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Background Kawasaki disease (KD) is the leading cause of acquired childhood heart disease, with untreated cases progressing to giant coronary artery aneurysms (GCAAs) in 2-3% of patients. While outcomes are well-documented in high-income settings, data from low- and middle-income countries (LMICs) remain limited. This study examines clinical profiles, treatment responses, and outcomes of children with GCAAs managed at a tertiary care center in South India. Methods We conducted a retrospective review of 18 children with KD-associated GCAAs (z-score >10 or diameter >8mm) treated between 2019-2024 at a tertiary care center in Kerala, India. All patients were referred from external centers with documented IVIg resistance. Treatment intensification included infliximab, corticosteroids, cyclosporine, and anakinra. Primary outcome was GCAA dimension change at last follow-up; secondary outcomes included thrombotic events, mortality, and anticoagulation cessation. Results The cohort comprised predominantly of males ( n = 14, 77.8%) with median symptom onset at 1.1 (IQR 0.3 – 2.7) years; 50% were under one year old. While ten children (55%) experienced delayed diagnosis (>10 days after symptom onset) with median diagnosis at 11 (IQR 7 – 13.5) days; all faced delays in treatment intensification. Excluding two children lost-to-follow-up, during the median follow-up of 8.6 (5.2 – 19.4) months, complete GCAA resolution occurred in three (18.8%), reduction to small/medium aneurysms in six (37.6%), while five (31.3%) remained static. One mortality (6.3%) from myocardial infarction and a patient requiring coronary artery bypass grafting were observed. Anticoagulation was discontinued in 56.25% of children whose aneurysms resolved or reduced significantly. Conclusion Delayed diagnosis and treatment intensification may have contributed to mixed outcomes in this GCAA cohort. Early recognition, appropriate risk stratification, and timely immunosuppression intensification are essential to improve prognosis and reduce severe coronary complications in resource-limited settings.

Introduction: Nearly 10% of children and adolescents suffer from functional somatic symptoms (FSS) which are persistent, bothersome bodily symptoms not having demonstrable organic pathology. Associations have been reported between children’s FSS, anxiety, depression, and certain parental factors. Aims: (i) compare the anxiety and depression levels between children with FSS and children not having FSS, (ii) identify the child and parent-related risk factors of FSS. Methods:  Case-control design was used for this cross-sectional study. Cases constituted a hospital sample of 60 children, aged 9-15 years, who presented with FSS. Age, gender and class-matched 60 school children, who did not disclose any FSS while screening with Children’s Somatic Symptoms Inventory-24 constituted the control group. Both the groups were administered with Revised Children’s Anxiety and Depression Scale, Illness Behaviour Encouragement Scale, and Lum Emotional Availability of Parents (Child report). Results: Cases scored significantly higher than the control children on anxiety, depression (p<0.001), and parental illness behaviour encouragement (p<0.001), but reported low emotional availability of both parents (p<0.01). Significantly large number of children in the FSS group showed anxiety disorder (p<0.05), separation anxiety disorder (p<0.001) generalized anxiety disorder (p<0.01), and major depressive disorder (p<0.01). The stepwise multiple logistic regression analysis revealed over all anxiety, separation anxiety, generalized anxiety and high parental illness encouragement as the risk factors of FSS. Conversely, higher perception of maternal emotional availability significantly reduced its risk. Conclusion: Children with FSS must be screened for anxiety and depressive disorders as well as parental reinforcing responses and emotional availability before initiating treatment.

Idiopathic inflammatory myopathies (IIMs) are a diverse group of diseases characterized by proximal muscle weakness and inflammation in skeletal muscle. Phenotypically, the subtypes include dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. The most common IIM in children is juvenile dermatomyositis (JDM). In contrast to adult dermatomyositis (DM), children are likely to have frequent relapses, vasculopathy, and long-term metabolic and other complications like lipodystrophy, insulin resistance, and calcinosis. Significant advances in our understanding of pathogenesis, disease course, and treatment of JDM has changed the therapeutic landscape and improved outcomes in children. Myositis-specific autoantibodies and myositis-associated autoantibodies have unique clinical associations, disease course and help predict response to therapy. A multidisciplinary approach including exercise programs and psychosocial support is essential. The first line of treatment is a combination of corticosteroids and methotrexate (MTX). Other targeted immunosuppressive therapy is used in refractory cases. Early recognition and timely referral to a specialist center remain pivotal to improving the mortality and morbidity associated with this disease.

