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Objectives: To improve the hand-hygiene (HH) compliance among the health care personnel from 69 to 85% by 4 mo plan-do-study-act (PDSA) cycles, based on the WHO's five moments of hand hygiene and to study the impact of HH compliance upon the health care–associated infections (HAI) rate in the authors' special newborn care unit. Methods: HH compliance study was undertaken based on the prioritization matrix. The barriers to HH compliance were identified and evaluated using fishbone analysis. An operational team was formed, and measures for improvement were chalked out. The baseline score was recorded through observation. A total of three PDSA cycles were carried out. Appropriate education and counseling regarding the WHO's five moments of hand hygiene were taught to the health care personnel. Interventions such as posters and supply of nonmedicated liquid hand soap, autoclaved paper towels, and alcohol-based hand sanitizer were provided. The effect of interventions on HH compliance was assessed at the end of each PDSA cycle. HAI data were collected and compared with the previous records. Results: The HH compliance recorded during baseline, PDSA 1, PDSA 2, and PDSA 3 are respectively, as follows: 69% (16.75 ± 3.46), 74.58% (43.07 ± 7.50; p = 0.043), 63.75% (24.43 ± 5.16; p = 0.083), and 84.70% (47.45 ± 10.59; p = 0.014). The sum of HH scores from the three PDSA cycles when compared to the baseline is significant (p = 0.022). The HAI rate decreased from 13.81 to 1000 patient days to 10.43 per 1000 patient days (p = 0.566). Conclusion: HH compliance among health care personnel can be improved through information, education, and communication with constant monitoring. [ABSTRACT FROM AUTHOR]

In India, considerable progress has been made in reducing child mortality rates. Despite this achievement, wide disparities persist across and socio-economic strata, and persistent challenges, such as malnutrition, poor sanitation, and lack of clean water. This paper provides a comprehensive review of the state of child health in India, examining key risk factors and causes of child mortality, assessing the coverage of child health interventions, and highlighting critical public health programs and policies. The authors also discuss future directions and recommendations for bolstering ongoing efforts to improve child health. These include state- and region-specific interventions, prioritizing social determinants of health, strengthening data systems, leveraging existing programs like the National Health Mission (NHM) and Ayushman Bharat Pradhan Mantri Jan Arogya Yojana (PM-JAY), and the proposed Public Health Management Cadre (PHMC). The authors argue that reducing child mortality requires not only scaled-up interventions but a comprehensive approach that addresses all dimensions of health, from social determinants to system strengthening. [ABSTRACT FROM AUTHOR]

Stillbirth is a major public health challenge and a multifaceted issue that leads to significant financial, physical, mental, financial, and psychosocial implications. India has made substantial progress in stillbirth reduction. Yet, many challenges continue and the absolute number of stillbirths remain high. This paper presents the national and state level burden of stillbirths and discusses about the magnitude, risk factors, causes and inequities in stillbirths. A few additional approaches for reduction of preventable stillbirths have been suggested. The authors argue that the institutional mechanisms need to be developed to ensure all stillbirths are registered in a timely manner. There is a need for standard definition for classification of stillbirths and document the cause, to roll-out suitable interventions. There is a need for state specific interventions to address different causes, as Indian states have variable stillbirth rates. The stillbirth audits should be institutionalised as a continuous quality improvement exercise to bring local accountability and reduce stillbirth rate. The healthcare system and providers must be trained to offer bereavement support to the affected mothers and families. These approaches should be implemented through primary healthcare system as well. [ABSTRACT FROM AUTHOR]

Adrenal hypoplasia congenita (AHC) is a rare disorder of the adrenal gland due to mutations in the nuclear receptor superfamily 0, group B, member 1 (NR0B1) gene. It commonly presents with adrenal insufficiency and hypogonadotropic hypogonadism, but there is a wide variability in the age as well as the clinical phenotype at presentation. Also, the mechanism of pubertal abnormalities in this condition, which include not just delayed or absent pubertal development, but also precocious puberty in a small proportion of cases, is not clear. In this paper, three unrelated patients with 3 different mutations are described, who presented with variable age of onset of adrenal insufficiency, gonadal development, and puberty, highlighting the need for high index of suspicion of this condition in children presenting with atypical features of adrenal insufficiency. [ABSTRACT FROM AUTHOR]

