Natural history of Wolcott-Rallison syndrome: A systematic review and follow-up study.

BACKGROUND AND AIMS: To systematically review the literature for reports on Wolcott-Rallison syndrome, focusing on the spectrum and natural history, genotype-phenotype correlations, patient and native liver survival, and long-term outcomes. METHODS: PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow-up were extracted. Survival and correlation analyses were performed. RESULTS: Sixty-two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5 months (IQR 2) and of death was 36 months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver-pancreas in one and combined liver-pancreas-kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (p = .0057). One-, five- and ten-year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (p = .013). CONCLUSION: Wolcott-Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver- or multi-organ transplantation is a feasible treatment option to improve survival.

Effect of Calcium and Vitamin D Supplementation (Dairy vs. Pharmacological) on Bone Health of Underprivileged Indian Children and Youth with Type-1 Diabetes: A Randomized Controlled Trial.

BACKGROUND: Bone health is affected by chronic childhood disorders including type-1 diabetes mellitus (T1DM). We conducted this randomized controlled trial with the objective of investigating the effect of 1-year supplementation of vitamin-D with milk or with pharmacological calcium on bone mass accrual in underprivileged Indian children and youth with T1DM. METHODS: 5 to 23year old (n = 203) underprivileged children and youth with T1DM were allocated to one of three groups: Milk (group A-received 200 ml milk + 1000 international unit (IU) vitamin-D3/day), Calcium supplement (group B-received 500 mg of calcium carbonate + 1000 IU of vitamin-D3/day) or standard of care/control (group C). Anthropometry, clinical details, biochemistry, diet (3-day 24-h recall), physical activity (questionnaires adapted for Indian children) and bone health parameters (using dual-energy X-ray absorptiometry and peripheral quantitative computed tomography- DXA and pQCT respectively) were evaluated at enrolment and end of 12 month intervention. RESULTS: Total body less head(TBLH) bone mineral content (BMC(g)) and bone mineral density (BMD(gm/cm2)) were significantly higher at end of study in girls in both supplemented groups (TBLHBMC-A-1011.8 ±â€¯307.8, B-983.2 ±â€¯352.9, C-792.8 ±â€¯346.8. TBLHBMD-A-± 0.2, B-0.8 ±â€¯0.2, C-0.6 ±â€¯0.2, p < 0.05). Z score of lumbar spine bone mineral apparent density of supplemented participants of both sexes was significantly higher than controls (Boys- A-0.7 ±â€¯1.1, B-0.6 ±â€¯1.4, C- -0.7 ±â€¯1.1; Girls- A-1.1 ±â€¯1.1, B-0.9 ±â€¯3.4, C- -1.7 ±â€¯1.3, p < 0.05). A significantly higher percentage increase was found in cortical thickness in girls in both supplemented groups (A-17.9 ±â€¯28.6, B-15.3 ±â€¯16.5, C-7.6 ±â€¯26.2); the differences remained after adjusting for confounders. CONCLUSION: Supplementation with milk or pharmacological calcium (+vitaminD3) improved bone outcomes-particularly geometry in children with T1DM with more pronounced effect in girls. Pharmacological calcium may be more cost effective in optimising bone health in T1DM in resource limited settings.

BoneXpert-derived bone health index reference curves constructed on healthy Indian children and adolescents.

BACKGROUND: Artificial intelligence (AI)-based applications for the assessment of the paediatric musculoskeletal system like BoneXpert are not only useful to assess bone age (BA) but also to provide a bone health index (BHI) and a standard deviation score (SDS) for both. This allows comparison of the BHI with age- and sex-matched healthy Caucasian children. OBJECTIVE: We conducted this study with the objective of generating BHI curves using BoneXpert in healthy Indian children with BA between 2 and 17 years. METHOD: We retrospectively reviewed anthropometric parameters, BHI, and BHI SDS data of digitalized left-hand radiographs (joint photographic experts group [jpg] format) of a cohort of 788 paediatric patients from a previous study to which they were recruited to compare various methods of BA assessment. The recruited children represented all age groups for both sexes. The corrected BHI for jpg images was calculated using the formula corrected BHI=BHI*(stature/(avL*50))^0.33333 where stature is height of subject and avL is average length of metacarpal bones. The reference Indian BHI curves and centiles were generated using the Lambda-Mu-Sigma method. RESULT: The mean BHI and BHI SDS of the study group were 4.02±0.57 and -1.73±1.09, respectively. The average increase in median BHI from each age group was between 2.5% and 3% in both sexes up to age of 14 years after which it increased to 4.5% to 5%. The mean BHI of Indian children was lower than that of Caucasian children with maximum differences noted in boys at 16 years (21.7%) and girls at 14 years (16%). We report 8.4% SD of BHI for our study sample. Reference percentile curves for BHI according to BA were derived separately for boys and girls. CONCLUSION: Reference data has been provided for the screening of bone health status of Indian children and adolescents.

