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Background: Connective tissue diseases (CTDs) are chronic in nature and carry a high risk of morbidity, disability, and mortality if not recognized and treated promptly. Data on clinical spectrum of CTDs in the pediatric age group are sparse from developing countries such as India. Objective: To study the clinicoepidemiological spectrum of CTDs in the pediatric age group. Methodology: A hospital-based, cross-sectional, observational study was undertaken over a period of 16 months from January 2021 to June 2022 in the dermatology outpatient department and pediatric rheumatology clinic in a tertiary care hospital in North India. Clinical and laboratory findings were recorded. Results: A total of 100 children diagnosed with a CTD were included in the study. The mean age was 11.11 ± 4.20 years. Out of 100 children, 55% were female and 45% were male with a male-to-female ratio of 1:1.2. Our study included 63% juvenile idiopathic arthritis, 17% systemic lupus erythematosus, 7% juvenile dermatomyositis, 6% Kawasaki disease, 3% systemic sclerosis, and 2% Henoch-Schonlein purpura and mixed CTD patients each. Mucocutaneous features commonly seen were rash (40%), oral ulcers (22%), and photosensitivity (18%). Commonly seen systemic features were joint pain (77%), followed by fever (63%). Conclusion: Mucocutaneous manifestations are frequently seen in CTDs and constitute a frequent and an early manifestation of CTD. Early recognition of CTD in the pediatric age group will help in timely initiation of treatment to prevent disabilities and mortality. [ABSTRACT FROM AUTHOR]

Objectives: To describe the clinical presentation, phenotype and outcome of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) from a tertiary care center in southern India. Methods: 257 children fulfilling the inclusion criteria of MIS-C were prospectively enrolled from June, 2020 to March, 2022. Results: Median (range) age at presentation was 6 year (35 day to 12 years). Presenting features were fever (98%), vomiting (75.8%), red eyes (63%), rashes (49%), pain abdomen (49%), shock (45.9%), lymphopenia (73%, thrombocytopenia (58.3%) and anemia (45%). 103 (39.7%) children required intensive care admission. Shock phenotype, Kawasaki-like phenotype and no specific phenotype were diagnosed in 45.9%, 44.4%, and 36.6% children, respectively. Left ventricular dysfunction (30.3%), acute kidney injury (13%), acute liver failure (17.4%), and hemophagolymphohistiocytosis (HLH) (13.6%) were the major system involvement in MIS-C. Mitral regurgitation (P=0.029), hyperechogenic coronaries (P=0.006), left ventricular dysfunction (P=0.001) and low ejection fraction (P=0.007) were significantly associated with shock. Overall mortality was 11.7%. Conclusions: Kawasaki-like and shock-like presentation were common in MIS-C. Coronary abnormalities were seen in 118 (45.9%) children. Children with acute kidney injury, HLH, need for mechanical ventilation, and echocardiogram evidence of mitral regurgitation in MIS-C have a poor outcome. [ABSTRACT FROM AUTHOR]

Introduction: Multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2 caused significant health concerns worldwide. In our center, we had encountered the first wave of MIS-C from June 2020 to January 2021, whereas the second wave surged up from April 2021 to August 2021. In this study, we have done a comparative analysis of different phenotypes of MIS-C seen during these two waves. Subjects and Methods: This was a single-center observational study where the children fulfilled the WHO criteria for MIS-C were included in the study. Clinical and laboratory findings, course of the illness, treatment, and outcome were noted down, and the patients were followed up. Depending on the presentations, cases were classified in four different phenotypes (Type 1: MIS-C overlapping with acute COVID-19, Type 2: MIS-C with shock/MIS-C with multiple organ dysfunction syndrome (MODS), Type 3: MIS-C Kawasaki disease phenotype, Type 4: Mild MIS-C/Febrile inflammatory state), and a comparative analysis of these phenotypes in the two waves was done. Results: There were 86 cases in 7 months during the first wave, whereas 102 cases in 5-month duration during the second wave. The clinical manifestations and laboratory findings were compared, type 2 phenotypes increased in proportion requiring more pediatric intensive care unit admissions. Mortality was seen during the 2nd wave which was absent in our cohort during the first wave. Conclusions: MIS-C typically showed a spectrum of disease manifestations starting from a mild febrile inflammatory state to full-blown MODS. Early phenotypic differentiation and targeted immunomodulatory therapy depending on the phenotype had shown to be useful. [ABSTRACT FROM AUTHOR]

