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INTRODUCTION: In the hypothalamic-pituitary-gonadotrophin axis, estrogen plays a key role in the regulation of bone maturation and growth plate closure. This study was designed to explore the link between single nucleotide polymorphisms (SNPs) in the estrogen receptor 1 (ESR1) gene with idiopathic short stature (ISS) susceptibility in a North Indian population. METHODS: Four SNPs of ESR1 (rs543650, rs6557177, rs2234693 and rs9340799) were genotyped by Sanger sequencing in ISS patients and controls. Linkage disequilibrium (LD) and haplotyping were done by SNPStat and SHEsisPlus software. The extent of LD was determined by calculating D’ and R2 values in SNP paired combinations. RESULTS: Fifty-two ISS patients were compared with 68 controls. A significant positive association was found between rs6557177 and rs543650 genotype and ISS susceptibility. The frequencies of the rs6557177 CC genotype [p=0.030; odds ratio (OR)=0.13; 95% confidence interval (CI): 0.01-1.10] and rs543650 genotype TT (p=0.043; OR=0.29; 95% CI: 0.09-0.92) were increased in the ISS group compared with controls. However, no significant correlation was observed between clinical parameters of patients and these SNPs. rs543650 showed strong LD with rs2234693 and rs9340799, similarly rs2234693 and rs9340799. DISCUSSION AND CONCLUSION: Our study showed that the CC genotype at rs6557177 and TT genotype at rs543650 of ESR1 constituted a risk factor for developing ISS in North Indian children. These findings may lead to a better understanding of the SNPs associated with ISS susceptibility.

Background: The most impactful research on pediatric type 1 diabetes (T1D) remains unknown. Objective: We aimed to evaluate the characteristics and impact of the 100 most-cited articles on pediatric T1D. Materials and Methods: Using a predefined bibliometric strategy, the Scopus database was searched for high-cited papers (HCPs) published from 2001 to 2020. Articles were evaluated for data on the publication year, countries, authors, journals, topics, and types. Social network analysis was performed to visualize the interaction among countries, organizations, and authors using VOSviewer software. Results: The top 100 HCPs received 390 to 4634 citations, averaging 773.5 citations per paper (CPP). The funded HCPs (n = 50) had a higher impact (CPP 791.5). The majority of HCPs (n = 83) were collaborative. Classifying by research type, 65 studies were clinical (n = 65), risk factors (n = 27), epidemiology (n = 26), pathophysiology (n = 16), treatment outcome (n = 13), genetics (n = 12), complications (n = 3), quality of life (n = 2), and prognosis (n = 1). The number of authors involved was 1,101, affiliated with 545 organizations in 27 countries; the USA (n = 64) and the UK (n = 24) were the most productive countries, whereas Australia and the UK were the most impactful. D.M. Nathan and J.M. Lawrence were the most prolific authors, while P. Raskin and J.M. Lachin were the most impactful. Conclusions: High-income countries such as the USA, UK, and Australia contribute significantly to high-impact pediatric T1D research. Funding and collaboration improve the impact of citations in publications. Less researched areas such as treatment outcomes, genetics, complications, quality of life, and prognosis should be the focus of future research on pediatric T1D.

INTRODUCTION: Short stature homeobox (SHOX) haploinsufficiency underlies idiopathic short stature (ISS) and Leri-Weill dyschondrosteosis. The worldwide prevalence of SHOX variations in ISS varies from 2.5% to 15.0%. The aim of this study was to assess the implication of SHOX variation in ISS in North Indians and compare this with other cases of SHOX variations from Asian population. METHODS: SHOX gene analysis was carried out by multiplex ligation-dependent probe amplification followed by Sanger sequencing in 54 patients with variable phenotypes. Comparison with other reports in a meta-analysis comprising the current study and 11 previous studies (n=979) was performed. RESULTS: SHOX analysis resulted in 12.9% positivity (7.4% deletions and 5.5% duplications). SHOX association was seen significantly related to gender, with predominance in females (p=0.047). Short arms and forearms were the only significantly associated trait seen in 51.9% of children. The overall prevalence of SHOX variation was 15.2% in Asians with ISS. No significant difference was found in geographical region-specific analysis. DISCUSSION AND CONCLUSION: This study summarises 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. Further high quality, large investigations including functional validation is warranted to validate this association.

