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Introduction: Energy metabolism in type 1 diabetes (T1D) is known to be different. Resting metabolic rate (RMR) accounts for the largest portion of total energy needs. The objective of our study was to assess resting metabolic rate and its determinants in adolescents and young adults with T1D in comparison with age- and gender-matched healthy controls. Methods: This cross-sectional study included 97 children and young adults (10–19 years) with type 1 diabetes having a disease duration of at least 1 year. For the control population, 95 age- and gender-matched healthy adolescents were enrolled. Clinical examination and biochemical evaluation of parameters pertaining to diabetes and body composition were estimated, and RMR was measured using indirect calorimetry for both cases and controls. Results: Adolescents with T1D were significantly shorter, and had significantly lower calorie intake, higher RMR and volume of oxygen consumed (VO2) as compared to the healthy controls (P < 0.05). RMR adjusted for weight showed a significant positive correlation with lean body mass (LBM) percentage, and energy intake and a negative correlation with disease duration. Those with a T1D duration of less than 5 years demonstrated a significantly higher RMR, lower body fat percentage, higher LBM percentage, carbohydrate and energy intake/kg body weight and higher calculated insulin sensitivity (IS) as compared to those with greater disease duration. Muscle mass percentage and higher energy intake were found to be significant positive predictors and advancing age/diabetes duration was a negative predictor of weight-adjusted RMR (P < 0.05), whereas IS and male gender tended towards significant negative association (P = 0.06). Conclusion: Indian children with type 1 diabetes had a higher resting metabolic rate as compared to healthy children. Muscle mass, energy intake and diabetes duration were observed to be important predictors of RMR in T1D. Reduction in RMR with advancing age/disease duration may predispose to weight gain and subsequent double diabetes in T1D. [ABSTRACT FROM AUTHOR]

Introduction: Recent evidence suggests that vitamin D deficiency and type-1 diabetes (T1D) have a bidirectional cause–effect relationship. The objective of this study is to estimate the prevalence and determinants of vitamin D deficiency in Indian children and young adults with T1D and assess the relationship between vitamin D status and their bone health. Methods: It was a single-centre, cross-sectional study. Inclusion: Children, young adults aged 5–25 years with T1D duration >1 year. Exclusion: Already on vitamin D supplementation, conditions affecting bone health. Data collected: Demographic, clinical, anthropometry, biochemical, body composition, DXA, pQCT measurements. Results: A total of 453 participants (251 girls) with T1D, mean age = 13.5 ± 4.0 years, disease duration = 5.7 ± 3.9 years. Mean 25-hydroxy vitamin D concentration of study group was 20.4 ± 11.3 ng/mL. One hundred and eleven (24.5%) were deficient in 25-hydroxy vitamin D, 141 (31.1%) were insufficient and 201 (44.4%) were sufficient. 25-Hydroxy vitamin D concentrations had significant negative correlation with BMI Z -score, diastolic blood pressure, fat percentage Z -score and positive correlation with physical activity, haemoglobin concentrations and trabecular density (P < 0.05). Risk of developing vitamin D deficiency and insufficiency was significantly lower in subjects with good/intermediate glycaemic control versus poor control (P = 0.008). Higher diastolic blood pressure and female gender were significant risk factors for development of vitamin D deficiency. Conclusion: Vitamin D deficiency has high prevalence in children and youth with T1D and has detrimental effect on bone geometry of these subjects. Weight reduction increased outdoor physical activity, good glycemic control are some modifiable factors that may prove useful in preventing vitamin D deficiency. [ABSTRACT FROM AUTHOR]

Introduction: Adolescence is a critical period for the accrual of bone mass. However, few studies have assessed the bone geometry in slum-dwelling girls/young women. We thus conducted this study: (1) to assess bone health in Indian adolescent girls and young women residing in slum vs nonslum (NS) areas and (2) to identify factors associated with poor bone health. Methods: This cross-sectional case–control study was performed on 110 apparently healthy unmarried, nonpregnant, nonlactating, postmenarchal adolescent girls and young women aged 11 to 24 years residing in urban slums with the same number of age-matched controls from NS areas. Anthropometric, dietary, physical-activity and bone-health parameters (using dual-energy X-ray absorptiometry and peripheral quantitative computed tomography—DXA and pQCT—respectively) were evaluated using standard protocols. A P value of < 0.05 was considered statistically significant. Results: Slum girls were significantly shorter and lighter and had lower dietary intakes of calcium and protein after adjusting for total daily calorie intake than NS girls (P < 0.05). Areal bone-mineral density (BMD) at lumber spine (0.940 ± 0.13 vs1.042 ± 0.15 g/cm2), femur and total body (less-head), bone-mineral apparent density adjusted for volume at the lumbar spine (0.295 ± 0.04 vs 0.319 ± 0.04 g/cm3) and height-adjusted bone density at femur (0.862 ± 0.011 vs 0.905 ± 0.011 g/cm2) were significantly lower in slum dwelling participants as compared to their NS counterparts (P < 0.05). After adjusting for confounders like sunlight exposure, anthropometric parameters and dietary calcium intake, area of dwelling was still a significant factor for the difference in BMD. Conclusions: The potential determinants of poor bone density and geometry in girls and young women residing in slums include residential location, dietary habits, and physical activity levels. Despite adjustments for confounding factors, differences in bone health between those in slum and nonslum areas suggest adaptations developed over generations of deprivation in these individuals, necessitating urgent intervention. [ABSTRACT FROM AUTHOR]

Introduction: A good screening tool, such as a growth chart, should distinguish between children with normal growth and those with perturbed growth. Suitability of synthetic Indian growth references for diagnosing growth-related disorders for under-five children has not been evaluated. To assess the validity of World Health Organization (WHO) 2006 standards vs synthetic Indian references (2019) (by comparing weight, height, body mass index (BMI), standard deviation scores (SDS) and the composite index of anthropometric failure (CIAF)) in differentiating normal children and children with growth-related disorders. Methods: Records of 2188 children (0-60 months) attending a tertiary centre paediatric outpatient department (OPD) were retrospectively studied; 1854 children were healthy and 334 were diagnosed with growth-related disorders as per the European Society for Paediatric Endocrinology (ESPE) classification. The anthropometric parameters converted to Z-scores for weight-for-age (WAZ), height-for-age (HAZ), BMI-for-age (BAZ) and a CIAF were computed using WHO and synthetic charts; Student's t-test was used for assessing differences and Youden's index for validity. Results: Disease status of children and anthropometric failure on WAZ, HAZ, BAZ and CIAF on both WHO and synthetic charts had a significant association (P-value <0.05). WAZ, HAZ on both charts and CIAF on synthetic chart had a fair to moderate agreement (Kappa statistics) with disease status as per diagnosis (P-value <0.05). The sensitivity and negative predictive value for all anthropometric parameters were higher for synthetic charts. Conclusion: Indian charts were more sensitive for diagnosing growth-related disorders from birth to 60 months of age when compared to WHO growth standards. [ABSTRACT FROM AUTHOR]

Introduction: Bone age (BA) assessment is important in evaluating disorders of growth and puberty; the Greulich and Pyle atlas method (GP) is most used. We aimed to determine the weightage to be attributed by raters to various segments of the hand x-ray, namely, distal end of radius-ulna (RU), carpals, and short bones for rating bone age using the GP atlas method. Methods: 692 deidentified x-rays from a previous study (PUNE-dataset) and 400 from the Radiological Society of North America (RSNA-dataset) were included in the study. Mean of BA assessed by experienced raters was termed reference rating. Linear regression was used to model reference age as function of age ratings of the three segments. The root-mean-square-error (RMSE) of segmental arithmetic mean and weighted mean with respect to reference rating were computed for both datasets. Results: Short bones were assigned the highest weightage. Carpals were assigned higher weightage in prepubertal PUNE participants as compared to RSNA, vice-versa in RU segment of post-pubertal participants. The RMSE of weighted mean ratings was significantly lower than for the arithmetic mean in the PUNE dataset. Conclusion: We thus determined weightage to be attributed by raters to segments of the hand x-ray for assessment of bone age by the GP method. [ABSTRACT FROM AUTHOR]

