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Showing papers in "Indian Pediatrics in 2021"


Journal ArticleDOI
TL;DR: The Indian Society of Pediatric Nephrology as discussed by the authors proposed a revised guideline for Steroid Sensitive Nephrotic Syndrome (SSNS), which combines evidence-based recommendations and expert opinion.
Abstract: Steroid sensitive nephrotic syndrome (SSNS) is one of the most common chronic kidney diseases in children. These guidelines update the existing Indian Society of Pediatric Nephrology recommendations on its management. To frame revised guidelines on diagnosis, evaluation, management and supportive care of patients with the illness. The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by review of literature and evaluation of evidence by experts in two face-to-face meetings. The initial statements provide advice for evaluation at onset and follow up and indications for kidney biopsy. Subsequent statements provide recommendations for management of the first episode of illness and of disease relapses. Recommendations on the use of immunosuppressive strategies in patients with frequent relapses and steroid dependence are accompanied by suggestions for step-wise approach and plan of monitoring. Guidance is also provided regarding the management of common complications including edema, hypovolemia and serious infections. Advice on immunization and transition of care is given. The revised guideline is intended to improve the management and outcomes of patients with SSNS, and provide directions for future research.

30 citations


Journal ArticleDOI
TL;DR: In this paper, the authors describe clinical outcomes and risk factors for transmission in neonates born to mothers with perinatal severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection.
Abstract: BACKGROUND: Limited evidence exists on perinatal transmission and outcomes of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in neonates OBJECTIVE: To describe clinical outcomes and risk factors for transmission in neonates born to mothers with perinatal SARS-CoV-2 infection DESIGN: Prospective cohort of suspected and confirmed SARS-CoV-2 infected neonates entered in National Neonatology Forum (NNF) of India registry SUBJECTS: Neonates born to women with SARS-CoV-2 infection within two weeks before or two days after birth and neonates with SARS-CoV-2 infection OUTCOMES: Incidence and risk factors of perinatal transmission RESULT: Among 1713 neonates, SARS-CoV-2 infection status was available for 1330 intramural and 104 extramural neonates SARS-CoV-2 positivity was reported in 144 intramural and 39 extramural neonates Perinatal transmission occurred in 106 (8%) and horizontal transmission in 21 (1 5%) intramural neonates Neonates roomed-in with mother had higher transmission risk (RR1 16, 95%CI 1 1 to 2 4;P=0 01) No association was noted with the mode of delivery or type of feeding The majority of neonates positive for SARS-CoV2 were asymptomatic Intramural SARS-CoV-2 positive neonates were more likely to be symptomatic (RR 5, 95%CI 3 3 to 7 7;P<0 0001) and need resuscitation (RR 2, 95%CI 1 0 to 3 9;P=0 05) compared to SARS-CoV-2 negative neonates Amongst symptomatic neonates, most morbidities were related to prematurity and perinatal events CONCLUSION: Data from a large cohort suggests perinatal transmission of SARS-CoV-2 infection and increased morbidity in infected infants

20 citations


Journal ArticleDOI
TL;DR: In this paper, the clinical profile and outcome of children with MIS-C treated with methylprednisolone pulse therapy and/or intravenous immunoglobulin (IVIG) were studied.
Abstract: To study the clinical profile and outcome of children with MIS-C treated with methylprednisolone pulse therapy and/or intravenous immunoglobulin (IVIG). This prospective observational study included children satisfying CDC MIS-C criteria admitted from September to November, 2020. Primary outcome was persistence of fever beyond 36 hours after start of immunomodulation therapy. Secondary outcomes included duration of ICU stay, mortality, need for repeat immunomodulation, time to normalization of CRP and persistence of coronary abnormalities at 2 weeks. Study population included 32 patients with MIS-C with median (IQR) age of 7.5 (5–9.5) years. The proportion of children with gastrointestinal symptoms was 27 (84%), cardiac was 29 (91%) and coronary artery dilatation was 11 (34%). Pulse methylprednisolone and intravenous immunoglobulin were used as first line therapy in 26 (81%), and 6 (19%) patients, respec-tively. Treatment failure was observed in 2/26 patients in methylprednisolone group and 2/6 patients in IVIG group. C-reactive protein levels less than 60mg/L by day 3 was seen in 17(74%) in methylprednisolone group and 2 (25%) in IVIG group (P=0.014). There was no mortality. At 2 weeks follow-up coronary artery dilatation persisted in 4 in methylprednisolone group and 1 in IVIG group. In patients with SARS-CoV-2 related MIS-C, methylprednisolone pulse therapy was associated with favorable short-term outcomes.

