Recurrent Kawasaki disease in children: Four case reports

Descriptive epidemiologic studies of recurrent and non-recurrent Kawasaki disease (KD) may identify other potentially important differences between these illnesses. Data from the USA and Japan, the Centers for Disease Control and Prevention (CDC) national KD surveillance(1984-2008) and the 17th Japanese nationwide survey (2001-2002), respectively, were analyzed to examine recurrent KD patients <18 years of age meeting the CDC KD case or atypical KD case definition. These patients were compared with non-recurrent KD patients. Of the 5557 US KD patients <18 years of age during 1984-2008, 97 (1.7%) were identified as having had recurrent KD. Among the US Asian/Pacific Islander KD patients, 3.5% had recurrent KD, which was similar to the percentage identified among KD patients (3.5%) in the Japanese survey. Compared with non-recurrent KD patients, KD patients [with recurrent KD] were more likely to be older, fulfill the atypical KD case definition, and have coronary artery abnormalities (CAA) despite i.v. immunoglobulin (IVIG) treatment. Differences in the age, race, and frequency of CAA exist between recurrent and non-recurrent KD patients. The increased association of CAA with recurrent KD suggests that more aggressive treatment strategies in conjunction with IVIG may be indicated for the second episode of KD.

Background: Coronavirus disease 2019 (COVID-19) has been an emerging, rapidly evolving situation in China since late 2019 and has even become a worldwide pandemic. The first case of severe childhood novel coronavirus pneumonia in China was reported in March 2020 in Wuhan. The severity differs between adults and children, with lower death rates and decreased severity for individuals under the age of 20 years. Increased cases of Kawasaki disease (KD) have been reported from New York City and some areas of Italy and the U.K., with almost a 6–10 times increase when compared to previous years. We conducted this study to compare characteristics and laboratory data between KD and COVID-19 in children. Methods: We obtained a total of 24 children with COVID-19 from a literature review and 268 KD cases from our hospital via retrospective chart review. Results: We found that patients with KD have higher levels of white blood cells (WBCs), platelets, neutrophil percentage, C-reactive protein (CRP), procalcitonin, and aspartate aminotransferase (AST) and a higher body temperature, while patients with COVID-19 have a higher age, hemoglobin levels, and lymphocyte percentage. After performing multiple logistic regression analysis, we found that age, WBCs, platelets, procalcitonin, and AST are identical markers for distinguishing COVID-19 from KD in children. Conclusion: In this COVID-19 pandemic period, clinicians should pay attention to children with COVID-19 infection when high WBC, platelet, procalcitonin, and AST values are present in order to provide early diagnosis for KD or multisystem inflammatory syndrome in children (MIS-C).

Kawasaki disease (KD) has features that appear supporting an infectious cause with a secondary deranged inflammatory/autoimmune response. The association of KD in adults with human immunodeficiency virus infection and the presence of KD in patients with immunodeficiency disorders support the infectious theory. We present four KD patients associated with immunodeficiencies: one with X-linked agammaglobulinemia, one with HIV infection, and two with leukemia; one of these patients also had Down syndrome. We did a literature search to find out all reported cases of immunodeficiency with KD in children. In immunodeficiency disorders, the inability of the immune system to eradicate the pathogens coupled to an exaggerated inflammatory response, especially in chronic granulomatous disease, may lead to the development of KD. The study of patients with immunodeficiencies complicated with KD may shed light into the etiopathogenesis of the disease.

