Episode Of Kawasaki Disease Research Articles

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.

Distinguishing Incomplete Kawasaki and Nonsevere Multisystem Inflammatory Syndrome in Children.

Characterizing inflammatory syndromes during the coronavirus disease 2019 pandemic was complicated by recognition of multisystem inflammatory syndrome in children (MIS-C), contemporaneous with episodes of Kawasaki disease. We hypothesized a substantial overlap between the 2 and assessed the performance of an MIS-C likelihood score in differentiating inpatients with nonsevere MIS-C from prepandemic incomplete Kawasaki disease (iKD) without coronary involvement. A retrospective review of inpatient records was conducted; the nonsevere MIS-C cohort (March 2020-February 2021) met the 2023 definition for MIS-C; the iKD cohort (January 2018-January 2019) met the American Heart Association criteria for iKD without coronary involvement. We applied the likelihood score to both cohorts. We estimated the percent of children with iKD who could have met the clinical criteria of the MIS-C, had they presented in 2023. The 68 children in the nonsevere MIS-C cohort were older (8 vs 4 years, P < .001) than the 28 children in the iKD cohort. Those in the nonsevere MIS-C cohort had higher rates of thrombocytopenia (P < .001) and lymphopenia (P = .021); those in the iKD cohort had higher rates of pyuria (P < .001). Twenty-four (86%) children in the iKD cohort met the 2023 MIS-C definition. The scoring system correctly predicted 71% to 74% children with their respective clinical diagnoses. Though there was considerable clinical overlap, thrombocytopenia, lymphopenia, and the absence of pyuria were the most helpful parameters to distinguish children with nonsevere MIS-C from those with iKD.

Clinical characteristics of patients with recurrent Kawasaki disease: a nationwide cohort study of 19 456 patients with minimum 3-year follow up

ObjectiveTo identify the clinical characteristics of patients with recurrent Kawasaki disease (KD).DesignA nationwide, population-based, cohort study using the Korean National Health Insurance claims database between 2013 and 2016.PatientsA total of...

Coronary arterial abnormalities detected in children over 10 years following initial Kawasaki disease using cardiac computed tomography.

To evaluate whether Kawasaki disease predisposes to premature atherosclerosis and to assess status of coronary artery abnormalities at least 10 years after diagnosis. A prospective study was carried out on 21 patients who were diagnosed with Kawasaki disease at least 10 years back and are on regular follow-up. The study was conducted on 128 Slice Dual Source computed tomography scanner with electrocardiography-triggered radiation optimised protocols for assessment of coronary artery abnormalities and calcifications. Study cohort had 21 subjects - 15 males and 6 females (age range: 11-23 years; mean: 15.76 + 3.72 years). Mean age at time of diagnosis was 3.21 + 2.48 years. Mean time interval from diagnosis of Kawasaki disease to computed tomography coronary angiography was 12.59 + 2.89 years. Four children had evidence of coronary artery abnormalities on transthoracic echocardiography at time of diagnosis. Of these, two had persistent abnormalities on computed tomography coronary angiography. One subject (4.76%) had coronary calcification that was localised to abnormal coronary artery segment. Four coronary artery abnormalities (one saccular; three fusiform aneurysms) were noted in two subjects. Prevalence of coronary artery calcification is low and, if present, is localised to abnormal segments. This calcification is likely dystrophic rather than atherosclerotic. It appears that coronary artery abnormalities can persist for several years after acute episode of Kawasaki disease. Periodic follow-up by computed tomography coronary angiography is now a feasible non-invasive imaging modality for long term surveillance of patients with Kawasaki disease who had coronary artery abnormalities at time of diagnosis.

