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Murugan Sudhakar, Rashmi Rikhi, Sathish Kumar Loganathan, Deepti Suri, Surjit Singh Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaCorrespondence: Deepti SuriDepartment of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, IndiaEmail surideepti@gmail.comAbstract: Wiskott–Aldrich syndrome (WAS) is an uncommon X-linked combined-immunodeficiency disorder characterized by a triad of thrombocytopenia, eczema, and immunodeficiency. Patients with WAS are also predisposed to autoimmunity and malignancy. Autoimmune manifestations have been reported in 26%– 72% of patients with WAS. Autoimmunity is an independent predictor of poor prognosis and predisposes to malignancy. Development of autoimmunity is also an early pointer of the need for hematopoietic stem–cell transplantation. In this manuscript, we have collated the published data and present a narrative review on autoimmune manifestations in WAS. A summary of currently proposed immunopathogenic mechanisms and genetic variants associated with development of autoimmunity in WAS is also included.Keywords: thrombocytopenia, vasculitis, genetics, hematopoietic stem–cell transplant, bleeding, malignancy

The Kingdom of Rantauprapat was a small kingdom that initially emerged in the upper reaches of the Bilah River. This research focuses on reconstructing the history of the Rantauprapat Kingdom through the utilization of historical relics found and historical research methods, as well as oral history methods. In particular literature, the starting point of the Rantauprapat kingdom is referred to as Poeldung. It is estimated that this kingdom existed from the late 18th century to the early 19th century, and it is known that the founder, named Patuan Bolatan, originated from Toba and was laid to rest behind the Sioldengan Village Castle housing. The existence of this kingdom became more evident during the reign of Patuan Bolatan's son, King Muda, around the mid-19th century. This was marked by the construction of the Palace in Sibuaya, formerly known as "Lobu in Bendahara Street," which has left a legacy as an ancient tomb complex. The existence of this kingdom subsequently extended from the king's residence on Padi Street to the relics of shop houses with ancient building typology on Pasar Lama Street and Veteran, as well as a tomb belonging to the last king of Rantauprapat, located in the Paindoan TPU complex

Summary: A 5‐year‐old boy presented with generalized cutaneous erosions, severe scarring, depigmentation and contractures affecting major joints. The lesions had initially affected his ears, nose, feet, and the genital and ocular mucosa, leading to significant depigmentation, scarring, contractures and mutilation. The whole of the trunk and limbs were involved at the time of presentation, with the exception of some islands of spared skin on the proximal thighs, legs, nipples and external genitalia. Electron microscopy revealed a split in the sublamina densa with the absence of anchoring fibrils, suggestive of dystrophic epidermolysis bullosa (EB). Immunofluorescence antigen mapping demonstrated a broad reticulate pattern of staining with collagen IV, VII, and laminin 332 in the floor of the blister, suggestive of Kindler syndrome. Next‐generation sequencing revealed a de novo heterozygous missense mutation (a variant of unknown significance) in exon 22 of the phospholipase‐C gamma 2 gene (PLCG2), which resulted in a substitution of serine by asparagine at codon 798 (p.Asp798Ser), a result that was validated using Sanger sequencing. The child was diagnosed with PLCG2‐associated antibody deficiency and immune dysregulation (PLAID)/autoinflammation and PLCG2‐associated antibody deficiency and immune dysregulation (APLAID) syndrome. The cutaneous and corneal erosions, inflammation and scarring of this magnitude, and the eventual result of death have not been described previously for the PLAID/APLAID spectrum previously. In conclusion, this was an unusual acquired autoinflammatory severe EB‐like disease that may be associated with de novo PLCG2 mutation. Click here for the corresponding questions to this CME article. [ABSTRACT FROM AUTHOR]

