Complete Kawasaki Disease Research Articles

Kawasaki Disease: A Single-Center Study from a Tertiary Care hospital in Dhaka, Bangladesh

Background: Kawasaki Disease (KD) is recognized as the leading cause of acquired heart disease in children in developed countries. This is a pioneer study from Bangabandhu Sheikh Mujib Medical University (BSMMU), documenting the common epidemiological, clinical, laboratory and echocardiographic findings, including management and immediate outcome of KD children. Method: This retrospective study includes all patients with a diagnosis of KD, who attended the pediatric rheumatology division at BSMMU from January 2015 to December 2019. Data were collected from medical records at diagnosis and immediately after management. Relevant statistical tests were done and a p value < 0.05 was considered as statistically significant. Result: A total of 129 cases were diagnosed with KD, among them complete KD cases were 62% and incomplete KD was 38%. The mean age at onset was significantly higher in incomplete KD than complete KD. The majority of the cases (67%) were between 1 to 5 years of age and male (72%) children were predominant. Fever was the most common presenting feature (100%), followed by bilateral conjunctivitis, mucosal involvement, rash, changes in extremities, cervical lymphadenopathy and others. Coronary artery dilatation was present in 30.5% cases with no significant differences between complete and incomplete KD cases. Aspirin was given to all the cases and IVIG was given to only 46.6% children. Rests of the children were treated with methyl prednisolone. All the cases improved after treatment and there was no immediate mortality. Conclusion: It seems that KD is not an uncommon disease and incomplete KD cases are relatively more prevalent. About 30% KD cases had coronary artery abnormalities. Immediate outcome of management is excellent but needs long term follow-up. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 9-16

Bilateral facial nerve palsy complicating Kawasaki disease: A case report and literature review.

Kawasaki disease (KD) manifests as an acute, self-limited vasculitis disease that constitutes the primary cause of acquired heart disease in children under 5 years of age. Facial nerve palsy (FNP) is a rare complication associated with coronary artery lesions (CALs) in patients with KD. Patients with KD and FNP usually present atypically, leading to a delayed diagnosis and treatment of KD. A 4-month-old boy with fever, left FNP and bilateral conjunctival injection with spontaneous resolution, was admitted to the hospital, received a short course of intravenous dexamethasone, and experienced rapid FNP recovery on the first admission. The patient experienced a resurgence of fever, bilateral conjunctival injection, and right FNP, which led to readmission. Physical examination revealed redness at the site of Bacillus Calmette-Guérin inoculation, reddening of lips, and desquamation of the distal extremities. Echocardiography revealed right-sided CALs. The patient initially missed KD on the first admission, and was later diagnosed with complete KD with FNP on the second admission. After a short course of intravenous dexamethasone, the left FNP resolved quickly. However, right FNP recurred after corticosteroids withdrawal. Meanwhile, more typical symptoms were observed, and KD was diagnosed. Treatment ensued with intravenous immunoglobulin (IVIG), aspirin, and dexamethasone. The patient achieved rapid remission, without recurrence. Echocardiography continued to show normal findings during 1-year follow-up after discharge. The clinical symptoms of FNP complicating KD in children are atypical and can easily lead to delayed diagnosis and treatment. FNP in patients with KD may serve as a risk factor for CALs, which are more challenging to resolve than the FNP itself. Adding corticosteroids to IVIG may be recommended to reduce IVIG resistance, decrease the risk of developing CALs, and alleviate CALs.

The changes of coagulation profiles in Kawasaki disease and its associations with clinical classification, intravenous immunoglobulin responsiveness and coronary artery involvement