Early diagnosis of autism spectrum disorder (ASD) is essential for improved outcomes. There is a paucity of data on the prevalence of ASD in low- and middle-income countries (LMIC), but early identification may be further delayed in those communities. In this paper, recent studies on strategies for the early detection of ASD, and the prevalence of ASD in LMIC are reviewed. The limitations that can arise in the early identification of ASD in LMIC communities are discussed, and screening tools and strategies that can be helpful are identified. The goal is to recommend models that are culturally appropriate and scientifically valid, easily integrated within community settings while strengthening community systems and reducing disparities in the early identification of ASD. Starting locally by simplifying and demystifying the ASD identification process and building community connections will inform global researchers and policymakers while making a difference in the lives of the children and families affected by ASD. [ABSTRACT FROM AUTHOR]

Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: To estimate and compare the prevalence of epilepsy during childhood using several approaches and also to determine whether school-based screening campaigns can capture epilepsy cases efficiently., Methods: Epilepsy prevalence determined from cases captured through the Rashtriya Bal Swasthya Karyakram (RBSK), a nationwide school-health screening framework, were compared with estimates derived from school- and community-based surveys in one Indian district. Level-1 screen comprised perusal of child health registers maintained by the RBSK teams over one year to estimate the documented number of children with epilepsy; Level-2 screen comprised a questionnaire-based school survey among 10,000 school children; and Level-3 screen-a door-to-door community-based survey among 10,000 children in the district., Results: Prevalence estimates of childhood epilepsy varied significantly across screening methods. The child health register identified lower crude and age-adjusted prevalences of 40 (95% CI, 24 to 55) and 36 (95% CI, 20 to 51)/1,00,000 vis-à-vis both the school survey [crude and age-adjusted prevalences of 354 (95% CI, 221 to 487) and 340 (95% CI, 181 to 517) per 100,000] and the community survey [crude and age-adjusted prevalences of 759 (95% CI, 591 to 927) and 746 (95% CI, 579 to 914) per 100,000]. The community survey identified 15 children with epilepsy (20%) who had dropped out of school. Also, it recaptured a small number of children previously identified by the school or child health register surveys., Conclusions: The present findings underscore the need to scale up the capacity of public programs to screen epilepsy among school children and underline the high frequency of school dropouts among children with epilepsy in resource-limited settings., (© 2024. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Desbuquois dysplasia (DBQD) is an uncommon, autosomal recessive disorder with multiple joint dislocations. It is caused by pathogenic variants in CANT1 (calcium-activated nucleotidase 1) [NM_001159773.2]. This study adds to the scant data of nine reported antenatal phenotypes of DBQD. The present paper describes two unrelated consanguineous families with antenatal features of lethal skeletal dysplasia. The defining radiological changes were identified in only one patient who presented in the late second and third trimesters. Solo exome sequencing was performed and two previously reported homozygous variants c.896C>T (p.Pro299Leu) in patient 1 and c.902_906dup (p.Ser303fs*20) in patient 2 were identified. This study highlights the fetal presentations in DBQD and adds to its phenotypic spectrum. A complete clinical workup, including fetal autopsy and radiographs is essential to confirm the diagnosis of lethal skeletal dysplasia. Molecular diagnosis remains the diagnostic modality to define the causative variant. A definitive diagnosis is essential to inform management and offer reproductive care. [ABSTRACT FROM AUTHOR]

Neonatal seizures are the commonest neurological emergency and are associated with poor neurodevelopmental outcome. While they are generally difficult to diagnose and treat, they pose a significant clinical challenge for physicians in low- and middle-income countries (LMIC). They are mostly provoked seizures caused by an acute brain insult such as hypoxic–ischemic encephalopathy (HIE), ischemic stroke, intracranial hemorrhage, infections of the central nervous system, or acute metabolic disturbances. Early onset epilepsy syndromes are less common. Clinical diagnosis of seizures in the neonatal period are frequently inaccurate, as clinical manifestations are difficult to distinguish from nonseizure behavior. Additionally, a high proportion of seizures are electrographic-only without any clinical manifestations, making diagnosis with EEG or aEEG a necessity. Only focal clonic and focal tonic seizures can be diagnosed clinically with adequate diagnostic certainty. Prompt diagnosis and timely treatment are important, with evidence suggesting that early treatment improves the response to antiseizure medication. The vast majority of published studies are from high-income countries, making extrapolation to LMIC impossible, thus highlighting the urgent need for a better understanding of the etiologies, comorbidities, and drug trials evaluating safety and efficacy in LMIC. In this review paper, the authors present the latest data on etiology, diagnosis, classification, and guidelines for the management of neonates with the emphasis on low-resource settings. [ABSTRACT FROM AUTHOR]

