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Objectives Long-term care of children with congenital adrenal hyperplasia (CAH) has psycho-social implications for parents. Experts recommend a customized educational program for parents to facilitate their psychological adaptation and improve disease management. Such educational programs often provided by nurse counsellors are well evaluated in developed countries. There is a dearth of data on nurse-led counselling in the context of less developed countries. We aimed to evaluate the effect of a nurse-led counselling intervention on various psycho-social outcomes among parents of children with CAH. Methods Fifty consecutive parents of children with CAH attending an outpatient clinic at a tertiary-care teaching hospital were enrolled. Parents’ stress level, stigma, knowledge, practices, and treatment adherence were assessed by using Cohen’s Perceived Stress Scale (PSS), DSD Stigma scale, and HILL-Bon Medication Adherence Scale (HB-MAS). Three educational counselling sessions were conducted within a month’s gap, using PowerPoint presentations and a booklet. Assessment of outcomes was done at baseline and at the end of the third session. Results At baseline, the majority (90%) of the parents had moderate stress. Half of the parents had mild and the rest had moderate stigma. After the intervention, the majority (94%) of parents had shifted to mild levels of stress and stigma. At baseline, 86% of the parents had poor knowledge about the disease whereas post-intervention, 80% were having good knowledge. Disease management practices and treatment adherence also improved after the intervention. Conclusions Nurse-led counselling was effective in reducing psycho-social problems, increasing knowledge, as well as improving practices and treatment adherence.

We aimed to provide a scientometric assessment of global research in stem cell therapy (SCT) for type 1 diabetes (T1D) during 1999-2020.The published data on SCT in T1D were retrieved from Elsevier's Scopus database and analyzed using select bibliometric tools. We used VOSviewer software and the Biblioshiny app to construct and visualize bibliometric networks.The global yield totaled 1806 publications in the 22-year study period, registering a 17.7% annual growth peaking at 196.9% in the last 11 years. The average citations per publication (CPP) decreased from 62.0 during 1999-2009 to 24.3 during 2010-2020. The funded publications were 727 (40.2%). Randomized controlled trials (RCTs) were only 2.4% (45). Amongst 70 participating countries, the USA led with a 38.6% share. Of the 388 global organizations, Harvard Medical School, USA, San Raffaele Scientific Institute, Italy, and the University of Florida, USA were the topmost contributors. Florina, Couri, and Trucco were the top productive authors, whereas Melton, Abdi, and Simoes were the most impactful. Only 129 (3.1%) publications were highly-cited; their total and average CPP were 31,228 and 214.0 (range 101-1841), respectively.The quantity of research in SCT for T1D has increased during the last two decades while the quality has dipped. The research landscape is dominated by high-income North-American and Western-European countries. There is a need for conducting large-scale RCTs and promoting research collaborations between high- and low-income countries for long-term sustainability and global impact.

Objectives Growth hormone deficiency (GHD) in adults is associated with an increased risk of cardiovascular morbidity and mortality. Although children with GHD are also believed to have a similar cardiovascular disease (CVD) risk beginning at an early age, the available data in children is scarce. We aimed to determine the various CVD risk parameters in children with isolated GHD (IGHD). Methods A cross-sectional case-control study was conducted at a tertiary care centre in North India comparing various auxological, biochemical, and echocardiographic parameters between 20 IGHD children aged 5–15 years and their age and sex-matched healthy controls. Results The mean age of children with IGHD and controls was similar (10.5 ± 2.6 yr vs. 9.9 ± 2.7 yr, p=0.48). Children with IGHD had significantly higher waist-hip-ratio (p=0.01), total cholesterol (p=0.02), non-high-density lipoprotein–cholesterol (p=0.02), serum homocysteine (p Conclusions Children with IGHD showed abnormalities in several biochemical and cardiac parameters that may be associated with an increased CVD risk in later life. More extensive studies, including younger children with IGHD, are needed to determine the lower ages at which the CVD risk is detectable.

Type 1 diabetes (T1D) incurs substantial out-of-pocket expenses (OOPE) on insulin and diabetes-related supplies. The information on OOPE is scarce from low- and middle-income countries. We aimed to estimate annual OOPE for children with T1D attending our diabetes clinic located in North India.An online survey was conducted among parents of 380 children with T1D (mean age: 10.3 ± 4.6 years). Modified BG Prasad scale was used to estimate the socioeconomic status (SES).The mean duration of T1D was 3.6 ± 2.6 years; 54.9% of children were boys. The median HbA1c (IQR) was 7.9% (5-15%). 51.9% belonged to lower or lower-middle SES. Mean annual spending on glucose monitoring, insulin administration, and laboratory investigations were Indian Rupee (INR) 21,576, INR 28,965, and INR 5069, respectively (total INR 55,185, IQR: 26,575-105,027). The cost of a single visit to the doctor was approximately INR 2889. Thirty children required hospitalization during the last year, which costs INR 27,495 on average. 30.3% had more than 50% of their total family income spent on diabetes care, with a significant negative correlation with their SES (There is a high financial burden of T1D care for North Indian children, almost on the verge of losing sustainability. Further studies are warranted to furnish larger OOPE data to guide policy decisions aimed at reducing direct costs to patients.