Comparison of Risk of Recrudescent Fever in Children With Kawasaki Disease Treated With Intravenous Immunoglobulin and Low-Dose vs High-Dose Aspirin

Timely initiation of intravenous immunoglobulin plus aspirin is necessary for decreasing the risk of recrudescent fever and coronary artery abnormalities in children with Kawasaki disease (KD). The optimal dose of aspirin, however, remains unclear. To evaluate whether initial treatment with low-dose compared with high-dose aspirin in children with KD is associated with an increase in fever recrudescence. A retrospective cohort study of 260 children with KD at Riley Hospital for Children, Indianapolis, Indiana, between January 1, 2007, and December 31, 2018, was conducted. Children aged 0 to 18 years with a first episode of KD, identified by International Classification of Diseases, Ninth Revision and International Statistical Classification of Diseases and Related Health Problems, Tenth Revision diagnosis codes treated within 10 days of symptom onset with high-dose intravenous immunoglobulin plus aspirin were eligible. Patients who received an alternative diagnosis, experienced a second episode of KD, did not receive intravenous immunoglobulin plus aspirin for initial treatment, were not treated within 10 days of symptoms, or had incomplete records were excluded. High-dose (≥10 mg/kg/d) or low-dose (<10 mg/kg/d) aspirin therapy. The primary outcome was recrudescent fever necessitating retreatment of KD. The secondary outcomes were coronary artery abnormalities and hospital length of stay. Among the 260 patients included, the median (interquartile range) age was 2.5 (1.6-4.3) years, 103 (39.6%) were girls, 166 (63.8%) were non-Hispanic white, 57 (21.9%) were African American, 22 (8.5%) were Asian, 11 (4.2%) were Hispanic, and 4 (1.5%) were of unknown race/ethnicity. One hundred-forty-two patients (54.6%) were treated with low-dose aspirin. There was no association between recrudescent fever and aspirin dose, with 39 children (27.5%) having recrudescent fever in the low-dose group compared with 26 children (22.0%) in the high-dose group (odds ratio [OR], 1.34; 95% CI, 0.76-2.37; P = .31), with similar results after adjusting for potential confounding variables (OR, 1.63; 95% CI, 0.89-2.97; P = .11). In a subset analysis of 167 children with complete KD, however, there was nearly a 2-fold difference in the odds of recrudescent fever with low-dose aspirin (OR, 1.87; 95% CI, 0.82-4.23; P = .14), although this difference did not reach statistical significance. In addition, no association was identified between treatment group and coronary artery abnormalities (low-dose, 7.4% vs high-dose, 9.4%; OR, 0.86; 95% CI, 0.48-1.55; P = .62) or median (interquartile range) length of stay (3 [3-5] days for both groups; P = .27). In this study, low-dose aspirin for the initial treatment of children with KD was not associated with fever recrudescence or coronary artery abnormalities. Given the potential benefits, further study of low-dose aspirin to detect potentially clinically relevant outcome differences is warranted to inform treatment decisions and guideline development.

The time option of IVIG treatment is associated with therapeutic responsiveness and coronary artery abnormalities but not with clinical classification in the acute episode of Kawasaki disease

BackgroundIn the last decade, incomplete Kawasaki disease (KD), intravenous immunoglobulin (IVIG) non-response and coronary artery abnormalities (CAA) have experienced the increasing trends in China. In addition, the enhancement of pediatricians’ awareness may also raise the diagnostic rate of incomplete KD and stimulate more aggressive initial therapy in the acute episode of KD. Given this background, we hypothesize that the time option of IVIG treatment should be in parallel with peak time of systemic inflammation; either earlier or later IVIG treatment may affect the clinical classification, therapeutic responsiveness and CAA occurrence in KD patients. Therefore, the major objective of the present study is to identify whether the time option of IVIG treatment could be associated with the clinical classification, therapeutic responsiveness and CAA occurrence in the acute episode of KD.Materials and methodsA total of 153 children with KD were recruited between July 2015 and May 2018. All patients received the standard therapy of KD, including a single infusion of IVIG (2 g/kg) and aspirin (30–50 mg/kg/d). Blood samples were collected from all subjects within 24 h pre-IVIG treatment, respectively. Echocardiography was performed during the period from 2 days to 14 days after IVIG treatment.Results(1) The clinical classification presented no significant heterogenicity among different treatment time (x2 = 1.59, p > 0.05) (2) Eleven KD patients resisted to IVIG treatment and 7 of them (63.60%) received the initial IVIG dose on day 5 and 6. (3) The distribution of CAA onset was subjected to a significant difference according to timing option of IVIG treatment (x2 = 11.94, p < 0.05).ConclusionsThe time option of IVIG treatment is associated with therapeutic responsiveness and CAA but not with clinical classification in the acute episode of KD.

