Long term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD 5. Yet, 82% of adult patients have CKD 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.

Background and objectives The optimal treatment regimen for correcting 25-hydroxyvitamin D (25OHD) deficiency in children with chronic kidney disease (CKD) is not known. We compared cholecalciferol dosing regimens for achieving and maintaining 25OHD concentrations ≥30ng/ml in children with CKD stages 2-4. Design An open-label multicenter randomized controlled trial randomized children with 25OHD concentrations<30ng/ml in 1:1:1 to oral cholecalciferol 3,000 IU daily, 25,000 IU weekly, or 100,000 IU monthly for 3-months (maximum 3 intensive courses).In those with 25OHD ≥30ng/ml 1,000IU cholecalciferol daily (maintenance course) was given for up to 9 months. Primary outcome was achieving 25OHD≥30 ng/ml at end of intensive phase treatment. Results 90 children were randomized to daily(n = 30), weekly(n = 29) or monthly(n = 31) treatment groups. At end of intensive phase, 70/90(77.8%) achieved 25OHD ≥30ng/ml; 25OHD concentrations were comparable between groups (median 44.3, 39.4, and 39.3 ng/ml for daily, weekly, and monthly groups respectively; p = 0.24) with no difference between groups for time to achieve 25OHD ≥30ng/ml (p = 0.28). There was no change in calcium, phosphorus, and parathyroid hormone, but fibroblast growth factor 23(p = 0.002) and klotho(p = 0.001) concentrations significantly increased and were comparable in all treatment groups. Irrespective of dosing regimen, children with glomerular disease had 25OHD concentrations lower than non-glomerular disease (25.8 vs 41.8 ng/ml; p = 0.007). One child had 25OHD concentration of 134 ng/ml and 5.5% had hypercalcemia without symptoms of toxicity. Conclusion Intensive treatment with oral cholecalciferol as daily, weekly or monthly regimens achieved similar 25OHD concentrations between treatment groups without toxicity. Children with glomerular disease required higher doses of cholecalciferol compared to those with non-glomerular disease.

<b><i>Background:</i></b> There is a paucity of information on epidemiology, diagnosis, and treatment outcomes of congenital nephrotic syndrome (CNS) in developing countries. <b><i>Methods:</i></b> Retrospective (2012–2017) review of case records undertaken across 12 Indian pediatric nephrology centers. <b><i>Results:</i></b> Sixty-five children (58% male, median birth weight 2.4 kg [interquartile range (IQR) 2.1–2.86]) were identified with CNS. Nearly half (45%) were preterm with previous history of fetal loss/sibling death in 22% and history of consanguinity in a third. No infective etiology was confirmed. Genetic reports available for 15 (23%) children identified causal mutations in 10 (8 in NPHS1 [1 novel variant], 1 in WT 1 [novel variant], and 1 in PLCE-1 gene). In addition, 1 child was clinically diagnosed as Galloway Mowat syndrome. Next-generation sequencing showed 80% yield and Sanger sequencing 20%. Albumin infusion and angiotensin-converting enzyme inhibitors were used initially in around two-third of cohort, while only 12% of children received indomethacin. Totally, 22 (34%) children were lost to follow-up after initial visit, and among the rest median follow-up was 69 days (IQR 20–180) with 18 (42%) deaths. Eight children showed partial response (including 2 with NPHS1 compound mutation), 1 complete response, and all of them were alive at last follow-up in contrast to 53% mortality among nonresponders, <i>p</i> = 0.004. <b><i>Conclusion:</i></b> This largest reported series on CNS from India revealed suboptimal management with poor outcome as well as low number of CNS being subjected to genetic evaluation.

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Background: External ear is a significant feature for human face identification. Its size, shape and spatial location on human face are vital from aesthetic point of view. The knowledge of morphometry of normal human auricle and its symmetry is also required for the surgical resection. Aim: The aim of the current study was to estimate the morphometry of external ear and its anatomical landmarks with respect of identification. Methods: In the current, morphometric, cross sectional study was done on 400 Indian students (males = 200 and females = 200) of Teerthanker Mahaveer Medical College and Research Centre.The parameters was analysed by using camera and adobe Photoshop software (version 7.0). The data was statistically analyzed by using student's t test. p <0.05 was considered statistically significant. Results: In our cohort, all parameters were higher in males except left lobular width, which was higher in females. The mean observation of the right and left ear length of males were 4.61± 0.41 cm, 4.54± 0.44 cm respectively, while in females were 3.68± 0.42 cm, 3.67±0.54 cm respectively. The mean value of the right and left ear width of males were 3.17±0.37 cm, 3.03±0.47 cm respectively, and while in females 2.57 ± 0.32 cm, 2.55±0.41 cm respectively. The mean value of the left right and left lobular length of males were 1.56±0.12 cm, 1.50± 0.19 cm and in females were 1.09± 0.13 cm, 1.08±0.13 cm respectively. The mean values of the right and left lobular width of males were 1.58± 0.26 cm, 1.51± 0.37 cm and in females were 1.53± 0.35 cm, 1.53±0.35 cm respectively. Free lobule = 88.5% and the attach lobule = 11.5% respectively. Conclusion: This study makes possible the identification of an individual such as race, sex and age whose identity is unknown. Medico legally it is considerable parameter for forensic investigation to optimize the crime.

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