ObjectivesTo study (1) epidemiological factors, clinical profile and outcomes of COVID-19 related multisystem inflammatory syndrome in children (MIS-C), (2) clinical profile across age groups, (3) medium-term outcomes and (4) parameters associated with disease severity.DesignHospital-based prospective cohort study.SettingTwo tertiary care centres in Kerala, India.ParticipantsDiagnosed patients of MIS-C using the case definition of Centres for Disease Control and Prevention.Statistical analysisPearson χ2 test or Fisher’s exact test was used to compare the categorical variables and independent sample t-test or Mann-Whitney test was used to compare the continuous variables between the subgroups categorised by the requirement of mechanical ventilation. Bonferroni’s correction was used for multiple comparisons.ResultsWe report 41 patients with MIS-C, mean age was 6.2 (4.0) years, and 33 (80%) were previously healthy. Echocardiogram was abnormal in 23 (56%), and coronary abnormalities were noted in 15 (37%) patients. Immunomodulatory therapy was administered to 39 (95%), steroids and IVIg both were used in 35 (85%) and only steroids in 3 (7%) patients. Intensive care was required in 36 (88%), mechanical ventilation in 8 (20%), inotropic support in 21 (51%), and 2 (5%) patients died. Mechanical ventilation requirement in MIS-C was associated with hyperferritinaemia (p=0.001). Thirty-seven patients completed 3 months follow-up by April 2021, of whom 6 (16%) patients had some residual echocardiographic changes.ConclusionsPatients with MIS-C in our cohort had varied clinical manifestations ranging from fever with mild gastrointestinal and mucocutaneous involvement to fatal multiorgan dysfunction. Immediate and medium-term outcomes remain largely excellent except for the echocardiographic sequelae in a few patients which are also showing a resolving trend. Hyperferritinaemia was associated with the requirement of mechanical ventilation.

We report a case of juvenile dermatomyositis (JDM) with cytomegalovirus (CMV) colitis which was further complicated with perforation. The patient, a 6-year-old girl, was diagnosed with JDM 1 month prior to the current presentation. After 2 weeks of optimising her treatment with steroid, intravenous Ig and methotrexate, she was readmitted with diffuse abdominal pain. Erect abdominal X-ray revealed gas under diaphragm. An exploratory laparotomy showed perforation of the large intestine. A biopsy showed inclusion bodies of CMV with immunohistochemistry for CMV positive. Strong positive CMV DNA PCR from tissue specimen, positive IgG CMV and negative IgM CMV in blood suggested a reactivation of CMV. The treatment followed included surgery and strategic use of antiviral agents as well as immunomodulators. CMV enteritis with complications should also be suspected in optimally treated autoimmune disease patients, including JDM, when they present with abdominal symptoms.

It is not uncommon in pediatric clinical practice to encounter children with musculoskeletal symptoms. A number of disparate conditions can present with joint complaints in children. In this article, the author describes the clinical approach to a child presenting with joint complaints. A detailed clinical history, including the family history, along with a complete physical examination can provide vital clues to the underlying condition in most cases. A structured screening examination of the musculoskeletal system that has been recently developed (i.e., pGALS) is also discussed. It is also pointed out that the pattern of joint involvement gives us one of the most important clues to the etiology of arthritis. The pediatrician has to be aware of the conditions that can have arthritis as one of the manifestations so as to investigate and treat the child accordingly.

Autoinflammatory diseases affecting the NLRP3 gene are rare autosomal dominant disorders presenting with episodic organ limited and systemic inflammation. We report three patients with cryopyrinopathies. Our first case is a 4-year-old boy with a history of periodic fever, failure to thrive, and raised intracranial pressure. The second case is a 6-year-old boy with similar complaints, also with bilateral uveitis. The third is a 24-year-old gentleman with periodic fever and early hearing loss, also with a novel presentation of sacroiliitis. Our case series demonstrates that there should be a low clinical threshold indicating genetic testing in any child who displays features of autoinflammation in combination with an urticarial rash, musculoskeletal manifestations, hearing loss, and chronic aseptic meningitis with macrocephaly. Furthermore, despite anakinra being a cornerstone in treating NLRP-3 AID, there is an unmet clinical need to provide access to alternatives such as colchicine and thalidomide in resource-limited settings.