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Multiple interventions have been studied for benzodiazepine-resistant status epilepticus (SE) in children and adults. This review aimed to summarize the available evidence and provide estimates of comparative effectiveness and ranking of treatment effects.All randomized controlled trials studying patients (1 month of age) with benzodiazepine-resistant SE were included. Outcomes including seizure cessation within 60 min, seizure freedom for 24 h, death, respiratory depression warranting intubation and cardiovascular instability were studied. Conventional and network meta-analyses (NMA) were done.Seventeen studies were included (16 in NMA). Phenobarbital and high-dose levetiracetam were significantly superior to phenytoin with respect to seizure cessation within 60 min. Network ranking demonstrated that phenobarbital had the highest probability of being the best among the studied interventions followed by high-dose levetiracetam and high-dose valproate. Network meta-analysis was limited by predominant indirect evidence and high heterogeneity.On pairwise comparisons, phenobarbital was found to be associated with a higher risk of need for intubation and cardiovascular instability. Levetiracetam had a better safety profile than fosphenytoin.Based on low quality evidence, phenobarbital appears to be the most effective agent for seizure cessation within 60 min of administration in patients with benzodiazepine resistant status epilepticus. High-dose levetiracetam, high-dose valproate and fosphenytoin are probably equally effective. Choice of medication may be guided by effectiveness, safety concerns, availability, cost and systemic co-morbidities.

Our objective was to compare the point prevalence of insulin resistance (IR) in children taking sodium valproate (VPA) and phenytoin sodium (PS) monotherapy for >1 year. 150 children, aged 6–18 years, were categorized (50 each) into - group A (VPA), group B (PS) and group C (healthy controls age-sex matched with group A). Groups were compared for metabolic complications and risk factors assessed. The point prevalence of IR and non-alcoholic fatty liver disease was significantly higher in children on VPA (12% and 34% respectively) than on PS and healthy controls, regardless of age, sex, pubertal and nutritional status. The presence of central obesity, acanthosis, hypertension, dyslipidaemia was significantly associated with IR but none showed an independent association on multivariate analysis. Therapy with VPA makes children susceptible to metabolic complications. Close monitoring will facilitate early detection and timely intervention.

This is a Brighton Collaboration case definition of thrombosis and thromboembolism to be used in the evaluation of adverse events following immunization, and for epidemiologic studies for the assessment of background incidence or hypothesis testing. The case definition was developed by a group of experts convened by the Coalition for Epidemic Preparedness Innovations (CEPI) in the context of active development of SARS-CoV-2 vaccines. The case definition format of the Brighton Collaboration was followed to develop a consensus definition and defined levels of certainty, after an exhaustive review of the literature and expert consultation. The document underwent peer review by the Brighton Collaboration Network and by selected expert reviewers prior to submission.

Early childhood development (ECD) provides building blocks for future educational achievement, emotional and moral development; the early years of life provide a critical window of opportunity for intervention. Experts across the world are emphasizing on promotion of ECD through the 5-pronged Nurturing Care Framework (NCF). The Sustainable Development Goals have focussed on optimum development for all children by 2030. For India, with 164.5 million population of children between 0-6 years, the magnitude of the problem is huge. We have been focusing on ECD since the launching of the Integrated Child Development Scheme (ICDS) in 1976. Many national policies and programs have evolved since then to promote ECD. Inspite of all efforts, the overall picture of early childhood development in India is still not optimal, due to multiple factors. All five components of nurturing care framework have not been included comprehensively in the services offered. If India focuses on these areas and comes up with a convergent ECD delivery system through a single portal that can be provided with equity at the grassroot level, coupled with proper documentation, we may proceed towards our goals at a better pace. Multiple stakeholders (the government, public and private health care providers, non-government organizations, professional bodies) need to work in synergy to enable us to reach the 2030 goals.

A large proportion of children under the age of five years who do not attain their expected developmental potential belong to low- and middle-income countries (LMICs). The strategies used for identifying children with high risk for developmental delay/disorders include developmental screening, surveillance, and monitoring. Suitability criteria for developmental screening tools in LMICs have been established, but few tools meet all the benchmarks. Based on these, the authors identified two tools that may be considered suitable in the Indian context; the International guide for monitoring child development and the Monitoring child development in the integrated management of childhood illnesses context. However, implementing and sustaining a universal developmental screening program using these is not feasible in the present circumstances. There is emerging evidence that parent intervention programs have significant impact on outcomes related to early childhood development (ECD). The nurturing care framework encompasses five strategies known to enhance ECD in young children even in the presence of adversities; good health, adequate nutrition, responsive caregiving, opportunities for early learning and safety and security. This article discusses the paradigm shift to incorporation of nurturing care-based preventive, supportive and promotive health care services in office practice with active parental involvement. This may prove to be a better option with a more positive, long lasting and quicker impact on ECD.

