scholarly journals Coronary Aneurysm and IVIG Resistance Prediction in Kawasaki Disease

2020 ◽  
Author(s):  
Yusuf Ziya Varlı ◽  
Kazim Oztarhan

Abstract Background: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. This study aimed to determine the clinical characteristics, demographic features, frequency of coronary involvement, and resistance to intravenous immunoglobulin (IVIG) treatment in Turkey based on our data.Methods: Patients with KD were evaluated with demographic data, clinical, laboratory, and echocardiographic findings. Results: Between 2010–2019, a total of 259 patients (male/female: 1.67) were treated in our hospital, with 48 (%19) cases < 1 year of age. According to diagnostic criteria, 31% were diagnosed with typical KD and 69% with atypical (incomplete) KD. The frequency of clinical findings were as follows: changes in the lips and oral mucosa (79%); polymorphic rash (69%); conjunctivitis (65%); changes in the extremities (54%); and cervical lymphadenopathy (48%). There was no significant difference between typical and atypical KD in the frequency order. CAA development and IVIG resistance occurred in 11.6% and 12.3% of cases, respectively. IVIG resistance was more common in infants and hospitalization times were longer in this group. Coronary artery lesions existed in 45 patients; right coronary artery (RCA) alone (20%), left coronary artery (LCA) alone (44.5%), and RCA and LCA together were involved (35.5%). The left main coronary artery affected 20 patients, the left anterior descending artery (LAD) affected nine patients (45%), the left circumflex artery (LCx) affected two patients (10%), and the LAD and LCx together affected two patients (10%). None of the patients had myocardial infarctions or died during follow-up. Conclusion: KD is a systemic vasculitis common in pediatric infants in which coronary artery involvement affects prognosis. Due to IVIG resistance and increased coronary involvement accompanying this vasculitis, it is an important problem in countries where the disease is common. It is important to know the factors that increase the risk of coronary involvement and IVIG resistance development.

2018 ◽  
Vol 23 (suppl_1) ◽  
pp. e29-e30
Author(s):  
Steffany Poupart ◽  
Audrey Dionne ◽  
Cathie-Kim Le ◽  
Léamarie Meloche-Dumas ◽  
Jean Turgeon ◽  
...  

Abstract BACKGROUND Kawasaki disease (KD) is a paediatric systemic vasculitis that can be associated with concomitant viral or bacterial infections. Patients with persistent or recurrent fever 36 hours after the end of intravenous immunoglobulin (IVIG) are considered to be resistant to treatment and are at increased risk for coronary complications. OBJECTIVES However, it is unknown how concomitant infection influences the response to IVIG treatment. The aim of this study was to determine the impact of concurrent infection on the prevalence of IVIG resistance and coronary outcome. DESIGN/METHODS Retrospective study of 154 children (mean age at diagnosis: 3.4 ± 2.8 years) diagnosed with KD, between 2008–2016 in a tertiary paediatric university hospital, of which 59 (38%) had concomitant infection. RESULTS Delay in diagnosis (>10 days of fever) was similar between patients with and without concomitant infection (7% vs 7%, p=0.89). Patients with concomitant infection were more likely to have fever 48 hours after initial treatment (36% vs 20%, p=0.05) and to be treated with a second dose of IVIG (33% vs 18%, p=0.04). Patients with infection had higher C-reactive protein at the time of diagnosis (148 vs 112 mg/L, p=0.04), which persisted after IVIG administration (111 vs 59 mg/L at 48 hours, p=0.003). However, there was no statistically significant difference in the prevalence of coronary artery (CA) complications (coronary artery Z-score > 2.5) between patients with and without concomitant infection (36% vs 39%, p=0.68). CONCLUSION Children with KD and concomitant infection are more likely to have persistent fever and elevated inflammatory markers after treatment requiring a second dose of IVIG. Nevertheless, this is not associated with an increased risk of CA complications. Larger scale studies are needed to help distinguish IVIG resistance from infection in children with persistent fever and guide management of this population. Table/Chart or graphic upload


2021 ◽  
Vol 12 ◽  
Author(s):  
Toshiyuki Kitoh ◽  
Tsuyoshi Ohara ◽  
Taichiro Muto ◽  
Akihisa Okumura ◽  
Reizo Baba ◽  
...  