Objectives: To assess the risk factors of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) and to evaluate the performance of the three Japanese risk-scoring systems, namely the Kobayashi, Egami, and Sano scores in predicting IVIG resistance among the Indian patients. Methods: Prospective observational study on children admitted with KD at Institute of Child Health, Kolkata, over a period of 16 months, from January 2019 to April 2020. The study included 70 KD patients all of whom were treated with IVIG. Clinical parameters, laboratory variables, and risk scores were compared between the IVIG-responsive and the IVIG-resistant groups. Results: A total of 31.4% were IVIG non-responders. Skin rash was found to be significantly associated with IVIG-resistant KD. The IVIG-resistant group had higher total bilirubin, lower albumin, higher CRP levels, and higher ALT and AST levels. High Kobayashi score, high Egami score, and high Sano score were significantly associated with IVIG resistance, individually. Sano score had the highest sensitivity (81.8%) and Kobayashi score had the highest specificity (77.1%) in our cohort. Conclusion: The presence of skin rash, high total bilirubin, high CRP, high AST, high ALT, and low albumin were important predictors of IVIG resistance in our population. Among the three scores, Sano score is the most reliable in identifying potential non-responders to IVIG. But Sano score lacked good specificity. Therefore, Indian KD patients may need an exclusive scoring system to predict non-responsiveness to IVIG so that a more aggressive therapy can be instituted at the earliest. Key points • Early prediction of IVIG-resistant KD is necessary to limit cardiac injuries. • Sano score has high sensitivity to predict IVIG resistance in Indian population. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease is a pediatric, systemic, vasculitic disorder. Its exact etiology is still unknown. Genetic polymorphisms are being investigated as susceptibility factor for this disorder. These are likely to vary among different populations. Aim: To investigate the association of single nucleotide polymorphism (SNP) rs113420705 of CASP3 in Kawasaki disease (KD) from North India. Settings and Design: Observational, case–control study. Methods: Polymerase chain reaction and bidirectional Sanger sequencing was used for determining genotypes of SNP rs113420705 in 45 cases of KD and 50 healthy age- and sex-matched controls. Allele and genotype frequencies were assessed and compared between the groups. Results: Among 45 cases, 32 had TT (71.1%), 13 had CT (28.9%) and none had CC genotype of SNP rs113420705. No significant differences in allele, genotype, or carrier frequencies of rs113420705 were found between the two groups. A comparison was also made between subgroups of KD with coronary abnormality (7 children; 15.5%) and KD with normal coronaries (38 children; 84.4%). The C allele was significantly overexpressed in KD with coronary abnormality group (P = 0.005). However, no difference was noted in the genotype frequencies. Conclusion: CT genotype of rs113420705 of CASP3 showed a trend to significance with the occurrence of KD in children in North India. However, we could not establish any association between minor allele C and susceptibility to KD. C allele appeared to be over expressed in children with KD with coronary abnormalities. Larger studies will help us to reach conclusive evidence applicable to all ethnicities. [ABSTRACT FROM AUTHOR]