This is a Brighton Collaboration case definition of thrombosis and thromboembolism to be used in the evaluation of adverse events following immunization, and for epidemiologic studies for the assessment of background incidence or hypothesis testing. The case definition was developed by a group of experts convened by the Coalition for Epidemic Preparedness Innovations (CEPI) in the context of active development of SARS-CoV-2 vaccines. The case definition format of the Brighton Collaboration was followed to develop a consensus definition and defined levels of certainty, after an exhaustive review of the literature and expert consultation. The document underwent peer review by the Brighton Collaboration Network and by selected expert reviewers prior to submission., The SPEAC Project is funded in whole by CEPI.

OBJECTIVE To assess the point prevalence of peripheral neuropathy (PN) in children with type 1 diabetes mellitus (T1DM) and to determine their predictors. METHODS In this cross-sectional study, children aged 8-18 y with T1DM on insulin therapy for > 2 y and free from acute complications were enrolled. All participants were evaluated for symptoms of PN with diabetic neuropathy symptom (DNS) score and underwent a detailed neurological examination. Assessment of nerve dysfunction was done using nerve conduction studies (NCS). The disease-related factors that increase the risk of PN were determined. RESULTS Fifty children (52% boys) were enrolled with mean age of 12.2 ± 2.8 y and duration of diabetes 5.1 ± 2.1 y. No subject had clinical evidence or DNS score suggestive of PN. Twenty-eight (56%) children demonstrated subclinical neuropathy on NCS. Proportion of children with pure motor, pure sensory, and mixed motor-sensory neuropathy was 40%, 2%, and 14%, respectively. The peroneal nerve was the most common motor nerve affected. Poor glycemic control (HbA1c > 9%) and longer duration of diabetes (> 5 y) were significantly associated with the risk of PN (p value < 0.05). CONCLUSION A large proportion of children with T1DM have subclinical PN. Poor glycemic control and longer duration of diabetes are risk factors for nerve dysfunction. Neurophysiological studies should be performed in these children to facilitate early detection.

Objectives This cross‐sectional study was designed to test the applicability of the 1989, 2010, and 2017 International League Against Epilepsy (ILAE) classification of epilepsy in children from a resource‐limited setting in India. Methods Classification of seizure types and syndromes was done through parental interviews and review of medical records in children with epilepsy aged one month to 18 years. Available investigations including EEG, MRI, and metabolic/genetic tests were used in classifying patients as per the 1989, 2010, and 2017 ILAE (level II‐epilepsy type) classification. We compared the proportion of children remaining unclassified by each scheme. Results Seven hundred and twenty‐six children (436 males, mean age 6.4 ± 4.6 years) were enrolled. Using the 1989 ILAE classification, we were able to classify 95.7%, and 82.6% children by the 2010 scheme. The 2017 ILAE classification could classify all 726 children at level I (seizure type), 664 (91.0%) children at level II (epilepsy type), and an electroclinical syndrome could be identified in 409 (56.1%) of the children. An etiology could be identified in 75%, perinatal brain injury being the most frequent. West syndrome was the most common electroclinical syndrome, identified in 22.7% patients. The 1989 ILAE classification system was superior to the 2010 system (P = .01) in epilepsy classification. There was no difference between the 1989 and 2017 schemes (P = .31) or the 2010 and 2017 schemes (P = .10). Significance The 2017 ILAE classification, being multidimensional, allowed classification of children who could not undergo extensive evaluation due to economic constraints and also provided room for overlapping etiologies.

Objective To assess clinical profile and short term treatment outcomes of pediatric status epilepticus (SE) at a tertiary-care center in northern India. Methods Prospective cohort study enrolled children aged 1 month to 18 years presenting with SE to the emergency department. Enrolled children (109) were treated as per hospital protocols. Clinical features during hospitalization were noted. Pediatric overall performance category (POPC) scale was used for classification of outcome at the time of discharge. Results Acute symptomatic etiology was identified in 66 (60.6%) cases (CNS infections were predominant). Previous diagnosis of epilepsy was found in 32 (29.4%) children; and benzodiazepine responsive SE were seen in 65 (59.6%) children. Predictors of unfavorable outcome were acute symptomatic etiology (adjusted OR 4.50; 95% CI 1.49, 13.62) and no treatment administered prior to hospital (adjusted OR 3.97; 95% CI 1.06, 14.81). Conclusions Acute symptomatic etiology, mainly acute CNS infections, is the leading cause of SE in this region. Early and pre-hospital management with benzodiazepines may improve SE outcome.