Kawasaki disease (KD) is a febrile disease of childhood characterized by systemic vasculitis that can lead to coronary artery lesions (CAL). This was a prospective cohort study to determine the levels of the pentraxin 3 (PTX3), soluble CD24-Subtype (Presepsin) and N-terminal pro-brain natriuretic peptide (NT-pro BNP) in consecutive KD patients. From January 2013 to March 2015, all patients with KD admitted to Aichi Medical University Hospital who provided consent had their plasma saved before IVIG administration. In total, 97 cases were registered. 22 cases of incomplete KD were excluded from the outcome analysis. The total 75 cases were used for statistical analyses. A PTX3 threshold of &gt;7.92 ng/ml provided a specificity of 88.5 %, a sensitivity of 94.4 %, and a likelihood ratio as high as 15.92 for the diagnosis of KD compared with febrile non-KD controls. Although an echocardiographic diagnosis of CAL in the early course of the disease was confirmed in 24 cases, it was not in the remaining 51 cases. Neither NT-proBNP nor Presepsin had statistical significance for the prediction of the echocardiographic CAL diagnosis. Only PTX3 was significantly predictive of the echocardiographic CAL diagnosis (p=0.01). The PTX3 level was significantly higher in the intravenous immunoglobulin (IVIG) non-responders (45.9±7.45) than in the IVIG responders (17.0 ± 1.46 ng/ml) (p&lt; 0.001). The PTX3 level also correlated with the number of IVIG treatment courses needed to resolve fever (R² =0.64). Persistent CAL (pCAL) formation was observed in three cases; one of aneurysm only and two aneurysms with dilatations. The patients with pCAL had significantly higher PTX3 levels (85 ± 8.4 ng/ml) than patients without pCAL (22 ± 2.2 ng/ml) (p&lt; 0.0001). In terms of pCAL prediction, the area under the curve (AUC) of receiver operating characteristic ROC curve of PTX3 was 0.99, and it was significantly greater than that of Presepsin (0.67) or NT-proBNP (0.75). PTX3 is a soluble pattern recognition molecule that acts as a main component of the innate immune system. These data suggest that PTX3 can be utilized as a definitive biomarker for the prediction of IVIG resistance and subsequent CAL formation in patients with KD.


Rheumatology ◽  
2018 ◽  
Vol 58 (5) ◽  
pp. 770-775 ◽  
Author(s):  
Jong Gyun Ahn ◽  
Yoonsun Bae ◽  
Dongjik Shin ◽  
Jiho Nam ◽  
Kyu Yeun Kim ◽  
...  

Abstract Objectives Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that affects infants and young children. Recent reports of elevated serum high mobility group box 1 (HMGB1) level during the acute phase of KD and its relationship to poor response to IVIG treatment suggest a possible association of HMGB1 polymorphisms with KD. We investigated the association between the polymorphisms of the HMGB1 gene, KD susceptibility, coronary artery lesions, and KD response to IVIG treatment. Methods Whole genome sequencing of the HMGB1 gene was performed to identify causative variants. Two tagging single nucleotide polymorphisms of the HMGB1 gene were selected using linkage disequilibrium analysis. The tagging single nucleotide polymorphisms were genotyped using the TaqMan Allelic Discrimination assay in a total of 468 subjects (265 KD patients and 203 controls). Results The HMGB1 single nucleotide polymorphisms were not associated with KD susceptibility. However, in KD patients, there was a significant association of rs1412125 with coronary artery lesions formation in the recessive model (GG vs AA + GA: odds ratio = 4.98, 95% CI = 1.69–14.66, P = 0.005). In addition, rs1412125 was associated with IVIG resistance in the recessive (GG vs AA + GA: odds ratio = 4.11, 95% CI = 1.38–12.23, P = 0.017) and allelic models (G vs A: odds ratio = 1.80, 95% CI = 1.06–3.06, P = 0.027). Conclusion The rs1412125 in HMGB1 might be a risk factor for the development of coronary artery lesions and IVIG resistance in KD patients.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
lijian xie ◽  
Cuizhen Zhou ◽  
Renjian Wang ◽  
Tingting Xiao ◽  
Jie Shen ◽  
...  