Coagulation disorders are common in Kawasaki disease (KD). The main objectives of the present study were to probe the associations of coagulation profiles with clinical classification, IVIG responsiveness, coronary artery abnormalities (CAAs) in the acute episode of KD. A total of 313 KD children were recruited and divided into six subgroups, including complete KD (n = 217), incomplete KD (n = 96), IVIG-responsive KD (n = 293), IVIG-nonresponsive KD (n = 20), coronary artery noninvolvement KD (n = 284) and coronary artery involvement KD (n = 29). Blood samples were collected within 24-h pre-IVIG therapy and 48-h post-IVIG therapy. Coagulation profiles, conventional inflammatory mediators and blood cell counts were detected. Echocardiography was performed during the period from 2- to 14-day post-IVIG infusion. In addition, 315 sex- and age-matched healthy children were enrolled as the controls. (1) Before IVIG therapy, coagulation disorders were more prone to appear in KD patients than in healthy controls, and could be overcome by IVIG therapy. FIB and DD significantly increased in the acute phase of KD, whereas reduced to normal levels after IVIG therapy. (2) PT and APTT were significantly longer in patients with complete KD when compared with their incomplete counterparts after IVIG therapy. (3) The larger δDD, δFDP and the smaller δPT, δINR predicted IVIG nonresponsiveness. (4) The higher δDD and δFDP correlated with a higher risk for CAAs (DD: r = −0.72, FDP: r = −0.54). Coagulation disorders are correlated with complete phenotype, IVIG nonresponsiveness and CAA occurrence in the acute episode of KD, and can be rectified by synergistic effects of IVIG and aspirin.

Clinico-Laboratory Profile and Outcome of Kawasaki Disease in Children in a Tertiary Care Hospital in Kathmandu

Introduction: Kawasaki disease (KD) is one of the most common vasculitis of childhood. It contributes significantly to acquired heart disease in children and results in coronary artery abnormalities including aneurysm in 25% of untreated children. This study aims to retrospectively assess the clinical course, laboratory results, and presenting symptoms of patients with complete and incomplete KD treated at tertiary care hospital in Kathmandu.Method: This retrospective study was conducted in the Department of Pediatrics. Data from January 2020 to December 2020 were retrieved from medical records department of the hospital and analyzed using Microsoft excel.Result: The total number of cases evaluated in our study was 9. The median age at the time of the diagnosis of KD was 24 months (median age for incomplete KD-21 months, complete KD-37 months). Male to female ratio was 1.2:1. The peak season of KD occurrence was Spring (March – May). Mean duration of stay in the hospital was 7 days. All children presented with fever, with an mean fever duration of 7 days at the time of presentation. Fever was present in every case. The most common principal symptom was a polymorphous skin rash, observed in 88% of the cases. Elevated CRP levels (≥3 mg/dl) were noted in all 9 cases. Cardiovascular complications were present in 88% of the patients, with coronary artery aneurysm (CAA) detected in 11%, coronary artery dilation in 22%, and mild pericardial effusion documented in 66% of the cases. Eight patients (88%) received IVIG, all 9 patients (100%) received aspirin, and 2 patients received steroids. There were no fatalities.Conclusion: KD is a common vasculitis in children which do not have significant sex predilection in oppose to most of the studies. Majority of cases occurred in spring season. The most common symptom was polymorphous rash. A high level of suspicion for diagnosing KD is essential, and early treatment is crucial to prevent complications and reduce the future economic burden on families and the nation.

Comparison of Characteristics and Outcomes of Multisystem Inflammatory Syndrome and Prepandemic Kawasaki's Disease

Abstract Objective In this study, our objective is to compare the demographic, clinical, laboratory, and echocardiographic findings of patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki's disease (KD) diagnosed in the prepandemic period. Methods We retrospectively collected data from all pediatric patients who met the Centers for Disease Control and Prevention's MIS-C case definition and who met the American Heart Association's definition of complete KD before the coronavirus disease 2019 pandemic. Results A total of 37 patients diagnosed with MIS-C and 40 patients diagnosed with complete KD were included. Gastrointestinal findings were significantly higher in the MIS-C group than in the KD group (vomiting [p = 0.009], diarrhea [p = 0.009]). The incidence of thrombocytopenia (48.6%) was significantly higher in the MIS-C group. Regarding inflammatory markers, procalcitonin and ferritin were significantly higher in the MIS-C group (p = 0.032 and p = 0.006) and the erythrocyte sedimentation rate was higher in the KD group (p < 0.001). Pericardial effusion and mitral valve regurgitation were significantly more frequent in the MIS-C group (p = 0.024 and p = 0.001). Conclusion Although they have similar findings, our current study findings show that MIS-C and KD differ from each other with different clinical and laboratory features. We think that these differences will help clinicians in diagnosis and patient management.