Recurrent Kawasaki disease at a tertiary care center in Chandigarh, North West India: 24 years of clinical experience.

Kawasaki disease (KD) is emerging as the most common medium vessel vasculitis in children. Recurrence of KD (0.8%-3.0%) is unusual and not commonly reported. To study the clinico-laboratory profile of children with recurrent KD. Case records of 714 children with KD, registered from January 1994 to April 2018 in the Pediatric Rheumatology Clinic at the Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research Chandigarh, were reviewed and data were analyzed for children having had recurrent KD. Seven (0.98%) children (five boys; two girls) had recurrent KD in our cohort of 714 patients. Mean age at diagnosis was 4.3years (range, 2-9years). Six (85.7%) had recurrence of KD within 1.5years. Incomplete KD was more common during recurrence (71.4%) as compared to the first episode (33.3%). Five children with recurrent KD developed desquamation during the first week of illness. Laboratory investigations did not show any significant difference during the two episodes of KD. Six of the seven children in the first episode received treatment with intravenous immunoglobulin (IVIG). Five of the seven children received IVIG during recurrence. One child had transient left main coronary artery ectasia during the first episode of KD as well as during recurrence and it normalized after treatment on both occasions. Recurrences in KD are unusual. Over a period of 24years the recurrence rate in our cohort of children with KD was 0.98%. Further, children with recurrences tended to have milder and incomplete form of the disease.

Carotid Intima-Media Thickness and Lipid Profile in Children With Kawasaki Disease: A Single-Center Follow-up Study After a Mean Duration of 6.9 Years.

Kawasaki disease (KD) has a predilection to involve coronary arteries, leading to several long-term cardiovascular sequelae. Apart from coronary artery abnormalities, children with KD are also prone to develop premature atherosclerosis, endothelial dysfunction, and lipid abnormalities. Some of these complications may occur even in children who have received appropriate treatment with intravenous immunoglobulin in the acute phase. In 2009, we had studied carotid intima-media thickness (cIMT) and lipid profile in 27 children with KD at least 1 year after the acute episode. In the present study, we have followed up the same cohort of 27 children at least 5 years after the acute episode of KD. We measured the cIMT, a surrogate marker for premature atherosclerosis, and fasting lipid profile in the cohort and compared the results with values obtained in our previous study. There was significantly higher mean cIMT in children with KD as compared with control subjects. However, there was no significant difference in cIMT among children in the cohort at 1 and 5 years of follow-up. Abnormal lipid profile was seen in 7 of 27 children in the present study, 5 of whom also had had lipid abnormality at 1-year follow-up. This suggests that lipid abnormalities in KD may be long lasting. Children with KD need careful long-term follow-up even when they do not have overt and persistent coronary artery abnormalities. It is possible that consequences of KD in childhood may impact health status of young adults several years later.

Sequential Changes in Left Ventricular Systolic Myocardial Deformation Mechanics in Children with Recurrent Kawasaki Disease.