Introduction: The incidence of Kawasaki disease (KD) in China is increasing for years. The current coronary artery lesion (CAL) incidence is 5-10% in KD with intravenous immunoglobulin (IVIG) treatment. And the 10-20% KD patients still exhibit IVIG resistance. However, little clinical evidence on the occurrence of either CAL or IVIG resistance for big KD sample study in China during the past decade. Objective: In order to find clinical risk factors of CAL and IVIG resistance of KD in China. Methods: We retrospectively analyzed the clinical manifestations, laboratory results, treatment and complications of cardiac vascular of 602 KD cases from 2007 to 2012 admitted at Shanghai Children’s Hospital. The SAS 9.2 edition was used for statistical analysis. The mean ± standard deviation or the median were used for measurements. Case numbers and percentages were used for the count number. The t-test and the Mann-Whitney test were both used for mean comparisons. Single factor and multi-factor logistic regression analyses were used to analyze the risk factors. Results: 1. The KD gender male to female ratio was 1.85: 1. The KD median age was 2.0 years old (one month to 11.7 years old). 20.1% cases (121 of 602) exhibited CAL. There was no difference of CAL incidence between the gender (p=0.09). 2. The incidence of bright red cracked lips (p=0.001), peeling of the skin of the toes (p=0.021) and perianal skin peeling (p=0.031) are less in group with CAL. 3. Among the 602 cases, there were 525 cases that were sensitive to IVIG therapy. 100 of those cases had CAL with an incidence of 19.1%. Among the 26 IVIG resistance cases, there were 9 cases with CAL with an incidence of 34.6%, which was higher than the IVIG sensitive group (p=0.05). 4. ESR (p=0.014), CRP (p=0.017), PLT (p=0.003) and Hb (p=0.032) were much higher in the IVIG resistance group than the IVIG sensitive group, even though the IVIG resistance group started the IVIG treatment earlier (p=0.003). 5. Logistic regression analysis was conducted to show that GPT≥80IU/L was the independent risk factor of IVIG resistance, risk ratio was 2.945 (p=0.012) . Conclusion: This research suggests that risk factors of clinical evidence for IVIG resistance and CAL in KD.


2014 ◽  
Vol 25 (6) ◽  
pp. 1124-1129 ◽  
Author(s):  
Jalaj Garg ◽  
Parasuram Krishnamoorthy ◽  
Chandrasekar Palaniswamy ◽  
Rajiv Paudel ◽  
Saurav Chatterjee ◽  
...  

AbstractBackground: Accelerated coronary atherosclerosis in patients with Kawasaki disease, in conjunction with coronary artery aneurysm and stenosis that characterise this disease, are potential risk factors for developing coronary artery disease in young adults. We aimed to determine the prevalence and predictors of coronary artery disease in adult patients with Kawasaki disease. Methods: All patients aged 18−55 years of age diagnosed with Kawasaki disease were sampled from Nationwide Inpatient Sample database using International Classification of Diseases 9th revision (ICD 9 code 446.1) from 2009 to 2010. Demographics, prevalence of coronary artery disease, and other traditional risk factors in adult patients with Kawasaki disease were analysed using ICD 9 codes. Results: The prevalence of Kawasaki disease among adults was 0.0005% (n=215) of all in-hospital admissions in United States. The mean age was 27.3 years with women (27.6 years) older than men (27.1 years). Traditional risk factors were hypertension (21%), hyperlipidaemia (15.6%), diabetes (11.5%), tobacco use (8.8%), and obesity (8.8%), with no significant difference between men and women. Coronary artery disease (32.4%), however, was more prevalent in men (44.7%) than in women (12.1%; p=0.03). In multivariate regression analysis, after adjusting for demographics and traditional risk factors, hypertension (OR=13.2, p=0.03) was an independent risk factor of coronary artery disease. Conclusion: There was increased preponderance of coronary artery disease in men with Kawasaki disease. On multivariate analysis, hypertension was found to be the only independent predictor of coronary artery disease in this population after adjusting for other risk factors.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kenji Suda ◽  
Hiroshi Nishino ◽  
Yoshiyuki Kudo ◽  
Hironaga Yoshimoto ◽  
Shintaro Kishimoto ◽  
...  

Objective: We report four patients with incomplete Kawasaki disease (KD) successfully treated by antibiotics without intravenous immunoglobulin (IVIG) treatment with ≦ 5 days of fever but left with coronary artery lesion. Result: The patients were 2 babies and 2 young children age ranged from 2 months to 2 years old and 9 months. They showed fever and other signs of KD, but did not fulfill diagnostic criteria. The numbers of symptoms compatible with KD were 2 or 3 and included conjunctival injection, oral or lip injection, and eruption. Within 5 days, they became afebrile by antibiotics without IVIG treatment. However, they were noted to have coronary artery aneurysm (CAA) on day from 7 to 33 of illness. All patients had been placed on antithrombotic treatment including aspirin or aspirin plus warfarin and, fortunately, showed regression of CAA within 2 years. Conclusions: This case series indicates that there are patients with incomplete KD successfully treated by antibiotics without IVIG but left with CAA. These cases must be underdiagnosed if intentional echocardiographic examination are scheduled and might be left without appropriate treatment, antithrombotic treatment. Further collection of data of these patients is indispensable.


2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tracey Dyer ◽  
Paul Dancey ◽  
John Martin ◽  
Suryakant Shah

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis can be made in a patient who presents with a prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes (including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervical lymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present. We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations for elevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed with atypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treated with high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol were added due to the presence of giant aneurysms.


