Involvement In Kawasaki Disease Research Articles

Relationship between ocular manifestations, laboratory findings, echocardiographic findings, and intravenous immunoglobulin resistance in Kawasaki disease.

This study investigates the incidence of ocular involvement in Kawasaki disease (KD) and evaluates the relationship between ocular manifestations, laboratory findings, echocardiographic findings, and intravenous immunoglobulin (IVIG) resistance. We conducted a cross-sectional study with 58 KD patients from June 2021 to March 2023. For all patients, a complete ophthalmologic examination and echocardiography were performed in the acute phase before starting the treatment. We analyzed the age, sex, mean of white blood cell (WBC) count, platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST), echocardiographic findings and IVIG responses for all patients and compared the group with ocular involvement with the group without involvement. The incidence of bilateral acute conjunctivitis was 70.7%, while that of acute uveitis was 30%. Patients with uveitis had significantly higher rates of Coronary artery dilatation and IVIG resistance, as well as higher mean levels of WBC, platelet, and CRP compared to those without uveitis. (P < 0.05). Additionally, the age of patients with uveitis involvement was lower than those without involvement. No significant relationships existed between ESR, AST, or ALT values and uveitis (P > 0.05). Furthermore, no significant correlations existed between any examined items and acute bilateral conjunctivitis. Uveitis in KD is significantly associated with coronary artery dilatation, IVIG resistance, higher WBC count, platelet count, and CRP level.

Coronary external diameter index for assessing coronary artery involvement in Kawasaki disease

Aim: Transthoracic echocardiography is commonly used to assess coronary artery dilatation in Kawasaki disease (KD). However, existing criteria often miss early abnormalities. This study examines the utility of a new parameter, coronary external diameter index (CEDi), for early diagnosis and monitoring in KD. Methods: CEDi of left main (LM) and right coronary artery (RCA), calculated as the ratio of coronary artery external diameter (i.e., the distance between the outer coronary edges measured in the proximal segment of the artery) and the diameter of the aortic annulus, was evaluated in 34 patients (age 23 mouths ± 13 months) with KD at the hospital admission and after 2 weeks and 8 weeks of treatment. The control group consisted of 210 healthy children aged 20 months ± 13.4 months. Z-score charts for LM and RCA coronary external diameter (CED) were obtained. Results: Compared with controls, KD patients had a markedly higher mean value of LM CEDi (0.53 ± 0.06 vs. 0.33 ± 0.04; P &lt; 0.0001) and RCA CEDi (0.48 ± 0.05 vs. 0.31 ± 0.04; P &lt; 0.0001) at hospital admission. By ROC analysis, LM CEDi of 0.41, and RCA coronary artery thickness index (CATi) of 0.39 were the best cut-offs to confirm the clinical diagnosis of KD, both exhibiting 100% sensitivity and specificity. Mean LM CEDi and RCA CEDi values decreased significantly (P &lt; 0.0001) after 2 weeks of follow-up and were similar to controls (P = 0.53 and P = 0.12, respectively) 8 weeks after admission. Conclusions: In patients with KD, CEDi of LM and RCA is an accurate parameter to evaluate coronary artery involvement in the early phase of the illness and during follow-up.

Cathelicidin (LL-37) causes expression of inflammatory factors in coronary artery endothelial cells of Kawasaki disease by activating TLR4-NF-κB-NLRP3 signaling.

Kawasaki disease (KD) is a type of vasculitis with an unidentified etiology. Cathelicidin (LL-37) may be involved in the development of the KD process; therefore, further research to investigate the molecular mechanism of LL-37 involvement in KD is warranted. Enzyme-linked immunosorbent assay (ELISA) was used to detect the levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-1β, NLRP3, and LL-37 in the sera of healthy subjects, children with KD, and children with pneumonia. Subsequently, human recombinant LL-37 or/and toll-like receptors 4 (TLR4)-specific inhibitor TAK-242 stimulated human coronary artery endothelial cells (HCAECs), CCK-8 was used to detect cell proliferation, flow cytometry to detect apoptosis, transmission electron microscopy to observe cytoskeletal changes, Transwell to measure cell migration ability, ELISA to detect inflammatory factor levels, Western blot analysis to analyze protein levels of toll-like receptors 4 (TLR4) and NF-κB p-65, and quantitative real-time polymerase chain reaction (qRT-PCR) to determine LL-37, NLRP3 mRNA levels. In this study, we found that the level of LL-37 was highly expressed in the serum of children with KD, and after LL-37 stimulation, apoptosis was significantly increased in HCAECs, and the expression levels of TLR4, NLRP3 and inflammatory factors in cells were significantly enhanced. Intervention with the TLR4-specific inhibitor TAK-242 significantly alleviated the LL-37 effects on cellular inflammation, TLR4, NLRP3 promotion effect. Our data suggest that LL-37 induces an inflammatory response in KD coronary endothelial cells via TLR4-NF-κB-NLRP3, providing a potential target for the treatment of KD.

