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Objective Precise evaluation of coronary artery abnormalities (CAAs) in Kawasaki disease (KD) is essential. The aim of this study is to determine role of CT coronary angiography (CTCA) for detection of CAAs in distal segments of coronary arteries in patients with KD. Methods CTCA findings of KD patients with distal coronary artery involvement were compared with those on transthoracic echocardiography (TTE) during the period 2013–21. Results Among 176 patients with KD who underwent CTCA (128-Slice Dual Source scanner), 23 (13.06%) had distal CAAs (right coronary—15/23; left anterior descending—14/23; left circumflex—4/23 patients). CTCA identified 60 aneurysms—37 proximal (36 fusiform; 1 saccular) and 23 distal (17 fusiform; 6 saccular); 11 patients with proximal aneurysms had distal contiguous extension; 9 patients showed non-contiguous aneurysms in both proximal and distal segments; 4 patients showed distal segment aneurysms in absence of proximal involvement of same coronary artery; 4 patients had isolated distal CAAs. On TTE, only 40 aneurysms could be identified. Further, distal CAAs could not be identified on TTE. CTCA also identified complications (thrombosis, mural calcification and stenosis) that were missed on TTE. Conclusions CAAs can, at times, occur in distal segments in isolation and also in association with, or extension of, proximal CAAs. CTCA demonstrates CAAs in distal segments of coronary arteries, including branches, in a significant number of children with KD—these cannot be detected on TTE. CTCA may therefore be considered as a complimentary imaging modality in children with KD who have CAAs on TTE. [ABSTRACT FROM AUTHOR]

KD can also be complicated by MAS, which has been reported in up to 2% of all cases.6 Diagnosis of MAS in KD is mostly extrapolated from studies in systemic juvenile idiopathic arthritis and other inflammatory conditions, with sparse literature on the specific use of these diagnostic criteria in KD. Severe myocarditis and macrophage activation syndrome (MAS) can complicate Kawasaki disease (KD) and a high index of suspicion is necessary for prompt recognition and timely management. Reported therapies for KD-associated MAS include intravenous glucocorticoids, IVIg, cyclosporine A, etoposide and biologics.7 In the index case, myocarditis and MAS were refractory and required additional therapy. [Extracted from the article]

Kawasaki disease (KD), an enigmatic medium vessel vasculitis, presents as an acute febrile illness predominantly affecting young children. KD appears to be a hyper-inflammatory response elicited by environmental or infectious agents (including respiratory viruses) in genetically predisposed individuals. Numerous reports from the current era of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic have described the occurrence of KD/KD-like illness in close temporal proximity to SARS-CoV-2 infection or exposure. Notably, KD has been reported in association with H1N1-pdm09 virus that caused the previous pandemic a decade ago. Non-H1N1 influenza infections as well as influenza vaccination have also been reported to trigger KD. Herein, we report a case of H1N1-pdm09 influenza who developed KD. We review the published literature on influenza infection or vaccination triggering KD. This may help in a better understanding of the KD/KD-like illness associated with SARS-CoV-2. Besides, we also evaluate the safety of aspirin in influenza-triggered KD as aspirin administration in children with influenza is associated with the risk of development of Reye syndrome. [ABSTRACT FROM AUTHOR]