BACKGROUNDSequential changes in left ventricular (LV) systolic function over time in patients with recurrent episodes of Kawasaki disease (KD) remain unclear.METHODSTwenty-five children with recurrent KD were retrospectively studied. Using conventional echocardiographic parameters and myocardial deformation analysis, systolic LV function in children in initial and recurrent KD episodes were compared with separate control groups, comprising 15 controls each. Recurrent KD was defined as occurring at an interval of ≥2 months between the initial and recurrent episodes.RESULTSThe interval range between initial and recurrent episodes of KD was 3–103 months. In children with KD, 8 (32%) were <1 year of age at the initial episode, 10 (40%) had a recurrence within 1 year of the initial episode, and 4 (16%) and 5 (20%) were intravenous immune globulin nonresponders in initial and recurrent episodes, respectively. In both the initial and recurrent episodes of KD, the mean LV longitudinal peak systolic ε was all within normal range. However, when compared to controls, mean LV longitudinal peak systolic ε was decreased in patients with KD in the acute phases of both the initial and recurrent episodes. When compared to controls, mean LV longitudinal peak systolic ε was decreased in patients with KD in the convalescent phase of the recurrent episodes.CONCLUSIONSSubclinical decreases in myocardial systolic deformation, as evidenced by decreased LV longitudinal peak systolic ε, may persist in children in the convalescent phase of recurrent KD; further studies involving larger numbers of patients may be needed for verification.

Recurrent Kawasaki disease: USA and Japan.

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.

Abstract 2: Historical Trends in Kawasaki Disease over Three Decades in a Single Institution

Purpose: Kawasaki disease (KD) awareness and therapy have evolved since its description in 1967. We explored historical trends since 1984 in patients (pts) treated for KD at a single institution. Methods: Inclusion criteria were 1) first episode of KD, and 2) treated with IVIG within 3 weeks of illness onset. Exclusion criteria were 1) evaluation solely for a second opinion, and 2) presence of congenital heart disease. We reviewed age at diagnosis, sex, race, presence of complete vs. incomplete (fever + &lt;4 clinical criteria), days of fever at initial IVIG treatment, and incidence of retreatment. Changes in pt characteristics over time were evaluated using tests of trends. Results: Of 1739 pts, 1133 met eligibility criteria. Race included 61% White, 10% Black, 11% Asian: 6% other, and 12% unknown. At diagnosis, 16% were age &lt;1 years (yrs), 58% were 1&lt;5 yrs, 24% were 5&lt;12 yrs, and 2% were ≥12 yrs. The median age at fever onset was 3 yrs and has not changed significantly over time. Male to female ratio was 59% to 41% and was stable over time. Median fever duration before IVIG treatment in the whole cohort was 7 days. Within the complete vs. incomplete KD groups, illness day at treatment was stable over time. However, median days of fever before IVIG treatment were lower in pts with complete vs. incomplete criteria (6 vs. 8 days, respectively, p&lt;.001). The % of pts treated with IVIG for incomplete KD increased significantly over time. Before 1989, only 1 pt (2%) was treated with IVIG for incomplete KD. From 1990-1994, 11 pts (15%) pts had incomplete criteria, whereas from 2010-2014, 45 pts (27%) received IVIG treatment with incomplete clinical criteria (p=.001). The incidence of retreatment with IVIG also increased, from 9% (1984-1989) to 23% (2010-2014, p&lt;.001). The use of adjunctive therapies (i.e., steroids, cyclosporine, cytoxan, abciximab and/or infliximab) were documented in 104/1133 pts (9%) and increased over time (p&lt;.001). Conclusions: At a single center over 3 decades, treatment of incomplete KD, as well as retreatment with IVIG and use of adjunctive therapies have become increasingly common. Pts who had incomplete diagnostic criteria received IVIG later in their illness. Future studies should assess whether these secular trends have improved outcomes in pts with KD.