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a new entity affecting a small percentage of children during the COVID-19 pandemic. Materials and methods: Demography, clinical, and laboratory variables of children admitted from April to September 2020 with MIS-C were studied retrospectively at eight hospitals in Delhi, India. Results: We identified 120 patients [median age: 7 years (interquartile range (IQR): 4-10)] with male-to-female ratio of 2.3:1. Overall, 73 out of 120 children (60.8%) presented with shock, 63 (52.5%) required inopressor support, and 51 (43%) required respiratory support. We categorized the cohort into three observed clinical phenotypes: MIS-C with shock (n = 63), MIS-C with Kawasaki disease (KD) (n = 23), and MIS-C without shock and KD (n = 34). Atypical presentations were hypothermia, orchitis, meningoencephalitis, demyelination, polyneuropathy, pancreatitis, and appendicitis. Ninety-four percent had laboratory evidence of SARS-CoV-2 (78.3%, seropositive and 15.8%, RT-PCR positive). The median C-reactive protein (CRP) was 136 mg/L (IQR, 63.5-212.5) and ferritin was 543 ng/mL (IQR, 225-1,127). More than 90% received immunomodulatory therapy (intravenous immunoglobulins and/or steroids) with an excellent outcome (96% survived). CRP and absolute neutrophil count (ANC) were correlated statistically with severity. Conclusion: MIS-C data from Delhi are presented. Rising CRP and ANC predict the severe MIS-C. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Multisystem inflammatory syndrome in children (MIS-C) is a new hyperinflammatory variant that evolved during the coronavirus disease 2019 pandemic. Although the precise pathophysiology of MIS-C is uncertain, it is thought to be due to immune dysregulation occurring after recovery from acute infection. Purpose: Our study aimed to analyze the clinical spectrum, laboratory parameters, imaging characteristics, treatment strategies, and short-term outcomes of children with a diagnosis of MIS-C. Methods: This retrospective and prospective observational study included children less than 16 years of age who were admitted to the pediatric unit of a tertiary care teaching hospital in south India between August 2020 to January 2021 with a diagnosis of MIS-C according to World Health Organization criteria. Results: Twenty-one children were included in the analysis; all had fever with variable combinations of other symptoms. The mean age was 6.9 years; 71.4% were male. Gastrointestinal (80.9%) and cardiovascular (80.9%) systems were the most commonly affected. The majority of children had elevated inflammatory markers, and 16 (76.2%) had echocardiographic abnormalities mimicking Kawasaki disease. Eleven children (52.4%) required intensive care admission, 3 (14.3%) required supplemental oxygen, and 4 (19%) required inotropes. Nine (42.9%) were treated with intravenous immunoglobulin alone, 6 (28.6%) with steroids alone, and 3 (14.3%) with steroids and immunoglobulin. The median hospital stay was 6 days; there were no fatalities. Overweight/obesity, elevated ferritin, and mucocutaneous involvement were significantly associated with a prolonged hospital stay (=7 days). Sixteen children (76.2%) were followed up till now and all of them had no clinical concerns. Conclusion: MIS-C is an emerging disease with variable presentation. A high index of suspicion is necessary for its early identification and appropriate management. Further research is essential for developing optimal treatment strategies. [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]

Aim: This is a retrospective study to report our experience with a cohort of 73 patients with Kawasaki disease (KD) over 2.5 years. Method: The study was conducted in the Department of Pediatrics. Data were retrieved from medical records of Pediatric Rheumatology and Immunodeficiency Clinic collected from April 2017 to October 2019 and analyzed. Results: Male‐to‐female ratio in our cohort was 2:1. The median age at diagnosis of KD was 3 years (IQR, 4.25). Fever was present in all patients. Oral mucosal changes are the second most common symptom (N = 64, 87%) followed by extremity changes (N = 58, 79%), and rash (N = 56, 76%). Nineteen (26%) children had cardiovascular complications like coronary artery abnormalities (N = 15, 20%), cardiac tamponade (N = 2, 2%), and shock (N = 1, 1%). The effusion in the patients with cardiac tamponade contained inflammatory cells and plenty of red blood cells. Sixty‐eight (93%) patients with KD had received treatment with IVIg. Patients in our cohort had completed a mean follow‐up of 13.6 ± 9.4 months. No fatality or any long term adverse effects were observed on follow‐up. Conclusion: Kawasaki disease is a common rheumatological disorder in children at our center with diverse clinical presentations. The disease needs to be considered as a differential diagnosis in an acute febrile illness in children persisting up to 5 days. [ABSTRACT FROM AUTHOR]

Objectives: We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India. Methods: This is an observational study conducted at four tertiary hospitals in Mumbai. Parameters including demographics, symptomatology, laboratory markers, medications and outcome were obtained from patient hospital records and analyzed in patients treated for MIS-C (as per WHO criteria) from 1 May, 2020 to 15 July, 2020. Results: 23 patients (11 males) with median (range) age of 7.2 (0.8–14) years were included. COVID-19 RT-PCR or antibody was positive in 39.1% and 30.4%, respectively; 34.8% had a positive contact. 65% patients presented in shock; these children had a higher age (P=0.05), and significantly higher incidence of myocarditis with elevated troponin, NT pro BNP and left ventricular dysfunction, along with significant neutrophilia and lymphopenia, as compared to those without shock. Coronary artery dilation was seen in 26% patients overall. Steroids were used most commonly for treatment (96%), usually along with intravenous immunoglobulin (IVIg) (65%). Outcome was good with only one death. Conclusion: Initial data on MIS-C from India is presented. Further studies and longer surveillance of patients with MIS-C are required to improve our diagnostic, treatment and surveillance criteria. [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]