OBJECTIVES The data on the use of shear wave elastography (SWE) in children with thyroid disorders is limited. We aimed to assess the role of SWE in the evaluation of the thyroid gland in children newly diagnosed with Hashimoto's thyroiditis (HT). METHODS The thyroid gland was evaluated in 18 children (5 boys and 13 girls, age range: 5-12 years) with newly diagnosed HT and 27 (21 boys and 6 girls, age range: 4-12 years) healthy controls using grayscale ultrasound followed by SWE. The values of SWE (in kPa) were compared between cases and controls and were also correlated with various demographic variables and serum thyroid hormone concentrations. RESULTS The overall median of SWE values in cases and controls was 20.6 kPa (IQR = 19.16-26.94) and 10.7 kPa (IQR = 9.9-16.32), respectively, and the difference was statistically significant (W = 438.5, P

Background Short stature homeobox (SHOX) haploinsufficiency underlies idiopathic short stature (ISS) and Leri-Weill dyschondrosteosis (LWD). The worldwide prevalence of SHOX variations in ISS varies from 2.5–15%. This study aims to assess the implication of SHOX variation in ISS in North India and its overall prevalence in Asian population. Methods SHOX gene analysis carried out by Multiplex ligation dependent probe amplification (MLPA) followed by Sanger sequencing in 54 patients with variable phenotypes. Meta-analysis was done to evaluate its prevalence which comprises 11 previous studies including present, carried out on a total of 979 participants. Results SHOX gene analysis resulted in 12.9% of positivity (7.4% deletions and 5.5% duplications). SHOX association was seen significantly related to gender, with predominance in female sex (P-value: 0.047). Short arms and forearms were the only significantly associated trait seen in 51.85% of children. We have found a rare association of multiple exostosis with LWD in a child with deletion. The prevalence of SHOX variation came as 15.2% in Asians. No significant difference was found in geographical region-specific analysis. Conclusion This meta-analysis compiles findings from the last decade and provides an updated picture of the prevalence of SHOX variations in Asians, emphasizing their potential as therapeutic targets in ISS patients.

Background: Obesity is considered to impact physical and cardio-respiratory fitness in children. There is scarce data on indices of physical fitness in Indian children with simple obesity even as they are at an increased risk for metabolic complications at lower body mass index (BMI) than their Western counterparts. We aimed to determine the effects of weight status on indices of physical fitness in North Indian children with simple obesity. Methods: Vital parameters such as blood pressure, pulse rate, respiratory rate, and rate of perceived exertion were measured before and after the six-minute walk test (6MWT) in obese children (BMI ≥95th percentile) (Group 2, n=70) aged 5-15 years and were compared to non-obese children (BMI 25th to 75th percentile) (Group 1, n=70). President's Challenge Fitness test was used to measure the physical fitness of each subject. Results: The mean age, weight and BMI of children in Group 1 and Group 2 were 9.07±2.88 and 8.93±3.13, 27.42±12.08 and 48.87±17.89 and 16.54±4.26 and 27.30±5.2, respectively. The mean blood pressure recorded before 6MWT was similar in the two groups but pulse rate, respiratory rate, and rate of perceived exertion were higher in Group 2. Children in Group 2 also showed significant elevation of vital parameters compared to Group 1 after the 6MWT. Additionally, children in Group 2 performed poorly in all the physical fitness parameters compared to those in Group 1 following the President’s Challenge Fitness test. Conclusions: Children with obesity showed several alterations in vital parameters and indices of physical fitness. Further studies are required to access the effect of interventions aimed at improving the physical fitness in children with obesity.

Purpose The objectives of this umbrella review were to describe various aspects of diabetes self-management education and support (DSMES)-related interventions and their effect on clinical, behavioral, and psychosocial outcomes among children and young adults with type 1 diabetes (T1DM) and identify gaps in current DSMES-related research. Methods An umbrella review of systematic reviews on DSMES interventions was conducted in accordance with the PRISMA 2009 statement. Four international medical databases were searched for eligible review articles published in English in the last 10 years that dealt with children and youths with T1DM and were conducted in accordance with a priori protocol. Results Out of the 234 citations screened, only 8 systematic reviews representing 166 studies were considered eligible for further analysis. Glycemic management (A1C) is the most common outcome variable (108 studies). Common behavioral and psychosocial outcome variables examined are episodes of hypoglycemia (27 studies) and diabetic ketoacidosis (21 studies) and self-care behavior and generic quality of life (20 studies each). Several gaps in DSMES-related research studies are identified, including uneven geographical distribution of study population, methodological weaknesses in study designs, missing important outcome variables, use of nonstandardized assessment tools, and lack of assessment of the sustainability of effects. Conclusion The effects of DSMES interventions on clinical, behavioral, and psychosocial outcomes are varied. The heterogeneity of implementation and evaluation makes it difficult to draw clear conclusions about elements of DSMES that are most effective. There is a need for long-term assessment of the psychosocial and behavioral outcomes using validated and generalizable instruments.

Context: Childhood obesity is a global health problem. A percentage of 2.3 of Indian boys and 2.5 of Indian girls are obese. Childhood obesity is associated with many morbidities like diabetes mellitus, coronary artery disease, musculoskeletal problems, and increased mortality. Aims: The aim of this study is to estimate burden of metabolic complications of obesity in child and parents of obese children and compare it with normal-weight children. Settings and Design: The study was done at a tertiary health center in northern India. It was a cross-sectional study. Methods and Material: We enrolled 60 obese children and age- and sex-matched 26 controls, based on Indian Academy of Pediatrics (IAP) 2015 body mass index (BMI) charts. Anthropometric parameters and metabolic complications in family were compared between cases and controls. Clinical markers of metabolic derangements and laboratory metabolic profile were assessed for obese children. Statistical Analysis Used: Descriptive statistics was used to describe frequencies. Chi-square test and Mann–Whitney test and Spearman correlation were used for comparison. Results: The prevalence of obesity and obesity-related complications was high in families of obese children. Ten percent of obese children had impaired fasting glucose and 30% had Haemoglobin A1c (HbA1c) in prediabetes category. Forty percent of obese children had dyslipidemia, 45% had transaminitis, and 46.7 were vitamin D deficient. A percentage of 41.7 of obese children had fatty liver on ultrasound. Conclusions: The family health and child weight are linked through home environment and genetics. The metabolic complications of obesity prediabetes, dyslipidemia, fatty liver, and lower vitamin D level are common in childhood obesity. Regular screening and interventions of metabolic complications are essential for saving child's future health.