E45 Kawasaki syndrome-related heart disease before and during the COVID-19 pandemic

Abstract Introduction Kawasaki disease (kDa) is an acute febrile systemic panvascularitis that affects medium-caliber arteries with a strong predilection for the coronary arteries. It is the leading cause of acquired heart disease in children under 5 years of age. However, after the COVID-19 pandemic, we see a change in the profile of cardiac involvement. Objective To describe the clinical, paraclinical, and evolutionary characteristics of cardiac damage in children managed for Kawasaki or Kawasaki-Like syndrome before and during the Covid19 pandemic. Materials and Methods This is a retrospective, descriptive study conducted over a period of five years (1st January 2017 to 31st December 2021). Result We collected 30 cases of patients hospitalized with Kawasaki disease, between January 2017 and December 2021: 11 cases with Kawasaki syndrome before COVID-19; and 19 cases with Kawasaki syndrome after COVID-19. The mean age and standard deviation (SD) at diagnosis was 42 (±37.8) months before the pandemic vs 81 (±34.7) months after the pandemic. The sex ratio was 0.5 (4 girls and 7 boys) before the pandemic vs 0.7 (8 girls and 11 boys) after the pandemic. Heart damage was found in 2 cases before the pandemic (coronary aneurysm type) vs 3 cases after the pandemic, mainly cardiogenic shock. The mean time from the first transthoracic ultrasound to fever was 11.3 days before the pandemic, vs 5.8 days after the pandemic. Transthoracic ultrasound showed 2 cases of coronary aneurysm before the pandemic, with a Z score exceeding 2.5SD; vs 7 cases of heart failure after the pandemic with a decrease in left ventricular ejection fraction. The ASAI score (a score that assesses the risk of coronary artery disease) was &amp;gt;6 in 1 case before the pandemic vs in 0 cases after the pandemic. The average predictive score for cardiac involvement was 4.2 (±1.4) before the pandemic, vs 3.5 (±1.5) after the pandemic. The outcome was favorable in all cases despite a more severe picture after the COVID-19 pandemic. Conclusion The change in the pattern of cardiac involvement in Kawasaki disease following the emergence of COVID-19 must be taken into account in order to manage an influx of patients with severe Kawasaki disease.

Neurological manifestations of Kawasaki disease and multisystem inflammatory syndrome in children associated with COVID-19: A comparison of two different clinical entities.

Kawasaki disease (KD) is one of the most frequent idiopathic vasculitis in children, affecting medium- and small-sized vessels. Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 has recently emerged as a new systemic hyperinflammatory condition affecting children some weeks after an acute COVID-19 infection. KD and MIS-C share different aspects and differ in many others: patients affected by MIS-C are usually older, with prominent gastrointestinal manifestations, diffuse adenopathy, extensive conjunctivitis, myocardial damage, leukopenia, and thrombocytopenia at the laboratory exams. Both conditions can present neurological complications. The aim of this manuscript is to provide a narrative review of neurological involvement in KD and MIS-C. A comprehensive review literature has been performed, and the main clinical features have been analyzed, contributing to neurological differential diagnosis.

Cardiovascular Compromise in Pediatrics in the Times of COVID-19: SARS-CoV-2 Infection and Kawasaki Disease