The present paper reports 10 patients (9 families) with Fanconi–Bickel syndrome managed during 2010–2021. Patients presented with polyuria, polydipsia, hepatomegaly, rickets, and stunting at a median of 5 (3, 7.3) mo; one had transient neonatal diabetes. Glucosuria, generalized aminoaciduria, β2-microglobinuria, urinary phosphate wasting, and hypercalciuria were present in all patients; 3 patients had nephrocalcinosis. Other metabolic abnormalities included hypertriglyceridemia (n = 5/6), fasting hypoglycemia (n = 5/8), and postprandial hyperglycemia (n = 3/6). Genetic analysis showed 7 homozygous or compound heterozygous variants in SLC2A2. A pathogenic variant c.952G>A, common to 4 patients (3 families), might be a potential hotspot. At a median follow-up of 43 mo, 4 patients died at a median of 25 mo; short stature persisted in all except one patient who showed catch-up growth with uncooked corn-starch diet. The present findings suggest that the Fanconi–Bickel syndrome has a severe phenotype with an unsatisfactory outcome. A high index of suspicion for diagnosis and efforts for facilitation of dietary therapy are necessary. [ABSTRACT FROM AUTHOR]

Pediatric overweight/obesity has assumed epidemic proportions in India. It is associated with several significant complications and tracks into adulthood. The mainstay of management is a holistic lifestyle modification that must be adopted by the family as a whole. It involves dietary changes, regular physical activity, and behavioral changes that favor a healthy way of life. Regular follow-up, and attention to keeping up the motivation of the child and family achieves good results. In the present paper, a stepwise approach to prevention and management of childhood obesity is presented along with the recommendations for screening and management of associated complications and the role of pharmacotherapy and bariatric surgery. [ABSTRACT FROM AUTHOR]

Children account for 1% to 5% of diagnosed COVID-19 infection with relatively mild presentation compared to adults. The frequency of neurological involvement in acute COVID-19 infection in children is unclear. COVID-19 is also considered to be a neurotropic virus, but so far, in the pediatric age group, very few cases with involvement of basal ganglia and no case of dentate nucleus involvement have been reported in the literature. The present paper reports two cases of acute encephalopathy with COVID-19, the first case with basal ganglia involvement and the second with dentate nucleus involvement. Both cases required aggressive management and had complete neurological recovery on follow-up. Hence, these cases are reported to make everyone aware of the neurological presentation with atypical neuroimaging finding of acute COVID-19 infection in the pediatric age group; timely management improves the outcome. [ABSTRACT FROM AUTHOR]

Biogenic amine neurotransmitters metabolism is a multistep pathway with pterin and pyridoxal phosphate (vitamin B6) as cofactors. A defect in biogenic amine and cofactor metabolism and vesicular transporters result in a primary neurotransmitter disorders. These are a well-recognized groups of inherited disorders and often present with features overlapping with other neurological conditions. Their diagnosis is made by analysis of biogenic amine metabolites in cerebrospinal fluid (CSF) and other body fluids and respective enzyme assays. Many of these disorders are treatable and deficits can be reverted by timely intervention. CSF biogenic amine or cofactor metabolite analysis is one of the primary indicators of a neurotransmitter disorder. In this paper, 3 cases are reported—2 of cofactor deficiency and 1 with enzyme deficiency wherein biogenic amine estimation has assisted in diagnosis. [ABSTRACT FROM AUTHOR]

Thalassemia is one of the most common hemoglobinopathies affecting a large number of people in India and other countries of South-East Asia. For patients with most severe form of the disease- Transfusion Dependent Thalassemia (TDT), stem cell transplantation or gene therapy are only curative treatment which are not available to most of the patients because of lack of experts, financial constraints and lack of suitable donors. In such situations, most cases are managed with regular blood transfusion and iron chelation therapy. With this treatment, over the years, survival of the patients has improved and 20-40% cases are entering into adulthood. In the absence of structured transition of care programs, currently most adult TDT patients are being managed by pediatricians. This article highlights the need for transition of care for TDT patients, barriers to transition and how to overcome the barriers and process of transition of care to adult care team. The importance of empowering the patients in self-management of the disease and educating the adult care team to achieve the desired outcome of transition program is highlighted., (© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