15 citations


Journal ArticleDOI
TL;DR: Recommendation of a booster dose of injectable polio vaccine at 4–6 years for children who have received the initial IPV doses as per the ACVIP/IAP schedule, re-emphasis on the importance of IPV in the primary immunization schedule, preferred timing of second dose of varicella vaccine at 3–6 months after the first dose, and uniform dosing recommendation.
Abstract: In view of new developments in vaccinology and the availability of new vaccines, there is a need to revise/review the existing immunization recommendations. Advisory Committee on Vaccines and Immunization Practices (ACVIP) of Indian Academy of Pediatrics (IAP) had a physical meeting in March, 2020 followed by online meetings (September-October, 2020), to discuss the updates and new recommendations. Opinion of each member was sought on the various recommendations and updates, following which an evidence-based consensus was reached. To review and revise the IAP recommendations for 2020–21 and issue recommendations on existing and new vaccines. The major changes include recommendation of a booster dose of injectable polio vaccine (IPV) at 4–6 years for children who have received the initial IPV doses as per the ACVIP/IAP schedule, re-emphasis on the importance of IPV in the primary immunization schedule, preferred timing of second dose of varicella vaccine at 3–6 months after the first dose, and uniform dosing recommendation of 0.5 mL (15 µg HA) for inactivated influenza vaccines.

14 citations


Journal ArticleDOI
Abstract: OBJECTIVE To assess the efficacy and safety of thalidomide in children with transfusion-dependent thalassemia. METHODS This prospective, single center, open-label study enrolled children aged 12-18 years, and who received thalidomide for a duration of 6 months at a starting dose of 2-3 mg/kg/day. Efficacy was assessed by reduction in transfusion requirement and rate of fall of hemoglobin. Efficacy was classified as major, moderate and minimal/no response depending on the reduction in transfusion requirement. Safety was assessed by adverse effects related to thalidomide. RESULTS 37 children [mean (SD) age, 14.7 (1.8) years were included. Rate of fall of hemoglobin reduced from a mean of 1.0 (0.24) g/week pre-thalidomide therapy to 0.58 (0.26) g/week after 6 months of thalidomide (P<0.001). 19 children (51.3%) had major response and 12 (32.4%) had moderate response. In 13.5% and 32.4% children response was observed within the first and second month of therapy, respectively. 15 (40.5%) children remained transfusion - free for a median (IQR) time of 6 (3-10) weeks of thalidomide therapy. Mean serum ferritin (SD) decreased from 1758.9 (835.1) to 1549.6(1016.9) (P<0.001). Mean HbF (SD) showed an increase from 2.95(2.6) to 49.2(33.3) (P<0.001). In 32 children, 47 adverse events were observed. Common adverse events were constipation and neutropenia (mostly mild). CONCLUSIONS Thalidomide resulted in major/moderate response in majority of children with transfusion-dependent thalassemia with satisfactory adverse effect profile.

13 citations


Journal ArticleDOI
TL;DR: In this paper, the authors present revised guidelines on diagnosis and evaluation, treatment and follow up, and supportive care of patients with the illness, which combine evidence-based recommendations and expert opinion.
Abstract: Justification The management of steroid resistant nephrotic syndrome (SRNS) is challenging. These guidelines update existing 2009 Indian Society of Pediatric Nephrology recommendations on its management. Objective To frame revised guidelines on diagnosis and evaluation, treatment and follow up, and supportive care of patients with the illness. Process The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by systematic review of literature, evaluation of evidence by experts and two face-to-face meetings. Recommendations Fourteen statements provide updated advice for managing steroid resistance, and underscore the importance of estimating proteinuria and baseline kidney function, and the need for kidney biopsy and genetic screening. Calcineurin inhibitors are recommended as most effective in inducing remission of proteinuria, the chief factor associated with long-term renal survival. Advice on managing allograft recurrence, congenital nephrotic syndrome, and monitoring and supportive care, including transition of care, are described. This revised practice guideline is intended to improve management and patient outcomes, and provide direction for future research.