Introduction: Recent evidence reveals that type 1 diabetes mellitus (T1DM) impairs muscle function (MF) in adolescents. However, despite its importance in physical well-being, data on dynamic MF in Indian children and adolescents (C and Y) with T1DM are scarce. We assessed MF using Jumping Mechanography (JM, a measurement method for motion analysis and assessment of muscle power and force). (1) To assess dynamic MF by JM in C and Y with T1DM as compared to healthy controls (2) To determine predictors of MF in children with T1DM. Methods: A cross-sectional observational study on 266 children (133 - T1DM duration >1 year with no known comorbidities + 133 age and gender-matched healthy controls) aged 6-19 years. Anthropometry, body composition, and MF (maximum relative power Pmax/mass, maximum relative force Fmax/BW by JM) were recorded. The lean mass index (LMI) was calculated as lean mass (kg)/height (m2). HbA1c was assessed in T1DM. Independent sample t-test and linear regression were performed. Results: MF parameters (Pmax/mass 33.5 ± 7.2 vs 38.0 ± 8.6 W/kg and Fmax/BW 10.5 ± 2.9 vs 11.4 ± 4.1 N/kg, P < 0.05) were significantly lower in T1DM group vs controls. Positive association of body mass index and LMI with both MF parameters and negative association of insulin requirement and HbA1c with Fmax was observed in T1DM. Predictors of MF identified were MMI (Pmax/mass:β = 1.6,95%CI = 0.6-2.6; Fmax/BW:β =2.0,95%CI = 1.6-2.4) and HbA1c (Pmax/mass:β = -2.1,95%CI = -4.5--0.5; Fmax/BW:β = -1.1,95%CI = -2.0--0.2) (P < 0.05). Conclusion: C and Y with T1DM exhibits compromised muscle function. Poor glycaemic control increases the risk of having decreased MF, irrespective of diabetes duration and may contribute to sarcopenia in adulthood. [ABSTRACT FROM AUTHOR]

Introduction: Insulin resistance (IR) and obesity are common presentations of double diabetes (DD) in subjects with type-1 diabetes (T1D). There is evidence that dietary composition has an impact on developing IR. Objectives were to assess the impact of macronutrient and fibre intake on glycaemic control and the role of macronutrient composition of diet in the development of DD in subjects with T1D. Methods: This cross-sectional study included 77 young adults (10-25 years) with T1D. Data related to demography, anthropometry, biochemistry and body composition were collected. Dietary data was collected by fourteen-day food diary. IR was calculated using eGDR, SEARCH and CACTI equations, and metabolic syndrome (MS) was diagnosed using the International Diabetes Federation Consensus Definition. Results: Subjects at risk of DD had higher age, leptin levels, percentage carbohydrate consumption in diet and IR. A positive association of insulin sensitivity with fibre intake and %protein intake was noted. Poor glycaemic control, adiponectin/leptin ratio, fibre intake and insulin/carbohydrate ratio were significant negative predictors of IR. Addition of dietary factors to the regression model improved the R square and percentage of subjects identified correctly. Inclusion of dietary parameters significantly improves the prediction of the risk of development of DD in subjects with T1D. Conclusion: Good glycaemic control and increased intake of dietary fibre may prevent the development of IR in subjects with T1D and reduce the burden of DD. [ABSTRACT FROM AUTHOR]

We report a 2.2 year-old-boy, born of consanguineous marriage, referred for short stature, with history of neonatal death and skeletal deformities in his older sibling. Rhizo-mesomelic dwarfism was detected antenatally. Within 24 hours of birth, he developed multiple seizures. Examination revealed severe short stature, dolichocephaly, broad forehead, deep set eyes, low set ears, bulbous nose, small, irregular teeth, pointed chin, and triangular facies. He had rhizomelic shortening, stubby fingers, pes planus, and scanty hair. Neurological evaluation revealed ataxia, hypotonia, and global developmental delay. Skeletal survey radiograph revealed shallow acetabuli, short femurs and humerus, short, broad metacarpals and short cone-shaped phalanges with cupping of phalangeal bases. Clinical exome analysis revealed homozygous mutations involving the POC1A gene and the SLC13A5 gene responsible for SOFT syndrome and Kohlschutter-Tonz syndrome respectively, which were inherited from the parents. Both these syndromes are extremely rare, and their co-occurrence is being reported for the first time. [ABSTRACT FROM AUTHOR]

Background: Chemomechanical debridement is insufficient to disinfect all bacteria from the root canals of primary teeth, and obturation of canals with an appropriate material thus acquires excellent importance and remains a critical step in the ultimate success of pulpectomy. Aim: The aim of the study was to compare and evaluate Endoflas, Metapex, and a mixture of calcium hydroxide (CH) and zinc oxide (ZnO) as obturating materials (OMs) in primary mandibular second molars. Materials and Methods: Seventy-five mandibular second primary molars requiring pulpectomies were identified in children aged 4-8 years. They were randomly allocated to the three treatment groups according to the type of OM received using the block randomization technique. After the completion of chemomechanical debridement, the canals were filled with Endoflas, Metapex, and CH-ZnO mixture, respectively. The intergroup clinical and radiographic comparison was made based on Coll and Sadrian criteria to decipher their clinical performance at 1, 3, and 6 months. Results: No statistically significant differences between the groups were observed at any evaluation time interval (P > 0.05). At 6 months, the clinical success rates were 95.2% in Endoflas, 96% in Metapex, and 95.8% in the CH and ZnO mixture groups, respectively. The materials, however, behaved differently in different clinical situations. Conclusion: Based on the observations, all three OMs showed similar clinical success in maintaining tooth functioning, but their use can be restricted to indications. However, prospective studies with longer follow-ups with more stringent eligibility criteria are required to reach more definitive conclusions. [ABSTRACT FROM AUTHOR]

Background: Non-genetic factors like microbial dysbiosis may be contributing to the increasing incidence/progression of type 1 diabetes mellitus (T1DM). Objectives: To analyse the gut microbiota profile in Indian children with T1DM and its effect on glycaemic control. Methodology: Faecal samples of 29 children with T1DM were collected and faecal microbial DNA was extracted and subjected to 16S rRNA (ribosomal RNA) sequencing and further analysis. Results: The dominant phyla in children with T1DM were Firmicutes and Bacteroidetes. Butyrate-producing bacteria Blautia and Ruminococcus showed a significant negative correlation with the glycosylated haemoglobin (HbA1C) levels (p < 0.05). Coprococcus and Propionibacterium were important negative predictors of glycaemic control (p < 0.05). Conclusion: Our study suggests that Indian children with T1DM have a distinct gut microbiome taxonomic composition and that short-chain fatty acid-producing bacteria like Ruminococcus and Blautia (butyrate-producing) may play an important role in the glycaemic control of subjects with T1DM. [ABSTRACT FROM AUTHOR]