Introduction: The pandemic caused by the new SARS-CoV-2 virus has reported an increase in morbidity and mortality worldwide during the years 2020 to 2022. Many pediatric infections have been reported as mild. The cardiovascular complications due to COVID-19 described in the literature are mainly in adults, however, reports in the pediatric population have been less frequent. Objective: To describe the clinical characteristics and evolution of patients treated in two centers with cardio-pediatric care services who presented cardiovascular involvement during the COVID-19 pandemic related to the presence of SARS-CoV-2 infection and Kawasaki disease (KD). Materials and Methods: Through a descriptive, retrospective study, the medical records, electrocardiographic and echocardiographic studies were reviewed in both groups of patients between May 2020 and May 2022 at the National Institute of Child Health and Alberto Sabogal Hospital. None of the cases had a history of previous structural heart disease. Results: The patients studied were 31 in total, with 21 cases of COVID-19 (SARS-CoV-2 infection) and 10 of KD. The female sex predominated, with an average age of 6.2 years (COVID-19) and 2.9 years (Kawasaki). In the echocardiogram, mild pericardial effusion was the most common finding. Coronary alterations were found in 60% of patients with KD and in only 18% of COVID-19 cases. We found 15 patients who met the criteria for the so-called multisystem syndrome (MIS-C) among COVID-19 cases, 5 of them with hemodynamic compromise. Conclusion: During the COVID-19 pandemic, the clinical picture in both groups: SARS-CoV-2 infection and KD presented some similar characteristics, mainly in relation to coronary involvement (greater involvement in KD), and in the evolution, a greater hemodynamic compromise was evidenced in cases of SARS-CoV-2 infection but without associated mortality.

PS-C05-8: ERYTHROCYTE SEDIMENTATION RATE LEVELS AND ITS ASSOCIATION WITH CORONARY ARTERY INVOLVEMENT IN KAWASAKI DISEASE

Objectives: The general objective of this study is to determine association of ESR with cardiovascular involvement in Kawasaki Disease (KD) patients. Specific Objectives: Design and method: This is a cross sectional study conducted at National Childrens Hospital (NCH), a tertiary pediatric hospital. Data was gathered using charts from the medical records of patients diagnosed with KD. Study population included patients diagnosed with KD with classical presentation from year 2013–2018. The diagnostic criteria for KD require presence of fever for five days or more and at least four of five of the following: bilateral conjunctival infection without exudates, polymorphous exanthema, strawberry tongue, desquamation of palms and soles, and cervical lymphadenopathy. ESR levels were recorded both as a numerical variable and categorical variable based on the level (below 80 mm/hr, more than 80 mm/hr). Patients with coronary artery involvement included perivascular brightness, coronary artery dilatation, or coronary aneurysm on two-dimensional echocardiography done during admission for KD. Descriptive statistics was done using frequency and percentages for categorical variables, and standard deviations for continuous variables was calculated to describe the profile of the respondents. Determination of the association between ESR and CAD among KD patients was analyzed using Chi square test and stratified analysis. Odds ratio and the 95% confidence interval was also calculated. Significance was set at p value of &lt; 0.05. Results: Of the 116 patients, 72.4% of the population were noted to have cardiac involvement and 50% had coronary artery involvement. The most common coronary artery involvement was on the proximal right coronary artery. Moreover, pericardial effusion and valvular regurgitation mostly involving the mitral valve were the most common cardiac involvement. In the population, 61 (52.6%) were male, and 26 (42.6%) of which developed CAD however no correlation between sex and CAD was revealed. Conclusion: This study showed no correlation between the level of ESR and the development of CAD. It is not recommended to obtain ESR in patients who qualify as complete KD. Hence the study does not support the practice of obtaining ESR upon admission of KD patients. Further studies on the association of ESR with CAD with higher sample size and adjusting for other confounding variables are needed.

Abstract 11464: Spectrum and Associated Factors for Coronary Artery Involvement in Kawasaki Disease versus Multisystem Inflammatory Syndrome in Children Associated With Covid-19

Introduction: Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 share clinical features and are both associated with coronary artery (CA) involvement. Methods: From January 2020 through January 2022, n=2566 contemporaneous KD, MIS-C and acute COVID-19 pediatric patients from 39 sites in 8 countries were enrolled into the International KD Registry. The study population was confined to 875 MIS-C patients meeting CDC criteria with confirmed or probable COVID-19 infection, and 492 KD patients meeting AHA guideline criteria without COVID-19 infection who had sufficient echo data. CA involvement was defined by maximum Z score in any branch at any timepoint (maxCAZ). Associated factors for each diagnosis were determined with multivariable logistic regression for maxCAZ&gt;2. Results: Median maxCAZ was higher for KD (+1.43) vs MIS-C patients (+1.33; p=0.004). The groups did not differ regarding the incidence of maxCAZ&gt;2 (KD 26% vs MIS-C 24%; p=0.42), although MIS-C patients had less severe Z score categories of involvement (FIGURE). In univariate analyses, higher peak troponin I (p&lt;0.05) and NTproBNP (p=0.06) were associated with maxCAZ&gt;2 for MIS-C but not KD patients. Lower LV ejection fraction was likewise associated with maxCAZ&gt;2 for MIS-C (p=0.009) but not KD patients. For KD patients, independent factors associated with maxCAZ&gt;2 were male sex, age &amp;lt;6 months, greater total days of fever, shock presentation, and higher peak platelet count and CRP (c-statistic 0.69). In contrast, for MIS-C patients the only independent factor associated with maxCAZ&gt;2 was male sex (c-statistic 0.56). Conclusions: Compared to KD patients, MIS-C patients have a similar incidence but lesser severity of CA involvement with few associated factors, male sex being the only one in common. CA involvement was significantly associated with higher cardiac biomarkers and lower LV ejection fraction for MIS-C patients only.