11 citations



Journal ArticleDOI
TL;DR: In this article, the authors study the various comorbidities and their impact on outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected children.
Abstract: Objective To study the various comorbidities and their impact on outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected children. Methodology Review of medical records of 120 children (58.4% males), aged 1 month to 18 years, admitted between 1 March and 31 December, 2020 with at least one positive RT-PCR test for SARS-CoV-2. Clinical and demographic variables were compared between children with and without co-morbidities. Results 62 (51.7%) children had comorbidities. The most common comorbidity was tuberculosis (32.3%) followed by other infections (27.4%) and hematological (19.4%) conditions. Fever (89.2%) was the most common clinical feature followed by respiratory (52.5%) and gastrointestinal (32.5%) manifestations. There was no significant difference in the severity of COVID illness, length of hospital stay and adverse outcomes (ventilation and mortality) among children with and without comorbidities. Conclusions The presence of a comorbid illness in pediatric inpatients with COVID-19 did not impact the illness severity, length of hospitalization, ventilation requirement and mortality.

10 citations


Journal ArticleDOI
TL;DR: In this article, the authors study clinical characteristics and outcome of children with admitted to a paediatric hospital in Mumbai, India, to assess the clinico-demographic characteristics, disease severity and factors predicting outcome in COVID-19 children.
Abstract: OBJECTIVE: To study clinical characteristics and outcome of children with admitted to a paediatric hospital in Mumbai, India METHODS: Review of medical records of 969 children admitted between 19 March and 7 August, 2020, to assess the clinico-demographic characteristics, disease severity and factors predicting outcome in COVID-19 children Variables were compared between children who were previously healthy (Group I) and those with co-morbidity (Group II) RESULTS: 123 (71 boys) children with median (IQR) age of 3 (0 7- 6) years were admitted, of which 47 (38%) had co-morbidities 39 (32 %) children required intensive care and 14 (11 4%) died Male sex, respiratory manifestation, oxygen saturation <94%;at admission, mechanical ventilation, inotrope, hospital stay of <10 days were independent predictors of mortality Oxygen saturation <94% at admission (OR 35 9, 95% CI 1 5-856) and hospital stay <10 days (OR 9 1, 95% CI 1 04-99 1) were significant CONCLUSION: COVID-19 in children with co-morbidities causes severe disease Association of mortality with oxygen saturation by pulse oximeter <94% on admission, and hospital stay <10 days, needs further evaluation

9 citations


Journal ArticleDOI
TL;DR: WINSROP, CHOP-ROP and ROPScore were not sensitive enough to replace the gestational age, weight and risk factor-based screening criteria for type 1 ROP.
Abstract: Algorithms for predicting retinopathy of prematurity (ROP) requiring treatment need to be validated in Indian settings to determine if the burden of screening can be reduced without compromising the sensitivity of existing gestation and weight-based cut offs. To evaluate the performance of the available algorithms namely, WINROP (Weight, Insulin-like growth factor I, Neonatal ROP), CHOP-ROP (children’s Hospital of Philadelphia ROP) and ROPScore in predicting type 1 ROP and time from alarm to treatment by each algorithm. Ambispective observational. Tertiary care neonatal intensive care unit in India. Neonates less than 32 weeks or less than 1500 g born between July, 2013 to June, 2019 (N=578), who underwent ROP screening. Sensitivity, specificity and time from alarm to treatment by each algorithm. The sensitivity and specificity of WINROP was 85% and 36%, for CHOP-ROP it was 54% and 71%, and for ROPScore it was 73% and 67%, respectively in detecting type 1 ROP. A total of 50/51 (98%) of neonates with type 1 ROP underwent treatment at median gestation of 9 weeks and median time from alarm to treatment by WINROP, CHOP-ROP and ROPScore was 7, 7 and 3 weeks, respectively. WINROP, CHOP-ROP and ROPScore were not sensitive enough to replace the gestational age, weight and risk factor-based screening criteria for type 1 ROP.