Background: Kawasaki disease (KD) is a clinical diagnosis, with common confusion among other causes of febrile illnesses. There are no confirmatory laboratory parameters for diagnosing KD. Objective: To investigate whether low mean platelet volume (MPV) is associated more with fever due to KD than due to the other common causes. Methods: This retrospective case-control study was done on febrile children between 6 months and 6 years of age admitted from January 2015 to January 2017. The MPV values of 28 KD and 50 non-KD febrile children admitted to our hospital were obtained from the hospital records. The diagnosis of KD was accepted only when (1) two pediatricians had agreed upon the diagnosis independently based on the American Heart Association guidelines 2004, (2) no other cause of fever coexisted with KD in a particular patient, and (3) prompt clinical response within 48 h of administration of intravenous immunoglobulin. Using suitable statistical software, the range of MPV in KD fever and non-KD fevers was compared. Results: MPV was lower in the KD group (9.75±0.98 femtoliter) than in the non-KD fever group (11.14±1.53 femtoliter). From the receiver operating characteristic curve, it was found that at MPV ?10.0 fl, KD can be diagnosed with 75% sensitivity and 80% specificity. This means that lower the value of MPV, lower is the probability that a non-KD patientwill be wrongly diagnosed as KD. Conclusion: Our study shows that low MPV is associated with KD. Hence, a low MPV can raise the index of suspicion for KD in febrile children, especially in cases of incomplete KD. Further, prospective studies involving larger sample size are needed to ascertain its diagnostic utility.

Background Several recent studies have validated the newfound significance of elevated eosinophils in Kawasaki disease (KD). However, this finding was not incorporated into the suggested laboratory criteria outlined by the Kawasaki Disease Committee of the American Heart Association for assessing children with incomplete KD. Purpose We aimed to determine the potential benefits of including eosinophil levels in the laboratory evaluation of KD. Methods A retrospective cohort study involved 77,934 febrile children (FC, not KD) and 1,970 subjects diagnosed with KD. Participants were recruited from four hospitals, comprising two medical centers and two regional hospitals. The AHA recommends specific laboratory tests—white blood cell count, hemoglobin, platelet count, ALT levels, albumin, and urinalysis—for evaluating incomplete KD in children with a higher CRP than 30mg/dL. The first four items are standard blood assays. Additionally, we introduced high eosinophil counts as a new diagnostic criterion for identifying children with KD. Results All these five laboratory findings (anemia for age defined as below the 5th percentile), platelet count &amp;gt;450,000/mm3, white blood cell&amp;gt;15,000/mm3, ALT&amp;gt;40 U/L, and eosinophil count &amp;gt;190/mm3) are significantly different between KD children and FC. The proportion of having these features is higher in KD children than in FC (19.3% vs. 8.0% in anemia for age, 20.5% vs. 10.3% in thrombocytosis, 37.0% vs. 18.1% in leukocytosis, 45.7% vs. 5.8% in elevated ALT, and 62.6% vs. 29.3% in high eosinophils). Elevated eosinophil counts are most commonly associated with KD among these five parameters. Furthermore, when incorporating the parameter of elevated eosinophil counts, the proportion of KD children with at least two abnormalities rose from 35.4% to 60.7%. In a subgroup analysis of children with KD presenting at least two abnormalities, the proportion rose from 38.7% to 63.5% in the high CRP group (≧ 3.0 mg/L) and 21.6% to 49.0% in the low CRP group (&amp;lt; 3.0 mg/L), respectively. Conclusions Higher eosinophil counts are vital markers in KD children. This aids in the identification of additional potential KD cases, especially in those exhibiting low CRP levels.

Kawasaki disease (KD) is a challenging diagnosis. Erythema and induration of the Bacillus Calmette-Guérin (BCG) site is increasingly recognised as a significant clinical clue. However, there is little data to support its specificity for KD as compared to other febrile illnesses. We aimed to evaluate BCG reaction or induration as a diagnostic tool for KD. A retrospective case-controlled study of patients discharged with a diagnosis of KD from 2007 to 2010 was conducted. Another group of patients admitted over the same period for possible KD, but later found not to have KD, served as control. Significantly more infants with KD (69.7%) had BCG site changes than older children (27.8%; p < 0.001). It also presented earlier in the course of KD; < 5 days (53.3%) compared to ≥ 5 days of fever (30.0%; p < 0.001). Positive predictive value of BCG site reaction or induration for KD was 90.8% (95% confidence interval [CI] 0.819-0.962) for infants and 96.2% (95% CI 0.868-0.995) for older children. The prevalence rate of changes at the BCG site was 9.9% among patients with non-KD febrile illnesses and 42.6% among patients with KD. BCG site reaction or induration is a useful clinical clue for the diagnosis of KD in both infants and older children, with a higher prevalence in infants. Physicians should consider KD in children with febrile illness and redness or crust formation at the BCG site, especially in view of low rates of BCG reaction or induration in non-KD febrile illnesses.