The implications of young childhood undernutrition on health, development and productivity are grave. In the last two decades, global and Indian studies on undernutrition in under five years have concluded that stunting and underweight rate increases steadily between 0-24 mo, and then stabilises. The available evidence highlights the significance of concentrating interventions to tackle child undernutrition in the first 1000 d of life, which should aim at universal coverage of essential nutrition interventions (ENIs) and maternal-child health care package comprising maternal nutrition care, adoption of appropriate infant and young child feeding practices (IYCF), iron-folic acid supplementation, immunization, deworming, appropriate management of childhood illness etc. Additionally, it is critical to address context specific nutrition sensitive measures such as socio-economic empowerment of women, preventing domestic violence, adolescent conception, appropriate water -sanitation-hygiene and family planning services etc. Mapping of the 'at risk' households having a member falling in the 1000 d window needs special attention and is central to the ongoing efforts in India through the National Nutrition Mission/ POSHAN Abhiyaan. However, for effective implementation, there is an urgent need to consider to shift the lead responsibility of ENIs in the first 1000 d of life to the health care system in India and strategize to integrate maternal nutrition care interventions and establishing of IYCF practices by ensuring optimum use of contacts with antenatal care and routine child immunization services., (© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Objectives: To assess the gaps and trends in child immunization coverage among urban and rural areas in India, and compare the success of immunisation program in each. Methods: PubMed, Scopus, and Crossref, and Google Scholar electronic databases were searched on October 9, 2019, and March 21, 2020, for studies that measured and reported immunization coverage indicators in India. Random-effects meta-analyses and meta-regressions were conducted. Results: The authors' search identified 545 studies, and 2 were obtained by expert suggestion. Among these 68 studies and 6 surveys were included. They found that full immunization coverage has grown yearly at 2.65% and 0.82% in rural and urban areas, respectively whereas partial immunization coverage declined by −2.44% and −0.69%, respectively. Percentage of nonimmunized children did not show a statistically significant trend in either. Conclusion: While rural immunization coverage has seen a large increase over the past two decades, the progress in urban areas is weak and negligible. This was largely attributable to a focus on minimizing dropouts in rural areas. However, a lack of significant reduction in unimmunized children may indicate left-out children or pockets in both rural and urban areas. The poor performance of immunization programs in urban areas, coupled with a larger impact of COVID-19, warrants that India urgently adopts urban-sensitive and urban-focused policies and programs. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: To develop a tool to assess pre-writing skills of 2-5 y old children in India. Methods: The tool development process followed the recommendations by Fitzpatrick et al. and the Consensus based Standards for the selection of health Measurement Instruments (COSMIN), and included 4 phases. In Phase I, an initial 35-item draft tool was developed by an expert panel for the tool-development. In Phase II, the 35-item draft tool was prevalidated through peer and expert reviews, pilot-study to assess the tool-comprehensibility, and assessment of test-retest and inter-rater reliability. In Phase III, the 35-item draft tool was administered on the 575 typically developing children aged 2-5 y, recruited from rural, urban, slum, and coastal areas through stratified random sampling. In Phase IV, the normative age-range for development of each item was generated by calculating the age-percentiles (10th, 25th, 50th, 75th, 90th). Factor analysis and item reduction was done for items in 2-3, 3-4, and 4-5 y age-groups. The final tool was converted to graphic format with 10th-90th age-percentile bars. Results: The final tool had 26 items with a three-factor structure. Cronbach's alpha was within acceptable limits for all three age-groups (0.723, 0.778, and 0.823 in 2-3 y, 3-4 y, and 4-5 y respectively). Kappa coefficients of the items ranged from 0.6-1 in interrater reliability and 0.64-1 test-retest reliability analysis reflecting substantial agreement between ratings. Conclusions: A 26-item screening tool "Prewriting skills Assessment Tool" (PAT) to assess writing readiness of 2-5 y old children was developed. Tool reliability and construct validity have been established. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, that mainly affects skin, joints and kidneys but can affect any organ in the body. It is characterized by presence of multiple autoantibodies like ANA, antibodies to dsDNA and RNA associated proteins. The major mechanism leading to tissue damage includes immune complex mediated complement activation, interferon alpha release by plasmacytoid dendritic cells, NETosis by neutrophils as well as defects in monocytes leading to poor clearance of cellular debris and direct cellular dysfunction mediated by antibodies. A child can present with pyrexia of unknown origin, immune mediated cytopenias, malar rash, oral ulcers, serositis, glomerulonephritis or nervous system dysfunction. As renal disease has a bearing on the long term impact, all children should have urine exam and blood pressure measurement done to rule out renal disease. The treatment varies depending on the severity and organs involved. In life or organ threatening situations, pulse methylprednisolone is used. Hydroxychloroquine, Mycophenolate mofetil, Azathioprine and Cyclophosphamide are the commonly used drugs in SLE. Over the years the prognosis of SLE has improved probably due to early diagnosis and better use of immunosuppressive treatment, regular follow up and treatment of co-morbidities. The 10-year survival now approaches 90% and with advent of new and targeted therapy it is hoped that the morbidity and organ damage can also be minimized. [ABSTRACT FROM AUTHOR]