8 citations


Journal ArticleDOI
TL;DR: In this paper, the authors compared sensory processing and mealtime problem behaviors among children with autism spectrum disorder (ASD) and typically developing controls, and examined the relationship between atypical sensory processing, and eating problems in children with ASD.
Abstract: To compare sensory processing and mealtime problem behaviors among children with autism spectrum disorder (ASD) and typically developing controls, and to examine the relationship between atypical sensory processing and eating problems in children with ASD. 50 children (4–10 years) with a diagnosis of ASD as per DSM-5 were recruited from the pediatric psychology clinic of a tertiary care center in India. The Brief Assessment of Mealtime Behavior in Children (BAMBIC) and the Short Sensory Profile (SSP) were administered to measure feeding and sensory processing problems, respectively. Parents were interviewed about their child’s dietary intake using a 3-day dietary recall. The ASD group showed greater mealtime behavior problems than the control group and had significantly higher total scores on the BAMBIC (P<0.001), and on two of the three subscales including food refusal (P<0.001) and disruptive behavior (P<0.001). The ASD group, relative to the neurotypical children, showed atypical response on majority of the subscales of the short sensory profile including tactile sensitivity (P<0.001), taste sensitivity (P<0.001), movement sensitivity (P<0.001), under responsiveness (P<0.001), auditory filtering (P<0.001), low weak/energy (P=0.02), and visual/auditory sensitivity (P<0.001). The study underscores the need for detailed evaluation of sensory processing and feeding problems of children with ASD so that the interventions can be tailored to address their unique sensory characteristics.

Journal ArticleDOI
TL;DR: Neonatal data regarding SARS-CoV-2 is sparse from India and only 50% of the positive babies had positive mothers/ caretakers, a contact could not be traced in others.
Abstract: Neonatal data regarding SARS-CoV-2 is sparse from India. On review of hospital records from April- August, 2020, 18/423 (4.25%) neonates were SARS-CoV-2 RT-PCR positive. 15 (83.3%) neonates recovered and 3 (16.6%) succumbed. Only 50% of the positive babies had positive mothers/ caretakers, a contact could not be traced in others.


Journal ArticleDOI
TL;DR: Severe lymphopenia, raised C-reactive protein, raised erythrocyte sedimentation rate, and high (>500ng/mL) levels of D-dimer were common and majority of children aged <18 years with SARS-CoV-2 infection had viral clearance within 14 days.
Abstract: Objective To study the clinical and laboratory profile and to assess period for viral clearance in COVID 19 children. Method We reviewed hospital records of children ( Results 81.2% of the 85 children studied were asymptomatic and 3 (8.5%) died. Severe lymphopenia (43.8%), raised C-reactive protein (93.8%), raised erythrocyte sedimentation rate (75%) and high (>500ng/mL) levels of D-dimer (37.5%) were common. Median (IQR) duration of viral shedding was 7 (5-10) days, with range of 2 to 45 days; 96.3% had viral clearance within 14 days. Conclusions Majority of children aged

Journal ArticleDOI
TL;DR: In this article, a review of the different types of vaping devices and of the substances contained in e-cigarette liquids is provided, including risks for the developing brain, acute lung injuries and long-term mental health effects.
Abstract: While the marketing and sale of electronic cigarettes (e-cigarettes) in India is forbidden since September, 2019, vaping represents a significant risk to the health and safety of Indian adolescents. Though the prevalence of youth e-cigarette use in India remains unknown, pediatricians are often brought to provide care to youth who vape. In this commentary, background information on e-cigarettes including a review of the different types of vaping devices and of the substances contained in e-cigarette liquids is provided. The short- and long-term health risks associated with vaping, including risks for the developing brain, acute lung injuries and long-term mental health effects, and a practical approach for clinical management of e-cigarette use for Indian pediatricians is presented. Public health measures to prevent and reduce youth vaping and a review of current Indian laws and policies around e-cigarette use are also mentioned.