Optimal glycaemic control is essential for the prevention of future micro- and macrovascular complications in type 1 diabetes (T1D). The type of insulin, the type of insulin delivery device, the caregiver's knowledge, the patient's age, duration of diabetes, and self-monitoring of blood glucose affect glycaemic control in type 1 diabetes. In the present study, we analysed glycaemic control and factors affecting it at a tertiary care centre in northern India.A retrospective review of records of patients registered between 2015 and 2018 was done. The data on demographic and disease-related factors were collected from the records. The different groups were compared with the t-test, one-way ANOVA, or Kruskal-Wallis test.The mean age at the time of evaluation was 10.43 ±4.04 years (2-21 years), and the mean disease duration was 46.61 ±28.49 months (16-141 months). Most of the patients were prepubertal and using a basal-bolus regimen. The mean glycated haemoglobin (HbA1c ) was 7.96 ±1.46%, but only 24% had HbA1c below the International Society of Paediatric and Adolescent Diabetes (ISPAD) recommended desirable level of below 7%. Forty-six patients suffered one or more micro-macrovascular complications, and dyslipidaemia was the most common complication. Children with a longer duration of disease (8.39 ±1.42% vs. 7.59 ±1.65%), an episode of DKA (diabetes ketoacidosis) within a year of evaluation (9.19 ±2.54% vs. 7.93 ±1.39%), lower maternal (8.22 ±1.37% vs. 7.56 ±1.45%) and paternal education (8.26 ±1.67% vs. 7.78 ±1.30%), and hyperthyroid state (9.43 ±2.28% vs. 7.91 ±1.45%) had higher HbA1c.Better diabetes education focusing on parents with lower education strata and children with longer disease duration and poor compliance can help improve glycaemic control in developing countries like India.

Syndromes causing short stature include Noonan syndrome (NS), Williams syndrome, and Silver–Russell syndrome (SRS). SRS is a primordial dwarfism with genetic heterogeneity. The SRS children present with prenatal growth retardation, neonatal hypoglycemia, feeding difficulties, physical asymmetry, with scoliosis and cardiac defect in some cases. The incidence is up to 1 in 100,000. Uniparental disomy, methylation abnormalities, and variants in some genes have been found underlying such phenotype. Growth hormone therapy has been used to improve the height gain in these patients. NS has genetic heterogeneity and most patients present with short stature with or without cardiac defect. Multiple genetic variants, mostly autosomal dominant, contribute to the phenotype. With the availability of next-generation sequencing, more and more genetic disorders causing short stature are being identified in different ethnic populations like Kabuki syndrome and Nance–Horan syndrome. Here, we present some cases of SRS and other additional syndromes with dysmorphism seen in past 5 years.

Objectives Boys with Duchenne Muscular Dystrophy (DMD) are at increased risk for compromised bone health, manifesting as low-impact trauma long bone fractures and vertebral compression fractures. Methods In a prospective observational study, we studied bone health parameters in North Indian boys with DMD. We consecutively enrolled ambulatory boys with DMD on glucocorticoid therapy. Bone health was evaluated with X-ray spine, Dual-energy X-ray absorptiometry (DXA), serum calcium, vitamin D3 (25[OH]D), 1,25-dihyroxyvitamin D3 (1,25[OH]2D3), serum osteocalcin, osteopontin, and N terminal telopeptide of type 1 collagen (Ntx) levels. Results A total of 76 boys with DMD were enrolled. The median age was 8.5 (interquartile range [IQR] 7.04–10.77) years. Among these, seven (9.2%) boys had long bone fractures, and four (5.3%) had vertebral compression fractures. Fifty-four (71%) boys underwent DXA scan, and among these 31 (57%) had low bone mineral density (BMD, ≤−2 z-score) at the lumbar spine. The mean BMD z-score at the lumbar spine was −2.3 (95% confidence interval [CI] = −1.8, −2.8), and at the femoral neck was −2.5 (95% CI = −2, −2.9). 25(OH)D levels were deficient in 68 (89.5%, n=76) boys, and 1,25(OH)2D3 levels were deficient in all. Mean serum osteocalcin levels were 0.68 ± 0.38 ng/mL (n=54), serum osteopontin levels were 8.6 ± 4.6 pg/mL (n=54) and serum Ntx levels were 891 ± 476 nmol/L (n=54). Boys with low BMD received glucocorticoids for longer duration, in comparison to those with normal BMD (median, IQR [16.9 (6–34) months vs. 7.8 (4.8–13.4) months]; p=0.04). Conclusions Bone health is compromised in North Indian boys with DMD. BMD at the lumbar spine is reduced in more than half of boys with DMD and nearly all had vitamin D deficiency on regular vitamin D supplements. Longer duration of glucocorticoid therapy is a risk factor for low BMD in our cohort.

Estimates of the soil and nutrient losses are essential for design and planning of soil conservation measures in Indian arid region. This study aims at estimating rainfall-runoff, soil and nutrient...

BACKGROUND: India plays an important role in global research on gestational diabetes mellitus (GDM), but a bibliometric assessment of this research is lacking. OBJECTIVE: To provide a comprehensive analysis of Indian GDM research during the last 30 years using select bibliometric indicators. METHODS: The Scopus international database was used to retrieve publication data, using a defined search strategy. The analysis focused on research output of Indian authors and organizations and their collaborations. The qualitative performance was assessed in terms of relative citation index and citations per paper (CPP). RESULTS: Overall, 100 countries participated in GDM research producing 13,193 publications during 1990–2019. India ranked ninth in global output (1182 publications, 3.1% share) and CPP of 18.6. Only 21.3% of publications had international collaboration and 9.4% were funded. Of the 235 organizations and 544 authors that participated in India’s research on GDM, the top 50 organizations and authors contributed 53.8 and 36.4% to national publication share, respectively. The leading productive organizations were AIIMS, New Delhi, KEMH, Pune and PGIMER, Chandigarh, whereas the most productive authors were S. Kalra, V. Seshiah and C.S. Yajnik. Indian Journal of Endocrinology and Metabolism, Journal of Clinical and Diagnostic Research, Journal of Obstetrics and Gynecology of India and Diabetes Research and Clinical Practice were the most productive journals. CONCLUSIONS: Indian research on GDM is lagging behind other countries which have a similar disease burden. Increasing national and international collaborations, and active support of national and international funding agencies is urgently required to produce quality research on GDM. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13224-021-01444-7.