The aim of this study was to estimate incidence of Kawasaki disease (KD) in Chandigarh, North India, during 2009-2014. Diagnosis of KD was based on American Heart Association guidelines. Records of all children diagnosed with KD below 15 years at a large tertiary care referral centre from North India for paediatric immunology were analysed from January 2009 to December 2014. Children residing in Chandigarh were identified. Incidence rates were calculated based on population of Chandigarh in National Census 2011. Methodology was similar to our previously reported study from 1994 to 2008. Incidence of KD in children below 5 was also computed. A total of 258 children were diagnosed to have KD. Of these, 54 (43 boys, 11 girls) resided in Chandigarh. Coronary artery abnormalities on echocardiography were noted in 6. Incidence rate varied between 1.11 (in 2012) and 4.71/100,000 children below 15 (in 2009). In children below 5, incidence rate varied between 1.0 (in 2012) and 9.1/100,000 (in 2009). Peak incidence of KD was in third year of life. There was clustering of cases in February, April, June and October with a nadir in July. While the overall number of KD cases has increased, the 2009-2014 Chandigarh incidence is comparable to our previous figures. Our study is based on hospitalized children with KD and may be missing patients diagnosed elsewhere but that number is likely to be small. Further, patients in whom the diagnosis has never been made would also be missed. Median age at diagnosis has reduced as compared to our previous study. This is probably a reflection of increased awareness about KD amongst paediatricians and physicians in the region as a result of which the proportion of infants and young children diagnosed to have KD has shown a significant increase. Seasonal pattern of occurrence of KD is consistent with our previous observation. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is now the commonest cause of acquired heart disease in children in developed countries. KD occurs all over the world, including developing countries. The present study steps out to explore our hypothesis, driven by clinical observation over the last 18 years, whether children with KD in North India are of a higher socioeconomic status than children with other rheumatologic diseases. One hundred consecutive children with KD, registered in Pediatric Rheumatology Clinic before January 2011, were enrolled as cases. Children with other rheumatologic diseases were taken as controls. Assessment of socioeconomic status was done by administering the Aggarwal scale. Data were collected through interview. Statistical analysis was done using SPSS package version 16. On univariate analysis, male sex, higher educational status of parents, urban residence, immunization status being complete, and higher scores on Aggarwal scale were found to be significantly associated with KD. On multivariate analysis, only male sex and urban residence were found to be significantly associated with KD ( p < 0.001). Families of children with KD tend to have a better sociodemographic profile when compared with other pediatric rheumatologic disorders in North India. These results, however, need to be replicated in a multicentric study for any firm conclusions to be drawn. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is a common cause of cardiovascular morbidity in young children. No study has attempted to stratify risk factors for coronary artery involvement in Indian children. We attempted to study prospectively the risk factors for coronary involvement in children with KD in a tertiary care hospital between October 2009 and November 2011. The clinical details and investigations for all children admitted with KD were tabulated, and echocardiography was performed; 37 children were admitted with KD; and 8 children (21%) had coronary artery abnormalities. Prolonged fever, wider dispersion of symptoms, and pyuria were significantly associated with the development of coronary lesions. Clinical factors such as wider dispersion of symptoms and prolonged fever along with presence of pyuria can increase the risk of coronary lesions. The presence of these factors may help direct aggressive management and prevent loss of precious time. [ABSTRACT FROM PUBLISHER]