Background: Existing scores to identify children at risk of hospitalized pneumonia-related mortality lack broad external validation. Our objective was to externally validate three such risk scores. Methods: We applied the Respiratory Index of Severity in Children (RISC) for HIV-negative children, the RISC-Malawi, and the Pneumonia Etiology Research for Child Health (PERCH) scores to hospitalized children in the Pneumonia REsearch Partnerships to Assess WHO REcommendations (PREPARE) data set. The PREPARE data set includes pooled data from 41 studies on pediatric pneumonia from across the world. We calculated test characteristics and the area under the curve (AUC) for each of these clinical prediction rules. Results: The RISC score for HIV-negative children was applied to 3574 children 0-24 months and demonstrated poor discriminatory ability (AUC = 0.66, 95% confidence interval (CI) = 0.58-0.73) in the identification of children at risk of hospitalized pneumonia-related mortality. The RISC-Malawi score had fair discriminatory value (AUC = 0.75, 95% CI = 0.74-0.77) among 17 864 children 2-59 months. The PERCH score was applied to 732 children 1-59 months and also demonstrated poor discriminatory value (AUC = 0.55, 95% CI = 0.37-0.73). Conclusions: In a large external application of the RISC, RISC-Malawi, and PERCH scores, a substantial number of children were misclassified for their risk of hospitalized pneumonia-related mortality. Although pneumonia risk scores have performed well among the cohorts in which they were derived, their performance diminished when externally applied. A generalizable risk assessment tool with higher sensitivity and specificity to identify children at risk of hospitalized pneumonia-related mortality may be needed. Such a generalizable risk assessment tool would need context-specific validation prior to implementation in that setting. publishedVersion

Background: Adverse drug reactions (ADRs) due to antiepileptic drugs (AEDs) in children contribute to poorer patient outcomes. However, reliable data ragarding such ADRs is not available. Objectives: Thus, the aim of the present study was to determine the incidence and patterns of ADRs of antiepileptic drugs in children aged 2-17 years presenting to a tertiary care teaching hospital. Methods: An observational study was conducted in the Department of Pediatrics, Kalawati Saran Children’s Hospital for a period of one year. Two hundred consecutive eligible patients (aged 2-17 yrs with epilepsy on AED) with consenting parents were enrolled. ADRs were noted using Paediatric Epilepsy Side Effect Questionnaire (PESQ) at clinic visits and any other ADRs reported by parents were also recorded. Causality, severity and avoidability assessments were done. Results: The mean age was 10.5 ± 3.6 years. A total of 139 ADRs occurred in 97 patients. One hundred and nine ADRs were reported by use of PESQ, in addition, 30 ADRs were reported by parents. Poor school result (33.8%) was the commonest ADR. Valproate (61.9%) was the main drug causing ADRs. Valproate, when used in polytherapy, was associated with more number of children experiencing ADRs (72.2%). The most common add on drug was clobazam (42.3%). Children with poorly controlled epilepsy were associated with more ADRs. Causality assessment revealed that 91.3% of the ADRs were probable. Most (94.9%) ADRs were of ‘mild’ category and 95.7% were probably preventable. Treatment was discontinued only in 6 patients of phenytoin toxicity.R Conclusion: Cognitive and neurological problems were the most common ADRs seen in children with epilepsy. Polytherapy significantly increases the likelihood of ADRs in children.

When a child is diagnosed with epilepsy, counseling regarding the same is done by the treating doctor. Most parents are frightened and have poor knowledge about epilepsy. Therapeutic advice including drug dosage, administration and side effects takes up the major part of physician's time, thereby neglecting important issues like home seizure management, follow up and others. These lacunae in knowledge require systematic patient and family education. To address these issues, an expert group meeting of pediatric neurologists and epileptologists in India along with social workers/epilepsy educators, legal experts, parents, and teachers was held. The various aspects regarding parental counseling in children with epilepsy were discussed and a consensus document was formulated. Here authors present the group consensus statement on counseling parents and caregivers of children with epilepsy. This document is intended to help physicians and pediatricians counsel the families when a child is diagnosed with epilepsy.

Congenital disorders of glycosylation (CDG) are multisystemic inherited metabolic disorders with marked phenotypic variability. The most frequent described type is PMM2-CDG (earlier known as CDG Type Ia) which presents either with pure neurologic features or with combined neurologic and systemic features. The classical presentation is characterized by varied combinations of developmental delay, hypotonia, ataxia, dysmorphism, inverted nipples, and abnormal fat distribution. Strokelike episodes and seizures are known acute complications that usually occur on a background of developmental delay, ataxia, or dysmorphism. We report here a developmentally normal young girl who presented with isolated strokelike episodes and was diagnosed to have CDG Type Ia. This condition should be kept in the differentials of unexplained strokelike episodes in children. The diagnosis has important therapeutic and prognostic implications.

To meet the sustainable development goals (SDG) target of reducing under-five mortality to 25 per 1000 live births, concerted efforts are required to end all preventable deaths of newborns and children under 5 y of age. There is evidence to support Integrated Management of Neonatal and Childhood Illness (IMNCI) as a cost- effective strategy which can improve child survival. IMNCI has 3 components- capacity building of health workers, health system strengthening and improving community and family practice. For best results, all three components of the IMNCI strategy should be implemented in a coordinated fashion. IMNCI implementation in india has been uneven. The main focus has been on capacity building and with little attention on system strengthening or improving community practices. Ill- sustained funding and poor monitoring and supervision system were additional factors which are major challenges. Since evidence based interventions remain same, IMNCI remains as relevant today as before. It would be appropriate to redesign it as per current needs and implement it with more planning with committed budget and inbuilt measures of quality improvement along with supportive supervision.