Background: A previous study compared insulin sensitivity indices for the detection of double diabetes (DD) in Indian adolescents with type-1 diabetes (T1D) and derived a cut-off to predict future risk for the development of metabolic syndrome (MS) in adolescents with T1D. We conducted the current study with the aim to validate these cut-offs for detecting DD among Indian subjects with T1D from various geographical locations. Methods: This multicentric cross-sectional study included 161 Indian adolescents with T1D. Demographic, anthropometric, clinical, and biochemical data were collected using standard protocols. Insulin sensitivity (IS) was calculated using various equations developed to determine insulin sensitivity in subjects with T1D. Metabolic syndrome was diagnosed using International Diabetes Federation (IDF) Consensus Definition 2017. Results: We report 4.3% prevalence of MS in Indian adolescents with T1D with an additional 29.8% of study participants at risk of development of MS. Low High density lipoprotein (HDL) (23.6%) was the commonest abnormal component of the MS definition. Insulin sensitivity calculated by an equation derived by the SEARCH group was the most appropriate index to identify MS and metabolic risk in Indian adolescents with T1D. The proposed cut-off of 5.48 had high specificity, positive predictive value, and negative predictive value in identifying the risk of the development of DD. Conclusions: Insulin sensitivity calculated by the equation proposed by the SEARCH group together with cut-offs derived in earlier study may be used effectively to identify risk of development of MS/DD in Indian adolescents with T1D from various geographical locations. [ABSTRACT FROM AUTHOR]

In pediatric patients, neurological manifestation of COVID-19 infection has been reported in acute and postinfectious stages. Hashimoto encephalopathy (HE) is the most common cause of hypothyroidism in children with prevalence being 1.2%. More than 200 cases of HE have been reported in adults. Only 60 cases have been reported in children. We present a case of a 2-year-old girl with status epilepticus and respiratory failure needing prolonged ventilation. Her COVID-19 antibody and antithyroid antibodies (anti-thyroid peroxidase and anti-thyroglobulin) were positive. Her thyroid profile was abnormal. Her clinical state improved after receiving an intravenous immunoglobulin and pulse dose of methylprednisolone. This suggests that complete recovery for such patients can be achieved with early detection and immunomodulatory treatment [ABSTRACT FROM AUTHOR]

Background: India has the highest number of prevalent type-1 diabetes (T1D) cases in the under-20-year age population. Data on the anthropometry of underprivileged Indian children with T1D are scarce. In economically disadvantaged countries like India, poor growth in patients with T1D is a major concern due to limited accessibility and affordability. Besides, due to the double burden of malnutrition, the prevalence of obesity is increasing mirroring the global trends, which may lead to the development of insulin resistance. Objectives: This study aims to assess the prevalence of malnutrition in Indian children and youth with T1D and to identify the determinants of short stature. Methods: A registry-based cross-sectional analysis of data collected from various centres across India enrolled in the Changing Diabetes in Children (CDiC) programme. Results: We observed that 6.4% were undernourished (3.4% severe undernutrition) and 17.7% (overweight 13.2%) had combined overweight/obesity. 21.2% of participants had short stature (adjusted for mid-parental height) with 7.4% cases of familial short stature. Longer duration of illness and insulin requirement were significant positive predictors of short stature while glycaemic control, insulin regimen and mid-parental height did not have a significant relationship with short stature. Participants on basal-bolus regimen had significantly higher insulin requirements and better glycaemic control than the ones on mixed-split regimen. Conclusion: We report that around one-fifth of children and youth with T1D were overweight/obese and around a fourth were stunted, especially those with longer duration of diabetes and higher insulin requirements. Close monitoring of anthropometric parameters is necessary for all children with T1D to optimize growth and nutrition. [ABSTRACT FROM AUTHOR]

Background and Objectives: Owing to paucity of data on adult height in Indian girls with Turner syndrome treated with growth hormone (GH), this study was conducted to assess improvement in height following GH therapy and adult height achieved with long-term GH therapy in Indian girls with Turner syndrome and to assess relationship between achieved and predicted height. Methodology: Retrospective analysis was performed on 12 girls with karyotype-proven Turner syndrome, who had attained adult height following mean duration of GH therapy of 4.8 years (range: 2.7-7.6). Adult height predictions were performed using index of responsiveness (IOR) and Ranke's prediction model. Results: Mean age at starting GH was 10.2 ± 1.9 years; Pubertal induction was between 11 and 15 years. Mean height gain was 29.3 ± 9.8 cm (range: 14-39.5) from onset of treatment to adult height. Significant improvement in height Z scores (IAP 2015 and Indian Turner reference data) following GH therapy (p = 0.002 and 0.012, respectively) was noted. Using Indian Turner reference data, the height Z score improved from pre-treatment 0.8 ± 0.8 to 2.0 ± 0.9 on stopping GH and adult height Z score of 1.3 ± 0.7. Using Ranke's equation for prediction of near adult height, predicted and achieved adult height showed a strong positive correlation (Spearman correlation coefficient = 0.827, significant at 0.01 level). Conclusion: At a dose in the lower range (40-50 mcg/kg/day) of recommendation and duration of 5 years, Indian girls with Turner syndrome can achieve adult height within the healthy Indian reference range. Dose individualization based on IOR would help in optimizing GH dosage and would turn out to be economically sustainable without compromising on height outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Increased prevalence of metabolic syndrome in Indian adolescents owing to the obesity epidemic leads to double diabetes (DD), which is associated with an increased risk of complications in type-1 diabetes (T1D). Metformin may be a useful intervention for the prevention and treatment of insulin resistance in T1D. We conducted this pilot randomized controlled trial with the objective of investigating the effect of metformin on insulin sensitivity in Indian adolescents with T1D. Method: This pilot randomized controlled trial was performed on 59 participants with T1D aged 10-19 years distributed uniformly by gender and puberty across two groups with a 3-month intervention period. The intervention group received metformin (weight less than 60 kg received 500 mg twice daily and more than 60 kg received 1 gm twice daily) and non-metformin group received standard of care for diabetes. Anthropometric, clinical details, biochemistry and insulin sensitivity indices (ISI) were evaluated using standard protocols at baseline and endline. Result: 22.2% of subjects from non-metformin group and 12.5% from metformin group were at the risk of the development of DD. The odds ratio and relative risk for the development of DD in non-metformin subjects were 2.0 and 1.4, respectively, as compared to participants in metformin group. The mean improvement in ISI ranged from 1.4% to 4.6% in participants on metformin as opposed to deterioration of -2% to -14.1% in non-metformin group. On performing the paired sample t-test, the reduction in ISI in non-metformin group was significant. Conclusion: Metformin may prevent deterioration in insulin sensitivity in Indian adolescents with T1D. [ABSTRACT FROM AUTHOR]

Background: Common etiologies of sciatic mononeuropathy are compressive, infiltrative, traumatic, or diabetic. However, in a proportion of patients, the etiology remains elusive despite extensive serological, electrophysiological, radiological, and histological investigations. Methods: Patients with unexplained sciatic mononeuropathy were studied with regard to their clinical, radiological, pathological, and treatment aspects. Results: We could identify five cases of sciatic mononeuropathy wherein the etiology remained unknown even after a comprehensive evaluation. The compressive, metabolic, hematological, and immune causes were ruled out with necessary investigations. The clinical, electrophysiological, radiological, and histological features of these patients are discussed. Conclusion: The etiology of sciatic mononeuropathy can remain obscure in certain instances in spite of the comprehensive workup. The role of investigations and the exclusion process of various diagnostic entities are discussed. [ABSTRACT FROM AUTHOR]

Background: Malnutrition among under-five children (U5C) in India is a major public health problem due to double burden caused by nutritional transition. WHO cut-offs are adopted as global growth standards which define how children should grow in condition of optimal nutrition and health. Growth references which are representative of population-specific existing growth patterns need to be updated regularly which is cumbersome; hence, the author's group published 'synthetic growth references' for Indian children of age 0-18 years. Objectives: The objective of this study is to field test the new synthetic growth references in U5C for height-for-age, weight-for-age and body mass index (BMI)-for-age against WHO charts in urban and rural Indian children to estimate prevalence of various indices of malnutrition. Methods: A cross-sectional anthropometric assessment of apparently healthy rural and urban Indian U5C attending vaccination centre was performed using standard protocols. They were converted to Z-scores using WHO and Indian synthetic growth references. The equality of proportion of parameters of malnutrition was tested by McNemar's test and P value <0.05 was considered significant. Results: WHO charts significantly overestimated stunting and malnutrition as compared to synthetic references with difference in sensitivity of 7.2% and 8.5%, respectively, and converse for over-nutrition by 2.1%. The most commonly affected parameter of malnutrition was underweight. Stunting was significantly higher in rural population using both cut-offs (P < 0.05). Conclusion: The synthetic references limit the spread of weight and BMI and do not overestimate stunting and wasting. They may be more useful for identification of malnutrition and may thus be recommended for routine screening in Indian U5C. [ABSTRACT FROM AUTHOR]