The role of serum lipid in predicting coronary artery lesions and intravenous immunoglobulin resistance in Kawasaki disease: a cohort study.

To assess the predictive value of the serum lipid profile for initial intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs) in patients with Kawasaki disease (KD). This retrospective cohort study enrolled patients with KD and divided them into IVIG-responsive and IVIG-resistant groups. They were also stratified based on the presence of CALs (CALs and non-CALs groups). Clinical, echocardiographic and biochemical values were evaluated. A subgroup analysis was performed on complete and incomplete KD. Predictors of initial IVIG resistance and CALs were determined by multivariate logistic regression analysis. A total of 649 KD patients were enrolled: 151 had CALs and 76 had initial IVIG resistance. Low-density lipoprotein cholesterol (LDL-C) was significantly lower in the IVIG-resistant group than in the IVIG-responsive group. LDL-C and apolipoprotein (Apo) B were significantly lower in the CALs group compared with the non-CALs group. Multivariate logistic regression failed to identify the serum lipid profile (LDL-C, Apo A or Apo B) as an independent risk factor for initial IVIG resistance or CALs in KD patients. KD patients might have dyslipidaemia in the acute phase, but the serum lipid profile might not be suitable as a single predictor for initial IVIG resistance or CALs.

Retrospective evaluation of 130 cases with kawasaki disease follow-up in a tertiary care center in Turkey between 1999 and 2019: a 20-year experience

ABSTRACT Objectives Kawasaki disease (KD), which is a medium vessel vasculitis, is common in Asian countries and is the most common cause of childhood-acquired heart diseases in developed countries. However, disease course and epidemiological data are limited in non-Asian developing countries like ours. We aimed to evaluate the clinical features and prognosis of patients with KD in our country and ethnicity, one of the referee centers of our country. Methods Patients with KD in our center for the last 20 years in the pre-COVID-19 pandemic era were included in the study. The clinical and laboratory findings, treatments, and follow-up findings were reviewed retrospectively in different age groups. Results Of the 130 patients, 82 (63%) were male. The median age at diagnosis was 2.97 years (2 months-11.5 years). Thirty-six (27.7%) patients were diagnosed with incomplete KD, and there was no significant laboratory difference between incomplete KD and complete KD patients. Thirty-three (25.3%) patients had coronary artery lesions (CAL), and it persisted in only 3 of 33 patients. One of 15 patients with IVIG resistance had CAL. The independent risk factors were days of illness at initial IVIG administration for CAL (p = 0.013, OR [95%CI] = 1.20 [1.04–1.38]) and low hemoglobin (p = 0.003, OR [95%CI] = 0.51 [0.33–0.79]) and low sodium for IVIG resistance (p = 0.012, OR [95%CI] = 0.81[0.69–0.95]). Conclusions The rate of CAL is approximately three times higher in our results than in the Japanese data in recent years. We showed that the time of IVIG administration is the most critical factor for preventing CAL. Wide-ranging studies are needed to decently predict the disease process according to the age and region of patients.

Determination of Serum Procalcitonin Level in Kawasaki Disease

Background: The most frequent cause of coronary artery aneurysm in children is Kawasaki disease (KD). Recently, limited studies on procalcitonin (PCT) were performed to find a biomarker for the diagnosis or prognosis of children with KD. Objectives: This study aimed to compare serum PCT levels between complete and incomplete KD and testify to the predictive validity of PCT for intravenous immunoglobulin (IVIG) resistance and predicting coronary artery lesions (CALs). Methods: This cross-sectional study was conducted at Namazi hospital in Shiraz, Iran, in 2019. All KD patients admitted to the hospital were included, with parental consent obtained. Kawasaki disease patients were categorized as complete KD (cKD) or incomplete KD (iKD). Two-dimensional echocardiography was performed, and peripheral venous blood was examined for PCT levels and other markers. All patients received IVIG and aspirin as standard treatment. The presence of coronary artery abnormalities (CAAs) was determined based on coronary artery size and morphology. We used Stata software version 14.0 for data analysis. Logistic regression models identified predictive factors for coronary complications. This study determined the optimal PCT cut-off point for predicted coronary complications using receiver operating characteristic (ROC) curve analysis. Approval was obtained from the Research Ethics Committees of Shiraz University of Medical Sciences. Results: Procalcitonin values in 38 children hospitalized with acute KD ranged from 0.2 ng/mL to 10 ng/mL, with a mean of 2.65 ng/m. There was no correlation of PCT with patients’ age or gender, incomplete KD, or IVIG resistance. The serum PCT concentration was higher in patients with CAL (P = 0.009). The best PCT cut-off value for CAL prediction was 2.5 ng/mL (corresponding sensitivity = 81.8% and specificity = 68.7%), considering CAL prevalence as the studied group. Conclusions: Supplementary research is needed to determine the sensitivity and specificity of PCT for the diagnosis and prognosis of KD. Procalcitonin might be of value in predicting which children are at increased risk for CALs to intensify therapy.