Fatty acid oxidation defects are a heterogeneous group of disorders related to the mitochondrial fatty acid oxidation pathway. Carnitine acylcarnitine translocase (CACT) is an enzyme responsible for the unidirectional transport of acylcarnitine across the inner mitochondrial membrane. This enzyme plays a crucial role in the oxidation of fatty acids. The autopsy pathology of the CACT deficiency is described in only a few cases. We describe the autopsy pathology of a child with CACT deficiency dominantly in the form of microvesicular steatosis of the hepatocytes, renal proximal tubular epithelia, cardiac myocytes, and rhabdomyocytes. The diagnosis was further confirmed on whole exome sequencing with compound heterozygous variants in the exon 1 (c.82G>T, p.Gly28Cys; likely pathogenic) and exon 5 (c.535G>A, p.Asp179Asn; uncertain significance) of the SLC25A20 gene. This case elucidates the histopathology of the liver and the detailed autopsy of a case of CACT deficiency from India.

Background: 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. Methods: 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. Results: 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. Conclusion: 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.

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.

Introduction: Ectodermal dysplasias (EDs) affect structures derived from the ectoderm such as skin, its appendages, nail, and teeth. In this series, we describe four patients presenting with a clinical phenotype of dysplasia of one or more ectodermal structures who underwent next-generation sequencing for mutational analysis. Case Series: The clinical phenotype of three patients was hypohidrotic ectodermal dysplasia (HED) and one patient was diagnosed with autoimmune polyglandular syndrome (APS) type 1. Two patients with classical clinical features of X-linked HED (XLHED) had mutations in EDA gene; variant c.924+ 8C>G (5′ proximal splice site) and c.760C>T (p.Gln254Ter). Case 3 had clinical phenotype of HED with urticaria pigmentosa, which was confirmed on skin biopsy and immunohistochemistry. This patient was found to have mutation in C1orf172; c.449G>A (p.Arg150Gln) which has not been reported previously. Case 4 was diagnosed to have APS type 1 with cutaneous features of discoloration of teeth and chronic mucocutaneous candidiasis. This patient had a compound heterozygous mutation of AIRE gene. The two variants detected were c.169C>T (p.Gln57Ter) and c.47C>T (p.Thr16Met). Conclusion: The present series highlights the clinic-genetic correlation in four patients with features of ED. Two variants of uncertain significance and two previously unreported variants were also found in this study.

Abstract 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 )].

Abstract Objective To investigate the effects of combination therapy with cholecalciferol and lansoprazole on residual β-cell function and glycemic control in children with new-onset type 1 diabetes. Methods Children aged 6-12 years with type 1 diabetes were allocated to receive cholecalciferol and lansoprazole (Group 1) or no treatment (Group 2). Children were maintained on their respective insulin regimens and kept records of blood sugar and insulin doses taken. Children were followed at three-month intervals for six months. Changes in mean fasting C-peptide and HbA1c levels, daily insulin doses, fasting blood glucose and mean blood glucose levels from baseline to end of the study were analyzed. Results Twenty-eight children (14 per group) met the eligibility criteria. Fasting C-peptide levels decreased significantly from baseline to study end in both groups (mean decrease -0.19±0.09ng/mL and -0.28±0.08ng/mL, p=0.04 and p=0.001; Group 1 and Group 2 respectively). However, fasting C-peptide level drop was significantly smaller in Group 1 compared to Group 2 (30.6% and 47.5% respectively; p=0.001). Likewise, daily insulin doses decreased significantly in both groups (-0.59±0.14units/kg and -0.37±0.24units/kg respectively; p=0.001). All patients recruited completed the study. No adverse events were reported. Conclusion Combined therapy with cholecalciferol and lansoprazole for six months was associated with smaller decline in residual β-cell function and lower insulin requirements in children with new-onset type 1 diabetes. Preliminary findings of this small-scale study need to be confirmed by larger studies. Registry of Clinical Trials (www.ctri.nic.in) under number REF/2021/03/041415 N.