Pediatric type 1 diabetes (T1D) is an intensively researched disease in the developed countries. However, the Indian contribution to global pediatric T1D research is limited. To provide a comprehensive analysis of Indian research on pediatric T1D over the past 3 decades. Articles on pediatric T1D published from 1990 to 2019 were retrieved from Scopus database. Specifically, the data on number of publications, top productive institutions and authors, citation analysis, highly cited articles, international collaboration, and active journals were extracted and presented using appropriate bibliometric indicators. The USA leads the global ranking of 90 countries with 29.29% publications share, followed by the UK, Germany, Italy, Sweden, and Australia with 10.77 to 5.48% share. India is ranked 17th and contributed 1.98% to global output. India’s publications averaged 9.74 citations per paper (CPP) compared to global average of 23.61. During 1990–2019, Indian research registered an average annual growth of 37.47% compared to global output of 12.45%. The most productive organizations were PGIMER, Chandigarh, and AIIMS, New Delhi, contributing 37 and 26 publications respectively. The most impactful publications were from MDRF, Chennai, and DRC, Chennai, with CPP of 20.36 and 19.56 and relative citation index of 2.09 and 2.01 respectively. Devi Dayal and V. Mohan were the most productive authors with 27 and 13 papers respectively. Indian contribution to global scientific output on pediatric T1D is lagging in quantity and quality compared to developed countries but has shown improvement in recent years. There is a need for Indian researchers to focus on collaborative research in pediatric T1D.

INTRODUCTION Homozygous familial hypercholesterolaemia (HoFH) carries a grave prognosis but is often underdiagnosed and undertreated. Confirmation of molecular diagnosis helps in planning effective management and determining prognosis accurately. Aim of the study: To determine the spectrum of mutations in the LDLR gene in a cohort of children with a clinical diagnosis of HoFH. MATERIAL AND METHODS Genomic DNA was extracted from peripheral blood samples of 8 patients, who were children of either sex, aged under 16 years, and diagnosed clinically with HoFH using the Simon Broome criteria. The potential variants in the LDLR gene were analysed by Sanger sequencing. RESULTS Fifty variations were found in the 8 patients; 39 (78%) were single nucleotide variations while 8 (16%) and 3 (6%) were deletions and insertions, respectively. The pathogenic variants in the LDLR gene were detected in four patients; three showed duplication in exon 17 (c.2416dupG) creating an amino acid change at position 806 (p.Val806GlyfsTer11) while one had a missense variant in the exon 9 at position c.1285G>A resulting in a change in amino acid at position 429 (p.Val429Met). The variants were found in heterozygous state in the parents or siblings of probands who showed pathogenic variants. CONCLUSIONS The frequency of disease-causing variants in the LDLR gene in our patients with HoFH was 50%. Further studies to characterise mutations in genes for apolipoprotein B, proprotein convertase subtilisin/kexin type 9, or LDL adaptor protein are suggested in all children with a clinical diagnosis of HoFH.

Disease chronicity, lifelong medications, Adrenal crisis, and genital surgeries affect the physical, mental, school and social aspects of a child's life and are a cause of great concern to parents regarding the future of their child with Congenital Adrenal Hyperplasia (CAH). The aim of the study was to assess quality of life (QoL) in children and parents of CAH and comparison with healthy children.This was a questionnaire-based cross-sectional study in 28 children with classical CAH attending the Pediatric Endocrine clinic at a tertiary-care center in northern India.CAH children had poorer QoL in School domain (73.6 vs. 90.0; p = 0.034) and significantly lower scores than their healthy peers in General (83.1 vs. 91.7, p = 0.025), Sleep (74.4 vs. 84.2, p = 0.017) domains and total score (80.0 vs. 87.8, p = 0.008) of the Fatigue scale. Parents reported Social (72.4 vs. 84.5; p = 0.009), School (63.8 vs. 90.0; p 0.01) and Total (74.3 vs. 84.2; p = 0.024) QoL were scores significantly lower than parents of healthy children. Parents perceived scores of Fatigue scale were significantly worse in all domains when compared to parents of healthy children. Failure to thrive was found to be a significant risk factor for impaired school (r = -0.533; p = 0.013) and overall (r = -0.563; p = 0.008) QoL as perceived by the child.Children and parents have different perception of QoL for their child. Routine periodic QoL assessment will help in better understanding of child and parent's hidden concerns which remain unaddressed in busy clinical practice.

Background: India has an enormous burden of type 2 diabetes mellitus (T2DM), and this plays an important role in global T2DM research. However, the quantity and quality of Indian T2DM research remain largely unexplored. Objective: To provide a detailed analysis of Indian T2DM research output during 1982–2019 using select bibliometric indicators. Materials and Methods: Data on T2DM publications were retrieved from the Scopus database. The analysis focused on the quantitative output of Indian authors and organizations. The qualitative performance was assessed in terms of relative citation index, citations per paper, and highly cited papers. Results: Globally, 157 countries participated in T2DM research, producing 208902 publications during 1982–2019. India ranks in the ninth place in global output, with 7844 publications (3.75% share). Only 19.06% of papers had international collaboration, whereas 12% received funding. The leading productive organizations were the Madras Diabetes Research Foundation, Chennai, All India Institute of Medical Sciences (AIIMS), New Delhi, and Post Graduate Institute of Medical Education & Research, Chandigarh; whereas King Edward Memorial Hospital, Pune, AIIMS, New Delhi, and Fortis Healthcare, Gurgaon were the most impactful. The most productive authors were V. Mohan, S. Kalra, and A. Ramachandran. Journal of the Association of Physicians of India, Diabetes and Metabolic Syndrome Clinical Research and Reviews, and Journal of Clinical and Diagnostic Research were the leading productive journals. Only 2.23% of publications were highly cited. Conclusions: Considering the burden of T2DM, India’s research is lagging behind. Increasing national and international collaborations, active involvement of national and international funding agencies, and prioritizing research on youth with T2DM is the need of the hour.