Catch-Up Growth in Infants and Young Children With Faltering Growth: Expert Opinion to Guide General Clinicians.

Faltering growth (FG) is a problem regularly seen by clinicians in infants and young children (<2 years of age). It can occur due to non-disease-related and disease-related causes and is associated with a wide range of adverse outcomes, including shorter-term effects such as impaired immune responses and increased length of hospital stay, and longer-term consequences, including an impact on schooling and cognitive achievements, short stature, and socioeconomic outcomes. It is essential to detect FG, address underlying causes and support catch-up growth where this is indicated. However, anecdotal reports suggest misplaced fear of promoting accelerated (too rapid) growth may deter some clinicians from adequately addressing FG. An invited international group of experts in pediatric nutrition and growth reviewed the available evidence and guidelines on FG resulting from disease-related and non-disease-related effects on nutritional status in healthy term and small for gestational age infants and children up to the age of 2 years in low-, middle-, and high-income countries. Using a modified Delphi process, we developed practical consensus recommendations to provide clarity and practical recommendations for general clinicians on how FG should be defined in different young child populations at risk, how FG should be assessed and managed, and the role of catch-up growth after a period of FG. We also suggested areas where further research is needed to answer remaining questions on this important issue.

Pre-pregnancy weight, the rate of gestational weight gain, and the risk of early gestational diabetes mellitus among women registered in a tertiary care hospital in India.

BACKGROUND: The impact of pre-pregnancy weight and the rate of gestational weight gain (GWG) together on the risk of early GDM (< 24 weeks gestation; eGDM) has not been studied in the Indian context. We aimed to study the influence of (1) pre-pregnancy weight on the risk of eGDM diagnosed in two time intervals; and (2) in addition, the rate of GWG by 12 weeks on the risk of eGDM diagnosed in 19-24 weeks. METHOD: Our study utilized real-world clinical data on pregnant women routinely collected at an antenatal care clinic at a private tertiary hospital, in Pune, India. Women registering before 12 weeks of gestation (v1), with a singleton pregnancy, and having a follow-up visit between 19-24 weeks (v2) were included (n = 600). The oral glucose tolerance test was conducted universally as per Indian guidelines (DIPSI) at v1 and v2 for diagnosing eGDM. The data on the onset time of eGDM were interval censored; hence, we modeled the risk of eGDM using binomial regression to assess the influence of pre-pregnancy weight on the risk of eGDM in the two intervals. The rate of GWG by 12 weeks was added to assess its impact on the risk of eGDM diagnosed in v2. RESULT: Overall, 89 (14.8%) women (age 32 ± 4 years) were diagnosed with eGDM by 24 weeks, of which 59 (9.8%) were diagnosed before 12 weeks and 30 of 541 (5.5%) women were diagnosed between 19-24 weeks. Two-thirds (66%) of eGDM were diagnosed before 12 weeks of gestation. Women's pre-pregnancy weight was positively associated with the risk of GDM in both time intervals though the lower confidence limit was below zero in v1. The rate of GWG by 12 weeks was not observed to be associated with the risk of eGDM diagnosed between 19-24 weeks of gestation. These associations were independent of age, height, and parity. CONCLUSION: Health workers may focus on pre-pregnancy weight, a modifiable risk factor for eGDM. A larger community-based study measuring weight and GDM status more frequently may be warranted to deepen the understanding of the role of GWG as a risk factor for GDM.

Influence of ecoregional and lifestyle factors on growth and body composition of Indian children and adolescents aged 9-18 years-A multicenter study.