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.

Introduction: This study was conducted to investigate the pathway from first symptoms to initiation of insulin regimen in children with new-onset Type 1 Diabetes Mellitus (T1DM) and explore the reasons behind diabetes ketoacidosis (DKA) at onset among children with T1DM. Materials and Methods: An exploratory study was conducted using a pretested questionnaire, among parents of children diagnosed with T1DM within preceding 3 months. Results: Out of the total 105 children, 56.1% were males. The median age was 7 years. The commonest reported symptoms were polydipsia (97.8%), polyuria (75.2%), and nocturia (75.2%). The mean time taken by parents from onset of symptoms to decide to visit the physician (appraisal gap) was 7.85 ± 7.95 days. The help-seeking gap (from decision-making to visiting a physician) was 3.01 ± 8.31 days, diagnostic gap (from first visit to diagnosis) was 4.19 ± 6.72 days, and the treatment gap (from diagnosis to the start of insulin) was 2.12 ± 6.87 days. The DKA at onset (was present in 39 out of 105 children 37.1%) and was higher among children with lower per-capita income (P-0.017), lack of previous experience among parents (P-0.017), longer appraisal (P-0.023), and treatment gap (P-0.009). Conclusion: Increasing awareness about the diabetes among children among the public and primary healthcare workers can help prevent DKA at onset.

Background: Diabetes is a common co-morbid condition that influences morbidity and mortality in patients with COVID-19 and has been a focus of intense research. However, a systematic assessment of global diabetes research concerning COVID-19 is unavailable. Aim: We aimed to provide a bibliometric assessment of research output on diabetes concerning the COVID-19 pandemic. Materials and Methods: Publications on diabetes in relation to COVID-19 were retrieved from the Scopus database and analyzed using appropriate bibliometric indicators. Results: The publications indexed till January 6, 2021 numbered 762. The share of the top 10 of the 82 countries was 90.6%. The USA, China, India, Italy, and the UK were the most productive countries, with publication share ranging from 11.4% to 17.8%. Brazil, China, Germany, Australia, France, and India registered higher publication impact. The average citations per paper were 7.3, and 19.4% of the publications were funded. The share of publications on type 2 diabetes, type 1 diabetes, and gestational diabetes was 23.2%, 16.5%, and 4.1%, respectively. Diabetic complications, clinical studies, and epidemiology were the most researched aspects of diabetes concerning COVID-19, with 47.9%, 23.3%, and 14.0% share, respectively. Publications on pathophysiology numbered only 93 (12.2%). The most productive organizations were Tongji Medical College, China, Huazhong University, China, Postgraduate Institute of Medical Education and Research, India, and INSERM, France. Obesity and Cholesterol Foundation, India, Imperial College, UK, and The Central Hospital of Wuhan, China were the most impactful organizations. R. Pal, A. Ceriello, and B. Cariou were the most productive authors, whereas A. Misra, R. Gupta, and A.K. Singh were the most impactful. The most active journals were Diabetes Research and Clinical Practice, Diabetes and Metabolic Syndrome: Clinical Research and Reviews, Journal of Diabetes Science and Technology, and Diabetes Care. Conclusion: Extensive high-quality research has been conducted on diabetes in relation to the COVID-19 pandemic in developed and developing countries. There is a need to focus on pathophysiological studies to elucidate further the underlying mechanisms that predispose COVID-19-affected diabetes patients to severe disease and death. Our scientometric assessment may help understand the research gaps and guide future research in this field.

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.

Abstract 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).

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.