Summary Background and aims Patients on long term anti-epileptic drug therapy are prone for Vitamin D deficiency for a myriad of reasons. The aim of this research was to study the effect of high dose vitamin D supplementation on vitamin D nutrition status of children newly started on anti-epileptic drug therapy. Materials This randomized controlled trial was conducted in a tertiary care Children's Hospital at New Delhi from November 2011 to March 2013. Eighty three children in the age group 5–10 years newly started on anti-epileptic drugs (AED) were randomized into two groups; group A – the intervention group, to whom 60,000 IU vitamin D3 was given orally/month under direct supervision along with AED for a period of 6 months, and group B- the control group, to whom AED without vitamin D3 was given. Serum 25(OH)D, ionized calcium (iCa), total calcium (tCa), inorganic phosphate (iP), alkaline phosphatase (ALP) and parathyroid hormone (PTH) levels were assayed at baseline and at the end of 6 months and were compared within and between the two groups. Results The mean 25(OH)D in Group A was maintained at 6 months follow up [ 26 ng/ml, 95% CI 20–34 ng/ml] compared to baseline [25 ng/ml, 95% CI −19 to 33 ng/ml] [ p = 0.83]. In group B, there was a significant decrease in 25(OH)D levels at 6 months [13 ng/ml (95% CI 9 ng/ml–17 ng/ml)] compared to baseline [18 ng/ml (95% CI 13–24 ng/ml)] [p = 0.01]. At 6 months, mean serum 25(OH)D was significantly higher in group A as compared to group B (p = 0.005). Conclusion To conclude, oral administration of 60,000 IU vitamin D3/month is sufficient to maintain serum 25(OH)D level and prevent development of vitamin D deficiency in children newly started on AED over a period of 6 months. Non supplementation leads to the lowering of serum 25(OH)D in these children. Trial Registration Number CTRI/2017/08/009234.

Background: The use of ultrasound for immediate management of life-threatening conditions is one among the main changes of these last decades. Lung ultrasound has become more and more popular in the first decade of the 21st century, both in neonatal and in paediatric age groups. The lung ultrasound may be tool in differential diagnosis of respiratory distress in newborns. Aims and objectives: To assess diagnostic role of point of care lung ultrasound in neonates presented with respiratory distress and analyse the possibility of initiating management protocol based on lung ultrasound finding.Methods: Present non-randomized study-a hospital based analytical cross-sectional study was carried out in 60 patients with respiratory distress for a period of 18 months (January 2020-30 June 2021). Neonates suffering from respiratory distress (Pulmonary cause with Downe’s score >4) were included. Routine investigations, lung USG and chest x-ray examination was recorded. Detailed perinatal history were obtained.Results: Mode of delivery was LSCS among 38.3% and vaginal delivery among 61.7% subjects. The mode of respiratory support was CPAP among 33.3% and ventilator among 66.7% subjects. Mean birth weight of the study population was 2003.51±767.35 grams. Final diagnosis of the present study shows that majority of neonates had respiratory distress syndrome (38.33%) followed by birth asphyxia with respiratory distress (28.33%) followed by TTNB (11.66%). A total of 25.0% neonates had Downes score 4, 16.7% had 5, 25.0% had 6, 26.7% had 7 and 6.7% neonates had 8 score. X-ray findings showed sensitivity 56.67% and positive predictive value 100% were observed. On the basis of USG findings, 93.22% sensitivity and positive predictive value 98.21% were observed.Conclusions: Lung ultrasound is a non-invasive, bedside and repeatable method that could improve the management of neonatal respiratory distress. Neonatal lung ultrasound is a useful predictor of the need for intubation over chest x-ray. Combined lung consolidation, pleural line abnormalities and absence of A line findings were the most specific lung ultrasound findings in RDS which were present among majority of the patients.

To assess the point prevalence of peripheral neuropathy (PN) in children with type 1 diabetes mellitus (T1DM) and to determine their predictors.In this cross-sectional study, children aged 8-18 y with T1DM on insulin therapy for2 y and free from acute complications were enrolled. All participants were evaluated for symptoms of PN with diabetic neuropathy symptom (DNS) score and underwent a detailed neurological examination. Assessment of nerve dysfunction was done using nerve conduction studies (NCS). The disease-related factors that increase the risk of PN were determined.Fifty children (52% boys) were enrolled with mean age of 12.2 ± 2.8 y and duration of diabetes 5.1 ± 2.1 y. No subject had clinical evidence or DNS score suggestive of PN. Twenty-eight (56%) children demonstrated subclinical neuropathy on NCS. Proportion of children with pure motor, pure sensory, and mixed motor-sensory neuropathy was 40%, 2%, and 14%, respectively. The peroneal nerve was the most common motor nerve affected. Poor glycemic control (HbA1c 9%) and longer duration of diabetes (5 y) were significantly associated with the risk of PN (p value0.05).A large proportion of children with T1DM have subclinical PN. Poor glycemic control and longer duration of diabetes are risk factors for nerve dysfunction. Neurophysiological studies should be performed in these children to facilitate early detection.