Objectives: To compare the effect of two strategies of breast pumping –power pumping (PP) vs. routine pumping (RP) over one week in mothers of very low birth weight (VLBW) infants with low milk output to improve breastfeeding rates at discharge. Methods: Mothers with low milk output, defined as inability to express sufficient breastmilk to meet the feeding requirements of their infant on or after post-natal day 14, were randomized to receive power pumping vs. routine pumping - once daily for 7 d coupled with routine lactation support and hand expression 3 hourly in both groups. The primary outcome was exclusive breastfeeding at discharge. Results: There was no difference in the two pumping strategies with respect to exclusive breastfeeding rates [61.1% in PP vs. 50% in RP group; (p = 0.477, RR 1.2; 95% CI 0.76 to 2.17)]. Median milk volume pumped in the individual power pumping session on 7th day of intervention was significantly higher than that in the individual routine pumping session on the 7th day (50 mL vs. 27 mL, p = 0.014). The cumulative median milk volume expressed per individual pumping session over the 7 sessions of power pumping was also higher than that with routine pumping (305 mL vs. 213 mL, p = 0.054). Conclusions: In this pilot trial, expressed milk volume was significantly higher after each individual power pumping session compared to routine pumping. However, the exclusive breastfeeding rates at discharge were similar in the two groups. [ABSTRACT FROM AUTHOR]

The spectrum of pediatric rheumatological disorders is diverse and they are important differential diagnoses in a variety of clinical scenarios. Basic investigations not only provide supporting evidence for the diagnosis of a rheumatological illness but also help in exclusion of other diseases as well as for monitoring the activity of disease. Among these, complete blood count, biochemical assays including tests for inflammatory response, urine analysis, and various autoantibodies are often used. In addition, depending on the clinical features, imaging and tissue biopsies are used to confirm the diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: To compare the growth of preterm neonates with fetal growth restriction (FGR) and preterm neonates born appropriate-for-gestational-age (AGA) from birth to 12–18 mo of corrected age (CA). Methods: In this prospective cohort study, 85 preterm neonates with FGR and 85 gestation- and gender-matched AGA neonates were followed up from birth till 12–18 mo corrected age. Anthropometric indices were compared at specific time points and the risk factors for underweight status were analyzed. Results: Mean gestational age of the cohort was 32.8 ± 2.1 wk. Mean birth weight was 1414 ± 248 g in the FGR and 1806 ± 416 g in AGA neonates. At 12–18 mo of corrected age, a significantly greater proportion of FGR infants were wasted (24.3% vs. 7.2%, P = 0.005). A greater proportion of FGR infants were underweight (27% vs. 17.4%, P = 0.11), stunted (41.9% vs. 36.2%, P = 0.30), and microcephalic (27% vs. 23.1%, P = 0.36), although the differences were not statistically significant. Significant catch-up growth from 40 wk postmenstrual age (PMA) to 12–18 mo corrected age in weight (52.8% vs. 13.1%, P <0.001) and length (37.9% vs. 8.7%, P <0.001) was observed in the FGR neonates. The z-score of weight for age at 3 mo (adjusted OR 0.65, 95% CI: 0.52–0.8; P <0.001), the median time to full feeds (aOR: 1.10, 95% CI: 1.04–1.15; P = 0.001), and hypothyroidism (aOR 2.44, 95% CI: 1.46–4.08; P = 0.001), were independent predictors of underweight status at 12–18 mo. Conclusions: At 12–18 mo of corrected age, a significantly greater proportion of preterm FGR neonates were wasted compared to AGA ones. The former also exhibited significantly greater catch-up growth than the latter. [ABSTRACT FROM AUTHOR]