This study highlights the spectrum of conditions that clinicians must be aware that can be seen in general pediatric wards and intensive care unit. This study also highlights the role of pediatric dermatologist in giving care to hospitalized children.

Steroide duyarlı nefrotik sendromu olan çocuklarda sık relapsların ve steroid bağımlılığının öngördürücülerini belirlemek amaçlandı. Gereç ve Yöntemler: Üçüncül bir merkezde 2003 ile 2015 yılları arasında steroide duyarlı nefrotik sendrom tanısı ile nefroloji kliniğine kaydedilmiş olan ve hastalık başlangıcından sonra en az bir yıl izlenen altı ay ile 18 yaş arasındaki bütün çocuklar çalışmaya alınmıştır. Bulgular: Hastalık başlangıcından itibaren en az bir yıl izlenmiş olan steroide duyarlı nefrotik sendrom tanılı 277 hasta çalışmaya alındı. Nadir relaps gösteren 157 hasta ve sık relaps gösteren 120 olgu çalışmaya alındı (sık relaps gösteren ya/ya da steroid bağımlı). Nadir relaps gösterenlerle karşılaştırıldığında, sık relaps gösterenlerde, hastalık başlangıç yaşı anlamlı derecede daha küçüktü (51,53±40,42 vs. 61,97±40,66 ay; p=0,035), ilk relapsa kadar geçen süre daha kısaydı [ilk tedavi başlangıcından ilk relapsa kadar geçen süre (8,65±11,99 vs. 23,46±24,05 ay; p<0,001)] ve enfeksiyonla birlikte daha yükek sayıda relaps vardı (8,65±11,99 vs. 1,25±1,85; p<0,001). Çok değişkenli lojistik regresyon analizinde, ilk relapsa kadar geçen sürenin altı aydan kısa olması (OR: 3,93; 95% CI: [1,97-7,82]) ve relapslar sırasında eş zamanlı enfeksiyon varlığı (OR: 1,82; 95% CI: [1,56-2,14]), sık relaps için anlamlı belirteçlerdi ve erkeklerde sık relaps olma olasılığı daha düşüktü (OR: 0,48; 95% CI: [0,24-0,93]). Kaplan-Meier analizi ve log-rank testi de, ilk relapsın altı ay içinde ortaya çıkmasının, sık relapslarla ilişkili olduğunu göstermiştir. Hastalık başlangıç yaşı ve başlangıçta yetersiz steroid tedavisi, sık relapslar için belirleyici değildi. Çıkarımlar: İlk relapsa kadar geçen sürenin daha kısa olması ve relapslar sırasında eş zamanlı enfeksiyon bulunması, gelecekte sık relaps olacağını öngördürebilir. Bu belirteçler, hastalara rehberlik vermek, onları daha yakından izlemek ve daha iyi tedavi protokolleri ve relapsa özgü müdahaleler geliştirmek için yararlı olabilir.

Objective:To estimate the incidence of invasive pneumococcal disease and pneumonia, distribution of pneumococcal serotypes, and antibiotic susceptibility in children aged 28 days to <60 months. Design: Hospital-based surveillance.Setting:; South Bangalore, India.Participants: 9950 children aged 28 days to <60 months with clinical suspicion of invasive pneumococcal disease or pneumonia.

Testicular steroidogenesis has significant implication in male reproductive function. Although the effects of various signalling molecules on testicular functions have been studied earlier, the influence of the plant hormone gibberellic acid (GA3 ) on steroidogenesis has not been investigated. Acute (4 h) and subacute (15 days) studies using this compound through oral administration (150 μg day(-1) ) to groups of normal and diabetic Wistar male rats were therefore carried out. Results indicate that (i) enhanced activity of steroidogenic markers 3β-hydroxysteroid dehydrogenase (3β-HSD), 17β-hydroxysteroid dehydrogenase (17β-HSD), elevated tissue testosterone (T) content, increased steroidogenic acute regulatory protein (StAR) and androgen binding protein (ABP) levels with reduced lipid peroxidation and improved antioxidant defence in this treatment group of normal and diabetic rat testis, and (ii) elevated lipid peroxidation and diminished antioxidant defence, with insignificant change in 3β-HSD and 17β-HSD activity and testosterone level in acute treatment group of normal and diabetic rats testis, were noted. The observed increase in the activity of testicular 3β-HSD and 17β-HSD along with elevated testosterone content established GA3 as an inducer of steroidogenesis in rat.