Journal ArticleDOI
TL;DR: In this article, the adverse effects of propranolol therapy in infantile hemangioma were assessed using an ambispective study from August 2011 to December 2019.
Abstract: To assess the adverse effects of propranolol therapy in infantile hemangioma. An ambispective study was conducted from August 2011 to December 2019. In retrospective arm all children managed for infantile hemangioma with propranolol were included and case records were assessed for adverse reactions. In prospective arm the adverse reactions were identified on the basis of predefined criteria. A total of 514 patients (358 retrospective records) were included. A majority, 378 (73.5%) patients had an excellent response, 75 (14.5%) had partial response and 61 (11.8 %) had no response. A total of 82 (15.9%) patients experienced at least one adverse effect. Diarrhea with weight loss (27, 32.9%) and irritability with decreased sleep (21, 25.6%) were the most common adverse effects. The adverse effects in 22 (4.2%) cases lead to the discontinuation of propranolol. Younger age, low body weight and early onset were risk factors for development of severe adverse reactions. Young children with low body weight were at higher risk for adverse effects of propranolol.

Journal ArticleDOI
Abstract: To assess if salivary C-reactive protein (CRP) can be detected in neonatal sepsis and correlate the levels of salivary and serum CRP. This analytical cross-sectional study included all neonates ≤28 days of life with suspected sepsis or with perinatal risk factors for sepsis. Saliva was collected using an absorbent swab and analyzed by enzyme-linked immunosorbent assay, along with serum CRP. Salivary CRP was detectable in 135 subjects (99%). An increase was seen in median (IQR) levels from 0.25 (0.13,0.3) ng/mL in clinical sepsis group to 0.6 (0.3,1.4) ng/mL in screen positive/blood culture negative group, and to 1.98 (0.54, 2.95) ng/mL in blood culture positive group. There was a moderate positive correlation between salivary and serum CRP (r=0.63, P value 0.01). On receiver-operator characteristics curve, the area under the curve of salivary CRP for predicting serum CRP ≥10 mg/L was 0.861 (95% CI, 0.78 to 0.94; P < 0.001), with the optimal salivary CRP cut-off being 0.6 ng/mL. Salivary CRP could be used as an alternative biomarker of neonatal sepsis.

Journal ArticleDOI
TL;DR: In this paper, the authors describe clinical features in Indian girls with Turner syndrome along with the phenotype-karyotype correlation, and report some novel associations of karyotype with non-endocrine parameters in Turner syndrome.
Abstract: OBJECTIVE To describe clinical features in Indian girls with Turner syndrome along with the phenotype-karyotype correlation. METHODS 103 girls with Turner syndrome were divided into karyotype-groups: Classic (45X), 45,X/46,XX mosaics, isochromosomeXq (46,X,iXq and 45,X/46,X,iXq mosaics), 45,X/46,XYmosaics and structural defects, and analyzed for phenotypic differences. RESULTS Majority (44.1%) had classic karyotype followed by isochromosome-Xq (26.5%). Classic Turner syndrome had higher prevalence of most skeletal and cutaneous stigmata, cubitus valgus (68.3%) and multiple nevi (68.2%) being the commonest. Bicuspid aortic valve was most common in 45,X/46,XX mosaics (5/15, 33.3%), and aortic coarctation in classic TS (3/42, 7.2%). Congenital renal anomalies occurred mostly in classic TS (6/42,14.3%). Overt hypothyroidism, conductive deafness and recurrent otitis media were commonest in isochromosomes (P<0.03). 45,X/46,XY mosaics had highest IQ (P<0.005). CONCLUSIONS We report some novel associations of karyotype with non-endocrine parameters in Turner syndrome. In resource-limited settings, underlying karyotype may help prioritize screening investigations in girls with Turner syndrome.

