patients in the specific season will be clear in future epidemiologic study.

Conclusion:
This study clarified part of the characteristic of the patient diagnosed in the different seasons.
Abstract 184: An Atypical Presentation of Incomplete Kawasaki Disease
Authors: Briceno-Medina Mario , Perez Michael , Waller Benjamin R , Sathanandam Shyam
Published: July 2021

Background:
Kawasaki disease (KD) is a systemic panvasculitis that can cause coronary artery aneurysms (CAA) in up to 25% if left untreated. Rarely, aneurysms of other medium-sized arteries have also been reported to occur. The incidence of systemic artery aneurysms (SAA) with typical KD can be as high a 2.2%. Incomplete KD with SAA is not well described. We report a case of diffuse SAA in a 12-year-old boy with incomplete KD and giant CAA.

Case Presentation:
The patient presented with fever, malaise, abdominal pain, rash and cervical lymphadenopathy. Suspicion for the presence of a pericardial effusion on an abdominal CT scan prompted echocardiographic assessment. By echo, multiple giant CAA in all three coronary arteries was noted. Incidentally, on an aortogram after performing pericardiocentesis, it was noted that he had diffuse ectasia and aneurysms of every arterial branch off the aorta. Selective angiograms confirmed the presence of aneurysms in all medium-sized arteries throughout the body including pulmonary arteries. The patient was treated with IVIG, methylprednisolone and high dose aspirin. Incomplete KD was suspected. However, because of the systemic vasculitis, cyclophosphamide therapy was administered. Patient responded well to therapy with improvement in clinical symptoms. Anticoagulation with heparin was transitioned over to maintenance warfarin therapy. Patient remains asymptomatic 2 years later with large, but stable CAA.

Conclusions:
Incomplete KD can manifest with giant CAA and SAA. Immunosuppressant therapy may be necessary for systemic involvement. Anticoagulation is needed to prevent thromboembolic manifestations.
Abstract 187: Endothelial Cell Function in Young Adults with History of Kawasaki Disease
Authors: Daniels Lori B , Vejar Matthew , Kahn Andrew M , Gordon John B , Burns Jane C
Published: July 2021

Background:
The endothelium is a single layer of epithelial cells that lines the blood vessels and is a major component in maintaining vascular homeostasis. Impaired endothelial cell (EC) function can contribute to cardiovascular disease states, but whether there is an association between endothelial dysfunction and the presence of coronary artery aneurysms after Kawasaki disease (KD) is unknown.

Methods:
We enrolled 110 teens and young adults, ages 16 to 30 years, with a history of KD (61% male), as well as 30 healthy controls (HC, 53% male). EC function (reactive hyperemic response after a 5 minutes brachial artery occlusion) was measured using the EndoPAT 2000. Reactive hyperemia index (RHI) and PAT ratios were determined. KD subjects were categorized based on their worst coronary artery status (normal, transiently dilated/resolved aneurysm, aneurysm).

Results:
The mean age of KD subjects and HC at the time of EndoPAT was 25 ± 9 and 26 ± 7 years, respectively (p=0.42). Mean time since onset of KD was 23 ± 19 years. Subjects with a history of transiently dilated/resolved aneurysms had a higher mean lnRHI compared to other KD subjects (0.91 vs 0.74, p=0.01) and HC (0.72, p=0.02). When PAT ratio was evaluated as a function of time after occlusion release, those with transiently dilated/resolved aneurysms had a trend toward a more favorable profile while other groups appeared similar (Figure).

Conclusion:
Most young adults with a history of KD have normal EC function. Individuals with a history of transiently dilated coronary arteries had a paradoxical trend toward better EC function, which may indicate healthier vasculature. The significance of this finding merits further study.
Abstract 192: Long Term Coronary Angiography of Giant Coronary Aneurysms after Kawasaki Disease in Mexican Children
Authors: Garrido-Garcia Luis M , Colin Jose L , Bobadilla-Aguirre Alfredo
Published: July 2021

Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Coronary artery aneurysms (CAA), is one of the most important aspects of this disease. Some patients with KD develop giant coronary aneurysms (z-score > 10) and coronary stenosis, leading to ischemic heart disease.

Objective:
To determine the outcome of giant coronary artery lesions caused by KD and the value of coronary angiography in the evaluation and follow-up of coronary artery lesions in Mexican children.

Materials and Methods:
From our Institutional database, 34 patients (23 men and 11 women) who developed giant aneurysms from 1995 to December 2013 were identified. Information on patient demographics, catheter and surgical interventions, and most recent status was collected from medical records.