Background: Effect of tobacco, areca nut, and alcohol consumption (vices) on orthopantomogram (OPG) indices. Aims: To assess the relationship between vices on OPG indices in Indian adult males aged 20–60 years. Subjects and Methods: This study was conducted on 172 males with a mean age of 34.2 ± 1 years. OPG was used for measuring mandibular indices and correlated with the history of the presence of vices. Statistical Analysis Used: Data were presented as mean ± SE or frequency (%). Point biserial correlation was used to assess the correlation between OPG indices and vices. Analysis of covariance was used to analyze the differences in age-adjusted OPG indices between males who had vices against males who had no vice. Results: Overall, 21.5% of the males had at least one vice. A significant decrease in antegonial index (AI) values (P < 0.05) was found between tobacco and alcohol consumption. There was a significant decrease in the values of the gonial index (GI) and AI, having at least one vice (P < 0.05). A higher percentage of participants who had at least one adverse habit had low OPG indices score than participants who had no such habits. Conclusions: Orthopantomogram indices specifically AI were negatively affected in males who had vices. [ABSTRACT FROM AUTHOR]

Peripheral neuropathies are traditionally categorized into demyelinating or axonal. It has been proposed that dysfunction at nodal/paranodal region may be a key for better understanding of pathophysiology in patients with immune mediated neuropathies. In last few years, antibodies targeting node and paranode of myelinated nerves have been increasingly detected in patients with immune mediated neuropathies. These patients have clinical phenotype similar common inflammatory neuropathies like Guillain Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy with some additional atypical neurological and systemic features, and they respond poorly to conventional first line immunotherapies like IVIG. This review summarizes the structure of the node, concept and pathophysiology of nodopathies. We provide an overview of clinical phenotypes in patients with specific nodal/paranodal antibodies, along with electrophysiological and other diagnostic features and suggest therapeutic line of management based on current evidence. [ABSTRACT FROM AUTHOR]

Further management of this condition with novel drug idebenone (and CO Q 10) can be undertaken, especially in adult patients with the m.11778G>A ND4 mutation, as in our patient as these patients would otherwise have the worst visual outcomes in their natural course. A knowledge of ethambutol-induced conversion in LHON as in our patient can help prompt suspicion of LHON. Keywords: Ethambutol-induced optic neuropathy; idebenone; Leber's hereditary optic neuropathy EN Ethambutol-induced optic neuropathy idebenone Leber's hereditary optic neuropathy 2760 2762 3 07/15/22 20220701 NES 220701 Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease which is blinding with variable penetrance. [Extracted from the article]

Background: There are several methods of bone age (BA) assessment, which include Gruelich-Pyle (GP), Gilsanz-Ratib (GR), and Tanner Whitehouse-3 (TW-3) methods. Although GP atlas is the most widely used, there are concerns about its accuracy in children of different ethnicities, making the use of the TW-3 method an attractive option in Indian children. Objectives: 1) To assess the relationship of BA with chronological age (CA) as assessed by different methods (GP, GR, and TW-3) in healthy Indian children 2) To assess which of the three methods of BA assessment is more suitable in Indian children. Methodology: X-rays of 851 children (438 boys and 413 girls, aged 2–16.5 years) were analyzed by four independent observers using three different methods of BA estimation (GP, GR, and TW-3). Mean BAs were converted to Z-scores. For purpose of deciding which method of BA was most suitable in our cohort, a test of proportions and root mean square (RMS) deviations were computed. Results: Using the test of proportions, the TW-3 method was most suitable overall (P < 0.05). TW-3 method was again most applicable in prepubertal boys (P < 0.05), in prepubertal girls (although not significant, P > 0.1), and pubertal girls (P < 0.05). However, in pubertal boys, the GR atlas method was most suitable (P < 0.05). The same results were obtained when root mean square (RMS) deviations were computed. Interestingly, BA was underestimated in Indian boys irrespective of the method used. In Indian girls, however, the BA was underestimated till the pubertal growth spurt, after which there was rapid advancement of BA. Conclusions: Among the three methods (GP, GR, and TW-3), the BAs estimated by the TW-3 method were closest to CAs. Hence, it seems reasonable to recommend the use of the TW-3 method for BA estimation in the Indian population till an Indian standard bone age atlas is developed. [ABSTRACT FROM AUTHOR]

Introduction: There is a growing body of evidence against using World Health Organization (WHO) charts for developing nations. Our objectives were: 1) To compare nutritional status of <5-year-old Indian children using WHO charts and synthetic Indian charts (SC) 2019. 2) To study nutritional status across wealth index categories. 3) To study nutritional status of predominantly breast-fed infants <6 months of age using both charts. Materials and Methods: Data from 4th National Family Health Survey (n ~ 236117, 0-59-month-old children) were used for assessing nutritional status using the WHO charts and SC. Z-scores were calculated for length/height, weight, and weight-for-height (WAZ) using both charts. Children were classified into degrees of malnutrition using appropriate cutoffs. Results: Stunting, wasting, and underweight were significantly higher using WHO charts. The prevalence of stunting (height for age) and wasting (WHZ) changed from high to medium and critical to poor when the reference changed from WHO to SC. All Z-scores showed an improving trend with increasing wealth index. On SC, almost all WHZ (wasting) from the richest to poorer were >-0.5 (clinically significant), whereas on WHO charts all wealth classes had WHZ <-0.5. For children under the age of 6 months, WHZ from richest to poorest was between -0.97 and -0.89 by WHO and 0.27 and 0.38 by SC. Conclusions: Use of Indian synthetic growth charts for growth monitoring of under-five children may be more appropriate; infants under 6 months and children from well off families performed well on these charts. [ABSTRACT FROM AUTHOR]

Context: Studies on prepregnancy body mass index (BMI), gestational weight gain (GWG), and pregnancy outcomes among urban Indian slums are sparse. Aims: To study BMI during early pregnancy, GWG, and maternal and neonatal outcomes among slum-dwelling women in Pune, India. Design: A retrospective study among pregnant women attending antenatal care (ANC) facility. Methods and Material: Anthropometric and clinical data throughout pregnancy and pregnancy outcomes postdelivery were collected during hospitalization for delivery using ANC cards. Asian BMI cut-offs were used to define underweight (UW), normal weight (NW), overweight (OW), and obesity (OB). GWG was classified into insufficient, adequate, and excessive categories (2009 Institute of Medicine). Statistical analysis was performed using R (v 4.0). Results: Slum-dwelling pregnant women (n = 509, mean age 24 (3.6) years) were studied. Seventy-five percent of the women visited ANC clinics at least thrice during pregnancy. Only 17.5% (n = 89) of the women registered before 12 weeks of gestation, and higher education and being primiparous were the correlates. A total of 28% of the women were UW, whereas 25% of the women were OW/OB as per early pregnancy BMI. The highest percentage of preterm deliveries and cesarean/instrumental deliveries were observed in OW/OB categories. A total of 27% gained appropriate gestational weight. This cohort had 508 live births (mean BW- 2.8 kgs) and one stillbirth. One baby had macrosomia (BW >4 Kg), whereas 19% were low birth weight (LBW) (BW <2.5 Kg). Conclusions: Double burden of malnutrition (UW and OW) was observed among young slum-dwelling women. The proportion of slum-dwelling women attending ANC clinics during early pregnancy is still low. Increased uptake of government programs is required to enhance maternal and child health. [ABSTRACT FROM AUTHOR]