OBJECTIVES: Ecoregion comprises all the environmental factors such as climate, vegetation, geomorphology, and soil taken together. The objectives of this study were to (1) assess differences in growth and body composition (BC) in 9-18-year-old children and adolescents (CA), across six ecoregions of India, (2) evaluate and compare the magnitude of the effect of ecoregional and lifestyle factors (LSF) on growth and BC in urban (U) and rural (R) children and adolescents, and (3) reassess the applicability of Bergmann's Rule and Allen's Rule to humans. SUBJECTS AND METHODS: Data on 1845 CA (925 boys; 920 girls) were collected (2016-2017) from urban and rural areas of six ecoregions of India. Anthropometric and BC parameters were measured; Z-scores were computed. Lifestyle Index score (LIS) based on LSF-(socioeconomic status [SES], diet, physical activity) was computed (categorized as low, medium, high-indicates better LSF). Univariate analysis was performed to estimate effect size. RESULTS: Significant differences were observed in growth and BC across urban and rural ecoregions. CA with high LIS were taller and heavier than those with low LIS (p < .05). Ecoregion had greater effect on growth (Partial eta square (η2 ) for R = 0.136 vs. U = 0.057) and BC (η2 for R = 0.094 vs. U = 0.058) of rural CA than urban (p < .01). LSF had more effect on the BC of urban CA (η2  = 0.017) than rural (η2  = 0.002, p < .01). CONCLUSION: Disparities among the six ecoregions were not large enough to fully support Bergmann's Rule and Allen's Rule. Ecoregion had greater effect on growth and BC of rural CA than urban, while lifestyle factors had more effect on the BC of urban CA.

The 2022 India Report Card on physical activity for children and adolescents.

Background: With strong evidence of physical inactivity's link to chronic disease and economic burden - particularly with childhood active living behaviors tracking into adulthood - it is imperative to promote physical activity among children and adolescents in India. Objectives: To evaluate active living patterns among Indian children and adolescents. Methods: The India Report Card (IRC) team, which consists of experts in India and Canada, systematically collected and appraised evidence on 11 indicators of active living, including 5 behavioral (Overall Physical Activity, Organized Sport Participation, Active Play, Active Transportation, Sedentary Behavior), 2 individual-level (Physical Fitness, Yoga) and 4 sources of influence (Family and Peers, School, Community and Built Environment, Government). Peer-reviewed articles were appraised based on national representativeness, sample size, and data quality. Grey literature was appraised based on comprehensiveness, validity of the sources, and representativeness. All indicators were assessed against parameters provided by the Active Healthy Kids Global Alliance. Results: Active Transportation and Government Strategies were ranked highest with a B- and C+ grade, respectively. Overall Physical Activity and Schools were assigned a C grade, while Sedentary Behavior and Community and Built Environment were given D grades. Yoga was the lowest ranking indicator with a D- grade. Organized Sport Participation, Active Play, Family and Peers, and Physical Fitness were all graded incomplete. Conclusions: Active Transportation, Government Strategies, and Overall Physical Activity have improved since the 2018 IRC, a positive trend that needs to be translated to other indicators. However, Sedentary Behavior has consistently worsened, with grades C, C-, and D-, in 2016, 2018, and 2022, respectively. Evidence generated by the 2022 IRC suggests opportunities for improvement not only in India, but also the 56 other countries taking part in Global Matrix 4.0.

Impact of Adolescent Pregnancy on Bone Density in Underprivileged Pre-Menopausal Indian Women.