Results:
The average age at onset of KD was 13.5 months, and the median observational period was 70 mo. (5 to 163 mo.). During this period 11 patients showed CAA regression, 21 patients persist with CAA and 2 patients died at follow-up. In 9 patients with persistent giant CAA or coronary stenosis we performed cardiac catheterization to evaluate the coronary anatomy and findings of myocardial ischemia. Coronary bypass was performed to alleviate coronary ischemia in 1 patient, this patient developed dilated cardiomyopathy one year after the surgery and died. The overall survival rate in our series is 97%.

Conclusions:
Despite being a small series, the long-term survival of patients with KD complicated by giant coronary aneurysms in our center is relatively good. However further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions.
Abstract 196: The Characteristics of Patients with Kawasaki disease Presented with Fever and Cervical Lymphadenopathy at Admission
Authors: kim yeo hyang , Shin Chae Ok , Hyun Myung Chul , Lee Dong Seok
Published: July 2021

Purpose:
Of the principal diagnostic criteria of Kawasaki disease (KD), cervical lymphadenopathy is the least common. However, it may be misdiagnosed as bacterial cervical lymphadenitis. We evaluated the characteristics of patients with KD presenting with only fever and cervical lymphadenopathy at admission.

Methods:
This study enrolled patients diagnosed KD from January 2013 to May 2014. All of patients were divided to three groups: group 1 had only fever and cervical lymphadenopathy at admission; group 2 had typical manifestations with cervical lymphadenopathy; group 3 had typical manifestations without cervical lymphadenopathy.

Results:
Ninety eight patients (group 1 in 13, group 2 in 31, group 3 in 54) were examined. The median age of group 1 was significantly older than group 2 and 3 (
P
=0.001). The duration of fever before admission at our hospital was more prolonged in group 1 than in group 2 and 3 (
P
=0.001). In comparison between groups, the laboratory results at the admission day were not significantly different. However, group 1 showed significantly elevated white blood cell counts, elevated neutrophil counts, and decreased lymphocyte counts after first intravenous immunoglobulin administration (
P
=0.001,
P
=0.001, and
P
=0.003). The frequency of additional intravenous immunoglobulin treatment did not have significant difference. Group 1 had significantly increased duration of hospitalization, and frequency of second line treatment such as systemic steroid or infliximab than group 2 and 3 (
P
=0.000,
P
=0.024, and
P
=0.007). The development of a coronary artery dilatation (z score >2.5) was higher in group 1 than in group 3 (
P
=0.008).

Conclusions:
KD with cervical lymphadenopathy as main presentation indicates a severe form of KD associated with increased risks of second line treatment such as systemic steroid or infliximab and coronary artery dilatation. KD should be suspected in the older children with antibiotics non-responsive, prolonged fever and cervical lymphadenopathy. For differentiation between responder and non-responder for first line treatment, white blood cell counts and their subset after first intravenous immunoglobulin administration may be beneficial.
Abstract 200: Afebrile Kawasaki Disease Beyond Diagnostic Guideline Evolving Coronary Artery Complication
Authors: Lee Sun Hwa , Kim Na Yeon
Published: July 2021

Kawasaki disease (KD) is diagnosed with clinical features. Presence of at least five days of fever is major criteria. Herein we report a 7 month -old boy diagnosed as afebrile kawasaki disease who initially presented with inflammation at the Bacille Calmette-Guerin (BCG) inoculation site (BCGitis) with multiple erythematous papular rash, followed by desquamation of finger tips at 11 day of illness. Laboratory test showed elevated ALT (110 U/L) and otherwise showed no specific finding. Clinical feature disappeared spontaneously except BCGitis. The patient did not fulfill the diagnostic criteria but progressive coronary arterial dilatation was noticed. Left coronary artery (LMCA) was dilated from Z-score 1.6 (3 day of illness) to 2.8 (11 day of illness). After treated with intravenous immunoglobulin (2g/kg), BCGitis disappeared and follow up echocardiogram showed normalized LMCA lesion (Z-score 1.0 at 17 day of illness). BCGitis was considered to be pathognomonic feature of this patient. Diagnostic algorithm and guidelines are useful tool for the incomplete KD patients but clinicians should also be cautious for the patient with BCGitis even they are excluded by diagnostic guideline.
Abstract 203: Long-term Outcome Of Kawasaki Disease With Giant Coronary Aneurysms Which Waste Cardiac Follow-up; A Case Report
Authors: Mizuno Masanori , Chikada Masahide , Tsuzuki Yoshimitsu , Ono Hirokuni , Arima Masaki , Goto Kenjiro , Aso Kentaro
Published: July 2021