Clinical prediction model: Multisystem inflammatory syndrome in children versus Kawasaki disease.

Multisystem inflammatory syndrome in children (MIS-C) is a rare but serious complication of severe acute respiratory syndrome coronavirus 2 infection. Features of MIS-C overlap with those of Kawasaki disease (KD). The study objective was to develop a prediction model to assist with this diagnostic dilemma. Data from a retrospective cohort of children hospitalized with KD before the coronavirus disease 2019 pandemic were compared to a prospective cohort of children hospitalized with MIS-C. A bootstrapped backwards selection process was used to develop a logistic regression model predicting the probability of MIS-C diagnosis. A nomogram was created for application to individual patients. Compared to children with incomplete and complete KD (N = 602), children with MIS-C (N = 105) were older and had longer hospitalizations; more frequent intensive care unit admissions and vasopressor use; lower white blood cell count, lymphocyte count, erythrocyte sedimentation rate, platelet count, sodium, and alanine aminotransferase; and higher hemoglobin and C-reactive protein (CRP) at admission. Left ventricular dysfunction was more frequent in patients with MIS-C, whereas coronary abnormalities were more common in those with KD. The final prediction model included age, sodium, platelet count, alanine aminotransferase, reduction in left ventricular ejection fraction, and CRP. The model exhibited good discrimination with AUC 0.96 (95% confidence interval: [0.94-0.98]) and was well calibrated (optimism-corrected intercept of -0.020 and slope of 0.99). A diagnostic prediction model utilizing admission information provides excellent discrimination between MIS-C and KD. This model may be useful for diagnosis of MIS-C but requires external validation.

Jaundice-predominant manifestation of Kawasaki disease in children.

A jaundice-predominant presentation of Kawasaki disease (KD) is atypical. A total of 12 children with KD with a predominant manifestation of jaundice at MacKay Children's Hospital were reviewed, along with 42 cases reported in the literature since 1990. The median age of the 12 patients was 1.85 years (range: 3 months-4 years), and 66.6% were male. All of the patients had elevated liver function at presentation, 50% had hydrops of the gallbladder, and almost 60% had gastrointestinal symptoms and signs. Complete KD was evident in 11 of the 12 patients (91.7%), and two patients (16.7%) had recurrent episodes. All of the patients received intravenous immunoglobulin (IVIG); however, one-third were refractory to treatment. Corticosteroids were used in five (41.7%) of the patients. Three (25%) of the patients had shock, and seven (58.3%) had coronary artery abnormalities, of whom one (8.3%) had persistent coronary artery aneurysm and the others recovered. A review of the 42 cases in the literature showed that the children with a jaundice-predominant presentation of KD had high rates of IVIG-refractory disease (25%), coronary artery abnormalities (25%), shock (13.2%), and corticosteroid treatment (24.2%). Children with KD presenting with a jaundice-predominant manifestation are at a higher risk of IVIG-refractory disease, coronary artery abnormalities, and more recurrent episodes. Physicians should be aware of the risk of shock in this population.