Humoral cross-coronavirus responses against the S2 region in children with Kawasaki disease

Multisystem Inflammatory Syndrome in Children (MIS-C), a post infectious complication of SARS CoV-2 infection, shares enough features with Kawasaki Disease (KD) that some have hypothesized cross-coronavirus (CoV) immunity may explain the shared pathology. Recent studies have shown that humoral cross-reactivity of the CoVs, particularly of OC43, is focused on the S2 region of the Spike protein.Due to efforts utilizing CoV S2 regions to produce a cross-CoV vaccine, we wished to assess SARS-CoV-2 S2 reactivity in children with KD and assess if cardiac involvement in KD correlated with S2 CoV antibody targeting. The presence of cross-reactivity does not distinguish KD from febrile controls and does not correlate with cardiac involvement in KD. These findings support that, in relation to cardiac vascular inflammation, vaccines targeting the S2 region appear to be a safe approach, but there is disparity in the ability of CoV species to raise cross-reactive S2 targeted antibodies.

A decision tree model for predicting intravenous immunoglobulin resistance and coronary artery involvement in Kawasaki disease

ObjectivesThis study aims to develop a new algorithm for predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement in Kawasaki disease (KD) through decision tree models.MethodsMedical records of children hospitalized for KD were analysed retrospectively. We compared the clinical characteristics, and the laboratory data in the groups with IVIG resistance and coronary artery dilatations (CADs) in KD patients. The decision tree models were developed to predict IVIG resistance and CADs.ResultsA total 896 patients (511 males and 385 females; 1 month-12 years) were eligible. IVIG resistance was identified in 111 (12.3%) patients, and CADs were found in 156 (17.4%). Total bilirubin and nitrogen terminal- pro-brain natriuretic peptide (NT-proBNP) were significantly higher in IVIG resistant group than in IVIG responsive group (0.62 ± 0.8 mg/dL vs 1.38 ± 1.4 mg/dL and 1231 ± 2136 pg/mL vs 2425 ± 4459 mL, respectively, P < 0.01). Also, CADs were more developed in the resistant group (39/111; 14.9% vs. 117/785; 35.1%, P < 0.01). The decision tree for predicting IVIG resistance was classified based on total bilirubin (0.7 mg/mL, 1.46 mg/dL) and NT-proBNP (1561 pg/mL), consisting of two layers and four nodes, with 86.2% training accuracy and 90.5% evaluation accuracy. The Receiver Operating Characteristic (ROC) evaluated the predictive ability of the decision tree, and the area under the curve (AUC) (0.834; 95% confidence interval, 0.675–0.973; P < 0.05) showed relatively higher accuracy. The group with CADs had significantly higher total bilirubin and NT-proBNP levels than the control group (0.64 ± 0.82 mg/dL vs 1.04 ± 1.14 mg/dL and 1192 ± 2049 pg/mL vs 2268 ± 4136 pg/mL, respectively, P < 0.01). The decision trees for predicting CADs were classified into two nodes based on NT-proBNP (789 pg/mL) alone, with 83.5% training accuracy and 90.3% evaluation accuracy.ConclusionA new algorithm decision tree model presents for predicting IVIG resistance and CADs in KD, confirming the usefulness of NT-proBNP as a predictor of KD.

Is There a Role for Laboratory Parameters in Predicting Coronary Artery Involvement in Kawasaki Disease?