High prevalence (31.5%) of adolescent pregnancies (AP) have been reported in India. Reports suggest that pregnancy during adolescence may have deleterious effects on peak bone mass. Very few studies have described the long-term effects of a history of AP on bone mass. The objective of this study was to compare bone mineral density (BMD) and bone geometry of premenopausal women with first childbirth during adolescence (i.e., before age of 19 years) or after 20 years. A cross-sectional study was conducted in 242 women (age 28.0-54.5 years) from Pune, India (November, 2015 to November, 2017). Women were divided into 2-groups: Group-1: women-who had 1st-pregnancy and childbirth before 19 years of age (AP n = 131) and Group-2: women-who had 1st pregnancy after 20 years of age (non-AP n = 111). Demographic data, anthropometric measurements, and biochemical tests were performed using standard protocols. Physical activity and nutrient intakes were recorded using validated questionnaires. Areal BMD and bone geometry were measured using Dual-Energy-Absorptiometry-DXA (Lunar-iDXA, GE Healthcare) and peripheral-quantitative-computed-tomography-pQCT (XCT2000, Stratec Inc.). Mean age of the study group was 37 ± 4.6 years; in women from group-1 mean age at first delivery was 16.9 ± 1.6 years as against 22.6 ± 3.1 years in group-2. Both groups were similar in body mass index and socioeconomic status. pQCT measured radial diaphyseal cortical thickness (1.97 ± 0.3 mm vs 1.88 ± 0.3 mm resp., p = 0.016, periosteal circumference (38.0 ± 3.6 mm vs 36.7 ± 2.5 mm, resp. p = 0.016), total bone area (114.3 ± 24.8 mm2 vs 108.7 ± 14.7 mm2 resp. p = 0.026) and stress-strain index (SSI = 217 ± 75 vs 201 ± 40 mm3 resp. p = 0.042) were significantly higher in group-1 than group-2. After adjusting for anthropometric and lifestyle parameters, pQCT measured cortical thickness (1.98 ± 0.03 mm in group-1, 1.87 ± 0.03 mm group-2, p = 0.01, mean ± SE) and iDXA derived aBMD at forearm were still significantly higher (0.599 ± 0.006 g/cm3 vs 0.580 ± 0.006 g/cm3, p = 0.023) in Group-1. Our data suggest that women with a history of adolescent pregnancy had better bone geometry and higher aBMD at radius in later years. We speculate that early exposure to pregnancy resulted in higher aBMD at the radius and induced changes at radial diaphysis with bones becoming thicker and wider among these women.

Determinants of muscle power and force as assessed by Jumping Mechanography in rural Indian children.

OBJECTIVES: To: 1. Assess muscle function (MF) of rural Indian children (6-11y, n=232), using Jumping Mechanography (JM) and hand dynamometer, 2. Investigate gender differences, 3. Identify determinants of MF. METHODS: Data on anthropometry, muscle mass%, diet, physical activity, sunlight exposure, MF (maximum relative power Pmax/mass, maximum relative force Fmax/BW by JM; relative grip strength (RGS) by hand dynamometer) were collected. Pearson's correlation and hierarchical linear regression was performed. RESULTS: Pmax/mass, Fmax/BW and RGS of the group were 31.7±5.0W/kg, 3.0±0.3 and 0.4±0.1 (mean±SD), respectively. The Pmax/mass Z-score was -1.1±0.9 and Fmax/BW Z-score was -0.9±1 (mean±SD) which was significantly lower than the machine reference data (p<0.05). Positive association of muscle mass% and protein intake was observed with all MF parameters and moderate+vigorous physical activity with Fmax/BW (p<0.05). Determinants of MF identified through regression for Pmax/mass were age (ß=1.83,95% CI=0.973 - 2.686), muscle mass% (ß=0.244,95% CI=0.131-0.358) and protein intake (ß=3.211,95% CI=1.597-4.825) and for Fmax/BW was protein intake (ß=0.130,95% CI=0.023-0.237) (p<0.05). Male gender was a positive predictor of having higher Pmax/mass (ß=1.707,95% CI=0.040-3.373) (p<0.05). CONCLUSION: MF was lower than in western counterparts. To optimize MF of rural Indian children, focus should be on improving muscle mass, ensuring adequate dietary protein, and increasing physical activity, especially in girls.

A comparison of bone age assessments using automated and manual methods in children of Indian ethnicity.

BACKGROUND: Bone age is useful for pediatric endocrinologists in evaluating various disorders related to growth and puberty. Traditional methods of bone age assessment, namely Greulich and Pyle (GP) and Tanner-Whitehouse (TW), have intra- and interobserver variations. Use of computer-automated methods like BoneXpert might overcome these subjective variations. OBJECTIVE: The aim of our study was to assess the validity of BoneXpert in comparison to manual GP and TW methods for assessing bone age in children of Asian Indian ethnicity. MATERIALS AND METHODS: We extracted from a previous study the deidentified left hand radiographs of 920 healthy children aged 2-19 years. We compared bone age as determined by four well-trained manual raters using GP and TW methods with the BoneXpert ratings. We computed accuracy using root mean square error (RMSE) to assess how close the bone age estimated by BoneXpert was to the reference rating. RESULTS: The standard deviations (SDs) of rating among the four manual raters were 0.52 years, 0.52 years and 0.47 years for GP, TW2 and TW3 methods, respectively. The RMSEs between the automated bone age estimates and the true ratings were 0.39 years, 0.41 years and 0.36 years, respectively, for the same methods. The RMSE values were significantly lower in girls than in boys (0.53, 0.5 and 0.47 vs. 0.39, 0.47 and 0.4) by all the methods; however, no such difference was noted in classification by body mass index. The best agreement between BoneXpert and manual rating was obtained by using 50% weight on carpals (GP50). The carpal bone age was retarded in Indian children, more so in boys. CONCLUSION: BoneXpert was accurate and performed well in estimating bone age by both GP and TW methods in healthy Asian Indian children; the error was larger in boys. The GP50 establishes "backward compatibility" with manual rating.