Clinical Features and Determinants of Coronary Artery Dilatation in Pediatric Patients with Complete and Incomplete Kawasaki Disease: A Single-Center Experience Over 15 Years in Hubei, China

Abstract Objective This study is aimed to elucidate age-associated clinical and echocardiography-defined coronary artery (CA) abnormalities features and to identify independent determinants of CA dilatation in pediatric patients with complete Kawasaki disease (cKD) and incomplete KD (iKD) over 15 years in a tertiary hospital in China. Methods This study comprised 241 patients with KD hospitalized in the pediatric department between 2003 and 2018. Patients were divided into three groups according to age at diagnosis: ≤12, 13 to 35, and ≥36 months. Results A total of 167 (69.3%) patients were defined as cKD (aged 24 months, male 60.5%) and 74 (30.7%) patients as iKD (aged 18 months, male 59.5%). The prevalence of cervical lymphadenopathy significantly increased with age in both cKD and iKD patients. In iKD patients, the prevalence of rash significantly decreased with age. CA dilatation most frequently occurred in patients with cKD and iKD aged ≤12 months. The absence of conjunctivitis or oral changes and higher serum creatinine levels were independent determinants of CA dilatation in KD patients aged ≤12 months, regardless of age, sex, and the type of KD. Conclusion Present study underlines the age-stratified disease characteristics, and the factors associated with CA dilatation in cKD and iKD patients. The absence of conjunctivitis or oral changes, higher serum creatinine levels, and mycoplasma, cytomegalovirus, and adenovirus infections might be associated with an increased risk of CA dilatation in KD patients. These findings provide additional hints for pediatricians in the early diagnosis and treatment of KD, especially for individual medical care of cKD and iKD patients in different age groups.

Comparison of Demographic, Clinical, and Echocardiographic Features Between Complete and Incomplete, and Early and Late Presenters of Kawasaki Disease: A 10-Year Single-Center Experience.

The diagnosis of Kawasaki disease(KD)is based mainly on clinical findings and supported by laboratory tests. Complete KD fulfills the main clinical criteria, while incomplete KD includes patients with fewer main criteria and compatible laboratory or echocardiographic findings. The study compares the demographic, clinical, laboratory, and echocardiographic parameters between the complete and incomplete KD and early and late presenters. Moreover, it describes the coronary manifestations of the study population. A retrospective review of all patients admitted with a diagnosis of KD during the period from January 2010 to September 2020 was conducted. Clinical presentation, laboratory features, echocardiographic observations, and follow-up data were examined. Moreover, the patients were further classified as early presenters (presented within 10 days of fever onset) and late presenters (presented after 10 days of disease onset). A comparison between complete and incomplete KD and early and late presenters was performed for demographic, clinical, and echocardiographic features. A total of 76 patients were admitted with a diagnosis of KD.The median age of presentation was 28 months, with a range of five to 144months, and the median timing was seven days, with a range of one to 30days. The median follow-up period was six weeks, with a range of one to 192weeks. Complete KD was present in 38 patients (50%), and 38 (50%) had incomplete KD.Skin manifestations, oral mucosal changes, skin desquamation, conjunctivitis, and lymphadenopathy were present more in patients with complete KD than incomplete ones. Complete and incomplete diseases did not differ regarding coronary artery lesions. Of the patients,53 (70%) presented 10 days or less after the onset of fever, and 23 (30%) presented after the 10th day of disease onset. Comparison between early and late presenters revealed significantly greater mucus membrane changes and lymphadenopathy manifestations among the early presenters and coronary artery lesions among the late presenters. The clinical features of KD should prompt early referral for evaluation, echocardiography, and early administration of intravenous immunoglobulin to prevent coronary artery complications. The complete form of Kawasaki does not have more frequent coronary artery lesions than the incomplete form. Additionally, late presenters may be at increased risk for coronary artery abnormalities than early presenters.

Clinical manifestations and risk factors of coronary artery lesions in children with Kawasaki disease.

