Exploring clinical features and therapeutic outcomes in Indian children with mixed connective tissue disease: A multicenter study.

INTRODUCTION: Mixed connective tissue disease (MCTD) is a rare entity in children. There is a paucity of studies on juvenile-onset MCTD (jMCTD) worldwide especially from Southeast Asia. OBJECTIVES: To describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India. METHODS: A predesigned detailed case proforma in an excel format was prepared and was sent to all the Pediatric Rheumatology centers in India. Eleven centers provided the clinical and laboratory data of their jMCTD patients, which was then compiled and analyzed in detail. RESULTS: Thirty-one jMCTD patients from 11 centers were included in the study. Our cohort had 27 females and four male patients over 12 months (August 2021 to July 2022). The median age at presentation was 12 years (range 5-18 years) and the median duration of symptoms was 24 months at diagnosis (range 2-96 months). The common features included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%). At a mean follow-up of 43 months (range 1-168 months), 45% of them were in remission. There were two deaths reported, due to macrophage activation syndrome and sepsis respectively. CONCLUSION: We present the largest multicenter experience on jMCTD from the Indian subcontinent. The study's findings serve as a crucial stepping stone toward unraveling the complexities of jMCTD and improving patient care and management strategies.

Management of Hyperparasitaemia in Severe Malaria: A Sea Change With the Availability of Artesunate.

OBJECTIVE: This study aims to study the efficacy of exchange transfusion in complicated malaria with hyperparasitaemia. METHOD: This is a retrospective case-control study conducted in a tertiary care referral hospital in northeastern India. A retrospective chart review was done, and 34 children with hyperparasitaemia were identified. Of these, 16 children received only antimalarial chemotherapy, and 18 received adjunct exchange blood transfusion (EBT). The data was analyzed for survival benefit as the main outcome measure. RESULTS: The subjects in each of the groups were comparable in terms of age, sex distribution, and mean haemoglobin at presentation. The patients treated with EBT were more ill than those who did not receive EBT; they met a greater number of WHO criteria for severe malaria (2.94 ± 1.16 vs. 1.81 ± 0.83; p=0.002) and had higher levels of parasitaemia (23.96 ± 17.55 vs. 16.14 ± 6.89; p=0.0007). The mean volume exchanged was 44.88 ± 20.49 ml/kg, against a target of 70 ml/kg due to the unavailability of fresh, compatible whole blood. There was no difference in mortality in the exchange transfusion group compared to the chemotherapy alone group (22.22% vs. 31.25%; p=0.83). CONCLUSIONS: In resource-limited areas, lower volumes of fresh whole blood for exchange transfusion can reduce hyperparasitaemia to a significant degree, and this may give some mortality benefit in very sick cases. Artesunate, by virtue of its rapid action, is capable of reducing hyperparasitaemia and may question the very place of blood transfusion in complicated malaria. It may also be worthwhile to accumulate more data comparing EBT against artesunate instead of quinine.

Evidence-Based Consensus Recommendations for Skin Care in Healthy, Full-Term Neonates in India.

Purpose: Neonatal skin care practices guided by personal experience and preferences might be substantially different across different hospital settings. The aim of this consensus recommendation is to provide clinical practice guidance to healthcare practitioners on evidence-based neonatal skin care practices from delivery-to-discharge, in hospital settings. Patients and Methods: A Scientific Advisory Board meeting on "Evidence-based Neonatal Skin Care Practices and Protocols" was held in December 2020 with an expert panel comprising neonatologists, pediatricians, obstetricians and gynecologists and pediatric dermatologist. Comprehensive literature search was performed up to 23 March 2021 using PubMed and Google Scholar to retrieve relevant evidence. Results: Recommendations were developed on critical aspects of skin care in healthy full-term neonates including cleansing at birth, skin-to-skin care, cord care, diaper area care, initial and routine bathing, cleansers and emollients use, and criteria to choose appropriate skin care products. Recommendations include inclusion of skin assessment in routine neonatal care, first bath timing after cardio-respiratory and thermal stabilization, 6-24 hours after birth; bathing with water alone or adding a mild liquid cleanser could be considered appropriate as it does not impact the developing skin barrier; use of emollients is recommended for neonates with higher risk of development of eczema to maintain and enhance skin barrier function and integrity; and inclusion of skin care advice in neonatal discharge checklist. Importance of rigorous quality control, high-quality clinical trials for assessment of baby products, usage of products that are formulated appropriately for newborns, and full label transparency for baby products were highlighted. The panel identified gaps in literature and discussed the scope for future research. Conclusion: These recommendations may help to standardize evidence-based skin care for healthy full-term neonates in Indian hospital settings to improve the quality of care that neonates receive in hospital and facilitate improvement in overall neonatal health outcomes.

Proceedings of the Expert Consensus Group meeting on meningococcal serogroup B disease burden and prevention in India.

Meningococcal disease is highly transmissible, life-threatening and leaves significant sequelae in survivors. Every year, India, which has a plethora of risk factors for meningococcal disease, reports around 3000 endemic cases. However, the overall disease burden and serogroup distribution are unknown, creating a setting of general disease negligence and unawareness. Vaccination with quadrivalent meningococcal conjugate vaccine A, C, W, and Y is only recommended for high-risk children, and there is no overall guidance for meningococcal serogroup B (MenB) vaccination. MenB vaccines, which recently have been licensed in many countries but not in India, have significantly aided the fight against meningococcal disease. However, these MenB vaccines are not available in India. An Expert Consensus Group meeting was held with leading meningococcal disease experts to better understand the current disease epidemiology, particularly serogroup B, the prevalence gaps, and feasible ways to bridge them. The proceedings are presented in this paper.

Mass gatherings: a review of the scope for meningococcal vaccination in the Indian context.

The risk of meningococcal transmission is increased with crowding and prolonged close proximity between people. There have been numerous invasive meningococcal disease (IMD) outbreaks associated with mass gatherings and other overcrowded situations, including cramped accommodation, such as student and military housing, and refugee camps. In these conditions, IMD outbreaks predominantly affect adolescents and young adults. In this narrative review, we examine the situation in India, where the burden of IMD-related complications is significant but the reported background incidence of IMD is low. However, active surveillance for meningococcal disease is suboptimal and laboratory confirmation of meningococcal strain is near absent, especially in non-outbreak periods. IMD risk factors are prevalent, including frequent mass gatherings and overcrowding combined with a demographically young population. Since overcrowded situations are generally unavoidable, the way forward relies on preventive measures. More widespread meningococcal vaccination and strengthened disease surveillance are likely to be key to this approach.