Hypothalamic hamartoma (HH) is one of the most common causes of central precocious puberty (CPP) in first few years of life. It can present with either seizures or CPP, although both manifestations coexist in the majority of the children. Gelastic seizures (GS), or laughing spells, are usually the first type of seizures seen in patients with HH. Although a wide variety of seizure types are known to occur in children with HH, GS are most common and consistent seizure type. The clinical presentation of HH may vary with the size and position of the mass, although large tumours typically present with both CPP and seizures. Although CPP can be managed with medical therapy, seizures can be very difficult to treat, even with multiple antiepileptic drugs. Noninvasive gamma knife surgery has been used with some success for the treatment of refractory epilepsy. We present a case of HH with very early onset seizures and CPP. The patient had an atypical form of seizures described by the parents as a "trance-like state" in which the patient had prolonged episodes of unresponsiveness lasting for hours with normal feedings during the episodes. GS occurred late in the course and were refractory to various combinations of antiepileptic drugs. A brain magnetic resonance imaging showed a large sessile HH (>20 mm). Later in the course of the disease, the patient experienced cognitive and behavioural problems. The patient underwent gamma knife surgery at nearly 5 years of age and experienced a modest response in seizure frequency. This case highlights the presentation of HH as a previously unreported seizure morphology described as a prolonged "trance-like state."

Context: Professional continuous glucose monitoring (P-CGM) has been approved for use in adults and adolescents with type 1 diabetes mellitus (T1DM). There is dearth of data on its use and feasibility in children 70% was seen in 31 (73.8%) children. Common adverse events were local redness in 11 (26.2%), local irritation in 16 (38.1%) and parental anxiety in 12 (28.5%) children. Conclusions: There were no significant adverse effects noticed with Professional CGM (using iPro™2) and is feasible and acceptable to children (2–10 years) with T1DM as well as their caregivers.

Banni grasslands in Gujarat are home to migratory pastoralists (Maldharis) for more than 500 years. Maldharis are landless and dependent on livestock for their livelihood. The study was conducted to measure the long-term growth and instability of different livestock species in Banni grasslands from 1977 to 2012. Pastoralists (280) were personally interviewed during 2015 to 2017 to prioritize the factors affecting trends in livestock population using Garrett ranking technique. The Compound Annual Growth Rate (CAGR) in population was highest for Banni buffaloes (5.89%) followed by Kankrej cattle (1.78%) and it was negative for goat (-0.29%) and sheep (–0.28%) population. Cuddy Della Valle Instability Index was very high for goat (89%) and sheep population (78%) whereas it was low for buffaloes (31%) and cattle (23%). The severity and duration of drought significantly affected the decline in population of goat and sheep. However, the shift in population of buffaloes and cattle was affected by complex of technological and policy factors. Decline in demand of Kankrej bullocks for agriculture use, low milk productivity of cows, and detrimental impact of regular feeding on Prosopis juliflora pods (weakening and dislocation of jaws and gradual death of cows) led to significant increase in population of Banni buffaloes when compared to Kankrej cattle. The population growth rate of Banni buffaloes (457%) was six times higher than the Kankrej cows (70%) from 1977 to 2012. This trend is most likely to continue in the following years as the migratory pastoralism is being gradually replaced by semi-migratory and sedentary pastoralism and establishment of organized dairy industry. Establishment of dairy units/Milk Collection Centres and improved road connectivity of villages in Banni have further facilitated in promotion of Banni buffalo based pastoralism as the primary source of livelihood. Composition of different livestock species in 2012 (Buffaloes: 72%, Cattle: 16%, Goat: 7% and Sheep: 6%) indicated the dominance of Banni buffalo both in terms of number and contribution to livelihood of pastoralists and overall economy of Banni grasslands.

Primary hypothyroidism related to morphological and volumetric abnormalities of the thyroid gland is one of the commonest of several endocrine dysfunctions in Williams-Beuren syndrome (WBS). We report a 10-month-old boy with WBS who presented with central hypothyroidism. During the neonatal period, he had prolonged jaundice, feeding difficulties and episodes of colic that continued during early infancy. Additionally, there was slowing of growth and mild developmental delay. He underwent surgical repair for supravalvular aortic stenosis at 6 months of age. An evaluation done to exclude cortisol deficiency before initiating levothyroxine lead to the detection of secondary adrenal insufficiency, unreported previously in WBS. In addition, insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 levels were low. This report of hypopituitarism in WBS indicates a need for complete evaluation of pituitary dysfunction in children with WBS.