Kawasaki disease (KD) may cause cardiac and coronary complications. Since definite markers to accurately predict coronary involvement is not present, we aimed to analyze the role of hematological indices [neutrophil-to lymphocyte ratio (NLR), platelet-to lymphocyte ratio (PLR), lymphocyte-to monocyte ratio (LMR), and mean platelet volume (MPV)-to lymphocyte ratio (MPVLR)], prognostic nutritional index (PNI) and systemic immune-inflammation index (SII) in predicting coronary involvement of KD. Patients The medical records of 134 KD patients admitted between January 2008 and December 2019 were investigated. Also, 268 age-matched healthy controls (HCs) were included in the study. KD patients were divided into two groups: KD with coronary artery lesions (KD-CALs) and KD without CALs. Logistic regression analysis was performed to determine parameters that may predict coronary involvement in children with KD. Among KD patients, 39 (29.1%) had CALs. When compared with HCs, the median levels of WBC, neutrophils, monocytes, eosinophils, platelets, MPV and, the values of NLR, PLR, MPVLR, SII were significantly higher; whereas lymphocyte count, PNI, platelet distribution width (PDW), LMR were markedly lower in the KD group (p˂0.001 for all, except for p=0.010 for eosinophil count). The CALs group's SII, PLR, and PNI values were significantly lower than those without (p=0.030, p=0.032, and p ˂0.001; respectively). Multivariable regression analysis revealed that PNI, SII, and gender (male) were associated with CALs in KD. Our analysis revealed that male sex, lower PNI, and lower SII levels were independently associated with CALs in children with KD.

Analysis of multiple organ involvement in Kawasaki disease

This study aimed to explore the incidence and clinical characteristics of multiple complications in children with Kawasaki disease (KD). All patients were diagnosed in our hospital between January 2016 and December 2020. A total of 640 cases were included, 43 patients had coronary artery aneurysm, 51 patients had coronary artery dilation, and 546 patients had no coronary artery damage. The patients were divided into three subgroups based on age: < 1year old, 1-5years old, and > 5years old. The multiple complications of all the KD children and the correlation between extracardiac complications and cardiovascular complications were analyzed. Among the 640 KD children, most were 1-5years old (415/640, 64.8%). Children < 1year old (31.6%) and > 5years old (28.3%) were more likely to have cardiovascular complications. The incidence of respiratory complications was highest in 1-5year olds (57.1%). Involvement of the digestive and the hematological systems gradually decreased with age, whereas involvement of the nervous system, the urinary system, and the joints gradually increased with age. The incidence of cardiovascular injury with extracardiac complications (22.3%) was higher than that without extracardiac complications (16.3%), although the difference was not statistically significant (p > 0.05).Conclusions: KD can be complicated by multiple-organ injury but there was no significant relationship between the occurrence of extracardiac complications and cardiovascular complications. What is Known: • Cardiovascular disease is generally believed to be the most common and serious complication of Kawasaki disease (KD). • Over recent years, we have found that extracardiovascular complications of KD are more common than generally supposed and, because they have some effect on prognosis, we believe that more attention should be paid to these complications. What is New: • There was no significant relationship between the occurrence of extracardiac complications and cardiovascular complications.

Neurological involvement in Kawasaki disease: a retrospective study

BackgroundKawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children. Neurological involvement is a known complication of KD, however, its association with KD severity remains elusive. We aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs).MethodsWe retrospectively reviewed data from 1582 patients with KD between January 2013 and December 2017. Profiles of patients with neurological symptoms (group A, n = 80) were compared to those of gender- and admission date-matched patients without neurological involvement (group B, n = 512). Multivariate logistic regression analyses were performed to determine whether neurological involvement was significantly associated with IVIG resistance.ResultsNeurological involvement was observed in 5.1% (80/1582) of patients with KD. The neurological manifestations were diffuse, presenting as headache (13/80, 16.3%), convulsions (14/80, 17.5%), somnolence (40/80, 50.1%), extreme irritability (21/80, 26.3%), signs of meningeal irritation (15/80, 18.8%), bulging fontanelles (7/80, 8.8%), and facial palsy (1/80, 1.3%). Neurological symptoms represented the initial and/or predominant manifestation in 47.5% (38/80) of patients with KD. The incidence of IVIG resistance and levels of inflammatory markers were higher in group A than in group B. However, neurological involvement was not an independent risk factor for IVIG resistance or CALs.ConclusionRates of neurological involvement were relatively low in patients with KD. Neurological involvement was associated with an increased risk of IVIG resistance and severe inflammatory burden. Our results highlight the need for pediatricians to recognize KD with neurological involvement and the importance of standard IVIG therapy.Trial registrationRetrospectively registered.