Abstract O.55: Utility of Adenosine Cardiac Stress MRI to Evaluate Ischemia in Patients with Kawasaki Disease

Background: Patients with Kawasaki Disease (KD) and a history of coronary artery disease (CAD) are at risk of myocardial ischemia/infarction. Adenosine stress cardiac MRI (CMR) has been increasingly used in adults to evaluate for atherosclerotic CAD. This modality has not been widely used in the evaluation of CAD in children and young adults, but may be useful in those with a history of KD. Methods: Patients with a history of Kawasaki disease and a clinical indication for a stress cardiac MRI were prospectively enrolled in the study. SSFP cine and delayed enhancement CMR (DE-CMR) were performed in a standard manner. Adenosine stress perfusion was performed with administration of adenosine (140 ug/kg/min) for 2-4 minutes and gadolinium (0.1 mmol/kg) using a standard adult protocol. Results: A total of 13 procedures were performed between 2010 and 2014 on 8 patients with a history of KD (ages 8 to 22, 3F/5M). Seven of eight patients presented with chest pain. Seven of eight patients had documented moderate to giant aneurysms and one had a previous coronary bypass operation. Scans were performed 3-16 years after initial episodes of KD. Three of 16 (19%) scans demonstrated inducible regional ischemia in the distribution of coronary abnormalities. Of these, all underwent cardiac catheterization and 1 patient subsequently underwent coronary bypass surgery. All patients with negative scans were followed clinically with no evidence of further symptoms. Conclusion: As a non-invasive imaging modality, adenosine cardiac stress MRI is feasible in patients with KD and coronary abnormalities and may obviate the need for invasive studies in order to rule out significant CAD. Further studies are needed to evaluate this imaging modality as a more definitive test in the evaluation of KD and chest pain.

Endothelial and Platelet Function in Children With Previous Kawasaki Disease

We investigated whether children with a previous Kawasaki disease (KD) have evidence of abnormal vascular and/or platelet function. We included 14 patients with previous KD and 14 matched controls. We assessed endothelial function by flow-mediated dilation (FMD), carotid intima-media thickness (cIMT), coronary microvascular function by coronary blood flow response (CBFR) to cold pressor test, and platelet reactivity by measuring monocyte-platelet aggregates (MPAs) and CD41-platelet expression by flow cytometry. No differences were found between the groups in FMD, cIMT, or CBFR to cold pressor test. The MPAs were similar in patients with KD and controls. CD41-platelet expression, however, was significantly increased in patients with KD compared with controls, both at rest (14.3 ± 1.9 vs 12.4 ± 1.9 mean fluorescence intensity [mfi], P = .01) and after adenosine diphosphate stimulation (19.3 ± 1.3 vs 17 ± 1.7 mfi, P < .001). In conclusion, children with a previous episode of KD showed increased platelet activation, compared with healthy participants despite no apparent vascular abnormality at follow-up.

Clinical features of recurrent Kawasaki disease and its risk factors

The clinical features and risk factors for recurrence of Kawasaki disease (KD) remain unclear. In order to summarize clinical features of recurrent KD and identify risk factors associated with recurrence, we conducted a retrospective review of the medical records of consecutive cases of KD from January 2002 to December 2010. Demographic, clinical, laboratory, and echocardiographic data were analyzed. The maximum coronary artery Z score normalized against body surface area was assessed using coronary artery diameters. At the first onset of recurrent KD, children had longer durations of fever before intravenous immunoglobulin (IVIG) treatment and higher levels of alanine aminotransferase, serum aspartate aminotransferase (AST), and lower hemoglobin levels than those with a single episode of KD. Multivariate logistic regression analysis showed that long durations of fever before IVIG treatment, high AST levels, and reduced hemoglobin levels were significantly associated with recurrent KD. Ten of the 22 recurrent KD children had coronary artery complications during the first onset episode, and six (60 %) of these also had coronary artery complications during the recurrence. Children with longer durations of fever, lower hemoglobin levels, and higher AST levels may be at increased risk for KD and coronary artery complications are more likely to occur in children with recurrent KD if they were present during the first episode.