To assess the diagnostic accuracy of Parent’s Evaluation of Developmental Status (PEDS), PEDS Developmental Milestones (PEDS:DM) and PEDS Combined for developmental screening of Indian children aged less than 2 y. A hospital-based study of diagnostic accuracy was conducted over 17 mo. Children under 24 mo (n = 180) were enrolled after exclusion of severe illnesses or known neurodevelopment disorders. The index tools included standardized Hindi translations of PEDS and PEDS:DM. The reference tool was Developmental Assessment Scale for Indian Infants (DASII). Both were administered by blinded researchers. Parameters of diagnostic accuracy were computed. There were 13 (7.2%) failures in PEDS, 119 (66.1%) in PEDS:DM and 119 (66.1%) in PEDS Combined. DASII identified 3 children with developmental delay. Sensitivity (Sn) [95% CI] of PEDS was 33.3 [0.8–90.6] and Specificity (Sp) 93.2 [88.5–96.5]. The Sn and Sp of both PEDS:DM and PEDS Combined were 100 [29.2–100] and 34.5 [27.5–42.0], respectively. Hindi translations of PEDS, PEDS:DM and PEDS Combined are not suitable for developmental screening of children less than 2 y due to suboptimal diagnostic accuracy.

There have been few case reports showing association of vitamin B12 deficiency with infantile spasms. We planned this study to see if there was an association of serum vitamin B12 deficiency in children with development of infantile spasms. Cases included children with infantile spasms of ages 6 months to 3 years. The controls were children in the same age group who had global developmental delay but no history of epileptic spasms. Mean serum vitamin B12, serum homocysteine, and urinary methylmalonic acid levels were measured in both groups and compared. Children with infantile spasms had lower mean serum vitamin B12 levels (354.1 pg/mL; standard deviation 234.1 pg/mL) as compared to children with global developmental delay without spasms (466.7 pg/mL; standard deviation 285.5 pg/mL) ( P value < .05). Mean serum homocysteine level (13.9 vs 7.8 μmol/L, P = .02) and mean urinary methylmalonic acid level (68.1 mmol/mol of creatinine vs 26.1 mmol/mol of creatinine, P = .03) were elevated in children with infantile spasms than in controls. Fourteen children (35.0%) with infantile spasms were vitamin B12 deficient compared with 3 (7.50%) controls ( P = .005). Thus, vitamin B12 deficiency may have an association with infantile spasms. More studies are needed before recommending routine measurement of serum B12 levels in children with infantile spasms.

BackgroundBacterial infections account for a significant proportion of neonatal and infant mortality globally. We aimed to identify predictors of death in infants with probable serious bacterial infection (PSBI) defined as signs/symptoms of possible serious bacterial infection along with baseline C-reactive protein (CRP) ≥12 mg/l.MethodsWe did a secondary analysis using the data collected from 700 infants with PSBI who participated in a randomized controlled trial in India in which zinc or placebo was given in addition to the standard antibiotics. Logistic regression was used to estimate the associations between relevant variables and death within 21 days.ResultsThose infants who were fed cow's milk or formula before the illness episode had 3.7-fold (95% confidence interval (CI) 1.5-9.3) and 5.3-fold (95% CI 2.0-13.6) higher odds of death, respectively. Lethargy (odds ratio (OR) 2.4, 95% CI 1.1-5.4) and CRP (OR 1.9, 95% CI 1.1-3.3) were also independent predictors of death. In the model including only clinical features, female gender (OR 2.25, 95% CI 1.0-5.0), abdominal distention (3.7, 95% CI 1.1-12.3), and bulging fontanelle (5.8, 95% CI 1.1-30.5) were also independent predictors for death.ConclusionFormula or cow milk feeding prior to the illness, lethargy at the time of presentation, and high serum CRP levels predicted death in infants with PSBI.