Objectives To carry out a review of clinical characteristics, laboratory profiles, management and outcomes of patients with Kawasaki disease (KD) and macrophage activation syndrome (MAS). Methods Medical records of patients treated for KD and MAS between January 1994 and December 2019 were reviewed. Patient demographics, clinical signs, laboratory values, coronary artery abnormalities, treatments and outcomes of patients with KD and MAS were recorded. We also performed a review published studies on the subject. Results Of the 950 cases with KD, 12 (1.3%; 10 boys, 2 girls) were diagnosed with MAS. The median age at diagnosis was 4 years (range 9 months–7.5 years). The median interval between onset of fever and diagnosis of KD was 11 days (range 6–30). Thrombocytopenia was seen in 11 patients. The median pro-brain natriuretic peptide value was 2101 pg/ml (range 164–75 911). Coronary artery abnormalities were seen in 5 (41.7%) patients; 2 had dilatation of the left main coronary artery (LMCA), 1 had dilatation of both the LMCA and right coronary artery (RCA), 1 had dilatation of the RCA and 1 had bright coronary arteries. All patients received IVIG as first-line therapy for KD. MAS was treated with i.v. methylprednisolone pulses followed by tapering doses of oral prednisolone. Additional therapy included i.v. infliximab (n  = 4), second-dose IVIG (n  = 1) and oral ciclosporin (n  = 1). Conclusion MAS is an unusual and underrecognized complication of KD. In our cohort of 950 patients with KD, 1.3% had developed MAS. KD with MAS is associated with an increased propensity towards development of coronary artery abnormalities. [ABSTRACT FROM AUTHOR]

Background: Platelet activation is an integral part of pathogenesis of Kawasaki disease (KD). However, there is paucity of literature on flow-cytometry based assessment of platelet activation in KD. We aimed to analyse monocyte-platelet aggregates (MPAs), one of the sensitive markers for platelet activation, by flow cytometry in children with KD. Findings: In this single-centre prospective study, we have enrolled 14 children with KD and results were compared with age-matched febrile (n = 15) and healthy (n = 13) controls. After gating monocytes in side-scatter plot, MPAs were identified based on CD14 and CD41 expression. Two (2) ml of blood samples for children with KD were collected at 3 phases of illness- acute stage before start of intravenous immunoglobulin or aspirin, 24 h after completion of IVIg infusion, and 3 months after acute episode of KD. Children with KD had a significantly higher MPA% values [Median (IQR)- 41.3% (26.6, 52.7)] when compared with febrile [Median (IQR)- 5.98% (2.98-9.72)] and normal [Median (IQR)- 4.48% (2.57-5.59)] controls, p<0.01. On follow-up, the MPA% showed a gradual decline in children with KD, but even at 3 months, the value [Median (IQR)- 7.55% (4.15-14.6)] was higher compared to healthy controls [Median (IQR)- 4.48% (2.57-5.59)]. Conclusions: Our results suggest that MPA% was significantly elevated in acute stages in children with KD and activated platelets may continue to persist even after systemic inflammation has subsided. Future studies are warranted whether objective evidence of platelet activation may guide the use of immunomodulatory and anti-platelet therapy in KD. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is associated with several musculoskeletal manifestations. Although arthritis has been reported to occur in 2.3-31% of children with KD, there is paucity of detailed studies on the subject. We report our experience on arthritis in children with KD. Data were collated from a review of records of patients diagnosed with KD and arthritis during the period January 1994-June 2019. Eight hundred sixty-five children (male:female 29:11) were diagnosed with KD during this period-of these, 40 (4.6%) had arthritis. Median day of diagnosis of KD was 17 days. Twenty-nine (72.5%), 8 (20%), and 3 (8.6%) children developed arthritis in acute, subacute, and convalescent phases of KD, respectively. Oligoarticular involvement was observed in 32 (80%) children and among these, 7 (20%) had monoarthritis. Predominant joints involved were knee (74.3%), ankle (40%), and hip (28.6%). Thirty-two children (80%) were treated with non-steroidal anti-inflammatory drugs (NSAIDs). Median duration of arthritis was 10 days (range, 2-180 days) with uneventful recovery in all cases. Three (7.5%) children had coronary artery ectasia which regressed on follow-up.Conclusion: Arthritis in KD is usually non-erosive, self-limiting, and responds well to a short course of NSAIDs.What is Known:• Arthritis has been reported to occur in 2.3-31% of children with KD.• Arthritis in KD is usually oligoarticular, non-erosive, and responds well to short course of non-steroidal anti-inflammatory drugs.What is New:• Children with KD and arthritis do not appear to be at increased risk of development of coronary artery abnormalities.• Arthritis in children with KD can result in diagnostic confusion, and diagnosis of KD may get delayed. [ABSTRACT FROM AUTHOR]