Assessment of height growth in Indian children using growth centiles and growth curves.

BACKGROUND: Growth centiles and growth curves are two ways to present child anthropometry; however, they differ in the type of data used, the method of analysis, the biological parameters fitted and the form of interpretation. AIM: To fit and compare height growth centiles and curves in Indian children. SUBJECTS AND METHODS: 1468 children (796 boys) from Pune India aged 6-18 years with longitudinal data on age and height (n = 7781) were analysed using GAMLSS (Generalised Additive Models for Location Scale and Shape) for growth centiles, and SITAR (SuperImposition by Rotation and Translation) for growth curves. RESULTS: SITAR explained 98.7% and 98.8% of the height variance in boys and girls, with mean age at peak height velocity 13.1 and 11.0 years, and mean peak velocity 9.0 and 8.0 cm/year, respectively. GAMLSS (Box-Cox Cole Green model) also captured the pubertal growth spurt but the centiles were shallower than the SITAR mean curve. Boys showed a mid-growth spurt at age 8 years. CONCLUSION: GAMLSS displays the distribution of height in the population by age and sex, while SITAR effectively and parsimoniously summarises the pattern of height growth in individual children. The two approaches provide distinct, useful information about child growth.

Prevalence of Sarcopenia and Relationships Between Muscle and Bone in Indian Men and Women.

Both ethnicity and age are important determinants of musculoskeletal health. We aimed to determine the prevalence of sarcopenia, assess the suitability of current diagnostic guidelines, and explore muscle-bone relationships in adults from India. A total of 1009 young (20-35 years) and 1755 older (> 40 years) men and women from existing studies were collated and pooled for the analysis. Dual-energy x-ray absorptiometry measured areal bone mineral density (aBMD) at the hip and spine, and fat and lean mass; hand dynamometer measured hand grip strength (HGS). Indian-specific cut-points for appendicular lean mass (ALM), ALM index (ALMI) and HGS were calculated from young Indian (-2SD mean) populations. Sarcopenia was defined using cut-points from The Foundations for the National Institutes of Health (FNIH), revised European Working Group on Sarcopenia in Older People (EWGSOP2), Asian Working Group for Sarcopenia (AWGS), and Indian-specific cut-points. Low lean mass cut-points were then compared for their predictive ability in identifying low HGS. The relationship between muscle variables (ALM, ALMI, HGS) and aBMD was explored, and sex differences were tested. Indian-specific cut-points (men-HGS:22.93 kg, ALM:15.41 kg, ALMI:6.03 kg/m2; women-HGS:10.76 kg, ALM:9.95 kg, ALMI:4.64 kg/m2) were lower than existing definitions. The Indian-specific definition had the lowest, while EWGSOP2 ALMI had the highest predictive ability in detecting low HGS (men:AUC = 0.686, women:AUC = 0.641). There were sex differences in associations between aBMD and all muscle variables, with greater positive associations in women than in men. The use of appropriate cut-points for diagnosing low lean mass and physical function is necessary in ethnic populations for accurate sarcopenia assessment. Muscle-bone relationships are more tightly coupled during ageing in Indian women than men.

Trabecular Bone Score has Poor Association With pQCT Derived Trabecular Bone Density in Indian Children With Type 1 Diabetes and Healthy Controls.