Journal ArticleDOI
TL;DR: In this paper, the authors used observed data rather than simulation to estimate the number of children (0-14 years), as well as children and adolescents (0 -19 years), in India who develop cancer every year at the national and state/union territory (UT) level.
Abstract: Background Hitherto, incidence burden of childhood cancer in India has been derived from GLOBOCAN data. Recent analyses have challenged whether this accurately measures the true incidence of childhood cancer. Objective To use observed data rather than simulation to estimate the number of children (0-14 years), as well as number of children and adolescents (0-19 years), in India who develop cancer every year at the national and state/union territory (UT) level. Methods Age-specific (five year groups), sex-specific, and state/UT specific population data from India Census 2011 was used. Global average incidence rates from the International Incidence of Childhood Cancer 3 (IICC3) report were used. Incidence rates per million person-years for the 0-14 years and 0-19 years age groups were age-adjusted using the world standard population to provide age-standardized incidence rates, using the age-specific incidence rates for individual age groups (0-4 years, 5-9 years, 10-14 years, and 15-19 years). Results The national number of children (0-14 years) and, children and adolescents (0-19 years) that may develop cancer every year based on 2011 census are 52,366 and 76,805 persons respectively. Cancer type specific incidence is provided for each state/UT for these age ranges. This national incidence is approximately double of the GLOBOCAN 2018 estimates of incidence of children diagnosed and registered with cancer and the differential is greater in girls. Conclusions Our analysis proposes new estimates of incident childhood cancer cases in India for children and adolescents. Future regional, national and international research on childhood cancer epidemiology and healthcare accessibility would help further refine these estimates.

Journal ArticleDOI
TL;DR: In this paper, the authors compared the clinical profile, treatment, and outcomes of PCR-positive and PCR-negative antibody-positive critically ill children with multisystem inflammatory syndrome (MIS-C).
Abstract: To compare the clinical profile, treatment, and outcomes of PCR-positive and PCR-negative antibody-positive critically ill children with multisystem inflammatory syndrome (MIS-C). This retrospective observational study was done at a tertiary care coronavirus disease 19 (COVID-19) pediatric intensive care unit in India. The baseline characteristics, clinical profile, treatment, and outcomes in seventeen critically ill children diagnosed with MIS-C were analyzed from 1 July to 31 October, 2020. Sixteen out of 17 children presented with hypotensive shock and respiratory distress. Mean (SD) age of PCR-negative antibody-positive and PCR-positive children was 11 (4.4) and 5 (3.7) years, respectively (P=0.007). The former group had significantly higher mean (SD) D-dimer levels [16,651 (14859) ng/mL vs 3082 (2591) ng/mL; P=0.02]. All received intensive care management and steroid therapy; 7 children received intravenous immunoglobulin. 14 children survived and 3 died. The outcome of children with MIS-C was good if recognized early and received intensive care.

Journal ArticleDOI
TL;DR: In this article, the authors describe the broader clinical spectrum of COVID-19 in children, including fever, excessive cry and dry cough; digestive symptoms were frequently found (24%) and most common signs were pharyngeal erythema and irritability.
Abstract: To describe the broader clinical spectrum of COVID-19 in children. In this descriptive, prospective study, we included confirmed pediatric patients with COVID-19 who presented to the emergency department of a pediatric tertiary care center from April to July, 2020. All patients were confirmed by the SARS-CoV-2 RT-PCR test, and we analyzed 24 symptoms and 25 signs. Among the 50 patients with COVID-19, the most common symptoms were fever, excessive cry and dry cough; digestive symptoms were frequently found (24%). The most common signs were pharyngeal erythema and irritability. Clinicians should recognize that the clinical spectrum of COVID-19 in children is wider than previously described, often with nonspecific signs and symptoms, and digestive symptoms should raise suspicion.

Journal ArticleDOI
TL;DR: In this article, the authors compared the efficacy of the modified Atkins diet (mAD) and low glycemic index treatment (LGIT) among children with drug-resistant epilepsy.
Abstract: To compare the efficacy of the modified Atkins diet (mAD) and low glycemic index treatment (LGIT) among children with drug-resistant epilepsy. Randomized, open labelled, controlled clinical trial. Tertiary care referral center. Children aged 6 months to 14 years with drug-resistant epilepsy. mAD (n=30) or LGIT (n=30) as an add-on to the ongoing antiseizure drugs. Proportion of children who achieved seizure freedom as defined by complete cessation of seizure at 12 weeks as primary outcome measure. Secondary outcome measures were proportion of children who achieved >50% and >90% seizure reduction at 12 weeks, and adverse effects of the two therapies. Of the 60 recruited children, 3 in the mAD group, and 3 in LGIT group were lost to follow-up. The proportion of children with seizure freedom [16.6% vs 6.6%; relative risk reduction (RRR) (95% CI), 1.5 (−10.9, 0.5); P=0.42] and >90% seizure reduction [30% vs 13.3%; RRR, −1.2 (−5.5, 0.2); P=0.21] was comparable between the mAD and LGIT group at 12 weeks. The proportion of children with >50% seizure reduction was significantly higher at 12 weeks among those who received LGIT as compared to the mADgroup [73.3% vs 43.3%; RRR (95% CI) 0.4 (0.1–0.6); P=0.03] although the effect size was small. The diet was well tolerated with lethargy being the most common adverse effect in children in mAD (53.3%) and LGIT (66.7%) groups. The present study with limited sample size shows that seizure freedom at 12 weeks was comparable between mAD and LGIT for the treatment of drug-resistant epilepsy.