Introduction Studies in adults with hypothyroidism suggest an equal efficacy of bedtime versus early morning intake of levothyroxine. There is limited data on timing of levothyroxine administration in children. Material and methods Children with hypothyroidism on early morning levothyroxine, and clinically and biochemically euthyroid, were assigned to receive levothyroxine at bedtime (group A) or were continued on early morning levothyroxine intake (group B). Clinical, anthropometric and laboratory evaluation (thyroid and lipid profiles, liver enzymes and creatinine) was done at baseline, and at 3 and 6 months. Results Eighty-four children, 42 in each group, completed the study. The clinical and anthropometric parameters remained similar in the two groups at baseline and at 3- and 6-month follow-up visits. There was no difference in the mean serum concentrations of triiodothyronine, thyroxine and thyrotropin at the 3 time-points in the study. In addition, mean serum aspartate transaminase, alanine transaminase, creatinine and parameters of lipid profiles remained similar in the two groups. The requirement of levothyroxine was similar at baseline (48.6 ±16.9 µg vs. 49.6 ±19.5 µg, p-value 0.80) and at the endpoint (48.3 ±17.2 µg vs. 51.9 ±18.0 µg, p-value 0.46) in both groups. At the study end, 25 (60%) patients in group A and 17 (40%) in group B preferred bedtime dosing of levothyroxine. Conclusions We found an equal efficacy of bedtime intake compared to early morning intake of levothyroxine in maintaining an euthyroid state in children with hypothyroidism. Further studies are required to see if bedtime levothyroxine administration improves the quality of life of patients.

Context Type 1 diabetic children exhibit poorer oral health than general population. However, no oral health preventive protocol exists for attending to the oral health needs of such children. Aim To evaluate the effect of an oral health preventive protocol on salivary parameters and gingival health of children with type 1 diabetes mellitus over a period of 6 months. Materials and methods Fifty diabetic children, aged 6–12 years were selected and divided into two groups. Children in group I received a comprehensive oral health preventive protocol. The parameters recorded were oral hygiene practices, salivary flow rate, pH, buffer capacity, viscosity, electrolytes, and plaque and gingival indices. These were compared at baseline, 3-, and 6-month intervals. Statistical analysis Statistical analysis was done using IBM SPSS STATISTICS (version 22.0). Tests were based on the type of data. Results The intervention group (group I) showed favorable improvements in the parameters assessed. A greater number of participants adopted the correct oral hygiene methods. Unstimulated salivary flow rate increased from 0.36 ± 0.21 to 0.82 ± 0.16 mL/minute in group I and from 0.32 ± 0.24 to 0.58 ± 0.16 mL/minute in group II after 6 months (p = 0.001). Salivary buffer capacity increased from 3.07 ± 2.64 to 10.40 ± 0.82 in group I while in group II, it improved from 3.20 ± 1.47 to 9.33 ± 1.44 (p = 0.02). Salivary viscosity decreased in group I from 1.97 ± 0.42 to 1.15 ± 0.06 and from 1.97 ± 0.35 to 1.23 ± 0.11 in group II after 6 months (p = 0.02). Gingival scores changed from 1.07 ± 0.35 to 0.20 ± 0.23 in group I and from 1.04 ± 0.28 to 0.85 ± 0.25 in group II (p = 0.001). Conclusion The preventive protocol used in the present study showed a significant (p < 0.05) improvement in the parameters assessed. How to cite this article Singh V, Gauba K, Goyal A, et al. Effect of an Oral Health Preventive Protocol on Salivary Parameters and Gingival Health of Children with Type 1 Diabetes. Int J Clin Pediatr Dent 2021;14(1):109–114.

Cordia sinensis, locally known as ‘Goondi’ in India, is an underexploited multipurpose fruit species found in hot arid regions that is well adapted to drought, salt and hot conditions. The present study was undertaken to collect fruit samples from different locations in the Kachchh region of Gujarat, India, and to determine their field establishment for characterization, conservation and utilization. The maximum distribution of the species was observed in Bhuj (45%) and Mandvi (25%). Field boundaries (35%) and scrub forests (30%) had greater frequencies, whereas backyards had rarer frequencies (10%). The species most commonly occurred on levelled topography (60%) with a soil pH in the range of 8–8.5 (63%). Morphological data of three-year-old plants in the field gene bank showed a maximum coefficient of variation in the number of leaves per plant (66.6), followed by the number of branches per plant (45.62) and collar diameter (27.69). Wide variations were recorded in plant height (121.67–212 cm), spread (118–223 cm2) and the number of branches per plant (6–24.33). Specific accessions were identified for fodder (CBCG-12, CBCG-13 and CBCG-16), early flowering and fruiting (CBCG-12, CBCG-13 and CBCG-14), easier propagation by seeds (CBCG-12 and CBCG-13) and salt tolerance (CBCG-15 and CBCG-16). Preliminary findings and information provided about this species' utilization and other aspects might be useful for future research on its domestication, sole plantation and conservation aspects, improving the exploitation of this species by present and future generations.

Background: In era of innovations it times to use nanotechnology, to maintain the ever-increasing market for food crops. Methods: Indian Farmers Fertiliser Cooperative Ltd. (IFFCO) has introduced its nanotechnology-based products i.e., Nano-N, Nano-Zn and Nano-Cu for initial testing on 7 major crops grown during Rabi season in Gujarat. Result: We tested 5 treatment combinations among which treatment T5 i.e., 50% farmer fertilizer practices plus 1 spray of Nano-N, 1 spray of Nano-Zn and 1 spray of Nano-Cu give higher return than all there treatments in all crops. It conclude that application of nano-fertilizers significantly increase crop yield over control or without application of nanofertilzer it is mainly because of increasing growth of plant parts and metabolic process such as photosynthesis leads to higher photosynthates accumulation and translocation to the economic parts of the plant. Foliar application of nano particles as fertilizer significantly increases in yield of the crop.