BACKGROUND: In children with type 1 diabetes mellitus (T1DM), low trabecular volumetric bone mineral density (Trab vBMD) has been reported. However, studies using the trabecular bone score (TBS) are scarce. The objective of our study was to assess areal bone mineral density at the lumbar spine (LS aBMD), the TBS and Trab vBMD in children with type 1 diabetes in comparison with healthy controls and to assess the relationship of Trab vBMD with TBS. METHODS: A total of 205 children were assessed for their LS bone mineral content (BMC) and LS aBMD by dual energy x-ray absorptiometry (DXA) and Trab vBMD at distal radius by peripheral quantitative computed tomography (pQCT). Machine generated Z-scores for both LS aBMD and Trab vBMD were used. The retrospective DXA LS scans in children with T1DM (n=137, age 13.1 ± 3.2 years) and controls (n = 68, age 13.0 ± 2.7 years) were analysed with a research trial version of TBS iNsight software (Medimaps Group). The established TBS cut-offs were used to categorize TBS. RESULTS: The mean LS BMC, LS aBMD, TBS and Trab vBMDs were lower in children with T1DM. TBS was positively correlated with LS aBMD but not with Trab vBMD in both groups. Distribution of T1DM and control children was similar in the TBS categories. Over a fourth of the T1DM children with low Trab vBMD (below -2 Z score) had normal TBS, while, in children with LS aBMD Z-score > -2 from both groups, >50% had degraded or partially degraded TBS. Degraded TBS was seen in half the control children although none of them had low Trab vBMD. CONCLUSION: We found poor correlation between TBS and Trab vBMD in paediatric diabetic and healthy population. Our results also suggest establishing paediatric TBS cut offs in improving the classification of children having degraded trabecular bone.

Assessment of Bone Density by DXA in Poorly Controlled Children With ß-Thalassemia: Correction for Hepatic Iron Overload by Manual Analysis.

INTRODUCTION: Beta thalassemia major (BTM) is characterized by anemia and iron overload, especially with inadequate chelation therapy. Dual energy x-ray absorptiometry software (DXA) may misanalyse bone measurements due to iron deposition in organs such as the liver. Our objective was to study difference between the posterior-anterior spine measurements of bone mineral content (BMC), area (BA) and density (BMD) in poorly chelated beta thalassemia patients with and without inclusion of the liver in the DXA analysis. METHODS: We studied hemoglobin and serum ferritin concentrations in 208 patients with BTM (children n = 177, young adults n = 31). Posteroanterior spine measurements BMC, BA and areal BMD were performed using a GE iDXA. Using the tissue point typing feature (EnCore software, version 16), analysis was carried out including and excluding (manually) the iron overloaded liver. Machine generated Z-scores of L1-L4 BMD were used for analysis. RESULTS: The mean age of the study group was 12.9 ± 5.4 yr. Mean hemoglobin and serum ferritin concentrations were 8.0 ± 1.7 g/dl and 2256.9 ± 1978.0 ng/ml, respectively. The mean BMC, BA, and aBMD at the lumbar spine were 23.2 ± 11.4 g, 29.9 ± 8.5 cm2 and 0.736 ± 0.173 g/cm2 respectively with inclusion of liver that is standard machine analysis. After the liver was excluded from the analysis, the mean BMC, BA, and aBMD were 23.9 ± 11.6 g, 30.0 ± 8.6 cm2 and 0.757 ±0.173 g/cm2, respectively and the BMC and aBMD were significantly greater (p < 0.05). Mean BMD Z-score was -1.5 ± 1.2, which significantly (p < 0.05) improved to -1.3 ± 1.2 after exclusion of the liver from the analysis. CONCLUSION: In poorly chelated patients with thalassemia, inclusion of the iron-overloaded liver in the tissue analysis may exaggerate the deficit in bone parameters. Iron overloaded tissues need to be manually excluded during analysis of the PA spine.

Paradoxical Response of Parathyroid Hormone to Vitamin D-Calcium Supplementation in Indian Children.