Cholesterol is the template for steroid hormone biosynthesis. Cholesterol homeostasis is regulated by Cyt-P450 oxygenated cholesterols acting as ligands on LXR-α and LXR-β transcription factors that are now emerging as drug targets. Heterodimerization of LXRs with retinoic acid receptor is considered a prerequisite for target gene activation. Dietary plant oxysterol 28-homobrassinolide (28-HB) is a proven antihyperglycemic and a pro-steroidogenic agent in the rat. Whether 28-HB has a role in LXR gene expression was therefore investigated using oral gavage (15 days) of 28-HB (333 µg/kg b w) to normal and diabetic rat. PCR amplified LXR-α and β mRNA transcripts from treated rat liver and testis exhibited quantitative differences in their expression. Conformational differences in 28-HB docking to LXR-α and β binding domains were also noted through in silico studies, LXR-β adopting lesser specificity. We report that 28-HB transactivates LXR genes in the rat tissues.

Steroidogenesis in testicular cells depends upon the availability of cholesterol within testicular mitochondria besides the activities of 3b-hydroxysteroid dehydrogenase (3b-HSD, 17b-hydroxysteroid dehydrogenase [17b-HSD]), and the tissue levels of steroidogenic acute regulatory protein (StAR), androgen-binding protein (ABP), and testosterone (T). Cellular cholesterol biosynthesis is regulated by endogenous oxycholesterols acting through nuclear hormone receptors. Plant oxysterols, such as 28-homobrassinolide (28-HB), available to human through diet, was shown to exhibit antihyperglycemic effect in diabetic male rat. Its role in rat testicular steroidogenesis and lipid peroxidation (LPO) was therefore assessed using normal and streptozotocin-induced diabetic male rats. Administration of 28-HB (333 mg/kg body weight) by oral gavage for 15 consecutive days to experimental rats diminished LPO, increased antioxidant enzyme, 3b-HSD and 17b-HSD activities, and elevated StAR and ABP expression and T level in rat testis. We report that 28-HB induced steroidogenesis in normal and diabetic rat testis.

Warburg syndrome is a rare disorder characterized by microcephaly, microcornea, congenital cataract, developmental delay, and hypogonadism. Here, we report two siblings from India who presented with developmental delay, microcornea, microphthalmia, and bilateral congenital cataracts, born to the third-degree consanguineously married couple. Both children had hypoplasia of corpus callosum. In this report, we aim to highlight and compare clinical features of these two cases with previously reported cases.

Understanding the influence of ubiquitously present plant steroids on mammalian cell biology is currently of interest. Feedback inhibition of HMGCoA reductase (HMGCR) catalytic activity in the transformation of HMG-CoA to mevalonate is a significant regulatory step in sterol biosynthetic pathway. To assess the role of dietary steroids in this biochemical transformation, the phytosteroid isoform 28-homobrassinolide (28-HB), 90 % pure, obtained from Godrej Agrovet (India) was used to determine its effect on mammalian HMG-CoA reductase. Photometric assay of pure human and select rat tissue HMGCR post 28-HB oral feed, PCR-HMGCR gene expression, and in silico docking of 28-HB and HMGCoA on HMGCR protein template were carried out. Using an oral feed regimen of pure 28-HB, we noted a decrease of 16 % in liver, 17.1 % in kidney and 9.3 % in testicular HMGCR enzyme activity, 25 % in HMGCR gene expression and 44 % in the activity of pure human HMGCR due to this plant oxysterol. In silico docking studies yielded binding metrics for 28-HB-HMGCR lower than for HMGCoA-HMGCR, indicating stronger binding of HMGCR by this ligand. 28-HB exerts differential effects on rat tissue HMGCR, down regulates liver HMGCR gene expression and significantly inhibits HMGCR activity.