Accurate diagnosis is required for management of Congenital adrenal hyperplasia (CAH). The conventional method for detection of mutations in the CYP21A2 gene is targeted capillary sequencing which is labor intensive and has limited multiplexing capability. Next generation sequencing (NGS) provides data with high sequence coverage and depth. Our objective was to develop an accurate NGS-based assay to characterize the mutation spectrum in CYP21A2 gene in Indian patients suspected to have 21-OH CAH. Cases with 21-OH CAH from 12 endocrine units across India were studied. DNA was extracted from proband’s and parent’s(subset) blood. Locus-specific long-range PCR and gel electrophoresis of amplicons was followed by NGS where no visible 30 kb homozygous/whole gene deletion was observed. Orthogonal confirmation was performed by capillary sequencing (ABI 3500) and Multiplex Ligation-dependent Probe Amplification (MLPA, MRC-Holland). PCR products were purified and individual libraries were pooled and sequenced (Illumina). Of the 310 CAH cases, biallelic mutations (pathogenic/ likely pathogenic variants involving both CYP21A2 gene copies) were detected in 256 (82.6%), heterozygous mutations in 13 (4.2 %), and none in 41 (13.2%). Most common mutation was c.293-13A/C>G (29.03%), followed by 30 kb deletion (18.24%). Thirty samples tested orthogonally (by capillary sequencing or MLPA) showed 100% concordance with NGS assay. Nine novel variants were identified. We have developed and validated a comprehensive NGS-based assay for detection of variants in CYP21A2 gene in patients with 21-OH CAH. We describe CYP21A2 mutation spectrum and novel variants in a large cohort of Indian patients with CAH.

Background No validated measures exist for evaluating diabetes self-management in Indian type 1 diabetes (T1D) patients. Objective To cross culturally adapt and evaluate the psychometric properties of Hindi version of Diabetes Self-Management Profile-Self Report (DSMP-SR-Hindi) in Indian T1D patients. Methods Total 160 T1D patients and their parents participated in the study. The mean age of patients was 13.5 ± 2.5 years and HbA1c was 8.6 ± 2.2%. Results Exploratory factor analysis employing principle axis factoring with promax rotation was conducted. Monte Carlo parallel analysis identified three sub-scales instead of five sub-scales proposed in original version. Because of underlying ceiling and floor effects and insufficient loadings, five items were eliminated. Consequently, final Hindi version of DSMP-SR contained 19 items from DSMP-SR-24. Internal consistencies were adequate for overall scale (Cronbach's α = 0.835), identified sub-scales (Cronbach's α = 0.702-0.802) and comparable between genders. DSMP-19 total scores (r = -0.74) and three subscales correlated significantly with HbA1c (SMBG and Corrective Adjustments [r = -0.58], Exercise [r = -0.48], and Conformity to Diet and Insulin Routine [r = -0.64]). For every one SD improvement (11.2 marks) in DSMP-SR-Hindi score, odds of falling into poor glycaemic group (HbA1c > 7.5%) dropped to 0.242 times (95% CI 0.144-0.405; P Conclusions DSMP-SR-Hindi is a reliable and valid self-report measure of diabetes self-management behavior in Indian T1D patients. The revealed three subscales are reliable to use in isolation and across the genders. It will help in monitoring patient's progress in stepwise manner, ranging from their basic understanding of prescribed regimen to taking advance corrective actions in face of altered needs.

Recent reports suggest that the clinical course of coronavirus disease 2019 (COVID-19) in previously healthy children is usually milder as compared to adults. However, children with comorbid conditions such as diabetes are at increased risk of infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) as well as morbidity and mortality due to COVID-19. Experience in adults with diabetes shows that they are prone to faster metabolic decompensation, develop diabetes-related complications, and have a poor prognosis when hospitalized with COVID-19. Data on children are limited. The aim of this mini review was to discuss the possible risks to children and adolescents with diabetes during the current pandemic and the special considerations in management in those affected with COVID-19. The challenges for children who develop new-onset type 1 diabetes during the COVID-19 lockdown, especially in accessing health care, are also discussed.

Background Acute kidney injury (AKI) is an important complication encountered during the course of diabetic ketoacidosis (DKA). Plasma-Lyte with lower chloride concentration than saline has been shown to be associated with reduced incidence of AKI in adults with septic shock. No study has compared this in DKA. Methods This double-blind, parallel-arm, investigator-initiated, randomized controlled trial compared 0.9% saline with Plasma-Lyte-A as initial fluid in pediatric DKA. The study was done in a tertiary care, teaching, and referral hospital in India in children (> 1 month–12 years) with DKA as defined by ISPAD. Children with cerebral edema or known chronic kidney/liver disease or who had received pre-referral fluids and/or insulin were excluded. Sixty-six children were randomized to receive either Plasma-Lyte (n = 34) or 0.9% saline (n = 32). Main outcomes Primary outcome was incidence of new or progressive AKI, defined as a composite outcome of change in creatinine (defined by KDIGO), estimated creatinine clearance (defined by p-RIFLE), and NGAL levels. The secondary outcomes were resolution of AKI, time to resolution of DKA (pH > 7.3, bicarbonate> 15 mEq/L & normal sensorium), change in chloride, pH and bicarbonate levels, proportion of in-hospital all-cause mortality, need for renal replacement therapy (RRT), and length of ICU and hospital stay. Results Baseline characteristics were similar in both groups. The incidence of new or progressive AKI was similar in both [Plasma-Lyte 13 (38.2%) versus 0.9% saline 15 (46.9%); adjusted OR 1.22; 95% CI 0.43–3.43, p = 0.70]. The median (IQR) time to resolution of DKA in Plasma-Lyte-A and 0.9% saline were 14.5 (12 to 20) and 16 (8 to 20) h respectively. Time to resolution of AKI was similar in both [Plasma-Lyte 22.1 versus 0.9% saline 18.8 h (adjusted HR 1.72; 95% CI 0.83–3.57; p = 0.14)]. Length of hospital stay was also similar in both [Plasma-Lyte 9 (8 to 12) versus 0.9% saline 10 (8.25 to 11) days; p = 0.39]. Conclusions The incidence of new or progressive AKI and resolution of AKI were similar in both groups. Plasma-Lyte-A was similar to 0.9% Saline in time to resolution of DKA, need for RRT, mortality, and lengths of PICU and hospital stay. Trial registration Clinical trial registry of India, CTRI/2018/05/014042 (ctri.nic.in) (Retrospectively registered).