OBJECTIVES: To investigate the effect of oral vitamin D-calcium supplementation on serum intact parathyroid hormone (PTH), calcium, phosphorous, and alkaline phosphatase (ALK-P) concentrations in children with habitually low calcium intakes. STUDY DESIGN: In this follow-up study to a randomized controlled trial that aimed to assess the effect of vitamin D-calcium supplementation on immunity, data related to dietary intake, anthropometry, and biochemistry [serum 25(OH)D and bone profile] were collected from 178 children-79 in the vitamin D group and 99 in the non-vitamin D group. RESULTS: Dietary calcium to phosphorus intake ratio was 0.4:1. Baseline serum 25(OH)D concentration was 58.2 ± 10.9 nmol/L; 66% children were vitamin D sufficient and none deficient. After supplementation, vitamin D group, compared with the non-vitamin D group, had significantly (P < .05) greater 25(OH)D (83.9 ± 30.1 nmol/L vs 58.3 ± 15.7 nmol/L), significantly greater PTH (6.7 ± 3.6 pmol/L vs 5.5 ± 3.2 pmol/L), and positive correlation (rs = 0.24) between serum 25(OH)D and PTH (vs negative correlation [rs = -0.1] in non-vitamin D group). Mean concentrations of serum bone measures in the vitamin D group were calcium (2.2 ± 0.1 mmol/L), phosphorus (1.7 ± 0.2 mmol/L), and ALK-P (178.7 ± 40.7 IU/L). At follow-up, 1-year post-supplementation, in the vitamin D group, PTH concentrations continued to remain high (but not significantly different from levels at 6 months), with low normal serum calcium, high normal phosphate, and ALK-P in reference range. CONCLUSIONS: In children who are vitamin D sufficient but with habitually low dietary calcium intake, vitamin D-calcium supplementation paradoxically and significantly increased serum PTH concentrations with no apparent effect on other bone biochemistry. Chronic low dietary calcium to phosphorus ratio is likely to have caused this paradoxical response.

Prevalence of dyslipidemia in Indian children with poorly controlled type 1 diabetes mellitus.

BACKGROUND: Children with type 1 diabetes having dyslipidemia are at increased risk of developing premature atherosclerosis and cardiovascular disease. The present study aims to determine the prevalence of dyslipidemia and its predictors in poorly controlled Indian children with type 1 diabetes. METHODS: The cross-sectional study included 235 children and youth (3-18 years) with type 1 diabetes having disease duration of at least 1 year. Demographic data and laboratory findings were obtained from patients' records. RESULTS: The prevalence of dyslipidemia in our study was 47.2% with abnormal low-density lipoprotein cholesterol being the most common lipid abnormality. Poor glycemic control and higher thyroid stimulating hormone values were important predictors of likelihood of dyslipidemia and hypertriglyceridemia. Despite a low percentage of overweight and obese children in our study, body fat percentage was a significant predictor of likelihood of high total cholesterol and abnormal high-density lipoprootein. Interestingly, 28 children under the age of 10 years were found to have dyslipidemia, which constitutes 11.9% of the total study group. CONCLUSIONS: We found a high prevalence of dyslipidemia in children with type 1 diabetes including children under age of 10 years, which emphasize the need for early screening and regular monitoring of lipid profile in these children.

A Cross-Calibration Study of GE Lunar iDXA and GE Lunar DPX Pro for Body Composition Measurements in Children and Adults.

OBJECTIVE: To cross-calibrate dual energy X-ray absorptiometry machines when replacing GE Lunar DPX-Pro with GE Lunar iDXA. METHODS: A cross-sectional study was conducted in 126 children (3-19 years) and 135 adults (20-66 years). Phantom cross calibration was carried out using aluminum phantom provided with each of the machines on both machines. Total body less head (TBLH), lumbar spine (L2-L4) and left femoral neck bone mineral density (BMD), bone mineral content (BMC), and bone area were assessed for each patient on both machines. TBLH lean and fat mass were also measured. Bland-Altman analysis, linear regressions, and independent sample t test were performed to evaluate consistency of measurements and to establish cross-calibration equations. RESULTS: iDXA measured 0.33% lower BMD and 0.64% lower BMC with iDXA phantom as compared to DPX-Pro phantom (p < 0.001). In children, TBLH-BMC, femoral BMC and area were measured 10%-14% lesser, TBLH area was higher by 1%-2% and L2-L4 area by 10%-14% by iDXA as compared to DPX-Pro. iDXA measured higher TBLH fat [15% (girls), 31% (boys)] than DPX-Pro. In adults, TBLH-BMD (1.7%-3.4%), BMC (6.0%-10.9%) and area (4.2%-7.6%) were measured lesser by iDXA than DPX-Pro. L2-L4 BMD was higher [2.7% (men), 1.8% (women)] by iDXA than DPX-Pro. Femoral BMC was 2.11% higher in men and 4.1% lower in women by iDXA as compared to DPX-Pro. In children, R2 of cross-calibration equations, ranged from 0.91 to 0.96; in adults, it ranged from 0.93 to 0.99 (p < 0.01). After the regression equations were applied, differences in BMD values between both machines were negligible. CONCLUSION: A strong agreement for bone mass and body composition was established between both machines. Cross-calibration equations need to be applied to transform DPX-Pro measurements into iDXA measurements to avoid errors in assessment. This study documents a need for use of cross-calibration equations to transform DPX-Pro body composition data into iDXA values for clinical diagnosis.