To analyze the clinical manifestations of children with Kawasaki disease (KD), and risk factors of coronary artery lesion (CAL). A total of 223 patients admitted to Anhui Children Hospital from January 2017 to December 2019 were enrolled. According to the clinical data, the children with KD were divided into complete Kawasaki disease (CKD) and incomplete Kawasaki disease (IKD) groups. According to the results of the cardiac color ultrasound, the children were divided into the CAL and nCAL groups. The clinical symptoms of children with KD were compared between the CKD and IKD groups. The risk factors of CAL were analyzed by univariate and binary logistic regression analyses. The incidence constituent ratio of KD increased annually from 2017 to 2019 (P < .05). The proportion of fever duration no longer than 10 days, chapped lips, fingertip decrustation, perianal desquamation, and fever combined with rash in the CKD group was significantly higher compared to the IKD group (P < .05), while intravenous immunoglobulin non-response and CAL were significantly lower than those in the IKD group (P < .05). The proportion of males, age <1 year, fever duration longer than 10 days, and IKD in the CAL group were significantly higher compared to the nCAL group, while hemoglobin levels were significantly lower than that in the nCAL group (P < .05). Sex, age, fever duration, atypical KD, and hemoglobin levels were risk factors for CAL in children with KD. Persistent fever, conjunctival hyperemia, chapped lips, and rash were common clinical symptoms in children with KD. The risk of CAL was relatively higher in children with low hemoglobin levels and IKD, whose ages were <1 year old and whose fever time was more than 10 days, which requires high clinical vigilance.

INTRAVENOUS IMMUNOGLOBULIN THERAPY FOR KAWASAKI DISEASE (CASE REPORT)

Kawasaki disease (KD) is acute vasculitis with an unidentified cause. The prevalence of Kawasaki disease is greater in developed countries or regions, also known as developed areas. Throughout 2021, two cases of complete Kawasaki were identified at Perkebunan Jember Klinik Hospital. Using a Harada score of 3, intravenous immunoglobulin (IGIV) treatment screening was calculated. In general, IGIV is used to reduce the risk of abnormalities/damage to the coronary arteries. The purpose of this study is to identify the diagnostic criteria and indications for administering IGIV to children with Kawasaki disease. Qualitative Method with the case report approach. Patients diagnosed with Kawasaki disease must receive therapy and care based on their symptoms and conditions. Kawasaki disease can be treated with a number of different drugs. IGIV can be delivered to Kawasaki disease patients as a form of treatment. IGIV treatment based on Harada Score. Kawasaki disease is a condition that is rarely recognized by the general public. The resulting conclusions are as follows Complete Kawasaki disease is diagnosed by five days of persistent fever plus 4 of the 5 classic symptoms. Screening with a scoring system is needed in making decisions about giving IGIV.

Clinical significance of serum cytokine profiles for differentiating between Kawasaki disease and its mimickers

ObjectivesTo investigate the clinical significance of serum cytokine profiles for differentiating between Kawasaki disease (KD) and its mimickers. MethodsPatients with KD, including complete KD, KD shock syndrome (KDSS), and KD with macrophage activation syndrome (KD-MAS), and its mimickers, including multisystem inflammatory syndrome in children, toxic shock syndrome, and Yersinia pseudotuberculosis infection, were enrolled. Serum levels of interleukin (IL)-6, soluble tumor necrosis factor receptor type II (sTNF-RII), IL-10, IL-18, and chemokine (C-X-C motif) ligand 9 (CXCL9) were measured using enzyme-linked immunosorbent assay and compared them with clinical manifestations. ResultsSerum IL-6, sTNF-RII, and IL-10 levels were significantly elevated in patients with KDSS. Serum IL-18 levels were substantially elevated in patients with KD-MAS. Patients with KD-MAS and KD mimickers had significantly elevated serum CXCL9 levels compared with those with complete KD. Area under the receiver operating characteristic curve analysis showed that serum IL-6 was the most useful for differentiating KDSS from the others, IL-18 and CXCL9 for KD-MAS from complete KD, and CXCL9 for KD mimickers from complete KD and KD-MAS. ConclusionSerum cytokine profiles may be useful for differentiating between KD and its mimickers.