We report 2 cases of Kawasaki disease (KD) which required surgical interventions long after initial diagnosis. Case 1: Twenty-one years-old male. He was diagnosed as KD at the age of 1. Despite he received intravenous gamma-globulin therapy, bilateral giant coronary aneurysms were formed. In addition, multiple aneurysms include abdominal, iliac and axillary arteries were formed simultaneously. Coronary angiography (CAG) was thought to be dangerous because of iliac artery aneurysm formation. He received medical follow-up with aspirin administration only. He referred to our department at the age of 13. Ischemic changes were confirmed on both treadmill exercise test and cardiac scintigraphy. CAG showed 75% right coronary artery (RCA) stenosis and 99% left main trunk stenosis. Coronary artery bypass grafting (CABG) was performed and his postoperative course has been uneventful. Case 2: Thirty-seven years-old male. He was diagnosed as KD at the age of 1. Left giant coronary aneurysms were formed and subsequently aneurysm was occluded. Aspirin was administrated, but he interrupted medication and follow-up for himself. He referred to our institution due to cardiopulmonary arrest during exercise. After successful resuscitation, CAG revealed total occlusion of RCA and 99% left coronary artery stenosis. Emergent CABG was performed and no postoperative sequel has occurred. Both cases didn’t receive sufficient assessment during the follow-up period. As a result, fatal cardiac event was occurred in case 2. Coronary aneurysm tends to change into combine aneurysmal and stenotic lesion. Long time follow up is necessary even if patients have no symptom.

In addition, to decrease lost follow up patients, we should explain the risk of sudden cardiac death repeatedly and emphasis necessity of long term follow-up and medication.
Abstract 212: Acute Myocardial Infarction in the Acute Phase in Kawasaki Disease in Mexican Children.
Authors: Sarmiento-Robles William , Garrido-Garcia Luis M
Published: July 2021

Background:
Kawasaki disease (KD) is an acute febrile vasculitis of unknown origin. Despite treatment with intravenous immunoglobulin during the acute phase of the disease, up to 5% of those affected will develop coronary aneurysms predisposing them to thrombotic complications that could result in myocardial infarction (AMI). In Mexico there are few reports of ischemic complications secondary to KD.

Objective:
To describe the clinical features, the laboratory parameters, treatment used and the outcome of children who presented with myocardial infarction during the acute phase of KD in a third level facility in Mexico City

Methods:
From our Institutional Database of KD we search for children who presented AMI in the acute phase of the disease from August 1995 to August 2014. We analyzed gender, age, clinical manifestations, time from the onset of the symptoms to diagnosis, laboratory parameters, treatment used, and outcome in the acute phase of the disease.

Results:
Eight infants were diagnosed with AMI during the study period. The median age at diagnosis was 8 months (range 2 to 53 months). Seven patients were male (87.5%). The median from the onset of the clinical manifestations to diagnosis of KD was 22 days (range 4 to 26 days). All patients developed giant coronary aneurysms (median Z-score 18.98, with a range of Z-score from 11.58 - 27.70). An abnormal EKG and abnormal perfusion tests demonstrated the myocardial infarction in all cases. Two patients died in the acute phase of cardiogenic shock, one more patient died of dilated cardiomyopathy 12 months after coronary bypass surgery with an overall mortality of 62.5% of this group.

Conclusions:
AMI is a fatal complication of KD. In our small series it was associated with a delayed diagnosis of the disease and therefore the development of giant coronary aneurysms. Treatment of AMI in children after KD is a medical challenge with a poor prognosis in children.
Abstract 23: Trends in Kawasaki Disease Incidence - 2004-2014
Authors: Sexson Tejtel Sara K , Ramirez Andrea A , Liou Amy , Seery Thomas , Canter Debra , DeGuzman Marietta , Altman Carolyn
Published: July 2021

Background:
Kawasaki disease is the leading cause of acquired heart disease in children in the US. While it remains a rare disease, the purpose of this study is to evaluate the incidence of KD

Methods:
To evaluate our hypothesis we used the Pediatric Health Information Systems (PHIS), 2004-2013, including all discharges at 44 of the largest freestanding children’s hospitals in the US.

Results:
Between 2004-2013, there were 16,417 hospitalizations for KD, 10,388 (61%) were male. The race of patients admitted with KD showed 5276 (31%) Caucasian, 2370 (14%) African American, 1586 (9.3%) Other, and 941 (5.5%) Asian. The average length of stay 3.9 days. The number of patients with KD increased annually for the past decade, Figure 1.