Aim Kawasaki disease ( KD) has been associated with abnormal lipid profiles. The latter, in turn, have been linked to changes in subcutaneous fat. In this comparative cross-sectional study we have quantified distribution of subcutaneous fat during follow-up of a cohort of North Indian children with KD. Method We compared 35 KD children (at least 2 years after disease) and 33 healthy controls. Study parameters included weight, height and skinfold thickness ( SFT) over biceps, triceps, midaxillary, subscapular, medial calf and suprailiac areas. Waist and hip circumferences were also recorded. All parameters were measured four times at 6-monthly intervals using standardized techniques. Serum lipids were assayed in the study group. Results Study children were enrolled 3.7 ± 2.5 years after KD and mean age at enrolment was 8.26 ± 3.65 years. Suprailiac SFT measured higher in boys with KD ( P ≤ 0.05). Biceps SFT was higher in the study group, but the difference was not significant. Other SFT were not affected. Waist and hip circumference was higher in the study group than controls ( P ≤ 0.05). Waist/hip circumference ratio was not affected. Serum low-density lipoprotein cholesterol (LDL-C) and triglycerides were higher in the study group as compared to historical controls (95.60 ± 36.12 and 129.40 ± 64.62 mg/dL vs. 80.10 ± 2.20 and 91.1 ± 29.85; P ≤ 0.05). Total cholesterol and high-density lipoprotein cholesterol levels remained unaffected. Conclusion Children with KD (especially boys) had increased subcutaneous fat deposition in the suprailiac region and waist, during follow-up. Serum LDL-C and triglycerides were elevated. KD children may have a tendency to develop central obesity. Further studies, with longer follow-up, would be necessary to show whether this has implications for development of coronary artery disease later in life. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease (KD) is the commonest systemic vasculitis in children. It predisposes to development of coronary artery abnormalities (CAAs). Thrombomodulin (THBD) gene polymorphism rs1042579 is associated with high risk of cerebrovascular diseases. However, association of THBD polymorphism (rs1042579) and plasma thrombomodulin (TM) levels with susceptibility to KD and CAAs remains unclear., Methods and Results: Polymorphism in THBD gene (rs1042579) was analysed in 50 KD patients and 50 age, gender and ethnicity matched controls using Sanger sequencing. Plasma TM levels were measured by ELISA., Results: Mean plasma TM level (± SD) in KD patients was 2549.41 (± 853.18) pg/ml and in controls was 2298.03 (± 869.14) pg/ml; p = 0.042. Mean plasma TM levels in CC genotype was 2299.98 (± 834.88) pg/ml and in CT/TT genotype was 2837.96 (± 857.14) pg/ml; p = 0.005. Genotyping data did not reveal significant differences in patients with KD as compared to controls (p = 0.25), and in KD patients with and without CAAs (p = 0.407). Odds of finding T allele in cases were 2.07 times greater than in controls (p = 0.093)., Conclusions: This is the first study from India, and second in the world, that investigates association of THBD gene polymorphism with KD. This is also the first study to assess plasma TM levels in KD patients. Our data show that plasma TM levels were significantly higher in KD patients with CT/TT genotypes. Further, the polymorphism rs1042579 at exon 1 of THBD gene was found to be more common in KD patients than in controls although the difference was not statistically significant., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