Familial clustering of common forms of primary strabismus like esotropia (ET) and exotropia (XT) is observed in a proportion of the strabismus cohort. The genetic components of this remain unidentified. Linkage studies have demonstrated susceptibility locus for primary strabismus at the STBMS1 locus on 7p22.1 as well as other loci on 4q28.3 and 7q31.2. Recently next generation sequencing (NGS) technology has emerged as a powerful tool in discovery genomics and a large number of novel disease-causing variants are being reported. In this study, we recruited informative families for subsequent genetic analysis for disease-causing variant identification.All consecutive families with two or more affected members with primary concomitant horizontal strabismus were prospectively recruited at the ophthalmic outpatients department (OPD) of Lady Hardinge Medical College, New Delhi, from August 2014 to February 2017. Detailed phenotypic evaluation and pedigree documentation was performed.Of the 39 recruited families of north Indian origin, 18 families each had affected family members demonstrating either ET or XT. 100% concordance of the phenotype in the affected family members was observed in these families. While vertical transmission was observed in 17/18 families with XT, 7 with ET had affected members across one generation, 2 demonstrated consanguineous pedigree, and 2 comprised identical twin families. In 3 families, a combination of ET and XT was noted. This comprised one family with the ET and XT patients being from 2 separate arms of the family related by marriage, one family where one sibling had XT and the other had ET, and another family where the maternal aunt of the affected proband with ET had XT.Subjects with familial primary concomitant strabismus recruited in this study may provide a valuable resource to unravel the genetic determinants of this condition, which is a common disorder of early childhood with high ophthalmic morbidity.

Background In 2013, the World Health Organization (WHO) and CIOMS introduced a revised Causality Assessment Protocol (CAP) for Adverse Events following Immunization (AEFI). India is one of the first countries to adopt the revised CAP. This study describes the application of the revised CAP in India. Methods We describe use of CAP by India’s AEFI surveillance program to assess reported AEFIs. Using publicly available results of causality assessment for reported AEFIs, we describe the results by demographic characteristics and review the trends for the results of the causality assessment. Results A total of 771 reports of AEFI between January 2012 and January 2015, completed causality review by August 2016. The cases were reported as belonging to a cluster (54%; n = 302), hospitalized or requiring hospitalization (41%; n = 270), death (25%; n = 195), or resulting in disability (0.4%; n = 3). The most common combinations of vaccines leading to report of an AEFI were DTwP, Hepatitis B, and OPV (14%; n = 106), followed by Pentavalent and OPV (13%; n = 103), and JE vaccine (13%; n = 101). Using the WHO Algorithm, most AEFI reports (89%, n = 683) were classifiable. Classifiable AEFI reports included those with a consistent causal association (53%; n = 407), an inconsistent causal association (29%; n = 226) or were indeterminate causal association with implicated vaccine(s) or vaccination process (6.5%; n = 50) (Fig. 1); 88 reports remained unclassifiable. Conclusions The revised CAP was informative and useful in classifying most of the reviewed AEFIs in India. Unclassifiable reports could be minimized with more complete information from health records. Improvements in causality assessment, and standardization in reporting between countries, can improve public confidence in vaccine system performance and identify important vaccine safety signals.

Objective To evaluate the effect of zinc as an adjuvant therapy in radiologically confirmed pneumonia in children 2-24 months of age. Patients and methods We analyzed data of 212 children with pneumonia for whom chest X-ray films were available at enrollment and at least two radiologists agreed on the diagnosis of pneumonia. We compared the time to recovery in the two groups (n = 121, zinc group and n = 91, placebo group) using a Cox proportional hazards regression model. Results Time to recovery was similar in both groups [median interquartile range: zinc, 84 h (64, 140 h); placebo, 85 h (65, 140 h)]. The absolute risk reduction for treatment failure was 5.2% (95% confidence interval: -4.8, 15.1) with zinc supplementation. Conclusion There was no significant beneficial effect of zinc on the duration of recovery or risk of treatment failure in children with radiologically confirmed pneumonia.

Injection vincristine is an important component of therapy for acute lymphoblastic leukemia (ALL). An important adverse effect of vincristine is neurotoxicity. The incidence of this adverse effect is well studied. The present was undertaken to determine the incidence of vincristine-induced neurotoxicity in children with ALL after the induction of remission phase of chemotherapy and to ascertain its correlation with undernutrition, vitamin B12, folate and iron deficiency. Thirty children (1-18 years) with ALL were enrolled at the commencement of chemotherapy. The electrophysiological evaluation was done at baseline and repeated after four doses of vincristine (1.5 mg/m