The largest proportion of the increase was from those children between 1 and 5 years of age, Figure 2.

Most children were only hospitalized once (14,231, 84%) however some children required subsequent hospitalization (1,291,8%).

Conclusions:
There has been a steady increase in the number of admissions per year for Kawasaki disease predominately for those patients ages 1-5 years.
Abstract 43: Real Time Polymerase Chain Reaction Assays For Detection Of Respiratory Viruses In Kawasaki Disease Patients
Authors: Hong Young Mi , Lee Seul Bee , Choi Han Seul , Kwon Jung Hyun , Kim Hae Soon , Son Sejung
Published: July 2021

Purpose:
Respiratory symptoms are frequently observed in children with Kawasaki disease (KD) during the acute phase. The association rate of KD with antecedent respiratory illness has been reported to range from 56 to 83%. Clinical and epidemiologic features of KD support an infectious cause, but the etiology remains unknown. We investigated the association of respiratory viruses in children with KD using multiplex reverse transcriptase-polymerase chain reaction (RT-PCR).

Methods:
138 KD patients were enrolled from January 2010 to June 2013. Two study groups (Group 1; n=94, KD without respiratory symptoms, Group 2; n=44, KD with respiratory symptoms) were compared with a control group (Group 3; n=5, febrile patients with respiratory symptoms). Laboratory data were obtained from each patient including complete blood count (CBC), erythrocyte sedimentation rate (ESR), platelet count, alanine aminotransferase (ALT), aspartate aminotransferase (AST), serum total protein, albumin, C-reactive protein (CRP), NT-pro brain natriuretic peptide (BNP). Echocardiographic measurements were compared between the three groups. RT-PCR was performed using nasopharyngeal secretion to screen for the presence of 14 viruses (corona virus, parainfluenza virus 1, 2 and 3, influenza A and B, respiratory syncytial virus A and B, rhino virus A, B and C, metapneumo virus, adenovirus, and bocavirus) in groups 2 and 3.

Results:
The rate of KD with respiratory symptoms was 17.5%. The duration of fever was significantly longer and coronary artery diameter was significantly larger in group 2 than in group 1. Coronary artery diameter, CRP, platelet count, ALT, and NT-pro BNP were significantly higher and albumin lower in group 2 compared with group 3. Detection rate of adenovirus was 55.0% in group 2 and 28.6% in group 3.

Conclusion:
A positive RT-PCR for respiratory viruses may be helpful to elucidate the specific virus in KD patients with respiratory symptoms. NT-proBNP is a very important diagnostic tool in differentiating KD from other febrile viral respiratory infaction.
Abstract 49: Kawasaki Disease With Tsutsugamushi Disease: A Case Report
Authors: Song Min Seob
Published: July 2021

Background :
Clinical and epidemiologic features suggest infectious agents as a possible cause of Kawasaki disease; however, the etiology of Kawasaki disease still remains unknown. A number of microorganisms were hypothesized as an etiology of the illness. This is the first reported case of Kawasaki disease with tsutsugamushi disease.

Case presentation:
We report the case of a 4-year-old boy who presented with fever of 7 days duration and skin rash and bilateral conjunctival injection. He had a history of visiting a rural area with his grandmother. On admission, he had fever of 39.4 °C. His heart rate was 90/minute and his blood pressure was 90/60 mmHg. His pharynx was slightly injected and there was red lip. His neck was swollen with cervical lymphadenitis. He had erythematous macular rash on her trunk. Examination of his skin revealed an eschar on penile base of right scrotum. His laboratory results showed WBC 4,720/mm
3
, 42% polymorphonuclear leucocytes, 39% lymphocytes, hemoglobin 10.3 gm/dL, platelet count 148,000/mm
3
, CRP 3.23mg/dl, pro-BNP 316.5 pg/ml. The respiratory viruses using a multiplex real-time-PCR kit (Adenovirus, Influenza A, Influenza B, Metapneumovirus, Rhino A virus, Respiratory syncytiai virus, Parainfluenza ) were all negative. Mycoplasma pneumonia IgM was negative. R.tsutsugamushi Ab was positive. Echocardiographic findings 1 day after admission was mild dilatation of LCA (RCA=1.8mm, LCA=3mm). He was treated on oral roxithromycin for presumptive diagnosis of tsutsugamushi disease along with clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin and aspirin. Over the next 48 hours, he became afebrile and his rash improved. He was placed on low-dose aspirin for 8 weeks. His echocardiogram were within normal limit (RCA= 1.9mm, LCA= 2.7mm) at 2 months after the onset of his illness.