Lipohypertrophy has been suggested as an outcome of lipogenic action of insulin and/or injection-related tissue trauma. In a cross-sectional study, we evaluated the predictors of lipohypertrophy in 372 type 1 diabetes patients (mean age 17.1 years) receiving subcutaneous insulin with pen and/or syringes for ≥3 months. On examining injection sites with inspection and palpation technique, 62.1% patients demonstrated lipohypertrophy. Univariate analysis showed that gender, BMI, HbA1c, injection device, rotation, injection area, needle length, insulin regimen, and total daily dose of insulin were associated with lipohypertrophy (p < 0.05). Notably, the mean needle reuse was comparable in patients with or without lipohypertrophy (8.1 vs. 7.2, p = 0.534). In multivariate logistic regression, gender, HbA1c, TDD, injection devices, and needle length lost its significance. Further, injections over smaller area (≤8.5 × 5.5 cm) and non-rotation of sites were found to be strongest independent predictor of lipohypertrophy (p < 0.0005 for both) with increased odds of 23.2 (95% CI 9.1–59.2) and 6.3 (95% CI 3.4–11.9) times, respectively. Being underweight was also a significant independent predictor (odds ratio [OR] 13.0 [95% CI 2.2–75.2], p = 0.004). Compared to rapid plus long-acting analogs, regular insulin plus long-acting analogs and conventional premixed insulin users had 3.2 (95% CI 1.5–6.8, p = 0.003) and 4.6 (95% CI 1.4–15.7, p = 0.014) fold higher risk of lipohypertrophy (mean injection frequency 4.01 vs. 4.01 vs. 2.09, respectively). Sub-group analysis showed that lipohypertrophy was 79% less likely in patients with multiple daily injections (≥4) than twice-daily regimen (OR 0.21, p < 0.0005). Moreover, lipohypertrophy was reduced to half with bolus doses of rapid-acting insulin analogs than regular insulin (p = 0.003), even though mean injection frequency was comparable (4.01 vs. 3.93, p = 0.229). This difference was statistically insignificant for basal doses with NPH or long-acting analogs (p = 0.069). Therefore, injection area, rotation, BMI, and insulin regimen are the best predictors of lipohypertrophy and together could correctly identify lipohypertrophy status in 84.4% patients with excellent discrimination capability (AUC = 0.906, p < 0.0005). In conclusion, findings of our study suggest that delivering rapidly absorbed insulin analogs over large injection area along with greater split of total daily doses reduce insulin-induced lipogenesis and outplay tissue trauma added through frequent injections and needle reuse.

An experiment was conducted in micro-plots filled with sandy loam soil having 14% clay and 0.33% organic carbon to see the responses of halophytic grasses Sporobolus marginatus and Urochondra setulosa collected from the extreme saline-sodic Kachchh plains, Bhuj, Gujarat, India under alkalinity/salinity. Maximum photosynthetic rate was recorded in control treatment. Photosynthetic rate decreased at pH 9.0 + EC 20 dS/m (11.25 and 17.8 μmol CO2/m2/s) in S. marginatus and U. setulosa respectively, along with reduction in stomatal conductance and transpiration rate. In comparison to control, increased accumulation of total soluble sugars and proline may be due to increased osmotic adjustment for both halophytes. Similarly, at mixed stress of pH 9.0 with EC 20 dS/m, the epicuticular wax load increased in both S. marginatus and U. setulosa (24.0 and 40.0 mg/g). A positive correlation of stress was seen with Na+ and Cl‑ content while negative correlation with K+ content. Na+ content increased to about 3-7 fold at salinity level, EC 35 dS/m. Similarly, with treatment of mixed stress of pH 9.0 with saline level EC 20dS/m, 3-6 times increase in Na+ with decrease in K+ was observed in S. marginatus and U. setulosa respectively. These grass species maintained better gas exchange properties with higher osmolytes accumulations and balanced ionic relations under high stress conditions of salinity and alkalinity. These attributes might be providing physiological adaptable mechanisms for growth under salt affected environments.