Guillain-Barre syndrome (GBS) is a common acquired polyneuropathy in children.To describe the clinical and serial electrophysiological features along with short-term outcomes of children with GBS in north India.This was a prospective study conducted at a tertiary care pediatric hospital in north India.Consecutive children, aged 2 to 18 years, with GBS, presenting within 4-weeks of onset of weakness, diagnosed on clinical and/or electrophysiological grounds, were enrolled. The enrolled children underwent a detailed clinical-assessment followed by nerve conduction studies. Repeat nerve conduction studies were performed after 2-weeks of the first study to determine changes in the electrophysiological subtype. The patients were followed up for 3 months.Thirty-six children were studied. The mean age at presentation was 5.1 years [standard deviation (SD): 2.1]. The mean medical research council (MRC)-sum-score at admission was 24.1 (SD: 10.4). Thirty-three children (91%) had loss of ambulation, 24 (66%) had cranial nerve involvement, and 6 (16.6%) required ventilation. At presentation, 20 had acute motor axonal neuropathy (AMAN), 13 had acute inflammatory demyelinating polyneuropathy (AIDP), 2 had in-excitable nerves, and 1 had normal findings. Four children, initially diagnosed as AIDP, had AMAN with reversible conduction failure on the repeat study. The final classification was AMAN in 25 (69.4%; 95% confidence interval (CI), 51.9-83.7%) and AIDP in 9 children (25%; 95% CI, 12.1-42.2%). Only one patient was nonambulatory at a 3-month follow-up (n = 32). The Erasmus GBS outcome score was 2 in 2 (5.6%), 3 in 5 (13.9%), 4 in 26 (72.2%), and 5 in 3 (8.3%) patients.The serial electrophysiological studies were helpful in establishing the final correct diagnosis.

Objectives To explore the existing evidence for anti-convulsant drugs and their routes of administration in treating acute seizures in children and adults when intravenous access is not available. Methods All major databases including Medline via Ovid, PubMed, Cochrane CENTRAL, Embase, and Google Scholar were searched till May 2015. Randomized and quasi-randomized controlled trials comparing two anti-convulsant drugs (at least one comparator being administered through non-intravenous route) for treatment of acute seizures were included. Outcome measures Primary outcome measure was proportion of children with clinical seizure cessation within 10min of drug administration. Secondary outcome measures were time taken to clinical seizure cessation from the time of admission and from the time of drug administration, and incidence of significant adverse effects. Results Out of the 19,165 citations, 26 studies were finally included. Regarding the primary outcome measure, the quality of evidence was ‘moderate' for following 3 comparisons: buccal midazolam being superior to per-rectal diazepam (RR 1.14; 95% CI, 1.06–1.24), intra-nasal lorazepam being same as intravenous lorazepam (RR 1.04; 95% CI, 0.89–1.22) and intramuscular paraldehyde (RR 1.22; 95% CI, 0.99–1.52). The quality of evidence was ‘very-low' for 1 comparison: per-rectal lorazepam being superior to per-rectal diazepam (RR 3.17; 95% CI, 1.63–6.14). The quality of evidence was ‘low' for following 2 comparisons: sub-lingual lorazepam being inferior to rectal diazepam (RR 0.71; 95% CI, 0.62–0.81), and intranasal midazolam being superior to per-rectal diazepam (RR 1.14; 95% CI, 1.05–1.25). The rest of the comparisons did not show any difference, but the quality of evidence was ‘low' to ‘very low'. The time to seizure cessation after drug administration was lower in the intravenous group. However, time to seizure cessation after presentation (includes time for drug administration) was lower in the non-intravenous group. Significant adverse effects were infrequently reported and when present, were similar in both the groups. Conclusions When intravenous access is not available, non-intravenous routes of administration of benzodiazepines should be considered for the control of acute seizures in children/adults. The preference may be guided by availability, expertise and social preference. [PROSPERO No: CRD42015019012].

Objectives: To assess the neurodevelopmental outcome of West syndrome (WS) in Indian children, who differ in their clinical profile from the western population. Materials and Methods: This cross-sectional study enrolled children aged 2--5 years with prior diagnosis of WS between November 2013 and March 2015. They were assessed for epilepsy outcome and developmental outcome using developmental profile 3 (DP3) and vineland adaptive behavioral scale II (VABS II). Results: Sixty-one children were enrolled. Perinatal asphyxia (40.9%), neonatal hypoglycemia (14.8%), and neonatal meningitis (9.8%) were predominant causes among the children with known etiology. Favorable epilepsy outcome (seizure freedom for >6 months) was observed in 29/61 patients (47.5%). Moderate to severe developmental delay was observed in 55/61 children (91.8%). Favorable developmental outcome (GDS by DP3 >70) was observed in just 5/61 (8%) patients. Conclusions: This study highlights the high prevalence of developmental delay in this population of children with WS, with adverse perinatal events being the most common etiology.

There have been few case reports showing association of vitamin B

India has implemented the World Health Organization's revised Causality Assessment Protocol for adverse events following immunization (AEFI). We describe the number and types of serious/severe AEFIs, including deaths.Analysis of causality classification of reported serious/severe AEFIs from 1 January 2012 to 7 January 2016 was done. Classification includes (A) consistent with causal association to immunization; (B) indeterminate; (C) coincidental association; or (D) unclassifiable. We present descriptive statistics across each category.Analysis of causality assessment completed for 1037 reports of serious AEFIs: 499 (48%) were causally associated, 84 (8%) were indeterminate, 323 (31%) were coincidental, and 131 (13%) were unclassifiable. Of the 499 reports in the A category, the events were causally linked to vaccine product for 189 (18%), to immunization error for 135 (13%), and to immunization anxiety for 175 (17%). Among 279 reported deaths, more than half (55%; n = 153) were coincidental events and 37% were unclassifiable.Causality assessment of AEFI cases is an important component of vaccination programs and post-marketing surveillance of vaccines. Field reporting and investigation of AEFIs can be improved for many severe or serious reports, most of which are not causally linked to the vaccination program.