BACKGROUND: Dyslipidemias are on the rise and are increasingly being treated with statins. As the metabolism of cholecalciferol and cholesterol are interrelated, reduction in cholesterol synthesis by statins is likely to affect Vitamin D status. OBJECTIVES: (1) The aim is to study the effect of treatment with statins (Atorvastatin/Rosuvastatin) on 25-hydroxy-Vitamin-D (25OHD) among newly detected subjects with dyslipidemia for 6 months (2) To study the impact of 25OHD concentrations on the efficacy of statin treatment. MATERIALS AND METHODS: This was a prospective, balanced randomized (1:1), open-label, parallel-group study, in apparently healthy Indian adult men (south Asian, 40–60 years). At baseline, serum lipids and 25OHD concentrations were measured. Based on the Adult Treatment Panel III guidelines, subjects were divided as per lipid concentrations into controls (who did not require statin treatment) and intervention (who required statin treatment) groups. Random allocation of subjects was done in two groups for receiving intervention for 6 months: Atorvastatin group (n = 52, received Atorvastatin) or Rosuvastatin group (n = 52, received Rosuvastatin). Lipids and 25OHD concentrations were measured at the end line. RESULTS: Atorvastatin group presented significant reduction (P < 0.05) in 25OHD, total cholesterol (TC) and low-density-lipoprotein-cholesterol (LDL-C) concentrations at the end line. In the Rosuvastatin group, significant drop in TC, LDL-C and high-density lipoprotein cholesterol (concentrations (P < 0.05) was observed, while 25OHD concentrations showed no significant change. Mean 25OHD concentrations were significantly correlated with a reduction in LDL-C concentrations in Atorvastatin group. CONCLUSIONS: Treatment with Atorvastatin resulted in a reduction in 25OHD concentrations; further, its efficacy in reducing LDL-C concentrations was related to the 25OHD concentrations. [ABSTRACT FROM AUTHOR]

Background and Objectives: Disease specific growth charts are useful to monitor growth and disease progress in specific disorders such as Turner syndrome. As there is a paucity of data on spontaneous growth of Indian girls with Turner syndrome, the objectives were to construct reference curves for height and assess height velocity in Indian girls with Turner syndrome from 5 centers from western India. Material and Methods: Three hundred forty-eight readings of height and weight on 113 genetically proven girls with Turner Syndrome from 5 centers from western India were collected and retrospectively analyzed. Data were collected over the last 2 decades (GH treatment naive girls were included). The method described by Lyon et al. was used to compute smoothed standard deviations and percentiles for height. For computing growth velocities, longitudinal data were used on 104 untreated girls (longitudinal readings for height for a minimum of 3 years were used). Midparental height z scores (MPHZ) were computed. Results: In girls with Turner syndrome, the mean adult height was found to be 140.1 cm. Height velocity was low at all ages compared to normal girls with a notable difference beyond the age of 10 years where normally, a growth spurt is expected. The MPH Z-score correlated positively with the height Z-score. The 3rd, 50th, and 97th height percentiles of Turner girls at all ages were lower than normal girls' charts. Conclusion: Turner syndrome charts for height are presented; these charts may be used to monitor growth in girls with Turner syndrome. [ABSTRACT FROM AUTHOR]

Background: Reduced levels of estrogen have been associated with metabolic alterations and increased insulin resistance (IR) in postmenopausal women, thus predisposing them to cardiometabolic risks. The aim of this study was to assess alterations in parameters of cardiometabolic risk in apparently healthy pre- and post-menopausal women and to study the effect of IR on these metabolic parameters. Methods: A cross-sectional study was conducted on randomly selected apparently healthy women (n = 262). These women were categorized as premenopausal (n = 184) and postmenopausal (n = 78). Anthropometric measurements, blood pressure, lipid profile, fasting glucose, and insulin concentrations were estimated on all the participants using standard protocols. Homeostatic model assessment of IR was computed to estimate the level of IR. Results: Most lipid parameters, blood pressure, waist circumference, and fat percentage were significantly higher (P < 0.05) in postmenopausal women than premenopausal women. On subcategorizing women with respect to IR (<3, >3), metabolic parameters (e.g., triglyceride – 104.7 ±53.2 mg/dl, Blood Sugar Level Fasting (BSLF) – 103.3 ± 40.1 mg/dl, and fasting serum insulin – 23 ± 12.3 mIU/L) were also higher (P < 0.001) in premenopausal women having IR >3. Significantly higher low-density lipoprotein (132.7 ± 38.7 mg/dl vs. 114.4 ± 25 mg/dl) and total cholesterol (211.3 ± 40.5 vs. 184.8 ± 29.4 mg/dl) were observed in postmenopausal women with IR >3 (P < 0.05) along with higher BSLF (126.6±54.3 mg/dl**) and fasting insulin levels (22.3 ± 12.1 mIU/L) (P < 0.001). Conclusion: This study reveals that IR may predispose women to increased cardiometabolic risk. Urgent attention needs to be focused toward metabolic health of women. [ABSTRACT FROM AUTHOR]

Background: The traditional Neurology exit examination in India has remained unchanged over the last few decades. In developed countries, objective evaluation methods have replaced the traditional ones. A need for such methods has not been explored in India. Objective: We aimed to study the perceptions and key recommendations of Neurology examiners on the existing examination pattern. Material and Methods: We conducted an online survey of examiners perceptions and recommendations using a set of 10 multiple-choice questions and an open-ended question. Results: 46 examiners provided completed responses suitable for analysis. Nearly equal proportions (30%) of the examiners had 10 years, 10--25 years and >25 years' experience. 92% were not satisfied with current system, 95% did not find adequate time for correction of theory scripts, 90% felt that theory questions were random, and 95% had legibility issues. 84% felt that the practical exams do not test true learning, 98% felt the examination stress impairs the performance and 85% felt that there are no objective criteria to pass the candidate. 83% felt the current system-needed changes. The key suggestions provided by the examiners to improve the system included objective assessments like MCQ, OSCE, OSLER and DOPS, inclusion of larger number of short answer type questions and periodic internal assessments of the candidates. Conclusions: A vast majority of examiners favoured changes to the current examination system and provided key recommendations. A larger study is needed to extrapolate these findings to the rest of India [ABSTRACT FROM AUTHOR]

Subperiosteal abscess (SPA) is a known sequel of acute mastoiditis typically afflicting children and less often the elderly, caused by Streptococcus, Staphylococcus, and anaerobes. Atrophic rhinitis is a progressive disease with nasal mucosal and turbinate atrophy, thick dried crusts, and foul odor called ozaena, caused by Klebsiella ozaenae. We present an interesting case of SPA in the mastoid in a patient with atrophic rhinitis, astonishingly caused by a common pathogen, K. ozaenae, and a review of literature. The presence of coexistent atrophic rhinitis and mastoiditis should always compel otolaryngologists to consider K. ozaenae as the common etiology. [ABSTRACT FROM AUTHOR]

Trichilemmal carcinoma is a rare malignancy of hair follicles affecting sun-exposed and hair-bearing skin; occurrence over pinna is unusual. This case report describes one such uncommon presentation in a 71-year-old man who came with a pinna swelling clinically mimicking basal cell carcinoma. Diagnosis is an arduous task due to its close resemblance with more common cutaneous tumors. Misdiagnosis or delay in intervention may lead to recurrence and local metastasis. [ABSTRACT FROM AUTHOR]