Atopic Diathesis in Patients with Kawasaki Disease

To determine the association between Kawasaki disease (KD) and atopic diathesis (atopic dermatitis [AD], allergic rhinitis, and asthma) in children younger than 5 years of age. In this nationwide study, we aimed to analyze the association and temporal relationship between KD and atopic diathesis. Data were obtained from the National Health Insurance Research Database of Taiwan from 1997 to 2010. In total, 200 patients with KD younger than 5 years of age and 800 age- and sex-matched control subjects were enrolled. In the whole study population, an increased risk of any concomitant atopic diseases was observed in patients with KD (OR 1.61, 95% CI 1.15-2.26). The risk of AD was increased in male patients between 1 and 5 years of age (OR 3.02, 95% CI 1.22-7.50). More than 60% of the patients developed atopic diseases after the diagnosis of KD. There appears to be an association between KD and risk of AD. Most of the atopic diseases occurred after the episode of KD.

Rate, associated factors and outcomes of recurrence of Kawasaki disease in Ontario, Canada

Previous studies on recurrence of Kawasaki disease (KD) have mostly been limited to Japan, which has an incidence of KD 8-10-fold higher than North America. The aim of the present study was to determine the rate of KD recurrence for patients in Ontario, to identify factors potentially associated with increased odds of recurrence, and to compare the clinical course and outcomes of index and recurrent KD episodes. Review was undertaken of all patients with recurrence of KD identified in Ontario, Canada, from 1995 to 2006. All patients with recurrence of KD (defined as at least three clinical signs of KD in addition to fever ≥ 5 days), presenting ≥ 14 days after the return to baseline from the index episode were included. A total of 1010 patients were followed for 5786 patient-years. During this period a total of 17 recurrent episodes in 16 patients were identified at a median of 1.5 years after the initial episode (2 weeks-5 years). Rate of recurrence of KD was 2.9 episodes/1000 patient-years, which is higher than the expected annual incidence of KD in the same age group (26.2/100,000 per year). No factors associated with increased risk of recurrence were identified, perhaps due to the small number of events. Clinical course and outcomes of the index and recurrent KD episodes were similar. A previous history of KD should increase the index of suspicion for future episodes of KD to allow for rapid recognition, treatment and to achieve optimal outcomes.

Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

IntroductionKawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited.Case presentationWe report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease.ConclusionsIn this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries.

Ultrasound Tissue Characterization of the Myocardium in Patients After Kawasaki Disease

We sought to determine if changes in myocardial physical properties, detected by ultrasound tissue characterization (UTC), are present in asymptomatic children years after an acute episode of Kawasaki disease (KD) and if such changes are related to coronary artery aneurysms (CAs). Myocardial UTC analysis was performed 4.8 + or - 3.4 years after acute KD in 22 patients, mean age 6.6 + or - 3.4 years, with or without CA, who had a normal ECG and normal left ventricular (LV) systolic and diastolic function by echocardiography. Twenty-two age-matched subjects were studied as controls. Cyclic variation of integrated backscatter (cvIBS) and calibrated integrated backscatter (cIBS) were assessed in 16 LV myocardial segments in each patient and control. We found large differences in the UTC data between patients and controls: cvIBS, 7.8 + or - 0.8 vs 8.9 + or - 0.6 dB; cIBS, 28.6 + or - 3.2 vs 25.2 + or - 1.0 dB (P\10-3 for both). The average values of cIBS and cvIBS did not differ significantly between KD patients with and patients without CA or stenosis. In conclusion, UTC analysis demonstrated significant differences in myocardial tissue properties between KD patients and controls, despite similar measures of LV function, independent of coronary artery abnormalities. UTC analysis might improve risk stratification for KD patients.