Association between prenatal exposure to air pollution and risk of Kawasaki disease in young children of South Korea: Big children's environmental health study.

Aim:The aim of this study was to evaluate presenting symptoms, clinical features, and laboratory tests for the diagnosis of Kawasaki disease (KD) in children and their short-term follow-up at a tertiary care hospital of North India from April 2017 to March 2020.Materials and Methods:A total of 31 children (23 boys and 8 girls) up to 10 years of age were included in this study. The diagnosis of KD was made as per the American Heart Association 2017 guidelines. Clinical features, laboratory parameters, and coronary involvement were compared between the complete and incomplete KD groups.Results:The incidence of complete versus incomplete KD was 19 (61.2%) versus 12 (38.7%) children, respectively. Change in extremities and oral mucosal changes were more encountered in the complete KD group as compared to the incomplete KD group (100% vs. 58.3%, P = 0.004, and 78.9% vs. 33.3%, P = 0.002, respectively). Coronary artery aneurysm was seen in 54% of the patients on echocardiography which was greater in the incomplete KD group (83.3%) as compared to the complete KD group (36.8%). The median time from the onset of symptoms to intravenous immunoglobulin infusion was <10 days in 84.2% of the patients with complete KD versus 41.7% with incomplete KD which was statistically significant. Fifty percent of the children with coronary ectasia and small aneurysm had normal coronaries at follow-up of 6 months.Conclusion:KD is probably underdiagnosed in most developing countries, like that of ours, and requires a high index of suspicion.

Significant Relationship Between Serum High-sensitivity C-Reactive Protein, High-density Lipoprotein Cholesterol Levels and Children With Kawasaki Disease and Coronary Artery Lesions

To analyze the epidemiology of Kawasaki disease in Icelandic children and its complications. A retrospective analysis of all cases of Kawasaki disease and atypical Kawasaki disease in children in Iceland from 1996-2005. Chart records were reviewed and children diagnosed at Landspítali - University Hospital invited for a Follow up study with emphasis on heart complications. Thirty children were diagnosed with Kawasaki disease, annual incidence was 10.7/100.000 children <5 years of age. The boy:girl ratio was 2.3:1. All 30 children were treated with IVIG, without any major adverse events related to the treatment. The median time from the initial symptoms to treatment was six days (range 3-31 days). There was no mortality. Two children developed coronary aneurysms and three coronary ectasia. Follow up echocardiography was preformed in 23 of the children four to twelve years after Kawasaki disease. Two of the children still had coronary ectasia, and six (26%) had mitral regurgitation. The incidence of Kawasaki disease in Iceland was comparable to an earlier Icelandic study and reported incidence in the Nordic countries. Coronary involvement during the acute phase was mild, and all coronary aneurysm regressed. Serious cardiac complications were not seen. Children with Kawasaki disease in Iceland have favorable prognosis. Interestingly, mild mitral regurgitation and coronary ectasia were common at mid-term follow up.

Maladie de Kawasaki chez le grand enfant et l’adulte jeune : expérience marseillaise sur 10 ans

Aim: To investigate the association between different viral infections and the development of Kawasaki disease (KD) in children. Materials &amp; methods: Electronic databases were searched for relevant studies published from inception to May 2020. The pooled odds ratios (ORs) of the association of different viral pathogens with KD were estimated using a random-effects model weighted by the inverse variance method. Results: The strongest associations were found between KD and human parvovirus B19 viremia (OR = 41.05; 95% CI: 5.13–328.28; I-square = 0%), EBV IgM seropositivity (OR = 7.18; 95% CI: 3.65–14.12, I-square = 0%) and human herpesvirus-6 IgG seropositivity (OR = 5.83; 95% CI: 1.06–32.01). Conclusion: Human parvovirus B19, EBV and human herpesvirus-6 are highly suspected to be key contributors to the development of KD.