Background and Aims: For updating growth references, large datasets are usually required; collection of these data are expensive and cumbersome. Using a combination of regression equations, Preece Baines model and global LMS values, synthetic growth references for the target population can be generated. The objective of this study is to compare growth references created from continuous anthropometric data using LMS method versus those created synthetically from anthropometric means at key ages. Methods: De-identified data on 46421 children (26037 boys) from 0-18 years of age from several multicentric studies conducted by the authors' group (2007 to 2017) were included in this study; growth references were constructed using the LMS method. For the production of synthetic references, arithmetic means of heights and weights at key ages were used and global LMS values were used from literature. Results: There was no difference in the medians for height, weight and BMI between the references created by the two methods. The extreme percentile values for height were similar (P < 0.05). However, the spread of values for weight and BMI was narrower in the synthetic references. Conclusion: Growth references produced from continuous data differ from those produced synthetically using anthropometric means mainly at the extreme centiles for weight and body mass index; synthetic references take into consideration global trends over several decades. [ABSTRACT FROM AUTHOR]

Objective and Aims: Overweight/obese children are at risk of developing type 2 diabetes mellitus. Random glucose elevations provide early warning signs of glycemic dysregulation. To assess random blood glucose (RBG) concentrations and risk factors associated with prediabetes in children aged 3-18 years from six Indian regions. Method: Multicenter, cross sectional, observational school-based study; multi-stage stratified random sampling was carried out. Height and weight measured; body mass index (BMI) was computed. RBG measured using a glucometer. National sample survey was used for dietary patterns. Data were analyzed using SPSS 25.0 for Windows. Setting: Study centers were from Maharashtra, Gujarat, Chhattisgarh, Assam, Tamil Nadu and Punjab from 40 selected schools. Participant: Children aged 3-18 years were measured. Results: Data on 14339 subjects (7413 boys) were analyzed. Prevalence of obesity was 5.8% and overweight-10.6%. Overall, 1% had low (<3 mmol/L), 93.7% in reference range (3.9-7.2 mmol/L) and 5.3% had elevated RBG (>7.2 mmol/L). With increasing mean BMI, there was increase in RBG concentrations. Children from Tamil Nadu were more likely to have RBG outside reference range compared to other regions (P < 0.05). Assam and Punjab had highest prevalence of RBG and BMI within reference range. Energy intake partly explained regional variations. Multivariate analysis showed male gender, urban residency, age >10 yrs (girls) and 13 yrs (boys), and overweight or obesity were predictive of prediabetes. Conclusion: Increased prevalence of overweight, obesity and prediabetes in Indian children are a matter of concern. Regional differences suggest that strategies to prevent obesity and combat perturbations in blood sugar may have to be customized. [ABSTRACT FROM AUTHOR]

Aim: This questionnaire-based national survey is aimed at understanding the patterns of practice of various aspects of central nervous system (CNS) tuberculosis (TB) among neurologists.Settings and Design: Neurology department of a tertiary medical college.Materials and Methods: A questionnaire was sent through email to all practicing neurologists in India. The responses were analyzed.Statistical Analysis: Inferential statistics.Results: In all, 144 responses were received (out of the 853 questionnaires sent). The major discrepancies were in the primary antitubercular drug regimen (HRZE + HR), duration for tubercular meningitis (TBM) [12 months] and tuberculoma (12-18 months) to develop, follow-up (varied), linezolid use (varied), proportion of drug-resistant cases (<25%), and not taking histological aids (91%). The cerebrospinal fluid (CSF) TB polymerase chain reaction (PCR) utility (75%), not using CSF adenosine deaminase [ADA] (58%), the strategy to stop antitubercular drugs, and the use of steroids (77%) were according to guidelines.Conclusion: The present survey, for the first time, provides ground-level evidence of various aspects of CNS TB as practiced by neurologists in India. The major diversity was observed in therapeutics such as the choice of antitubercular drugs, its duration, linezolid use beyond the recommended duration, and knowledge of drug resistance. The monitoring aspects of CNS TB also showed variations. The investigational aspects of CNS TB such as using TB PCR, not using CSF ADA, and regular neuroimaging revealed a good clinical practice. Other CSF parameters require uniformity. This survey thus helps to identify areas of future work in CNS TB in India. [ABSTRACT FROM AUTHOR]

Background: Genital stage and testicular volume examination and assessment are essential for assessment of growth puberty in boys. There is paucity of Indian data for penile and testicular parameters particularly in pubertal years. Aim: The aim of this study is to present normative data for penile length, testicular volume, and testicular length from birth to 18 years in boys from western Maharashtra and to correlate these parameters with sexual maturity staging (SMR) (Tanner staging). Materials and Methods: A cross-sectional observational study was undertaken in apparently healthy boys from 0 to 18 years of age from western Maharashtra from October 2016 to October 2017. Age, anthropometric parameters, stretched penile length (SPL), testicular volume, and testicular length were measured. Pubertal stage was categorized using Tanner staging. Data were entered in Microsoft excel 2013 and using SPSS version 16. LMS method was used to construct age-specific Z-scores for SPL, testicular volume, and testicular length. Results: A total of 843 boys were enrolled in the study. Mean SPL was 4.1 ± 0.4, 5.4 ± 0.8, and 10.2 ± 1.7 cm at 1, 10, and 18 years of age, respectively, and showed a gradual rise from birth to 18 years. Mean testicular volume was 1.8 ± 0.5, 3.3 ± 1.2, and 24.1 ± 3.5 ml, whereas testicular length was 1.3 ± 0.4, 2.1 ± 0.5, and 4.4 ± 0.7 cm respectively, at 1, 10, and 18 years, respectively. The magnitude of increase for SPL and testicular volume was greater around 9–10 years. The increase in SPL and testicular size was hand in hand with SMR for genital development as well as for axillary and pubic hair stages. Conclusion: Normative data on SPL, testicular volume, and testicular length from western Maharashtra are presented. These data may be useful in disorders of puberty and genitalia in boys. [ABSTRACT FROM AUTHOR]

Background: Guillian--Barre' Syndrome (GBS) has been shown to be associated with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. The aim of our study was to study the clinical profile and outcomes of GBS in COVID-19 from the Western region of India, the State of Maharashtra. Methods: This was a retrospective, multicenter observation study from different hospitals in Maharashtra beginning from March 2020 until November 2020. Results: We report 42 patients with COVID-19 GBS. Mean age was 59 years (range, 24--85 years). 31/42 (73.8%) were men. GBS was the presenting symptom in 14/42 (33%), while six of them remained asymptomatic for COVID-19 despite positive SARS-CoV-2 on nasopharyngeal swab reverse transcriptase polymerase chain reaction. The median interval between COVID-19 and GBS was 14 days (SD + 11), with minimum of 1 and maximum 40 days. Clinical presentation was like that of typical GBS. Electrophysiological studies showed a predominant demyelinating pattern in 25/42 (59.5%). Inflammatory markers were elevated in 35/42 (83.3%) and 38/42 (90.5%) had an Abnormal high-resolution CT (HRCT) chest. 14/42 (33.3%) patients required a ventilator, with nine deaths. Intravenous immunoglobulin was the mainstay of treatment for GBS. Majority had a good outcome and were walking independently or with minimal support at discharge. In subgroup analysis, the postinfectious group had a better outcome than the parainfectious group. Conclusion: GBS in COVID-19 occurs as both parainfectious and postinfectious GBS. Parainfectious GBS needs more rigorous monitoring and may benefit from COVID-19 specific treatment. Routine screening for SARS-CoV-2 should be implemented in patients with GBS in view of the ongoing pandemic. [ABSTRACT FROM AUTHOR]

To report a case of non-Hodgkin lymphoma (NHL) that was diagnosed 35-month of initial ocular manifestation. Retrospective chart review. A 53-year-old male presented with painless diminution of vision in both eyes. He subsequently underwent extensive laboratory investigations including multiple vitreous biopsies with a suspicion of intraocular lymphoma. Cytology from the vitreous aspirate failed to diagnose any relevant pathology. After 35-month from the onset of his ocular symptom, a brain biopsy revealed a round cell tumor suggestive of NHL. Even with high index of suspicion, consultation with ocular oncologist, imaging, and diagnostic vitrectomy, the diagnosis of lymphoma remains challenging. [ABSTRACT FROM AUTHOR]