ACUTE RESPIRATORY DISTRESS SYNDROME UNFOLDING AS ATYPICAL KAWASAKI DISEASE

TYPE: Abstract Publication TOPIC: Pulmonary Manifestations of Systemic Disease PURPOSE: Kawasaki disease (KD) is a medium vessel multisystemic vasculitis and is the leading cause of acquired heart disease among children in developed countries. Pulmonary involvement in KD is uncommon. Here we present a case of atypical KD, with acute respiratory distress syndrome (ARDS) as presenting manifestation. METHODS: A 7-year old, previously healthy boy presented with fever for 5 days, rash since day 2 of illness and difficulty in breathing for 2 days. On examination, severe respiratory distress, desquamating macular rash over face, neck, chest and minimal conjunctival congestion were noted. X-ray chest with findings of diffuse bilateral opacities and oxygenation index of 11 on blood gas analysis were suggestive of ARDS. The child required mechanical ventilation for respiratory failure. RESULTS: Serum electrolytes, hemogram, liver and renal function tests were within normal limits. Blood culture came sterile and workup for tropical fevers came negative. On day 15 of illness, child developed periungual peeling. Repeat investigations showed hemoglobin of 82g/L, total leucocyte count 18.5x109/L, platelets 707x109/L, ESR 55 mm in 1sthour and CRP 27mg/dL. Diagnosis of atypical KD was considered and 2g/kg intravenous immunoglobulin was given. Clinical and laboratory parameters showed a trend towards normalization and child was extubated 60 hours later. No coronary artery abnormality was noted on echocardiography. Child was discharged on low-dose aspirin and is doing well on follow-up. CONCLUSIONS: ARDS is an extremely unusual initial presentation of KD. CLINICAL IMPLICATIONS: A high index of suspicion for KD must be kept in atypical pulmonary presentations to prevent delay in diagnosis and development of sequelae. DISCLOSURE: No significant relationships. KEYWORDS: Kawasaki disease, Acute respiratory distress syndrome, Intravenous immunoglobulin

Cardiac Biomarkers for Early Detection of Cardiac Involvement in Children with Kawasaki Disease: A Cross-Sectional Study

Background: Kawasaki disease (KD) is one of the most prevalent vasculitis diseases in children and can bring about serious cardiovascular complications. Early detection of cardiac involvement in KD can play an essential role in managing and preventing the cardiac sequels. We aimed to evaluate the applicability and diagnostic accuracy of cardiac biomarkers including cardiac troponin I (cTnI), and N-terminal pro-brain natriuretic (NT-proBNP) for early detection of cardiac involvement in children with KD. Materials and Methods In this cross sectional study, 32 children with KD who were admitted to the Children's Hospital of Tabriz University of Medical Sciences, Tabriz, Iran, in a three-year period, were consecutively included. Transthoracic echocardiography was performed to evaluate cardiac involvement. Also, the serum levels of NT-proBNP and cTnI were measured to evaluate their diagnostic accuracy. ROC curve analysis was conducted to evaluate the discriminatory power of NT-ProBNP for the diagnosis of cardiac involvement in KD and to determine the best cut-off point at which the sensitivity and specificity were optimal. Results: Of 32 enrolled patients, 4 (12.5%) had cardiac involvement including 3 patients with perivascular brightness of coronary arteries and one patient with small aneurysm of the coronary arteries. In all study patients, the cTnI levels were lower than 0.35 ng/ml and the NT-proBNP mesurment revealed an average of 678.5 pg/ml. Children with cardiac involvement had significantly higher NT-proBNP (p= 0.001). Both sensitivity and specificity of NT-proBNP at the optimum cut-off point of 1354 pg/ml were 100 percent (AUC=1.000, p=0.001). Conclusion: Unlike the cTnI, our results support the applicability of NT-proBNP as an excellent objective test for early detection of cardiac involvement in children with KD.

Delta-neutrophil index: a potential predictor of coronary artery involvement in Kawasaki disease by retrospective analysis.