A clinical possibility of incomplete Kawasaki disease (KD) was considered. 8822996 3 Petruni'c M, Drinkovi'c N, Stern-Padovan R, Mestrovi'c T, Lovri'c D. Thoracoabdominal and coronary arterial aneurysms in a young man with a history of Kawasaki disease. [Extracted from the article]

There is evidence for premature atherosclerosis and systemic arterial stiffening during follow-up of children with Kawasaki disease (KD) and coronary artery abnormalities (CAA). Moreover, patients with KD may also have subclinical myocardial involvement and inhomogeneous ventricular repolarization. The inhomogeneous ventricular repolarization manifests as increased QT dispersion on electrocardiography. There is a paucity of studies in endothelial dysfunction and QT dispersion in children with KD and transient CAA. Twenty children with KD and transient CAA were studied at least 1 year after resolution of CAA. Mean follow-up period between KD onset and enrolment in the study was 53.7 months. Twenty age and sex-matched controls were enrolled. High-resolution B-mode ultrasonography was used to analyze brachial artery dilatation in response to reactive hyperemia (cases and controls) and sublingual nitroglycerine (cases only). Carotid artery intima-media thickness (cIMT) and stiffness index were calculated. The difference between maximum and minimum QTc intervals on 12 lead electrocardiogram was calculated as QTc dispersion (QTcd). No statistically significant difference was noted in percent flow-mediated dilatation of brachial arteries in response to reactive hyperemia between cases (13.31 ± 10.41%) and controls (12.86 ± 7.09%). Sublingual nitroglycerine-mediated dilatation in children with KD was 14.88 ± 12.03%. Mean cIMT was similar in cases (0.036 ± 0.015 cm) and controls (0.035 ± 0.076 cm; p = 0.791). No statistically significant difference between groups was observed in mean QTcd values (0.057 ± 0.018 s vs. 0.059 ± 0.015 s in controls, p = 0.785). No evidence of significant endothelial dysfunction or increased QT dispersion in patients with KD and transient coronary artery abnormalities was found in our cohort when studied at a mean follow-up of 53.7 months. This is reassuring, and indicates that risk of subclinical atherosclerosis and myocarditis in a subset of children with KD and transient coronary artery abnormalities is not significant. [ABSTRACT FROM AUTHOR]

Epidemiological case definition of Kawasaki disease (KD) by the American Heart Association requires the presence of fever and four of the following: eye signs, oral mucosal changes, skin rashes, limb edema, and unilateral cervical lymphadenopathy. Incomplete KD is a well-known entity where there is lack of some of mucocutaneous features, and this occurs more often in infants. We report a 5-year-old boy with KD and giant coronary aneurysms, who presented only with fever and there is complete lack of skin and mucosal manifestations at presentation. [ABSTRACT FROM AUTHOR]

The article describes the case of 5-year-old boy with fever for seven days associated with red, dry, cracked lips and had hyper-keratotic indurated plaques on his palms and soles. Topics discussed include his diagnosis of Kawasaki disease and administration of intravenous immunoglobulin (IVIG), findings on the histopathology of the skin, and clinical characteristics of psoriasiform lesions associated with Kawasaki disease.

Given progressive thrombocytosis, sterile arthritis, elevated serum Pro-BNP levels, and the presence of Beau's lines, intravenous immunoglobulin (IVIg) 2 g/kg was administered for suspected Kawasaki disease (KD). He had a significant clinical response to IVIg therapy.[1] Gall-bladder hydrops occurs in 5-13% of children with acute KD.[2] Gall-bladder perforation has been reported once previously in a 2-year-old child with KD.[3] The histopathological findings seen in our patient are consistent with KD.[4] To conclude, gall-bladder perforation can be a presenting feature of KD. 4 Sun Q, Zhang J, Yang Y. Gallbladder hydrops associated with Kawasaki disease: A case report and literature review. [Extracted from the article]