Journal ArticleDOI
TL;DR: In this article, an expert group consisting of pediatric neurologists and epileptologists was constituted and the evidence was reviewed, and for areas where the evidence were not certain, the Delphi consensus method was adopted, and the final guidelines were circulated to all experts for approval.
Abstract: West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval. Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.

Journal ArticleDOI
TL;DR: In this paper, the authors studied the growth pattern of term infants from birth to 2 years, focusing on catch-up and catch-down growth (increase or decrease in z-score > 0.67) in weight and length.
Abstract: Catch-up in the first two years of life may help in reducing the growth deficit. To study growth pattern of term infants from birth to 2 years, focusing on catch-up and catch-down growth (increase or decrease in z-score >0.67) in weight and length. Prospective birth cohort. 262 healthy term infants with birthweight 1800–4000 g. Serial assessment of anthropometric parameters at birth, 3.5 month, 1 year and 2 year of age. Proportion, timing and determinants of catch-up and catch-down growth. Weight catch-up between birth to 3.5 mo, 1 y, and 2 y was seen in 18%, 41% and 38%; and weight catch-down in 27%, 25% and 23%, respectively. Between birth and 2 y, change in weight z-score was inversely related to birthweight (β −3.754, P<0.001) and directly to caloric intake at 2 y (β 0.003, P<0.001). Mean (SD) birthweights of infants with catch-up, steady growth and catch-down were 2.6 (0.4), 2.9 (0.4) and 3.1 (0.4) kg, respectively (P<0.001). Catch-up and catch-down in length between birth and 2 y were present in 30% and 33% of the infants, respectively. Length z-scores at 2 y but not at birth were positively correlated with mothers’ (r=0.21, P=0.002) and fathers’ height (r=0.22, P=0.001). Nearly two-thirds of healthy term infants experienced either catch-up or catch-down in weight and length first 2 years of life. Infants’ birthweight and length at birth, caloric intake, and parents’ heights are important determinants of their growth patterns.

Journal ArticleDOI
TL;DR: High flow nasal cannula therapy is an effective and safe primary mode of respiratory support in children with respiratory distress and children who succeed on HFNC show a favorable clinical response within first few hours.
Abstract: OBJECTIVE To assess efficacy and safety of High flow nasal cannula therapy (HFNC) as primary mode of treatment for children with respiratory distress. METHODS Consecutive patients (1 mo-16 years) with respiratory distress were assessed for respiratory clinical score, COMFORT score and saturation to FiO2 (SF) ratio. RESULTS A total of 188 (91.7%) patients out of 205 responded to HFNC alone. The respiratory clinical score and COMFORT score were lower with higher SF ratio in these than 17 patients who required ventilation (P<0.001). Median (IQR) time to failure was 2 (1.75-24) hours. Air leak was seen in 2 (1%) patients. CONCLUSION HFNC is an effective and safe primary mode of respiratory support in children with respiratory distress. Children who succeed on HFNC show a favorable clinical response within first few hours.