Conclusion:
This case report suggests that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.
Abstract 55: Histological And Rheological Evaluation At Coronary Artery Lesions In Patients With Kawasaki Disease Within 2 Years From Onset
Authors: Makoto Watanabe
Published: July 2021

Background:
Some paper reported that intravascular ultrasound (IVUS)- virtual histology (VH) showed atherogenesis in the evolution of coronary artery lesions (CAL) in young adults long after Kawasaki disease(KD). However, there is no report about those findings during early phase of KD.

Purpose:
We perform coronary intimal histologic evaluation by IVUS-VH for KD patients with CAL within two years from onset. Furthermore, we calculate shear stress in the target site and examine whether rheological potential affects to vascular histological change after KD.

Subjects and Methods:
IVUS-VH was performed in 12 Japanese KD patients (median age, 5.1 years) during 2 years after onset of KD (median, 10.2months) . All these patients had giant aneurysm in another branches. We investigated 20 coronary branches including 10 sites of small aneurysm (s-AN), 10 sites of regressed s-AN, and 20 sites of normal segment. Each of the 4 plaque components was assigned a respective color and defined as follows: fibrous area (green); fibro-fatty area (yellow) ; necrotic core area (red); and dense calcium area (white). Moreover, we measured average coronary peak flow velocity by Flow wire and calculated shear stress in the each sites.

Results:
10 sites of s-AN showed prominent endothelial hypertrophy with fibrous and/or fibro-fatty plaques. In 7 sites of these 10 sites, dense calcium and necrotic core localized which indicates early phase of atherosclerosis. 10 sites of regressed s-AN had circumferential endothelial hypertrophy occupying mainly fibrous and/or fibro-fatty plaques composition. In 8 sites of these regressed 10 sites, dense calcium and necrotic core locally existed. On the other hand, normal segment in 20 sites had no plaque in 19 sites and trivial plaque in 1 site. Moreover, shear stress in all evaluated VH sites were within normal limit, which shows rheological potential doesn’t affect to vascular remodeling in such coronary artery lesions.

Conclusions:
IVUS-VH study revealed that initial atherosclerotic findings locally existed not only at small aneurysm site but also at regressed site. Therefore, careful further investigation to vascular remodeling in KD patients with CAL including regressed s-AN will be need.
Abstract 57: Costimulation Mediated T-cell Survival Excerbates Kawasaki Disease
Authors: Parsaud Leon , Moolani Yasmin , Duong Trang T , Lau Andrew C , Keong Garrett T , Hoang Long T , Watts Tania H , Hibberd Martin L , Yeung Rae S
Published: July 2021

Introduction:
Kawasaki Disease (KD) is a multi-system vasculitis leading to coronary artery damage. Previous work has shown that co-stimulatory signals can rescue a subset of superantigen (SAg) reactive T-cells from apoptosis and one of the major pathways responsible for delivery of this co-stimulatory signal is CD28 signaling on T-cells.
Lactobacillus casei
cell wall extract (LCWE) contains a SAg among its active ingredients, leading to induction of coronary arteritis in mice that closely resembles human KD.

Methods:
Flow cytometry was used to measure the expression of pro-survival molecules and markers of apoptosis.
In vivo
studies were performed with C57BL/6 mice (4-5 weeks) injected i.p. with either LCWE, or LCWE and anti-4-1BB or Isotype control antibody. Cardiac tissue isolated, processed, stained and scored as per protocol. Gene expression analysis in KD patients was performed using the Illumina HumanHT-12v4.

Results:
Despite the fact that SAg-activated T-cells undergo apoptosis and are deleted, T-cells persist and are central to ongoing inflammation in affected arteries. Stimulation of CD28 leads to upregulation of pro-survival molecules cFLIP and BCLxL and reduction of caspase 3-annexinV double positive cells (markers of apoptosis) after SAg-stimulation, as detected by flow cytometry. In animals co-injected with anti-4-1BB (co-stimulation agonist), the incidence of coronary arteritis was dramatically increased to 94% compared to 54% with LCWE alone. Analysis of gene expression profile from 171 children with KD show elevated levels of molecules specific to the CD28 signaling cascade through Grb2, and VAV1 leading to the upregulation of Rac1 and CDC42, together with upregulation of pro-survival molecules cFLIP, MCL1 and NAIP. Interestingly, increased expression of cell survival molecules was associated with IVIG failure, with statistically significant elevation of NAIP and CDC42 and a trend towards increased expression for cFLIP and MCL, in IVIG non-responders compared to responders.