To evaluate the relationships between serum plasminogen activator (PA) and D-dimer levels, the severity of Kawasaki disease (KD) in children, and their ability to predict coronary artery lesions (CAL). This retrospective study analyzed the clinical data of 102 children diagnosed with KD at the Affiliated Hospital of Jiangnan University from January 2020 to September 2023. The cohort was divided into two groups: 31 children with CAL in the CAL group and 71 without it in the non-CAL group. The study assessed the incidence of CAL and investigated the correlations between serum PA and D-dimer levels and various inflammatory markers (white blood cell (WBC) count, platelet count, and erythrocyte sedimentation rate (ESR)). Receiver operating characteristic (ROC) curves were used to evaluate the predictive value of these biomarkers for CAL. CAL was present in 30.04% of the children. Pearson correlation analysis revealed that serum PA levels were inversely correlated with WBC count (P = 0.0187), platelet count (P = 0.0116), and ESR (P = 0.0041), while D-dimer levels were positively correlated with these markers (P < 0.001). A negative correlation between PA and D-dimer levels was also observed (P < 0.001). The combined use of PA and D-dimer levels to predict CAL achieved an area under the curve of 0.871. Serum PA levels were negatively associated with the severity of KD, whereas D-dimer levels were positively associated. Together, these markers showed significant predictive value for CAL, highlighting their utility in assessing disease severity and guiding management in children with KD.

Objective To study the clinical features of children with recurrent Kawasaki disease (KD). Methods PubMed, Web of Science, Embase, CNKI, Wanfang Med Online, and Weipu Data were searched for case-control studies on the clinical features of initial and recurrent KD. The articles were screened according to the inclusion and exclusion criteria. RevMan 5.3 software was used to perform the Meta analysis. Effect models were selected based on the results of heterogeneity test, and then pooled OR or weighted mean difference (WMD), and their 95% CI were calculated. Results A total of 9 case-control studies were included, with 12 059 children with KD in total, among whom 206 children had recurrent KD (127 boys/61.7%; 79 girls/38.3%). The results of the Meta analysis showed that compared with the initial KD onset, the children with recurrent KD had a shorter duration of fever (WMD=-1.81, 95%CI:-2.99 to -0.64) and a lower proportion of children with swelling of the hands and feet (OR=0.46, 95%CI:0.26 to 0.80). There was no significant difference in the incidence rate of coronary artery lesions between recurrent KD and initial KD (OR=1.34, 95%CI:0.84 to 2.14). Conclusions Current evidence shows that children with recurrent KD tend to have a shorter duration of fever and a lower incidence of swelling of the hands and feet. KD recurrence is more common in boys. Current evidence does not show an increased risk of developing coronary artery lesions in children with recurrent KD.

We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD) in children. Five males and a females, aged 5-15 years old (median, 9), underwent PTCRA. The interval from the onset of KD to PTCRA ranged from 5 to 12 years (median, 9). The target vessels were the left anterior descending artery (three patients), the left circumflex (two patients), and the right coronary artery (one patient). The immediate results of PTCRA were successful in all patients, and the mean stenosis degree improved from 89 +/- 10% to 27 +/- 12%. In follow-up coronary angiograms within 1 year, four vessels were restenosed, including two with complete occlusion. A 15-year-old male has had good patency for 4 years after undergoing re-PTCRA for restenosis using a larger burr size. PTCRA is feasible for severe localized stenosis with calcification caused by KD in children and the immediate results are good. However, restenosis often occurred within 1 year after PTCRA in small children and PTCRA is not always appropriate for them. When the use of a larger burr is possible, good patency can be expected and can be maintained by close follow-up and re-PTCRA.