DNI is the immature granulocyte fraction provided by a blood cell analyzer, which is determined by subtracting the fraction of mature polymorphonuclear leukocytes from the sum of myeloperoxidase-reactive cells. We aimed to evaluate the role of Delta-neutrophil index (DNI) in cardiac prognosis prediction in children with Kawasaki disease (KD). Medical records of 193 patients were retrospectively reviewed. The values of DNI, white blood cells, erythrocyte sedimentation rate, the percent of polymorphonuclear leucocytes, C-reactive protein, aspartate transaminase, alanine aminotransferase, total bilirubin data of children with KD were analyzed. Also, sex and age of children were compared. The value of DNI was higher in children with cardiac complications [median 0.8 (0-0.26) vs 5.3 (3.55-8.95); P < 0.001]. The ROC curves showed that DNI was a better predictor of cardiac complications than other parameters. The best cutoff value for DNI to predict cardiac complications was 5.55% with sensitivity of 80% and specificity of 82% (AUC 0.883, 95% confidence interval [CI] 0.807-0.959, P < 0.05). DNI could serve as a facile and useful marker to predict cardiac complications in children with KD, as it is included in a routine complete blood count.

Severe Lip Involvement in Kawasaki Disease: A Clinical Oddity.

Severe Lip Involvement in Kawasaki Disease: A Clinical Oddity.

Understanding the importance of cerebrovascular involvement in Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

Role of Kobayashi Risk Scoring for Determining Refractory Kawasaki Disease

Background: Coronary complications are common in children with Kawasaki disease (KD). Administration of intravenous immunoglobulin (IVIG) during the first 10 days will result in lower coronary complications. It is useful to predict if a patient will develop coronary complications or not. Objectives: We designed this study to evaluate the role of Kobayashi risk scoring for determining refractory KD. Methods: Medical records of all cases with KD were reviewed and demographic data, previous history, coronary artery involvement, laboratory findings, and days with fever were extracted for all cases. Kobayashi score was calculated. Results: A total of 168 cases enrolled. There was no significant difference regarding Kobayashi score in different groups regarding coronary involvement. In cases without coronary involvement, 93.5% were classified as low risk, in one and two vessels involvement groups, all were categorized as low risk, and among three vessels involvement cases, 75% were known as low risk. Conclusions: Kobayashi score is not useful for predicting severity of coronary involvement in Kawasaki disease.

Abdominal-pain-first presentation of Kawasaki disease

Clinicians from Italy cared for a child with high fever and abdominal pain as first complaints of Kawasaki disease. Abdominal computed tomography showed concentric thickening of jejunal bowel, dilatation of small and large intestine, ascitic fluid, and enlargement of mesenteric lymph nodes. As typical mucocutaneous signs and laboratory findings appeared, the diagnosis was considered. IGIV therapy was associated with rapid defervescence and resolution of abdominal pain. The authors reviewed 48 published cases of Kawasaki disease with intestinal involvement. Fever, abdominal pain, and vomiting generally were first or early manifestations, before appearance of mucocutaneous findings. Pseudo-obstruction was present in >80% of cases by plain radiograph or another imaging study. Hydrops of the gallbladder was not common. Kawasaki disease was not an early consideration and 25% of patients underwent surgery. Coronary artery aneurysms were present in 43% of cases at the time of Kawasaki disease diagnosis. Symptoms resolved following IGIV therapy. Authors of published cases did not report intestinal sequelae (but follow up was variable and usually was short). This editor has cared for a very young infant with Kawasaki disease before the IGIV therapy era who had bowel obstruction due to jejunal stenosis 2 months after the acute phase. Compilation of 57 cases of lymph-node-first presentation of Kawasaki disease was reported in The Journal in 2013 (J Pediatr 2013;162:1259-63).1 We now add abdominal-pain-first presentation. It should not be too surprising that intestinal vasculitis could be significant in some cases. Might it not suggest an intestinal portal of microbe or super antigen entry, as might cervical lymphadenitis a respiratory tract portal of entry? The 43% rate of coronary artery aneurysms at the time of diagnosis in cases of abdominal-pain-first Kawasaki disease in the literature likely represents both diagnosis and reporting biases. However, it behooves us all to consider Kawasaki disease in the differential when a child has high fever and abdominal pain. Article page 186 ▶ Intestinal Involvement in Kawasaki DiseaseThe Journal of PediatricsVol. 202PreviewTo describe a case of Kawasaki disease with intestinal involvement and to analyze other published reports to define clinical characteristics, diagnostic issues, and therapeutic approaches of gastrointestinal involvement in Kawasaki disease. Full-Text PDF