Journal ArticleDOI
TL;DR: In this paper, the efficacy of dexmedetomidine and midazolam for sedation in mechanically ventilated children aged 1 month to 15 years was compared in a randomized controlled trial.
Abstract: There is a paucity of data on use of dexmedetomidine as a sedative agent in mechanically ventilated children. To compare the efficacy of dexmedetomidine and midazolam for sedation in mechanically ventilated children aged 1 month — 15 years. Secondary objectives were to compare the need for top-up doses of fentanyl and paralytic agents, duration of mechanical ventilation, ICU stay and hospital stay, and adverse events. Open label, non-inferiority, randomized controlled trial. PICU of a tertiary care teaching hospital in India. Consecutive children aged 1 month to 15 years who were mechanically ventilated. Children were randomized to either dexmedetomidine or midazolam and the doses were titrated to maintain target sedation score of 4 or 5 as measured by Penn State Children Hospital Sedation algorithm. The percentage of time spent in level 4 or 5 of Penn State Children Hospital sedation algorithm for ventilated children. 49 children were randomized (24 to ‘midazolam group’ and 25 to ‘dexmedetomidine group’). There was no difference in the percentage of time spent in the targeted sedation between the groups [midazolam 67.3% (18.8) vs. dexmedetomidine 56.3 %. (28.6); P=0.12]. The absolute difference in the percentage of time spent was −10.9% [SE (95% CI) 7.05: (−25.15 to 3.25)]. The lower end of 95% CI for the difference breached the non-inferiority limit of −20%. Number of fentanyl boluses, duration of mechanical ventilation, ICU stay, and hospital stay were similar. Four (17.4%) children in dexmedetomidine group developed persistent bradycardia. Non-inferiority of dexmedetomidine compared to midazolam for sedation in children on mechanical ventilation could not be established.


Journal ArticleDOI
TL;DR: The study reveals that tribes have gross variation in child mortality rates and there is pressing need to prioritize tribe-specific action plans to improve their health indicators.
Abstract: To provide tribe- specific child mortality rates and health indicators from selected states in India. We used Census 2011 data and Coale Demney methodology to estimate the infant mortality rate (IMR), under-five mortality rate (U5MR) and expectation of life at birth (LEB) for 123 tribes of selected states of India. The estimated IMR and U5MR were higher in scheduled tribe population compared to respective state’s total population. The IMR varied from 124 in the Birhore tribe of Chhattisgarh and Jharkhand, and the Bharias of Madhya Pradesh to 48 per 1000 live births in the Gamit tribe of Maharashtra. Similarly, the U5MR varied from the highest (203) in the Birhore tribe of Chhattisgarh to the lowest (57/1000 live births) in the Gamit tribe. The LEB varied from 72 years in the Gamit tribe of Maharashtra to 51 years in the Birhore tribe of Chhattisgarh. The study reveals that tribes have gross variation in child mortality rates and there is pressing need to prioritize tribe-specific action plans to improve their health indicators.

Journal ArticleDOI
TL;DR: In this article, a whole exome sequencing was performed on samples from all children diagnosed with congenital nephrotic syndrome, presenting at centers collaborating in a nationwide registry and biorepository.
Abstract: Information on etiology of congenital nephrotic syndrome in non-Caucasian populations is limited. This study aimed to determine the genetic basis of congenital nephrotic syndrome in Indian patients. In this observational, cross-sectional study, whole exome sequencing was performed on samples from all children diagnosed with congenital nephrotic syndrome, presenting at centers collaborating in a nationwide registry and biorepository. Analysis was targeted to focus on reported or novel, pathogenic or likely pathogenic variants in 89 genes implicated in etiology of nephrotic syndrome. Sanger sequencing was used to confirm disease-causing variants in patients and allelic segregation of compound heterozygous variants in samples from parents. Inheritance of a shared haplotype was analyzed among ten individuals carrying the most common variant. During 2017–2019, 34 patients with congenital nephrotic syndrome were screened. Consanguinity and similar illness in siblings were reported in eleven patients each. Homozygous or compound heterozygous, pathogenic or likely pathogenic variants were found in NPHS1 in 24 cases, including two novel variants. One patient each had homozygous pathogenic or likely pathogenic known or novel variant in NPHS2, PLCE1, OSGEP and LAMB2 genes. Patients with OSGEP and LAMB2 mutations had phenotype typical of Galloway Mowat and Pierson syndromes, respectively. Three variants in NPHS1 were common to 16 individuals. One reported variant in exon 19 (c.2600G>A; p.Gly867Asp) appears to share a common founder. A genetic cause was determined for 82.4% patients with congenital nephrotic syndrome. Variants in NPHS1 are most common in Indian patients and founder mutations might be present.