Acute Painful Diabetic Neuropathy (APDN) is a reversible neuropathy that occurs in patients with diabetes usually after a fast improvement in glycaemic control. The condition is extremely rare in children with Type 1 Diabetes (T1D). We describe a 12-year-old girl T1D who developed APDN shortly after diagnosis of T1D. Neurological examination, nerve conduction studies showed severe asymmetric lower limb sensorimotor neuropathy. She was treated with carbamazepine and benfotiamine (vitamin B1 analogue), and NSAID analgesics and showed complete recovery 9 months after the onset. The treating physicians should recognize and understand this entity in view of the current recommendations for quick achievement of glycaemic targets in T1D, the need to provide relief from severe pain and to lay emphasis on complete recovery.

Context: Ectopic Thyroid Gland (ETG) is known to be the most common form of thyroid dysgenesis in children with permanent congenital hypothyroidism (CH). Recent reports indicate that agenesis or hypoplasia of thyroid gland may be commoner as compared to ETG in thyroid dysgenesis (TD). There is limited information available on the proportion of different variants of TD in Indian children. Aim: To characterize the different TD variants in a cohort of North Indian children with TD. Settings and Design: Endocrinology Unit of a large Multispecialty Pediatrics Center located in North India. Retrospective review of clinical records of children with CH due to TD diagnosed between April 2004 and March 2014. Results: Diagnoses of TD in 94 children (48 boys and 46 girls) were based on combined scanning with high-resolution ultrasonography, and technetium-99m pertechnetate thyroid scintigraphy. Thyroid agenesis, ectopia and hypoplasia were diagnosed in 74 (78.7%), 14 (14.8%) and 6 (6.4%) patients respectively. The mean initial serum total T4 and thyroid stimulating hormone concentrations at diagnosis were 3.03 ± 2.88 μg/dL (range 0.01-8.9) and 284.52 ± 300.67 mIU/L (range 10.03-1159.0) respectively. Patients with ETG were older at the time of diagnosis as compared to patients with hypoplasia or ectopia. The mean duration of follow-up was 3.7 ± 2.85 years (range 3 months-10 years). Conclusions : Thyroid agenesis was the most common form of TD in our children with permanent CH. Hypoplasia and ectopia were uncommon. Female preponderance, noted in many previous reports, was not seen in our patients with TD.

In patients with tuberculosis, abnormal extrarenal production of 1,25-dihydroxyvitamin D3 by activated macrophages in granulomatous tissues may result in hypercalcaemia. More commonly reported in adults with active pulmonary tuberculosis, this complication may rarely occur in extrapulmonary tuberculosis, and children. The hypercalcaemia may be precipitated by usually recommended vitamin D and calcium supplementation in patients with tuberculosis. We report here an infant with tubercular meningitis who developed hypercalcaemia 12 days after starting routine vitamin D and calcium supplementation. This communication highlights the importance of close monitoring of calcium levels in patients with tuberculosis, especially if started on vitamin D and calcium replacement before antitubercular therapy.

There is wide variation in the prevalence of pancreatic and other major autoantibodies in different patient populations of Type 1 diabetes (T1D) across continents and even within countries. The data on frequency of associated autoimmunity Indian children with T1D is limited. A retrospective record review of 310 children aged 7.28±3.3 y (range 0.7-15 y) with recently diagnosed T1D attending our Pediatric Diabetes Clinic between April 2004 to September 2014, showed positivity for anti-GAD65, anti-IA2b, anti-TPO and anti-tTGA of 50% (64/128), 16% (12/72), 18.7% (23/123) and 22% (47/212) respectively. The male:female ratio in patients with anti-GAD, anti-TPO and anti-tTG positivity was 1.3, 0.7 and 0.6 respectively. In conclusion, our patient cohort exhibited a moderate prevalence of anti-GAD 65, low prevalence of anti-TPO and high prevalence of anti-tTGA autoantibodies as compared to previous reports. Male preponderance was noted in children with GAD65 positivity.