Conclusions:
Enhanced co-stimulation contributes to T-cell survival after SAg-stimulation leading to persistent coronary artery inflammation and poor treatment response in KD.
Abstract 68: Echocardiographic Evaluation of the Kawasaki Disease Mouse Model
Authors: Olson Aaron , Ledee Dolena , Kajimoto Hidemi , Bruce Margaret , Portman Michael
Published: July 2021

Background:
Mouse echocardiography is an established, non-invasive method to evaluate experimental cardiovascular disease. The murine Kawasaki disease (KD) model demonstrates aortic and coronary artery inflammation by histology. The clinical relevance of these pathological changes has not been confirmed. In children, KD involves coronary artery abnormalities visualized by echocardiography. However, echocardiography has not been previously applied to the mouse KD model. We hypothesized that coronary vasculitis caused the
lactobacillus casei
cell wall extract (LCE) causes abnormalities detectable by echo.

Methods:
Male mice (ages 3-4 months) were injected with LCE or vehicle. Sedated echos were performed with a VisualSonics Vevo 2100 system. Twelve mice (8 LCE and 4 vehicle) had pre-injection echos followed by serial studies for 6-10 weeks. Evaluations were blinded to treatment group. 2-D measurements were taken at consistent locations in the arteries. Aortic regurgitation was rated based upon clinical criteria. Histology was evaluated at 6 weeks post-LCE.

Results:
Mild aortic regurgitation (AR) was present in 6 of 8 mice injected with LCE starting at 1 week post-injection. No AR was present at baseline or vehicle injected mice. LCE mice showed increased aortic root diameter at 6 weeks compared to baseline (1.78mm±0.01 vs 1.52±0.02, p<0.05). Overall left coronary artery dimensions were not changed from baseline at 6 weeks post-LCE, but coronary imaging was difficult. One mouse had a diffusely enlarged left coronary system and severely diminished left ventricular function at 10 weeks post-LCE. Histology findings were present in all LCE-injected mice including aortic valvulitis, myointimal proliferation and a single case of infarction.

Conclusion:
The most prominent echo findings in the mouse KD model are severe aortitis with dilation and valvular regurgitation. These features could serve as non-invasive experimental measurements. Coronary dilation was a rare finding; however the coronaries are difficult to evaluate in mice. Overall, the mouse KD model demonstrates greater aortic pathology than found in human KD. Further studies are underway to evaluate additional non-invasive measurements such as vascular strain.
Abstract 71: Abdominal Aorta Dilatation and Aneurysm in Kawasaki Disease Vasculitis Mouse Model: Role of IL-1 Signaling
Authors: Wakita Daiko , Lee Youngho , Shimada Kenichi , Chen Shuang , Crother Timothy R , Lehman Thomas J , Fishbein Michael C , Arditi Moshe
Published: July 2021

Background:
Kawasaki disease (KD) is the most common cause of acute systemic vasculitis and acquired cardiac disease among US children. KD causes coronary artery aneurysms in up to 25% of untreated patients, and less frequently aneurysms in other systemic arteries including the abdominal aorta. Lactobacillus casei cell wall extract (LCWE)-induced KD mouse model mimics histopathologically the coronary artery lesions seen in KD patients.

Objective:
To evaluate the development of abdominal aorta dilatation and aneurysm in KD mouse model and investigate the role of IL-1 signaling.

Methods and Results:
We investigated the incidence and progression of abdominal aorta aneurysm (AAA) and dilatation in the KD model at 1, 2, 5 weeks. Over 80% of the mice developed significant dilation of abdominal aorta at 2 wks with progressively greater dilatation at 5 wks, with greater severity in males. KD mice showed fusiform and saccular AAA, which were always below the renal artery. Immunohistochemistry showed significant intimal proliferation, massive myofibroblastic proliferation that breaks the elastin layer, infiltration of large numbers of neutrophils and macrophages into the media and adventitia. IL-1R- or IL-1beta-deficient mice were completely protected from the KD associated abdominal aorta dilatation and AAA. IL-1R antagonist (Anakinra) significantly prevented the abdominal aorta dilatation and AAA (in addition to blocking coronary arteritis) in the KD mice.