2020 ◽  
Vol 8 (B) ◽  
pp. 99-103
Author(s):  
Nguyen Huu Son ◽  
Tran Kiem Hao ◽  
Nguyen Thi Hoang Anh

INTRODUCTION: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology which affects mainly children <5 years of age. If the disease is left untreated, it can lead to serious complications such as inflammation of the blood vessels. AIM: We aim to evaluate the clinical and laboratory findings and response to therapy of KD at Hue Central Hospital. METHODS: This is a retrospective study of patients with KD at Pediatric Center of Hue Central Hospital between January 2010 and December 2019. Clinical and laboratory examinations as well as the echocardiograms finding were analyzed. RESULTS: All patients were under 5 years old, in which boys were more than girls. Fever lasting over 5 days, changing in the mouth mucosa, and peripheral extremities were seen in all patients. About 73.2% had bilateral conjunctivitis and 78.0% had rash. About 42.3% of patients had cervical lymphadenopathy. Laboratory findings were noted with 84.5% of patients had hyperleukocytosis (>12,000/ mm3), 76.2% of patients had high serum C-reactive protein (CRP) levels (>100 mg/dl), 56% of patients had erythrocyte sediment rate >60 mm in the 1st h, and 34.5% of patients had thrombocytosis (platelet count >500,000/mm3) at the time of diagnosis. About 26.2% of patients had coronary artery lesions. Most patients (84.4%) had good outcome since the first dose of gamma-globulin and 13% of patients needed the second dose. There was a significant correlation between coronary artery abnormalities and no or late treatment of gamma-globulin. CONCLUSION: KD was very common in children under 5 years old with the high rate of coronary artery lesion. Treatment with gamma-globulin on or before 10 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.


2020 ◽  
Author(s):  
Hyo Soon An ◽  
Gi-Beom Kim ◽  
Mi Kyoung Song ◽  
Sang Yun Lee ◽  
Hye Won Kwon ◽  
...  

Abstract BackgroundThis study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels. MethodsThis retrospective analysis was based on the nationwide survey of KD conducted in the Republic of Korea between 2015 and 2017. We enrolled 9131 patients and defined low (<3 mg/dL) and high (≥3 mg/dL) CRP groups. Demographic data, clinical characteristics, z-scores, and scores based on the Japanese criteria for CAL were compared between the two groups. Logistic regression analysis was used to identify CAL risk factors.ResultsThe low CRP group accounted for 23% of patients. A significant difference was observed for the mean age at diagnosis (high vs. low CRP, 34.4 ± 24.9 vs. 31.7 ± 24.8 months, p<0.001) and fever duration (high vs. low CRP, 6.6 ± 2.2 vs. 6.3 ± 2.5 days, p<0.001). A non-response to intravenous immunoglobulin treatment was found in 1377 patients (20.1%) and 225 patients (11.7%) in the high and low CRP groups, respectively (p<0.001). CAL were found in 12.9% and 18.3% of the high and low CRP patients, respectively (p<0.001), based on z-scores; and in 9.9% and 12.5%, respectively (p = 0.001), based on the Japanese criteria in the acute phase. The giant coronary artery aneurysm occurrence ratio was similar between groups (p=1.0).ConclusionsCAL occurred in patients with both high and low CRP. Therefore, patients with KD should be carefully monitored regardless of their CRP levels.


2021 ◽  
Author(s):  
jing zhang ◽  
Haobo Weng ◽  
Qiongfei Pei ◽  
Penghui Yang ◽  
Wentao Fan ◽  
...  

Abstract Background: Kawasaki disease (KD) is an acute, systemic vasculitis of unknown etiology that occurs predominantly in infants and children, and the most crucial complication of KD is coronary artery aneurysm (CAA). Tumor necrosis factor (TNF)-like protein 1A (TL1A) is a member of the TNF superfamily, which possesses the ability of maintaining vascular homeostasis and regulating immune response. This study aims to examine the serum TL1A levels in KD patients, and to investigate the relationship between TL1A and CAAs in children with KD.Methods: Blood samples were recruited from 119 KD patients, 35 febrile controls (FCs) and 37 healthy controls (HCs). The KD group was further divided into KD with CAAs (KD-CAAs) and KD non-CAAs (KD-NCAAs) groups. Serum TL1A levels were measured using enzyme-linked immunosorbent assays, and clinical parameters were collected in KD patients. Results: Serum TL1A levels in the acute phase of KD patients were significantly higher than that in the FC and HC groups. In particular, serum TL1A were substantially increased in the KD-CAA group than that in the KD-NCAA group. Furthermore, TL1A levels were positively correlated with the duration of fever, time point of IVIG and WBC levels, but negatively correlated with levels of RBC, Hb and Albumin in the KD group. Conclusions: TL1A might be involved in the KD-associated vasculitis, and might be a factor in the development process of CAAs.


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