Characteristics of persistent arthritis with refractory Kawasaki disease: a single-center retrospective study

Arthritis is one complication of Kawasaki disease (KD); however, the clinical features of arthritis in KD have not been well clarified. We retrospectively investigated the characteristics of persistent arthritis beyond the subacute phase of KD. In this cohort, 49 of 243 patients (20%) developed arthritis, with 33 patients (14%) experiencing persistent arthritis. Among these 33 patients, 31 (94%) had complete KD. Thirty (91%) were resistant to first intravenous immunoglobulin, and 15 (45%) required additional infliximab. Five patients (15%) developed coronary artery lesions, and 24 (73%) had oligoarthritis, mainly in large lower-extremity joints. Twenty-four patients (73%) complained of arthralgia. At arthritis onset, 16 patients (48%) presented with fever, including recurrent fever in 10 patients. Serum C-reactive protein concentration in patients with active arthritis significantly increased compared with after acute KD treatment (2.4 vs. 0.7 mg/dL, p < 0.001). Serum matrix metalloproteinase-3, a biomarker of arthritis, was significantly higher in patients with active arthritis than in remission (93.7 vs. 20.3 ng/mL, p < 0.001). Thirty (91%) and 14 (42%) patients, respectively, were treated with non-steroidal anti-inflammatory drugs and prednisolone, and they completely recovered. To summarize, persistent arthritis is a common complication in refractory KD, and adequate diagnosis and treatment are necessary.

Case report: corticosteroid as an alternative therapy for Kawasaki disease

Background: Kawasaki disease is an acute febrile illness with systemic vasculitis and predominantly causes acquired heart disease. The etiology is still unknown, and there is no specific diagnostic test. The diagnosis was made using clinical criteria, laboratory and echocardiographic findings. It is characterized by fever, bilateral non-exudative conjunctivitis, erythema of lips and oral mucosa, rash, cervical lymphadenopathy, edema and erythema of the hand and foot. The initial treatment for both complete and incomplete Kawasaki disease is a single high dose of intravenous immunoglobulin (IVIG) and aspirin. The place of corticosteroids in the initial therapy is still controversial. Case Description: A 3-year-old boy presented with a 7-day history of high fever and not relieved by antipyretic, which was followed by a maculopapular rash on his trunk and extremities, vomiting, reduced appetite, red on her lips and eyes who aspirin, IVIG and corticosteroid treated as initial therapy. He showed clinical improvement and did not have cardiovascular complications. Conclusion: We report a case of Kawasaki disease in a 3-year-old boy. This case highlighted that adding corticosteroids to IVIG and aspirin as initial therapy might improve outcomes in Kawasaki disease and could be selected as a treatment modality to treat Kawasaki disease.

Kawasaki disease in children with Bacillus Calmette-Guérin scar reactivity: Focus on coronary outcomes

Background/Purpose: Reactivity at the Bacillus Calmette-Guérin (BCG) scar is a pathognomonic feature of Kawasaki disease (KD). However, its value in predicting KD outcomes has not been emphasized. This study explored the clinical significance of BCG scar redness with respect to coronary artery outcomes. MethodsThis retrospective study collected data on children with KD from 13 hospitals in Taiwan during 2019–2021. Children with KD were categorized into four groups based on the KD type and BCG scar reactivity. Risk factors of coronary artery abnormalities (CAA) were analyzed in all groups. ResultsBCG scar redness occurred in 49% of 388 children with KD. BCG scar redness was associated with younger age, early intravenous immunoglobulin (IVIG) treatment, hypoalbuminemia, and CAA at the first echocardiogram (p < 0.01). BCG scar redness (RR 0.56) and pyuria (RR 2.61) were independent predictors of any CAA within 1 month (p < 0.05). Moreover, pyuria (RR 5.85, p < 0.05) in children with complete KD plus BCG scar redness was associated with CAA at 2–3 months; first IVIG resistance (RR 15.2) and neutrophil levels ≥80% (RR 8.37) in children with complete KD plus BCG scar non-redness were associated with CAA at 2–3 months (p < 0.05). We failed to detect any significant risk factors of CAA at 2–3 months in children with incomplete KD. ConclusionBCG scar reactivity contributes to diverse clinical features in KD. It can be effectively applied to determine the risk factors of any CAA within 1 month and CAA at 2–3 months.