Cognitive and behaviour assessment following Kawasaki disease—a study from North India

Kawasaki disease (KD) is an acute medium vessel vasculitis of childhood that predominantly affects the coronary arteries [1, 2]. It is the leading cause of acquired heart disease among children in developed countries. It has been described from all parts of the world, including several centres in India [3, 4]. The condition was first described by Dr. Tomisaku Kawasaki [1] in 1967 from Japan and was originally referred to as the ‘mucocutaneous lymph node syndrome’. The clinical features, diagnostic criteria, and treatment modalities have gradually evolved over time [1, 2]. The etiopathogenesis, however, remains obscure [2–5]. The disease can present with protean clinical manifestations which include high grade fever (lasting for several days to weeks), rash, redness of the lips and a typical strawberry tongue, cervical lymph node enlargement, oedema over hands and feet and, later during the convalescent stage, acral periungual desquamation. The diagnosis rests on the recognition of this characteristic temporal sequence of clinical events, although none of the clinical characteristics are, by themselves, pathognomonic [1–4]. Neurological and psychological complications associated with KD have also been noted, although research in this area is limited. Extreme irritability is a common feature during the acute febrile phase of the illness [1, 3, 5]. This usually settles down with treatment. Case studies have reported severe lethargy, semi-coma, facial nerve palsy [6, 7], sensori-neural hearing loss [8], hemiplegia [9], and cerebral infarction [10]. While major neurological sequelae can occur in a small number of those affected, most escape clinically apparent neurological sequelae [1, 3]. King et al. [11] found no differences in cognitive or academic measures of children with a previous episode of KD when compared with their siblings as controls. The authors, however, reported that the risk of a clinically significant behavioural score was 3.3 times greater than the sibling controls [11]. Carlton-Conway et al. [12] studied the behaviour sequelae following acute KD and used two sets of controls—a hospital control group and a sibling control group. The study revealed that 40% of the KD group showed elevated internalising scores as compared to 18% of hospital controls and 13% of sibling controls [12]. Dhillon et al. [13] reviewed 265 children with KD with the aim of identifying significant changes in physical and psychological health as perceived by the child’s caregiver. Results demonstrated that 34% of children suffered from behavioural changes lasting for more than 1 year following clinical recovery from the acute stages of KD [13]. We [14–18] and others [19] have previously reported that the clinical presentation of KD in India is different as compared to Japan and the Western countries. It is, therefore, important to analyse whether these differences extend to aspects of adaptive and cognitive behaviour functioning in affected children. To the best of our knowledge this would be the first study of its kind from India as also from any developing country.

Kawasaki Disease

Kawasaki disease (KD) is an acute systemic inflammatory illness that occurs predominately in children <5 years of age. The reported incidence varies widely depending on the ethnicity of the population and the method of case ascertainment. Recent reports would suggest the annual incidence is ≈20 to 25 per 100 000 children <5 years of age in North America, with the highest reported incidence of 188 being in Japan, where the disease was first described in 1967.1 The illness is self-limited and of unknown cause, but is complicated by a systemic vasculitis with a predilection for small- to medium-sized arteries, particularly the coronary arteries. The majority of patients will have either transient coronary artery dilation or no coronary artery luminal changes as noted on echocardiography. Long-term prognosis for these patients is considered to be excellent. Coronary artery aneurysms occur in 25% of patients, but the prevalence is reduced to ≈4% for patients treated with intravenous immunoglobulin infusion within 10 days of illness onset. Aneurysms are associated with an intense inflammatory cell infiltrate, destruction of the internal elastic lamina, and smooth muscle cell death. Coronary artery involvement is usually maximal within 6 to 8 weeks after the acute episode. Regression of aneurysms can occur primarily through myointimal proliferation, although the arterial structure and function remains abnormal,2 and there is an important ongoing risk of stenoses and occlusions.3 Long-term cardiology assessment and management is required, and some of these patients may require revascularization procedures or, rarely, cardiac transplantation. KD has become the most prevalent acquired cardiac disease in children in developed countries. Article see p 60 Although coronary artery complications are the predominant cause of morbidity and mortality, other cardiovascular abnormalities can occur. Valvulitis is a less prevalent complication, and there have been case reports of important long-term aortic and …