INTRODUCTION There are limited data on the alterations of serum copper and zinc, which have been proposed to have associations among children with obesity. MATERIAL AND METHODS A total of 173 children were enrolled and grouped into overweight/obese (n = 69) and non-obese (n = 104) according to CDC 2000 growth charts. Serum and whole blood zinc and copper concentrations were measured by validated ICP-MS method, and copper/zinc ratios were calculated and correlated to various anthropometric parameters. RESULTS Mean BMI in obese (24.78 ±3.93) was significantly higher as compared to non-obese (16.44 ±2.34; p

Introduction Scientometric studies help scientists to identify research gaps and plan future research. There is no scientometric assessment of research on childhood onset type 1 diabetes (T1D), despite an intensive research in this field over the past 2 decades. Aim of the study To provide a scientometric assessment of global research output in pediatric T1D. Material and methods Publications on pediatric T1D indexed in Scopus database over the 20-year period from 2000 to 2019 were examined. The most productive and impactful countries, organizations and authors, trends in research, the media of publications and characteristics of high cited publications were identified using appropriate bibliographic tools. Results Pediatric T1D research registered 7.84% and 79.99% annual and 10-year cumulative growth and averaged 19.35 citations per paper (CPP). The field witnessed an uneven participation of 141 countries, wherein 82.67% of the global research output share came from top 10 countries. USA leads the ranking with 29.76% share, followed by UK (10.56%), Germany, Italy, Sweden and Australia (5.15% to 7.88%), and Poland, Finland, Canada and Denmark (3.04% to 4.24%). Five countries which registered relative citation index (RCI) higher than their group average of 1.63 were Finland (2.30), UK (1.82), Canada (1.77), Denmark and USA (1.74 each). The number of participating organizations and authors was 3627 and 5596 respectively. Conclusions The USA and Finland are the most productive and the most impactful countries respectively in global pediatric T1D research. The contribution from developing countries especially from Southeast Asia is meager despite a large disease burden.

BACKGROUND: Camurati-Engelmann disease (CED) is a rare bone dysplasia characterized by diffuse diaphyseal osteosclerosis. Skull base involvement in CED can result in hypopituitarism but is seldom reported. Our objective was to report a patient with acquired hypopituitarism due to CED and assess the management challenges. CASE REPORT: A 20-year-old boy presented with lower limb pain. He had walking difficulty in childhood, which was diagnosed as CED and managed with prednisolone. He later discontinued treatment and was lost to follow-up. Current re-evaluation showed short stature (-3.6 standard deviation), low weight (-4.3 standard deviation), and delayed puberty with delayed bone age (13 years). He was found to have secondary hypogonadism (luteinizing hormone level, 0.1 mIU/mL [1.7-8.6 mIU/mL]; follicle-stimulating hormone level, 1.0 mIU/mL [1.5-12.4 mIU/mL]; and testosterone level, 0.087 nmol/L [9-27 nmol/L]), growth hormone deficiency (low insulin-like growth factor I level, 120 ng/mL [226-903 ng/mL] and peak growth hormone level of 7 ng/mL on insulin-induced hypoglycemia), and secondary hypocortisolism (cortisol level, 105 nmol/L [170-550 nmol/L] and adrenocorticotropic hormone level, 6 pg/mL [5-65 pg/mL]). Serum prolactin level was normal (8.3 ng/mL [5-20 ng/mL]), and he was euthyroid on levothyroxine replacement. Magnetic resonance imaging revealed a partially empty sella. Sanger sequencing revealed a missense mutation (p.R218C/c.652C>T) in exon 4 of the TGFβ1 gene. The patient was treated with zoledronate, losartan, and oral prednisolone and continued on levothyroxine and testosterone replacement, which resulted in symptomatic improvement. DISCUSSION: The index case manifested severe CED requiring multimodality therapy. Later, he developed combined pituitary hormone deficiencies, which were managed with thyroid and gonadal hormone replacement with the continuation of glucocorticoids. The partial efficacy of bisphosphonates in CED has been reported in the literature. CONCLUSION: Skull base involvement in CED can lead to structural and functional hypopituitarism as a result of intracranial hypertension. ispartof: AACE Clin Case Rep vol:8 issue:2 pages:58-64 ispartof: location:United States status: Published online

Fetus and neonate are dependent maternal supply of calcium for maintaining the calcium profile in physiologic range. The disturbances in maternal calcium homeostasis leads to changes in the baby's calcium. Maternal investigations in neonatal hypocalcemia not only reveal the etiology in the baby but are sometime helpful in unmasking maternal disorder of calcium homeostasis.

Background Acute kidney injury (AKI) due to Diabetic Ketoacidosis (DKA) is rather common. Novel biomarkers to diagnose AKI are being increasingly used in different settings. The use of urinary Neutrophil Gelatinase-Associated Lipocalin (uNGAL) in predicting persistent AKI in pediatric DKA cases is still not thoroughly investigated. Methods This was a secondary analysis of Saline versus Plasma-Lyte in Ketoacidosis (SPinK) trial data; 66 children (> 1 month-12 years) with DKA, defined by the International Society for Pediatric and Adolescent Diabetes (ISPAD), were analyzed. Children with cerebral edema, chronic kidney disease and those who received pre-referral fluids and/or insulin were excluded. uNGAL and urine NGAL-creatinine ratio (uNCR) at 0 and 24 h were measured in all. Persistent AKI was defined as a composite outcome of continuance of AKI defined by the Kidney Disease Improving Global Outcomes (KDIGO) stage 2 or 3 beyond 48 h from AKI onset, progression of AKI from either KDIGO stage 0 or 1 to a worse stage, need of renal replacement therapy or death. Main outcomes Thirty-five (53%) children had AKI at admission; 32 (91.4%) resolved within 48 h. uNGAL was significantly higher in the AKI group at admission [79.8 ± 27.2 vs 54.6 ± 22.0, p = 0.0002] and at 24 h [61.4 ± 28.3 vs 20.2 ± 14.5, p = 0.0003]. Similar trend was observed with uNCR at admission [6.7 ± 3.7 vs 4.1 ± 2.6, p = 0.002] and at 24 h [6.3 ± 2.5 vs 1.2 ± 1.0, p = 0.01]. Furthermore, uNGAL at admission showed a moderate positive linear correlation with serum creatinine. Additionally, elevated uNGAL at 0 and 24 h correlated with corresponding KDIGO stages. Admission uNGAL >88 ng/ml and uNCR of >11.3 ng/mg had a sensitivity of 66% and 67%, specificity of 76% and 95%, and Area under the receiver operating characteristic curve (AUC) of 0.78 and 0.89 respectively for predicting persistent AKI at 48 h. Conclusions Majority of AKI resolved with fluid therapy. While uNGAL and uNCR both correlated with serum creatinine and AKI stages, serial uNCR was a better predictor of persistent AKI than uNGAL alone. However, feasibility of routine uNGAL measurement to predict persistent AKI in DKA needs further elucidation. Trial registration This was a secondary analysis of the data of SPinK trial [CTRI/2018/05/014042 (ctri.nic.in)].