Single cell RNA-seq resolution revealed CCR1+/SELL+/XAF+ CD14 monocytes mediated vascular endothelial cell injuries in Kawasaki disease and COVID-19

IntroductionThe COVID-19 pandemic provide the opportunities to explore the numerous similarities in clinical symptoms with Kawasaki disease (KD), including severe vasculitis. Despite this, the underlying mechanisms of vascular injury in both KD and COVID-19 remain elusive. To identify these mechanisms, this study employs single-cell RNA sequencing to explore the molecular mechanisms of immune responses in vasculitis, and validate the results through in vitro experiments. MethodThe single-cell RNA sequencing (scRNA-seq) analysis of peripheral blood mononuclear cells (PBMCs) was carried out to investigate the molecular mechanisms of immune responses in vasculitis in KD and COVID-19. The analysis was performed on PBMCs from six children diagnosed with complete KD, three age-matched KD healthy controls (KHC), six COVID-19 patients (COV), three influenza patients (FLU), and four healthy controls (CHC). The results from the scRNA-seq analysis were validated through flow cytometry and immunofluorescence experiments on additional human samples. Subsequently, monocyte adhesion assays, immunofluorescence, and quantitative polymerase chain reaction (qPCR) were used to analyze the damages to endothelial cells post-interaction with monocytes in HUVEC and THP1 cultures. ResultsThe scRNA-seq analysis revealed the potential cellular types involved and the alterations in genetic transcriptions in the inflammatory responses. The findings indicated that while the immune cell compositions had been altered in KD and COV patients, and the ratio of CD14+ monocytes were both elevated in KD and COV. While the CD14+ monocytes share a large scale of same differentiated expressed geens between KD and COV. The differential activation of CD14 and CD16 monocytes was found to respond to both endothelial and epithelial dysfunctions. Furthermore, SELL+/CCR1+/XAF1+ CD14 monocytes were seen to enhance the adhesion and damage to endothelial cells. The results also showed that different types of B cells were involved in both KD and COV, while only the activation of T cells was recorded in KD. ConclusionIn conclusion, our study demonstrated the role of the innate immune response in the regulation of endothelial dysfunction in both KD and COVID-19. Additionally, our findings indicate that the adaptive immunity activation differs between KD and COVID-19. Our results demonstrate that monocytes in COVID-19 exhibit adhesion to both endothelial cells and alveolar epithelial cells, thus providing insight into the mechanisms and shared phenotypes between KD and COVID-19.

Complete and incomplete Kawasaki disease: Clinical differences and coronary artery outcome from a national prospective surveillance study in Switzerland.

The aim of this national prospective surveillance study was to compare the clinical presentation, laboratory findings, treatment, and coronary artery outcome in patients with incomplete and complete Kawasaki disease (KD). Between March 2013 and February 2019, children with a diagnosis of complete and incomplete KD were reported by the Swiss Paediatric Surveillance Unit and prospectively enrolled. Clinical data, laboratory values, treatment, and echocardiographic features were collected at diagnosis and 1 year of follow-up. Data were compared between children with complete or incomplete KD. A total of 351 questionnaires were registered from children with a diagnosis of KD. Of them, 219 (62.4%) children had complete KD, and 132 (37.6%) children had incomplete KD. Children with incomplete KD were younger and had a longer-lasting fever; however, there were no differences in the level of C-reactive protein. All but four children received intravenous immunoglobulin treatment, whereas 14% of children were treated with corticosteroids. Children with incomplete KD were more often treated with corticosteroids than children with incomplete KD (p = 0.01). At diagnosis, 39 (11.1%) patients had only coronary artery dilation and 57 (16.2%) had at least one coronary artery aneurysm. There were no differences in coronary artery involvement between the two groups. At follow-up, 273 of 294 (92.8%) patients had no coronary artery involvement, with no difference between the two groups (p = 0.609). The overall incidence of coronary artery aneurysms at diagnosis was 16.2%. At follow-up, most coronary artery aneurysms had regressed, and coronary artery aneurysms were present in only 5.8% of the patients. Coronary artery aneurysms were slightly more frequent in patients with incomplete KD at follow-up (p = 0.039) but not at diagnosis (p = 0.208). Although the clinical presentation in children with incomplete and complete KD differs, the absence of coronary artery involvement does not. The use of corticosteroids appears to be preventive against the development of coronary artery aneurysms in these patients. However, the results of this study suggest a lower rate of coronary artery aneurysm regression in patients with incomplete KD. Further studies on a larger scale are needed to assess the risk of non-regression of coronary artery aneurysms in this particular group of patients.