ABSTRACT
The outcome for children with rheumatic diseases has been dramatically altered by the use of biological therapies. Increasing use of these agents will need careful monitoring for long term safety, particularly in children. Current data on safety of these drugs stem exclusively from Western literature. There is clear need for a registry of all children with rheumatic diseases who are commenced on biological agents to ensure appropriate pharmacovigilance. In this perspective, we discuss the need for and the role of a biologics registry for children with rheumatic diseases in India.
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Objective To determine the outcomes in children with MIS-C receiving diferent immunomodulatory treatment. Methods In this multicentric, retrospective cohort study, data regarding treatment and outcomes of children meeting the WHO case defnition for MIS-C, were collected. The primary composite outcome was the requirement of vasoactive/inotropic support on day 2 or beyond or need of mechanical ventilation on day 2 or beyond after initiation of immunomodulatory treatment or death during hospitalization in the treatment groups. Logistic regression and propensity score matching analyses were used to compare the outcomes in diferent treatment arms based on the initial immunomodulation, i.e., IVIG alone, IVIG plus steroids, and steroids alone. Results The data of 368 children (diagnosed between April 2020 and June 2021) meeting the WHO case defnition for MIS-C, were analyzed. Of the 368 subjects, 28 received IVIG alone, 82 received steroids alone, 237 received IVIG and steroids, and 21 did not receive any immunomodulation. One hundred ffty-six (42.39%) children had the primary outcome. On logistic regression analysis, the treatment group was not associated with the primary outcome; only the children with shock at diagnosis had higher odds for the occurrence of the outcome [OR (95% CI): 11.4 (5.19–25.0), p<0.001]. On propensity score matching analysis, the primary outcome was comparable in steroid (n=45), and IVIG plus steroid (n=84) groups (p=0.515). Conclusion While no signifcant diference was observed in the frequency of occurrence of the primary outcome in diferent treatment groups, data from adequately powered RCTs are required for defnitive recommendations.
ABSTRACT
Mevalonate kinase deficiency (MKD) is a rare autosomalrecessive autoinflammatory disease caused by mutations inMVK. We report two siblings with MKD, presenting withrecurrent febrile illnesses, detected to have compoundheterozygous variants in MVK. MKD mimics common pediatricconditions and should be considered as a differential diagnosis.
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Pediatric cardiology is outgrowing from the shadows of adult cardiology and cardiac surgery departments in India. It promises to be an attractive and sought-after subspeciality of Pediatrics, dealing with not only congenital cardiac diseases but also metabolic, rheumatic and host of other cardiac diseases. The new government policy shall provide more training avenues for the budding pediatric cardiologists, pediatric cardiac surgeons, pediatric anesthetists, pediatric cardiac intensivists, neonatologists and a host of supportive workforce. The proactive role of Indian Academy of Pediatrics and Pediatric Cardiac Society of India, towards creating a political will at the highest level for framing policies towards building infrastructure, training of workforce and subsidies for pediatric cardiac surgeries and procedures shall fuel the development of multiple tertiary cardiac centers in the country, making pediatric cardiology services accessible to the needy population.
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Objectives: To determine the efficacy of oral zinc for treatment of idiopathic neonatal hyperbilirubinemia in near-term and term (35- 41 weeks) neonates. Design: Randomized placebo-controlled trial. Setting: Tertiary-care teaching hospital Participants: Eighty newborns with idiopathic neonatal hyperbilirubinemia. Intervention: Neonates were randomized to receive either oral zinc sulfate (10 mg/d) or placebo for 7 days. Main outcome measures: Primary: total serum bilirubin levels at 48 (±12) h, 96 (±12) h and 144 (±12) h after intervention. Secondary: duration of phototherapy, and serum zinc and copper levels. Results: Baseline mean (SD) total serum bilirubin levels were 14.8 (3.8) and 14.4 (3.5) mg/dL in zinc and placebo groups, respectively. No significant differences were observed in total bilirubin levels between the two groups after the intervention. Mean (SD) total serum bilirubin levels in zinc and placebo groups were 13.9 (2.5) vs. 13.4 (1.9) mg/dL (mean difference 0.566; 95% CI -0.535, 1.668, P=0.038) at 48 h, 13.1 (2.7) vs. 12.8 (2.3) mg/dL (mean difference 0.234; 95% CI -1.011, 1.479, P =0.708) at 96 h and 8.0 (2.0) vs. 8.6 (1.2) mg/dL (mean difference -0.569, 95% CI -1.382, 0.242, P=0.166) at 144 h. Although the mean duration of phototherapy in the zinc group was less by 21.3 h (95% CI 11.6, 30.9, P=0.052), the difference was not significant. Postintervention, serum zinc levels were significantly higher in the zinc-supplemented group while serum copper levels were comparable between the two groups. Conclusions: Oral zinc sulfate, in a dose of 10 mg/day, is not effective in the management of idiopathic neonatal hyperbilirubinemia.
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Objective:To describe profile and outcome in children with significant pericardial effusion. Methods: Hospital records of 25 children admitted with significant pericardial effusion during January 2010 to March 2013 were analyzed. Results: Thirteen (52%) children had tubercular, 6 (24%) had bacterial, 3 viral, 2 recurrent idiopathic and one had malignant pericardial effusion. Only 3 children in our series required surgical drainage. Conclusions: Echocardiography guided percutaneous pericardiocentesis and pigtail catheter placement was found to be safe and effective.