Conclusions:
We report a new model of AAA and aortic dilatation in the LCWE-induced KD mouse model. These studies suggest that in children with KD the incidence of abdominal aortic dilatation and AAA maybe higher than currently appreciated, thus requiring prospective studies to determine the frequency of these vascular complications. Our findings also demonstrate that IL-1 plays an important role in development of LCWE-induced abdominal aortic lesions and blockade of IL-1 signaling may be a promising therapeutic target not only for KD vasculitis and coronary arteritis, but also for abdominal aorta dilatation and AAA associated with the disease.
Abstract 74: Coronary Artery Lesions in Children with Kawasaki’s Disease: A 10-Year Follow-up in Moscow
Authors: Bockeria Olga , Lyskina Galina , Shirinskaya Olga , Satyukova Anna , Gagarina Nina
Published: July 2021

Background:
Kawasaki’s disease (KD) is an acute systemic vasculitis in childhood. Lesion in the coronary arteries (CA) causes an acute or chronic ischemia, myocardial infarction and sudden death.

Materials and Methods:
The study included 189 children with KD for the period of 9 years (2004 - 2013). The age of the patients at the time of the acute stage of the disease was 1 month - 12 years 8 months . Boys and girls ratio was 1.8:1. 92% of the children were ill at the age of up to 5 years. Mean follow-up period was 15,8 months. We performed coronary angiography by CT scan in 14 children and by catheter in 9 children in CCVS. Echo was done in all 189 children.

Results:
Aneurysms of CA were found in 59 (31.2%) patients according to echocardiography data. The frequency of aneurysm formation in children first year of life was higher than in children older than 1 year (41.3% and 29.3%). Thirty eight (20%) patients had multiple aneurysms. Small aneurysms with a diameter less than 5 mm were found in 16.4% of patients, average (5 - 8 mm) - in 9%, giant (greater than 8 mm) - in 5.8% of patients. Transient CA ectasia was diagnosed in 13.2% of children.

Sensitivity of echocardiography in diagnosis of CA aneurysms was 88.5% in comparison with MSCT coronary angiography, specificity - 92.3%. Mean aneurysms' diameter measured by ECHO was 7,1 ± 3,3 mm, while CT contrast study revealed 7,3 ± 3,1 mm (p> 0.05).

We found an involution of 40.2% of aneurysms during follow-up.

Thrombi were found in 8 of 11 patients with giant aneurysms by echocardiography. Five thrombi disappeared after conservative therapy, 2 increased and occluded the RCA. CA stenosis greater than 75% was revealed in 2 patients with giant aneurysms in 2 years 7 months and in 3.5 months after manifestation of KD by using coronary arteriography.

Conclusion:
Kawasaki’s disease is a frequent cause of aneurysms, thrombosis and stenosis in coronary arteries in early childhood. Giant aneurysms often predispose to occlusion of the CA. Echocardiography is a highly specific and sensitive method for the visualization of CA aneurysms and thrombi. Other methods are useful when distinguishing stenosis and thrombosis in small CA is required.
Abstract 76: Clinical Usefulness Of Serum Procalcionin Level In Kawasaki Disease Children.
Authors: Choi Hee Joung , Kwon Tae Chan
Published: July 2021

Purpose:
Procalcitonin (PCT) is one of acute phase reactants such as C-reactive protein (CRP), and is likely to elevate in systemic inflammation, especially bacterial infection. As we know, the CRP level rise in the patients with high fever including Kawasaki disease (KD) and bacterial infection. So we investigated the clinical usefulness of serum PCT level in Kawasaki disease (KD), which is a systemic inflammation caused by vasculitis.

Method:
From August 2013 to June 2014, a total 336 patients were studied serum PCT level during hospitalization. We enrolled 41 patients of KD, 83 patients with viral infection, and 21 patients with bacterial infection.

Result:
The patients with KD had significantly higher mean age (24.4±18.1 months), mean body weight (12.1±3.9 Kg), and mean duration of fever prior to admission (4.4±1.8 days) than other patients (p<0.05). The serum PCT level, white blood cell (WBC) and platelet count, neutrophil proportion, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were significantly higher in KD patients than viral infection patients (p<0.05). Although not statistically significant, the serum PCT level was lower in KD patients than bacterial infection patients, whereas the CRP was higher in KD patients than bacterial infection patients. No significant difference in serum PCT level was showed between complete KD patients and incomplete KD patients. Also there was no significant difference in serum PCT level between responders to an initial intravenous immunoglobulin treatment and nonresponders.