Considering the diagnosis of Kawasaki disease (KD), he was given intravenous immunoglobulin 2 g/kg, aspirin and became afebrile. Ultrasound examination revealed bulky left epididymis measuring 1 × 0.6 cm (Fig. b) with evidence of minimal fluid collection in left scrotal sac, suggestive of epididymitis. There is a paucity of reports on this aspect of KD.[1] The cause of epididymitis in KD is not well understood and ascribed to either increased vascular permeability or testicular arteritis that developed during the acute phase of KD.[[1]] The colour of the scrotum either become red or may remain normal. [Extracted from the article]

Kawasaki disease (KD) is the commonest medium vessel vasculitis in children. The etiology of KD remains an enigma despite extensive research. Infections are considered to be one of the triggers for KD, especially in genetically susceptible hosts. KD occurring within a short time interval among siblings is an important clinical observation supporting this hypothesis. In addition, siblings of children with KD are at a higher risk of developing the disease as compared with other children. Screening for KD in febrile siblings, therefore, seems prudent. This would help initiate timely therapy and prevent complications. We briefly review 16 English language reports of KD in siblings diagnosed within 1 month of each other to highlight its etiological and therapeutic implications. Key Points • KD should be suspected in febrile children who have a sibling recently diagnosed with KD. • Etiological studies should also focus on siblings who develop KD in close temporal proximity. [ABSTRACT FROM AUTHOR]

Juvenile systemic lupus erythematosus (SLE) is a heterogeneous multisystem autoimmune disease. Kawasaki disease is a common vasculitic disorder in children that manifests with fever and mucocutaneous involvement. While overlap of childhood SLE with other rheumatological disorders has been described, it is extremely unusual in the context of Kawasaki disease. We report two children who had SLE and developed features of Kawasaki disease simultaneously, and the second child had myocarditis which could be a manifestation of Kawasaki disease rather than SLE. Two or more rheumatological diseases may coexist at the same time and one must always be vigilant. [ABSTRACT FROM AUTHOR]

Objective: To investigate the value of two-dimensional ultrasound speckle tracking (2D-STI) and three-dimensional ultrasound speckle tracking (3D-STI) in evaluating myocardial function in children with Kawasaki disease. Methods 92 children with Kawasaki disease admitted to our hospital from February 2017 to February 2019 were retrospectively analyzed. 50 children who underwent 3D-STI examination were taken as observation group and 42 children who underwent 2D-STI examination were taken as control group. The left ventricular systolic function index, storage time and analysis time of the image, the diameter of coronary artery, the strain difference of left ventricular basal segment, middle segment, apical segment and whole segment were observed. Results The levels of left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), left ventricular myocardial mass (LVMI) in the observation group were higher than those in the control group (P < 0.05), but there was no statistical difference in left ventricular ejection fraction (LVEF) between the two groups (P > 0.05). The storage time and analysis time of the image in the observation group were significantly lower than those in the control group (P < 0.05). The left coronary artery (LCA) and right coronary artery (RCA) in the observation group were higher than those in the control group (P < 0.05). There was no statistical difference between left anterior descending (LAD) in the two groups (P > 0.05). The longitudinal peak systolic strain (LS), circumferential peak systolic strain (CS) and radial peak systolic strain (RS) in the observation group were higher than those in the control group (P < 0.05). The global longitudinal peak strain (GLS), global circumferential peak strain (GCS) and global radial peak strain (GRS) in the observation group were higher than those in the control group (P < 0.05). LS and CS in the middle segment of the observation group were higher than those in the control group (P < 0.05). Conclusions Compared with 2D-STI, 3D-STI can objectively and accurately reflect the myocardial function of children with Kawasaki disease. [ABSTRACT FROM AUTHOR]

Background: 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. Objective: To study the clinico‐laboratory profile of children with recurrent KD. Methods: 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. Results: Seven (0.98%) children (five boys; two girls) had recurrent KD in our cohort of 714 patients. Mean age at diagnosis was 4.3 years (range, 2‐9 years). Six (85.7%) had recurrence of KD within 1.5 years. 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. Conclusion: Recurrences in KD are unusual. Over a period of 24 years 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. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings. [ABSTRACT FROM AUTHOR]