Background: Trigeminal neuralgia (TN) is a painful condition, often leading to poor quality of life.Objective: The aim of this review was to discuss the various treatment modalities for the medical management of TN.Materials and Methods: We reviewed the available literature on TN in clinical databases including PubMed, Google Scholar, and the Cochrane Database of Systematic Reviews, with a specific focus on the pharmacological treatment and newer drugs under development for the treatment of TN.Results: Carbamazepine (CBZ) is the gold standard of treatment for TN. The first-line drugs for the treatment of TN are CBZ and oxcarbazepine (OXC). A proportion of cases (30%) are initially resistant to the first-line drugs. Alternative drugs need to be considered if the first-line drugs are not well tolerated or become ineffective with prolonged therapy. The second-line drugs comprise lamotrigine, baclofen, gabapentin, and pregabalin used as monotherapy or in combination with CBZ/OXC. Botulinum toxin A may be a promising presurgical option. Newer drug like vixotrigine has shown good results in phase two randomized control trials. About 50% of cases develop treatment resistance to oral drugs over the subsequent years of therapy and require surgical options.Conclusion: The first-line drugs for the treatment of TN (irrespective of the age group or type) are CBZ and OXC. Combination therapy with second-line or other drugs may become necessary with poor response to CBZ/OXC, or if adverse events occur. Patients should be offered surgical options if there is poor response or tolerance to the medical therapy. [ABSTRACT FROM AUTHOR]

Objectives: To present the clinical profile, diagnostic work-up, and management of children with Disorders of Sexual Development (DSD).Materials and Methods: A retrospective study from a tertiary pediatric endocrine unit of western India. We included 39 patients who presented over a period of 9 years from June 2009 to June 2018. Results: Nineteen patients (48.7%) were diagnosed with 46 XY DSD, 16 (41%) with 46 XX DSD, and 4 (10.3%) with sex chromosomal DSD. Out of 46 XY DSD, androgen insensitivity was observed in 8 (42.1%) patients, 5 alpha-reductase deficiency in 5 (26.3%), gonadal dysgenesis in 3 (15.8%), ovotesticular DSD in 2 (10.5%) and 17 beta-hydroxylase (17γ-HSD3) deficiency in 1 (5.3%). Congenital adrenal hyperplasia was the most common cause in 46 XX DSD observed in 11 (68.75%) out of 16 patients, ovotesticular DSD was seen in 4 (25%) patients and testicular DSD in 1 (6.25%) patient. In sex chromosomal DSD 3 (75%) patients had mixed gonadal dysgenesis and 1 (25%) had ovotesticular DSD out of a total of 4 patients. At presentation gender of rearing was assigned as male in 16 (41%) patients, female in 20 (51.3%) patients, and no gender was assigned in 3 (7.7%). The gender of rearing was changed after diagnosis in 6 (16.7%) children. Conclusion: CAH was the most common etiology of 46 XX DSD whereas androgen insensitivity among 46 XY DSD. Assigning the sex of rearing should not be hurried and should be done only after diagnosis and parental counseling. A multidisciplinary and systematic approach is required for children with DSD. [ABSTRACT FROM AUTHOR]

A case study of a 48‑year‑old lady developed constant stiffness in the right distal leg with painful spasms. Topics include Stiff person syndrome (SPS) is a rare disorder of the central nervous system, characterized by rigidity affecting the lumbar, trunk and limb muscles; and stiff limb syndrome (SLS) is diagnosed based on clinical features, serum anti‑glutamic acid decarboxylase (GAD65) antibody, and electrophysiology testing.

Background: Diabetes is a psychologically challenging medical condition. Diabetes distress (DD) refers to the unique, often hidden emotional burdens and worries that the patient experiences when managing diabetes. Objective: The objective of the study was to find the burden and identify the predictors of DD in adult Type 2 diabetes mellitus (T2DM) patients. Materials and Methods: Two hundred and fifty T2DM patients were recruited for this study from the endocrine outpatient department from February to April 2019. DD was measured using the Diabetic Distress Scale. Results: The prevalence of DD was 19.6%. The risk of DD was 4.25 times more in those aged ≤45 years as compared to those aged >45 years. Patients with hemoglobin A1c (HbA1c) >8% had 8.8 times more DD. Patients on insulin had more DD (5.4 times) as compared to patients who were on oral antidiabetic drugs. Patients with a history of treatment interruption had 11 times more risk of DD as compared to patients who did not. Conclusions: DD was found to be high among patients aged ≤45 years, illiterates, patients on insulin, patients with a history of treatment interruption, and those with HbA1c >8%. Patients with high DD were found to have higher HbA1c levels. [ABSTRACT FROM AUTHOR]

Background and Aims: A wide variety of neurological diseases result in clinical and/or radiological enlargement of nerves, roots and plexuses. With the advancement in techniques and use of magnetic resonance neurography (MRN), aided by electrophysiology, proximal segments of the lower motor neuron (LMN) can be well studied. The relative merits of investigative modalities have not been well defined and comprehensive information on this subject is sparse. Methods: This retrospective study included data from January 2010 to June 2018. Patients having clinical and/or radiological enlargements of lower motor neuron were included. Clinical and laboratory work up, electrophysiology, MRN and biopsy studies were documented and analyzed. Results: 133 patients fulfilled the inclusion criteria. The diagnostic categories were of leprosy (32%), immune neuropathies (27.8%), nerve infiltrations (8.2%), inherited neuropathies (9%), diabetic radiculopathies (9%) and others (12.7%). MRN was essential to diagnosis in 24.8% and supportive in 31.5% patients. Electrophysiology was essential in diagnosis in 70.6%, biopsy in 45.8% and genetic studies in 6.4% patients. Conclusion: The manuscript presents a large cohort of diseases causing enlargement of LMN with clinical and investigative aspects of 7 patients of the most unusual condition of chronic immune sensorimotor polyradiculopathy (CISMP) and details of 7 other patients with chronic mononeuropathies at non-entrapment sites. A table of comparative utility and an algorithm depicting the optimization of investigations has been presented. [ABSTRACT FROM AUTHOR]

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders that cause muscle weakness and also have extramuscular manifestations involving various organ systems; namely the lung, skin, heart, and joints. Previously classified broadly as dermatomyositis (DM) and polymyositis now the spectrum of the disease has evolved into more clinical subtypes. There are now five clinicoserological subtypes recognized worldwide DM, antisynthetase syndrome (AS), overlap myositis (OM), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis. Each of these subtypes has a unique phenotype and specific antibodies associated. With the evolving treatment options from the use of immunosuppressive medications to the use of targeted therapy with biologic agents, and further understanding of the pathogenesis of inflammatory myositis, we may have more effective treatment options. We discuss in this review, various myositis-associated antibodies associated with each clinicoserological subtype of IIM and their role. We also describe the evolving therapies and the evidence for the newer biologic therapies in the treatment of IIMs. [ABSTRACT FROM AUTHOR]

CNS tumours and their treatment commonly lead to endocrinopathies in children. Advances in surgical techniques, chemotherapy and radiotherapy have improved the survival in children with CNS tumours however they have also added to the late effect burden. Since the pituitary and hypothalamus regulate many important bodily functions, loss in their function leads to major derangements in growth and hormonal milieu. This article is a review of clinical presentation, diagnosis and management of endocrinopathies encountered in common sellar and supra-sellar CNS tumours in children. The management is divided into mainly two sections: immediate (peri and post-operative) and long term. [ABSTRACT FROM AUTHOR]