Conclusion:
Serum PCT level may help to differentiate KD from viral infection, but we did not find a significant difference in PCT level between KD and bacterial infection. And the utility of PCT level as clinical marker in KD may be limited.
Abstract 79: Is Harada Score a Useful Tool in Mexican Childrens with Kawasaki Disease?
Authors: Flores-Pineda Angel J , Garcia-Rodriguez Fernando , Tomala-Haz Javier E , Lara-Herrera Paola B , Salinas-Encinas Diego R , Rodriguez-Garcia Andres N , Maldonado-Velazquez Maria R , Moreno-Espinosa Sarbelio , Faugier-Fuentes Enrique
Published: July 2021

Background/Purpose:
In developed countries Kawasaki disease (KD) is the most common cause of acquired heart disease in childhood. Haradascore is one of the most used predictive tool for coronary arteries aneurysms (CAA) development in KD. The aim of the study is to describe clinical data, treatment, and to assess the Harada score in Mexican patients with KD.

Methods:
We retrospectively indentified all patients evaluated at Hospital Infantil de Mexico Federico Gómez during a 6-yearperiod between 2008 and 2014. We reviewed demographic, clinical, laboratory, echocardiographic, and treatment data;and Harada scores were derived to evaluate efficacy in predicting risk for CAA.

Results:
In our Institution 34 cases were reported during the study period, 16 (47.1%) males, and median age of 36 (8 - 120)months. Incomplete KD was diagnosed in 6 (17.6%) cases with no significant differences in demographic data, but with alower accuracy in establishing diagnosis at admission compared with complete presentations (100% vs 66.7%, P = 0.027).Most of the patients (76.5%) received at least one antibiotic prior to admission to our Hospital.

Patients presented with history of 6 (4 - 16) days with fever and conjunctivitis. Significant differences were observed between complete and incomplete presentations regarding oral changes (100% vs 66.7%, P = .027), strawberry tongue (92.9% vs 33.3%, P = .004), pharyngitis (100% vs 33.3%, P < .001), and rash (71.4% vs 16.7%, P = .021). No significant differences were observed in laboratory results. Seven (20.6%) echocardiograms werereported with CAA, one of them found valve insufficiency, and none identified effusion. Harada score was positive in 23 (67.6%) patients, with high sensitivity (85.7%) and low specificity (37%), OR 1.36 (CI95% 0.89 - 2.06, P = .384).

In total, 32 (94.1%) patients received intravenous immunoglobulin (IVIG). Retreatment with IVIG was given in 3 (8.8%) and steroid treatment in 1 (2.9%) patient. All of the patients received aspirin.

Conclusions:
This study revealed a high incidence of CAA in our population, added to lack of suspicion and antibiotic overuse. As in Japanese and US population, Harada score is a good screening tool to identify risk for CAA development in Mexican patients.
Abstract 92: Evaluation Of The Ascending Aorta Stifness Following Kawasaki Disease In Compraison With Systemic Hypertension
Authors: Maurice Roch Listz , Nandlall Ian , Fournier Anne , Dahdah Nagib
Published: July 2021

Background:
Kawasaki Disease (KD) is a systemic vasculitis that affects large and medium-size arteries. The coronary arteries draw most of the clinical attention whereas few studies have taken interest in involvement of other vessels. Using an imaging-based mechanical biomarker (ImBioMark), we measured the ascending aorta (AA) stiffness in pediatric KD subjects compared to healthy controls (CTL) and patients with systemic hypertension (HTN).

Methods:
We evaluated AA (Fig. 1a) in 20 CTL, 12 KD subjects (4 with and 8 without CA aneurysms). 8 children with HTN represented a comparative clinical model of vascular stiffness. We measured systolic and diastolic AA strains using ImBioMark. Strain was tested for normality against height for CTL. Strain values from KD and HTN subjects were normalized (Z-score) according to height. Comparisons were performed between groups using non-parametric statistics.

Results:
Systolic and diastolic pressures were normal in KD subjects (49.8±28.4 and 46.1±6.9 percentile, respectively). Age was similar between KD (9.1±5.3 yo) and HTN (9.9±5.3 yo), p=NS. Fig. 1b exhibits a strain curve computed with ImBioMark. Systolic and diastolic strain values were normally distributed against height in CTL. HTN subjects had abnormal systolic and diastolic strain values. KD subjects had normal diastolic strain, but significantly abnormal systolic strain (Fig. 2). There was no statistically significant difference in strain, based on the presence or absence of CA aneurysm.

Conclusion:
Despite normal blood pressure, AA in KD exhibited reduced strain during systole. This may reflect in situ rigidity of the AA.