Background: The modified Atkins diet (MAD) has been used predominantly in older children, adolescents, and adults. There is a paucity of data on the use of the MAD in refractory epilepsy in young children. Objectives: This study was planned to evaluate the efficacy and tolerability of the MAD in refractory epilepsy in young children. Methods: This study recruited children aged 9 months to 3 years with refractory seizures. Children received MAD for 6-month with the on-going anticonvulsant medications being continued unchanged. Reduction in seizure frequency was the primary outcome measure. Adverse effects were also studied. Results: Thirty-one children with daily seizures were studied with a median age of 18-month (range 9-30 months). West syndrome was the most common epilepsy syndrome (26, 86.6%). Twenty-one children remained on diet at 3 months and 13 at 6 months. The children who achieved >50% seizure reduction were 17 (54.8%) at 3 months and 9 (29%) at 6 months. Refusal to eat was a significant problem seen in eight children. Three children discontinued the diet due to adverse effects. Conclusion: The MAD was found to be feasible, effective, and well-tolerated.

Hereditary myoclonus-dystonia (DYT 11) is caused by the epsilon-sarcoglycan (SGCE) mutation. The clinical details and investigations of cases diagnosed with myoclonus-dystonia were reviewed. We describe 5 patients (3 families) with myoclonus-dystonia diagnosed at our center. Majority of the patients had the classical phenotype with few atypical features (adult-onset disease and onset in lower limbs). Four patients carried a mutant variant in the SGCE-gene. A diagnosis of myoclonus-dystonia should be considered in cognitively normal patients with early-onset myoclonus (that may occur both at rest and/or action) with or without dystonia and with or without psychiatric-disturbances.

Tuberculous meningitis, an extrapulmonary manifestation of tuberculosis, is associated with poor prognosis and high mortality in the pediatric age group with delayed treatment. The main causative agent in humans is Mycobacterium tuberculosis but other members of the M. tuberculosis complex especially Mycobacterium bovis, have also been implicated as potential human pathogens. Our study utilizes the hup B gene (Rv2986c in M. tuberculosis and Mb3010c in M. bovis) as a target for a two-step 'in house' nested polymerase chain reaction (PCR) in cerebrospinal fluid samples and can differentiate between M. tuberculosis and M. bovis. The products obtained for M. tuberculosis and M. bovis are 116 and 89 base pair respectively due to a 27 base pair deletion at the C-terminal of hup B gene of M. bovis. Fifty cases, suggestive of tuberculous meningitis, and fifty controls (admitted for other causes) were chosen as per Modified Ahuja's criteria and correlated with response to therapy. They were regrouped retrospectively as definitive tuberculous meningitis [25] and non-tuberculous meningitis [75]. Of the non-tuberculous meningitis category, 45 patients were diagnosed as pyogenic meningitis while the rest were diagnosed as febrile seizures [16], hypocalcemic seizures [11] and enteric encephalopathy [3]. Mycobacterial DNA extracted from cerebrospinal fluid samples were subjected to a two-step nested PCR. A sensitivity of 92% and specificity of 98.7% was obtained. M. tuberculosis and M. bovis infection was seen in 56% and 35% of the PCR positive cases respectively while 9% showed co-infection with both species. Thus this novel gene target shows promising potential as a rapid molecular diagnostic aid in pediatric tuberculous meningitis where rapid institution of antituberculosis therapy is essential.

Background SLC30A10 mutations cause an autosomal recessive disorder, characterized by hypermanganesaemia, polycythemia, early-onset dystonia, paraparesis, or late-onset parkinsonism, and chronic liver disease. This is the first identified inborn error of Mn metabolism in humans, reported in 10 families thus far. Methods Methods for this study consisted of clinical examination, neuroimaging studies (MRI), serum dosages, and SLC30A10 genetic analysis. Results We describe early disease manifestations (including videos) in 5 previously unreported Indian children, carrying novel homozygous SLC30A10 mutations. Gait and speech disturbances, falls, dystonias, and central hypotonia were the presenting neurological features, starting within the first 5 years of life. All children also had severe hypermanganesemia, polycythemia, variable degree of liver disease, and marked brain MRI T1 hyperintensities. Conclusions Our findings expand the mutational and clinical spectra of this recently recognized disorder. An early diagnosis is warranted, because treatment with manganese-chelating agents, iron supplementation, or their combination might improve symptoms and prevent progression of this otherwise potentially fatal disease. © 2015 International Parkinson and Movement Disorder Society