Objective: To study efficacy and safety of use of biosimilar growth hormone (GH) in Indian children with growth disorders. Materials and Methods: We studied 322 children (May 2012-2017) with growth disorders including growth hormone deficiency (GHD), multiple pituitary hormone deficiency (MPHD, idiopathic short stature (ISS), small for gestational age (SGA), and Turner syndrome (TS). Children were treated either with innovator molecule (Norditropin) or biosimilar GH (Headon) with standard dosage protocol for 1 year. Height and weight was measured using standard protocol. Height and BMI for age Z-scores (HAZ, BMIZ), height velocity (HV), and HV Z-score (HVZ) were computed from available data. Results: Mean age of the studied children (n = 322) was 9.6 ± 4.1 years, 32% children had GHD, 39% had ISS, 11% had MPHD, 12% had SGA, and 6% children had TS. There were no serious adverse events; three patients recorded eight instances of headaches, two had rash at injection site, and one each had hives and facial edema. Reactions were mild and were treated symptomatically. At the end of the 1 year of GH therapy, change in HAZ was similar in children from both the innovator and biosimilar GH groups. Similarly, the HV and HVZ were also similar in children from both groups and all the studied growth disorders. Conclusion: Biosimilar GH was effective and safe for treatment in children with growth disorders where GH use is indicated. However, in the view of scarcity of such data a longitudinal study with large sample size is warranted. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the duration of casual sunlight ultraviolet-B (UVB) exposure required to maintain optimal Vitamin D status (25-hydroxyvitamin-D [25(OH)D]) >50 nmol/L in urban Indian men, using polysulfone (PSU) dosimeters and a sunlight exposure questionnaire. Methods: In healthy men (aged 40-60 years) from Pune (18.52° N, 73.86° E), India, serum 25(OH)D was measured using enzyme-linked immunosorbent assay. Sunlight exposure was assessed using PSU dosimeter and by questionnaire. Results: Of 160 men (48.3 ± 5.6 years), 26.8% were deficient and 40.6% had insufficient Vitamin D concentrations. A hyperbolic function for the relationship between PSU measured sunlight exposure in standard erythema dose (SED) and serum 25(OH)D concentrations (r = 0.87, P < 0.01) revealed that daily exposure of 1 SED was sufficient to maintain serum 25(OH)D concentrations over 50 nmol/L. The curve plateaued around 5 SED (80 nmol/L) and extrapolation of the curve (>5 SED) did not increase 25(OH)D concentrations above 90 nmol/L. Receiver operating curve analysis confirmed that 1 SED-UV exposure was sufficient to maintain 25(OH)D concentrations over 50 nmol/L. Based on the questionnaire data, >1 h of midday casual sunlight exposure was required to maintain serum 25(OH)D concentrations above 50 nmol/L. Duration of sunlight exposure assessed by questionnaire and PSU dosimeter showed a significant correlation (r = 0.517, P < 0.01). Conclusion: In urban Indian men, >1 h of casual midday sunlight exposure daily was required to maintain serum 25(OH)D concentrations above 50 nmol/L, and >2 h of casual sunlight exposure was needed to maintain 25(OH)D concentrations above 75 nmol/L. Excess sunlight did not increase 25(OH)D linearly. The sunlight exposure questionnaire was validated for use in clinical studies and surveys. [ABSTRACT FROM AUTHOR]

Introduction: Due to the high prevalence of Vitamin D deficiency in spite of abundant sunshine and scarcity of studies investigating Vitamin D status in Indian children from rural and semirural areas, the objectives of this cross-sectional study were to: (1) assess the Vitamin D status of school-children in a semi-rural setting and (2) identify the determinants of Vitamin D status in these children. Materials and Methods: Data collected included anthropometric measurements (height and weight), body composition, three-one-day dietary recall method, demographic data, and sunlight exposure. Serum 25-hydroxyVitamin D (25(OH)D) was estimated by enzymelinked immunosorbent assay (ELISA) technique. SPSS software was used for statistical analysis. Results: Anthropometric characteristics of the children were similar and mean serum 25(OH)D concentration was 58.5 ± 10.3 nmol/L with no significant differences between genders. Around 80% children reported sunlight exposure of 2 h or more. A majority (71%) of children were Vitamin D insufficient with serum 25(OH)D concentrations between 50 and 74.9 nmol/L. Determinants of Vitamin D identified were duration of sunlight exposure and body fat percent. Significant (P < 0.05) positive association of duration of sunlight exposure was observed with serum 25(OH) D concentrations, while BF% showed a negative association with serum 25(OH)D (ß = -0.307; standard error = 0.1388; P < 0.05). Discussion: We have reported a high prevalence of Vitamin D insufficiency in school-children aged 6-12 years, from a semirural setting, in spite of a majority (80%) reporting >2 h of sunlight exposure. We have also demonstrated that duration of sunlight exposure and body fat percentage are the two important determinants of serum 25(O H)D concentrations in these children. [ABSTRACT FROM AUTHOR]

Purpose: The aim of study was to assess the prevalence of osteoporosis and changes in bone mass with increasing age and compare bone health status of apparently healthy men, premenopausal and postmenopausal women. Methods: Data were collected on anthropometric and sociodemographic factors in 421 apparently healthy Indian adults (women = 228), 40-75 years of age, in a cross-sectional study in Pune city, India. Bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry at two sites-lumbar spine (LS) and left femur. Individuals were classified as having osteoporosis or osteopenia based on the World Health Organization criteria of T-scores. Results: Mean age of study population was 53.3 ± 8.4 years. Of the total women, 44.3% were postmenopausal with 49.2 ± 3.5 years as mean age at menopause. Postmenopausal women showed a rapid decline in BMD with age till 50 years while men showed a gradual decline. Premenopausal women showed no significant decline in BMD with age (P > 0.1). Significantly lower T-scores were observed at LS in men compared to premenopausal (P < 0.05). At left femur, T-scores were lower in men compared to premenopausal women (P < 0.05) but not postmenopausal women (P > 0.1). The prevalence of osteoporosis in men at LS was lower than postmenopausal women but higher than premenopausal women. Conclusion: In Indian men, a low T-score compared to women indicates higher susceptibility to osteoporosis. In women, menopause causes a rapid decline in BMD. Therefore, both Indian men and postmenopausal women require adequate measures to prevent osteoporosis during later years in life. [ABSTRACT FROM AUTHOR]

Introduction: Maternal body composition (BC) changes during lactation. Increased prepregnancy obesity is associated with poor obstetric outcomes. The aim was to study changes in maternal BC postpartum (PP) to 1-year PP with reference to their prepregnancy body mass index (BMI) status. Methods: The study design was a 1-year follow-up study. Sixty-five apparently healthy primiparous women (28.6 ± 3.4 years delivered full-term infants) were randomly selected from December 2010 to June 2013 and postclassified on the basis of their prepregnancy BMI status. Anthropometry, sociodemographic status, physical activity, diet, clinical examination, biochemical tests, and BC at total body (dual energy X-ray absorptiometry, GE, Lunar DPX) were collected using standardized protocols. Results: Forty-one women were classified in Group A with normal prepregnancy BMI (20.4 ± 2.0 kg/m²) and 24 women in Group B with overweight/obese (OW/OB) prepregnancy BMI (26.1 ± 1.9 kg/m2). At 1 year, 75% of women returned to normal BMI in Group A, whereas all 100% of women from Group B remained in OW category at 1-year PP. Nearly 43% of Group B women showed the presence of at least two metabolic syndrome risk factors as compared to 36% in Group A at 1 year. Conclusion: Women with OW/OB prepregnancy BMI accumulated higher visceral fat with a higher prevalence of metabolic risk factors at 1-year PP. Our study underlines the importance of maintaining BMI status in reference range in reproductive years. [ABSTRACT FROM AUTHOR]