The objective of the study was to evaluate the epidemiologic trends (1993–2008) in Kawasaki disease (KD) and Henoch Schonlein purpura (HSP) among children as seen in a tertiary care north Indian hospital. A hospital-based retrospective chart analysis of all the patients diagnosed with KD and HSP in our pediatric rheumatology clinic from January 1993 to December 2008 was performed. The diagnosis of KD and HSP was based on the American Heart Association and American College of Rheumatology criteria respectively. The collected data were analyzed for any yearly or seasonal trends in the incidence of the two diseases. KD was diagnosed in 196 patients (135 males, 61 females, ratio 2.21:1), while HSP was diagnosed in 209 patients (155 males, 54 females, ratio 2.87:1). The median age at the time of diagnosis was 5.1 ± 3.04 years (range 4 months–14 years) for KD and 6.9 ± 2.98 years (range 1–17 years) for HSP. Over this 16-year period, the number of patients diagnosed with KD progressively increased from 1993 to 2008 ( p < 0.001, chi-square test) with a clear majority of these (162 cases, 82.6%) being diagnosed in the last 8 years. The number of children diagnosed with HSP progressively increased from 1993 to 1997, after which it has remained relatively uniform, and only 93 cases (44.5%) were diagnosed in the last 8-year period ( p > 0.05, chi-square test). However, both the diseases exhibited a comparable seasonal trend in the distribution with a noticeable peak being discernible in the months of October and November. KD has shown a rising trend over the recent years in our hospital. Since 2004; the annual number of KD cases has outnumbered the HSP cases. Seasonal predilection is noticeable for both conditions. This is the first hospital-based report on epidemiological trends of KD and HSP from a developing country. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) was first reported from Japan in 1967 by a young pediatrician, Tomisaku Kawasaki, while working at the Red Cross Hospital in Tokyo. Soon thereafter, Marian Melish independently reported children with a similar clinical profile from Hawaii in the United States. KD has now been reported from all parts of the world, including several centers in India. Based on the epidemiology and clinical features, an infectious etiology has been suspected for long but no definitive causative agent has been implicated so far. Like many other vasculitides, the diagnosis of this condition is based on the recognition of a temporal sequence of clinical features, none of which is pathognomonic in isolation. KD is believed to be the commonest vasculitic disorder of children. Incidence rates as high as 60-150 per 100,000 children below 5 years of age have been reported from several countries. In India (as also perhaps in many other developing countries), however, majority of children with KD continue to remain undiagnosed probably because of the lack of awareness amongst pediatricians. The clinical features of KD can be confused with other common conditions like scarlet fever and the Stevens Johnson syndrome, if the clinician is not careful. Development of coronary artery abnormalities (CAA) is the hallmark of KD and accounts for most of the morbidity and mortality associated with the disease. Prompt recognition of the disease and early initiation of treatment with intravenous immunoglobulin (IVIG) results in significant reduction in the occurrence of CAA. It is, therefore, imperative for the pediatrician to diagnose and treat KD expeditiously. KD should be considered in the differential diagnosis of all febrile illnesses in young children where the fever persists for more than 5-7 days. [ABSTRACT FROM AUTHOR]

Severe acute respiratory syndrome-coronavirus-2 (SARS CoV-2) is a novel virus. There has been an increasing number of case reports on multisystem inflammatory syndrome in children (MIS-C) but the global and population-specific incidence of MIS-C particularly in Asian countries, its causal relationship with SARS-CoV-2 and its immunopathogenesis remain unknown. Emerging questions on how the pathophysiology of MIS-C differs from that of Kawasaki disease (KD) and non-KD inflammatory syndromes need to be answered. Genetic factors influencing the incidence of MIS-C in the different ethnic populations are to be explored. What happens to the children with MIS-C, in the long run, remains unknown to date. Multicenter clinical trials are needed to establish optimal treatment and follow-up for MIS-C. [ABSTRACT FROM AUTHOR]

A study of organ-specific and organ-nonspecific autoantibodies was carried out in 21 children being followed up after a diagnosis of Kawasaki disease at a tertiary care center of North India. Anti-nuclear antibodies were detected in 9.5% patients while anti-thyroid microsomal antibodies were detected in 23.9% patients. Other autoantibodies (e.g. anti-parietal cell antibody, anti-liver kidney microsomal antibody anti-neutrophil cytoplasmic antibody, anti-mitochondrial antibody and anti-smooth muscle antibody) were not detected in any of our patients. Children with Kawasaki disease need to be monitored for the development of autoantibodies during follow-up. [ABSTRACT FROM AUTHOR]

Background: Post-COVID multisystem hyperinflammatory syndrome in children (MISC) has clinical and laboratory similarities with Kawasaki disease (KD). Inflammatory markers like C-reactive protein (CRP), interleukin 6 (IL6) as well as N-terminal probrain natriuretic peptide (NT-proBNP) are elevated in both. This study attempts a comparative analysis of the 3 markers in an attempt at early differentiation for planning appropriate management., Methodology: This analytical study conducted at the Institute of Child Health, Kolkata, India compared the levels of the above 3 markers at admission between 72 patients with KD, 30% of whom had coronary artery lesions (CALs) collected over a period of 18 months (Jan 2017-June 2018), with 71 MISC patients over a period of 6 months (July 2020-December 2020). The non-parametric Mann-Whitney U test was used to test for similarity in distributions of the samples of CRP, NT-proBNP and IL6 in KD and MISC patients using correction factor for similar ranks. The 3 parameters were compared using receiver operating characteristic (ROC) curve analysis., Results: Mean IL6 value in KD was 83.22 pg/mL and in MISC 199.91 pg/mL, which was not found to be statistically significant (P = .322 > .05).However mean NT-proBNP (914.91 pg/mL) with CRP level (96.32 mg/L) in KD was significantly lower (P < .05 for both cases) than that in MISC (9141.16 pg/mL and 145.66 mg/L respectively). ROC analysis showed NT-proBNP has the best sensitivity and specificity in predicting MISC., Conclusion: NT-proBNP and CRP are significantly higher among MISC patients; ROC analysis shows levels >935.7 pg/mL and >99.55 mg/L respectively might act as a guide to differentiate between them., (© 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