Context: High senstivity C-reactive protein (hsCRP) levels correlate well other parameters of obesity related metabolic syndrome (MS) and can be used as predictors of future cardiovascular disease risk. There is limited data on hsCRP levels in Indian children with simple obesity. Aim: To study the relationship of hsCRP levels with various anthropometric as well as metabolic parameters in children with simple overweight and obesity. Materials and Methods: This case control study was conducted in Paediatric Endocrinology clinic of a tertiary care hospital in Northern India. Levels of hsCRP were estimated in 100 overweight and obese children (BMI between 85th and 95th percentiles according to age & gender specific CDC 2000 growth charts) aged between 6 and 16 years and in 100 nearly age and sex matched healthy controls. These levels were then correlated to various anthropometric (body mass index, BMI; waist circumference, WC; hip circumference, HC; waist hip ratio, WHR; blood pressure) and biochemical (fasting blood glucose, FBG; total cholesterol, TC; high-density lipoproteincholesterol, HDL-C; low-density lipoprotein cholesterol, LDL-C; very low-density lipoprotein-cholesterol, VLDL-C; triglycerides, TG) parameters. Results: Mean levels of hsCRP were significantly higher in the study group (3.92±2.20 versus 2.15±1.05 mg/L) as compared to controls. Significantly more (58% versus 10%) subjects in the study group had hsCRP (>3 mg/L). Of all the parameters studied, only BMI showed a positive correlation with hsCRP levels in the study group. Multiple logistic regression analysis for predicting outcome of high hsCRP showed positive correlation with BMI; with every 1 kg/m2 increase in BMI, odds of high hsCRP level were increased by 37% (OR=1.37; 95% CI 1.23-1.53, p-value

A field experiment was conducted at CAZRI, Regional Research Station, Kukma-Bhuj during kharif 2009 to find out the management options for improving soil productivity and quality in shallow hard pan soils in arid Gujarat. There were four tillage treatments, viz no-tillage, minimum tillage, shallow tillage and deep tillage in main plots and four amendments (gypsum at 5 Mg/ha, farmyard manure (FYM) at 5 Mg/ha and both gypsum and FYM at 5 Mg/ha each and control) in sub plots. The results of the experiment showed that deep tillage along with the application of gypsum and FYM at 5 Mg/ha produced maximum yield and maximum net returns. The study found a decreased yield and negative net returns under no-tillage in these shallow hard pan soils. However soil biological and physical properties were better under no-tillage. Long term studies may be necessary to find out the applicability of no-tillage in these soils from a yield perspective.

Plasma levels of 25-hydroxyvitamin D [25(OH)D] were measured by competitive Electrochemiluminescence Immunoassay (ECLIA) in 92 children (67 boys, 25 girls) aged 3 months to 12 years at admission to hospital (timepoint 1, T1) and at discharge (timepoint 2, T2). There was a significant fall in the mean 25(OH)D from T1 (71.87 ± 27.25 nmol/L) to T2 (49.03 ± 22.25 nmol/L) (mean change = 22.84 nmol/L, P value = 0.0004). Proportion of patients having VDD (levels

Children with hypothyroidism generally have delayed pubertal development. Rare association with precocious puberty may occur especially in long standing untreated patients. The cardinal features of hypothyroidism induced pseudo precocious pubertal development include thelarche, galactorrhea and menarche. Other characteristics features are an absence of sexual hair and retardation of linear growth. Its manifestation as isolated menarche has been rarely reported. Recently, a five and half year old girl presented to us with history of one episode of vaginal bleeding. A pelvic ultrasonogram revealed multiple cysts in both ovaries and subsequent investigations led to a diagnosis of autoimmune hypothyroidism.