Background: India is a second largest producer of vegetables after China. Fruits and vegetables are essential parts of our diets and vegetables recommended highest by ICMR. Therefore, the vegetables require extra emphasis to improve production and productivity of it to feed the second largest population in the world. Methods: Indian Farmers Fertiliser Cooperative Ltd. (IFFCO) has introduced its nanotechnology-based products i.e., Nano-N, Nano-Zn and Nano-Cu for initial testing on 11 important vegetable crops grown during winter season in Gujarat with 5 treatment combinations. Result: We tested 5 treatment combinations among which treatment T5 i.e., 50% farmer fertilizer practices plus 1 spray of Nano-N, 1 spray of Nano-Zn and 1 spray of Nano-Cu give 5.34% higher return then all there treatments in all crops. Which conclude that application of nano-fertilizers through foliar spray significantly increase crop yield over control or without application of nano-fertilzer due to its higher Nutrient Use Efficiency (NUE). So it was recommended to use farmers fertilizers practices (FFP) 50% N, 1 spray of Nano-N, 1 spray of Nano-Zn and 1 spray of Nano-Cu to get more economic yield.

Celiac disease (CD) is frequently associated with type I diabetes mellitus (T1D), where its diagnosis may be a challenging task. This study aims to test the usefulness of the double staining immunofluorescence (dsIF) technique for the detection of intestinal anti-tissue transglutaminase specific IgA antibody (tTG-IgA) deposits in CD and T1D children with coexisting CD.A total of 46 patients (30 cases of CD and 16 cases of T1D with CD) and 16 non-diabetic, non-celiac children were recruited. Endoscopic biopsies were taken and analyzed by light microscopy, quantitative histology (QH), and a dsIF technique.Histologically, villous atrophy was most severe in CD, followed by T1D with CD, while all control biopsies except 1 were normal. QH showed a statistically significant difference in villous height (Vh), crypt depth (CrD), and Vh:CrD ratio between diabetic and non-diabetic patients with CD. dsIF technique could detect tTG-IgA deposits in 85.7% of cases of CD alone and 93.8% of biopsies from diabetic children. Surprisingly, deposits were more extensive in biopsies with minimal villous shortening. Also, all 5 biopsies from T1D patients with normal histology were dsIF positive.In-situ analysis of tTG-IgA immune deposits facilitates the detection of positive serology early-onset CD. Quantitative analysis may be used as an ancillary tool to increase the reliability of histological findings in these patients.

Hyperphosphatemic familial tumoral calcinosis (HFTC) is an extremely rare autosomal recessive disorder caused by variants in the GALNT3 (N-acetylgalactosaminyltransferase 3), FGF23 (Fibroblast Growth Factor-23) and αKL (α-Klotho) genes, which results in progressive calcification of soft tissues. We describe the case of a 9-year-old girl who presented with recurrent hard nodular swellings on her feet and knees which intermittently discharged chalky white material. Her younger brother also had a similar condition. Both siblings showed hyperphosphatemia, but the parentsbiochemical parameters were normal. The histological features of the material aspirated from a skin lesion were consistent with tumoral calcinosis. Sanger sequencing identified a novel homozygous non-synonymous sequence variant in exon 10 of the GALNT3 gene (NM_004482.3:c.[1681T>A];[1681T>A], NP_004473.2:p. [Cys561Ser];[Cys561Ser] in the proband and her affected brother. The parents were heterozygous carriers for the same sequence variant. In conclusion, we report a new variant in the GALNT3 gene that caused HFTC in a North Indian family.

To detect the prevalence of diabetic polyneuropathy (DPN) in children with type 1 diabetes (T1D) and to identify associated the risk factors.This cross-sectional study evaluated children aged between 2 and 16y with T1D for ≥2 y. Detailed neurological examination, neuropathy symptom score, and nerve conduction studies were done in all children to assess nerve dysfunction. Disease-related factors were evaluated for the prediction of neuropathy.Sixty-six children (67% boys) were enrolled. The mean age at the time of diagnosis of T1D was 7.1 ± 2.6 years. The mean duration of diabetes was 4 ± 1.8 years. None of the patients had neuropathy on clinical examination or on the neuropathy symptom score. The prevalence of subclinical DPN was 18.2% (n = 12/66). The type of neuropathy was pure motor (n = 11, 91.6%) and mixed sensorimotor (n = 1, 8.3%). The common peroneal nerve was most commonly affected (n = 6, 50%), followed by the tibial (n = 4, 33.3%) nerve. The most common patterns of nerve involvement were mixed axonal and demyelination (n = 7, 58.3%), followed by axonal (n = 3, 25%) and demyelinating type (n = 2, 16.6%). Children with subclinical DPN had a significant reduction in velocity of tibial, common peroneal, median motor, and ulnar motor nerves; delayed latency in common peroneal, median motor, ulnar motor, and median sensory nerves compared to those without DPN (p value0.05). A higher body mass index predicted the development of subclinical DPN (p value0.05).Nearly one-fifth of children with T1D have subclinical neuropathy as early as two years of the disease. A higher body mass index is significantly associated with DPN. Electrophysiological studies should be performed regularly to screen for nerve dysfunction and its progression.