A retrospective analysis was carried out to identify factors associated with delay in diagnosis of Kawasaki disease in a tertiary care pediatric hospital setting in Chennai, India. Over a period of 2 years, a total of 37 cases were studied. The cases were divided into Early Diagnosis Group (EDG) and Delayed Diagnosis Group (DDG) with the cut-off for early diagnosis being ten days. A greater proportion of cases in the EDG presented primarily to our institution ( P=0.004). In the DDG group greater number of cases had received medical attention from practicing pediatricians prior to referral. There was greater interval in onset of individual symptoms in the DDG group. There was no difference between the two groups with regard to age, gender, total blood counts, CRP, liver enzymes, urine analysis or serum albumin values. Platelet counts were higher in the DDG compared to the EDG ( P=0.004).Coronary abnormalities were more common in the DDG ( P=0.05). Our findings suggest that children presenting primarily to a tertiary care centre with symptoms of Kawasaki disease are more likely to be associated with early diagnosis and delay in onset of neck swelling or oral lesions may be associated with delayed diagnosis. There is a need for creating more awareness about Kawasaki disease among practicing pediatricians in India. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is an illness that involves the skin, mouth, and lymph nodes, and typically affects children who are under the age of five. The cause of KD is unknown, but if the symptoms are recognized early, children with the disease can fully recover within a few days. A generalized vasculitis of unknown etiology, early recognition and treatment of KD can reduce the development of potentially life-threatening coronary artery abnormalities. [ABSTRACT FROM AUTHOR]

Kawasaki disease also known as mucocutaneous lymph node syndrome is a generalised vasculitis of unknown etiology. Presenting a review of the literature and using our cases for illustration we discuss some of the pertinent features in the evaluation and management of patients with this condition. 2 cases of Kawasaki disease have been evaluated and treated in the department of ENT and Paediatrics who responded satisfactorily to treatment with I. V. Immunoglobulin and Aspirin. The case reports have been presented in view of the rarity of reporting of similar cases in India and the need for early diagnosis and prompt treatment to prevent coronary artery aneurysm and other serious complications. [ABSTRACT FROM AUTHOR]

Till date, there is paucity of published literature on clinical manifestations of Coronavirus disease 2019 (COVID-19) in children from low-middle-income countries (LMIC). Most of the reports are from Europe, USA or China. Our study aimed to capture data on varied and unusual clinical presentation and management of MIS-C (Multisystem Inflammatory Syndrome in Children) with COVID-19 and compare the MIS-C and non-MIS-C children. This was a single-centre cohort study of 41 COVID positive children 0-12 years age hospitalized between 1 April 2020 and 31 July 2020. Data were entered into standardized WHO Case Report Form and analysed using strata 15.0 statistical software. Twenty out of 41 children fulfilled the criteria of MIS-C. Male-to-female ratio in the cohort was 1.73:1. In MIS-C cases, predominant clinical manifestation was fever (100%), neurological manifestations (80%), lower respiratory tract infection (50%), rash (35%) and acute gastroenteritis (25%). They were categorized into Acute Encephalitis-like illness in 35%, Kawasaki-like disease, Toxic Shock-like syndrome and Comorbidity with systemic complications in 20% each. Ninety percent of MIS-C cases required oxygen supplementation with odds ratio (OR) 18 (3.22-100.48), whereas 65% required mechanical ventilation with OR 37.14 (4.08-338.10). Most of them had raised inflammatory markers and hepatic enzymes derangement. Steroids, Intravenous immunoglobulin and supportive therapy were mainstay of management for MIS-C group. Most MIS-C group children had multisystem involvement with predominant neurological manifestations at time of presentation. Delay in diagnosis and referral may have adversely affected the prognosis and outcome., (© The Author(s) [2021]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