Inter-regional differences in body proportions in Indian children and adolescents-a cross-sectional multicentric study.

Background: Sitting height (SH) and leg length (LL) help in assessing disproportionate growth. Anthropometric dissimilarity has been observed in different ethnicities.Aim: To (1) study sitting height and body proportions in children from different regions of India; and (2) compare sitting height and body proportions with data from other countries.Subjects and methods: This was a cross-sectional multicentric observational study, where 7961 (4328 boys) 3-18 year old children from five regions (north, south, east, west and central) were measured (height, weight and SH).Results: Boys from north India and girls from central India were taller and heavier (mean height 153.2 ± 18, 146.4 ± 11), while western boys and girls were the shortest (131.1 ± 20.7, 129.8 ± 19.5) (p < 0.05 for all). The highest SH was observed in the north (79.2 ± 8.5) and the lowest in the west (68.8 ± 9.1). Mean SH:LL ratio was highest in children from the northeast (1.13) followed by those from western, northern and central India (1.12, 1.10 and 1.07, respectively) and the ratio was the least in children from south India (1.05) (p < 0.0.5 for all except northeast and west). Children from the north and west were similar to the Dutch, children from the south were similar to South (black) Africans and the north-eastern children were similar to Chinese children.Conclusion: There were inter-regional differences in body proportions; similarities in body proportions with children from other ethnicities may throw light on the migration history of Indian people.

Low knowledge of osteoporosis and its risk factors in urban Indian adults from Pune city, India.

OBJECTIVE: To assess knowledge of osteoporosis and its risk factors and to explore associations between knowledge and various sociodemographic factors in Indian adults. DESIGN: Cross-sectional study. The Revised Osteoporosis Knowledge Test (OKT) was used to assess knowledge of osteoporosis. Four scores (OKT-total, range 0-32; OKT-exercise, range 0-20; OKT-nutrition, range 0-26; OKT-risk factors, range 0-14) were generated by giving 1 point to every correct answer and 0 points for incorrect or 'not known' answers. SETTING: Tertiary-care hospital in Pune city, India.ParticipantsAdults aged 40-75 years (n 477; 234 males) enrolled through voluntary routine health checks and health camps. RESULTS: Mean age of the study population was 54·6 (sd 9·5) years. Half the participants were aware of osteoporosis and could correctly define it. Women showed significantly higher median OKT-total and OKT-nutrition scores than men (P0·1). CONCLUSIONS: Understanding about osteoporosis and its risk factors is low in the present cohort of Indian men and women. There is need to create awareness programmes aimed at both men and women especially targeting those with lower education, lower socio-economic status and no previous exposure to osteoporosis.

Sitting height percentiles in 3-17-year-old Indian children: a multicentre study.

Background: Sitting height (StH) percentiles are not described for the Indian paediatric population.Aim: To generate multicentre StH percentile values for Indian children.Subjects and methods: A total of 7961 apparently healthy children (3-17 years old, Boys: 4328) randomly selected from 10 schools from six states of India were measured for height (ht), StH and weight during July 2016-October 2017.Results: The StH:Ht ratio was 0.52 (0.02) and was similar between boys and girls (p > 0.1). The ratio decreased in boys until the age of 14 years and then slightly increased; the lowest ratio was observed during 13-15 years. In girls, however, the StH:Ht ratio decreased until the age of 9 years and then plateaued until 15 years of age with a slight increase at 16 years; the lowest ratio was observed at the age of 12-13 years. Sitting height percentiles (3rd, 10th, 25th, 50th, 75th, 90th and 97th) were computed using LMS chartmaker.Conclusions: The results indicate that, during the pubertal years, the lower limb growth is more predominant than trunk growth. Further, this study provides smoothened percentile curves for sitting height in Indian children for the first time.