There is a global concern of increasing number of children presenting with inflammatory syndrome with clinical features simulating Kawasaki disease, during ongoing COVID-19 pandemic. The authors report a very similar case of 5-y-old boy from a COVID-19 hotspot area who presented in late April 2020 with acute febrile illness with abdominal pain and loose stools followed by shock. On examination, child had bulbar conjunctivitis and extremity edema. Initial investigations showed high inflammatory parameters, elevated serum creatinine and liver enzymes. Echocardiography showed moderate LV dysfunction and normal coronaries. Cardiac enzymes were also elevated, suggesting myocarditis. He was treated with inotropic support, respiratory support with high flow nasal cannula, IV immunoglobulins, aspirin, steroids and diuretics. RT PCR for SARS-CoV-2 was negative twice. His clinical condition improved rapidly, was afebrile from day 2, inflammatory parameters decreased, left ventricular function improved and was discharged after 6 d of hospital stay.

To evaluate serum levels of IL6 in patients with Kawasaki disease and compare it with CRP, and to assess the role of these biomarkers in predicting coronary changes and resistance to the first-line therapy of this disease in a subset of Indian population. A single centre prospective observational study was conducted amongst all Kawasaki disease patients for a period of 18 months from January 2017 at Institute of Child Health, Kolkata. Serum IL6 and CRP were compared at diagnosis and after 48 h of administering IVIG in patients who developed coronary changes with those who did not and also among the responders and non-responders to IVIG, the first-line therapy given to these patients. Out of total 72 patients of KD [mean age of presentation: 24 months, M:F = 1.22:1], 30% (n = 22) had coronary artery involvement (CALs), and 15% (n = 11) were IVIG non-responders. Mean IL6 prior to IVIG in those with CALs was 143.60 pg/ml, which was about three times higher than in those without CALs (mean = 52.90 pg/ml), the difference being significant (p < 0.01). Mean CRP values also were significantly raised in patients with CALs (p < 0.01) whereas post-IVIG levels of mean serum IL6 was found to be 108.15 pg/ml in non-responders which was about 17 times raised than that in the responders (mean IL6 = 6.22),the difference again was statistically significant (p < 0.001).Also, ROC analysis revealed a sensitivity and specificity of 81.0% and 82.0%, respectively, for IL6; 72% and 74%, respectively, for CRP for predicting CALs. This study also shows a sensitivity of 72% and specificity of 68% for IL6 in predicting IVIG resistance whereas that of CRP being 90% sensitive and 36% specific. These results suggest that higher levels of IL-6 and CRP at diagnosis are associated with occurrence of CALs and IVIG resistance in KD patients. Using the cutoff for IL6 and CRP from our study, chances of developing CALs and IVIG resistance can be predicted, which might prevent the development of future complications like aneurysms in such patients.

Aim: To study the profile of autoantibodies on long-term follow-up of children with Kawasaki disease (KD)., Patients and Methods: In this single-center observational cohort study, 50 children who had been diagnosed and treated for KD with a minimum follow-up period of 3 years were enrolled. The organ-specific autoantibodies that were assessed in the study included anti-thyroid microsomal antibody (TMA), anti-parietal cell antibody (PCA) and anti-liver kidney microsomal (LKM) antibody. The organ-nonspecific autoantibodies that were studied included anti-endothelial cell antibody (AECA), anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA), anti-mitochondrial antibody (AMA) and anti-smooth muscle antibody (SMA)., Results: The sample for assessment of serology was taken at a mean follow-up period of 6.41 years (±1.95 SD) after diagnosis. Autoantibodies were detected in 11/50 (22%) patients. ANA was detected in three patients, TMA was positive in seven and ANCA was positive in one., Conclusions: These autoantibodies likely develop in children with KD during the acute stage and may persist for many years. There is no concrete evidence to suggest that these children are at increased risk of developing an autoimmune disease in the future. However, there is some justification for prolonged surveillance for development of autoimmune manifestations., (© 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)