The current investigation was conducted to study the fractions of sulphur in nine districts of low and mid hills of Himachal Pradesh. For this purpose 31 representative soil sampling sites were selected from nine districts and the soil samples were analyzed for physicochemical properties and different fractions of sulphur (water soluble sulphur, exchangeable sulphur, available sulphur, non-sulphate sulphur, organic sulphur and total sulphur). The results indicated that the total sulphur in soils varied from 98.2 to 470.1 mg kg-1 in surface soil (0-15 cm) and 67.2 to 370.7 mg kg-1 in subsurface layer (15-60 cm). The organic sulphur varied from 80.5 to 401.1 mg kg-1 in surface and 44 to 306.1 mg kg-1 in subsurface layer. The water soluble sulphur, exchangeable sulphur, available sulphur and non-sulphate sulphur varied from 1.7 to 9.2, 2.7 to 18.4, 4.5 to 27.6 and 10.2 to 58.9 mg kg-1 respectively in surface soil and 0.5 to 5.4, 1 to 17.7, 3.7 to 23.5 and 12.5 to 50.2 mg kg-1, respectively in sub-surface soil. It was observed during course of study that with increase in the soil depth the content of different fractions of sulphur decreased. These soils had the major part of their total sulphur content in organic form followed by non-sulphate sulphur, available sulphur, exchangeable sulphur and water soluble sulphur. It can be concluded that the soil texture and organic carbon content played a major role in determining the quantity of different fractions of sulphur in these soils. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is an autoimmune disorder that can affect virtually any organ. Chylous ascites as a presenting manifestation of SLE has been described in a handful of cases in adults. However, to the best of our knowledge this presentation has never been reported in the pediatric age group. Podocytopathy in SLE was initially considered to be a chance association. However, more recently it has been suggested that minimal change disease is not only a chance association; it is part of the lupus nephritis spectrum. [ABSTRACT FROM AUTHOR]

Presents a study which investigated the effect on the superconducting and microstructural properties of YBa[sub2]Cu[sub3]O[sub7-x] screen printed thick films as a function of silver content. Experimental details; Results and discussion; Conclusion.

Allergy is still a health problem in Indonesia. One of the manifestations of allergies is allergic rhinitis. Many factors can trigger the recurrence of rhinitis, including exposure to house dust mites. This study aims to identify the population of house dust mites in the classrooms and student residences. This study was participated by 74 FK UNRI students with allergic rhinitis. The samples included dust collected from residences and classrooms consisting of four large classrooms, three small classrooms, four laboratories, two examination rooms, 12 skills lab rooms, and 15 tutorial rooms. Detection of dust was performed using the direct method. The results showed that no classroom (0%) was found with house dust mites. However, there were 37.8% of residences were positive. Based on the result, it can be concluded that the high population of house dust mites in the students’ residences becomes an essential factor as a chronic stressor for allergic rhinitis.

The ocular system can be affected in systemic lupus erythematosus (SLE) in one third of patients. However, optic nerve involvement is relatively uncommon, but is more so in pediatric SLE patients, where it can occur in 1% of cases. We report three children with SLE who presented with optic nerve involvement. Two children had optic neuritis, with optic neuritis being the first manifestation in one child. The third child had ischaemic optic neuropathy secondary to antiphospholipid syndrome. A careful work up for SLE should be performed in every child with optic nerve disease. Prompt diagnosis and early treatment results in a better prognosis. [ABSTRACT FROM AUTHOR]

The article discusses the clinical outcomes of childhood lupus nephritis in developing countries. It examines the actuarial survival and actuarial renal survival of children with lupus nephritis using Kaplan-Meier analysis. It notes that outcome of children with lupus nephritis from India is worse than developed countries.

Abstract: Glucocorticoids serve as key stress response hormones that facilitate stress coping. However, sustained glucocorticoid exposure is associated with adverse consequences on the brain, in particular within the hippocampus. Chronic glucocorticoid exposure evokes neuronal cell damage and dendritic atrophy, reduces hippocampal neurogenesis and impairs synaptic plasticity. Glucocorticoids also alter expression and signaling of the neurotrophin, brain-derived neurotrophic factor (BDNF). Since BDNF is known to promote neuroplasticity, enhance cell survival, increase hippocampal neurogenesis and cellular excitability, it has been hypothesized that specific adverse effects of glucocorticoids may be mediated by attenuating BDNF expression and signaling. The purpose of this review is to summarize the current state of literature examining the influence of glucocorticoids on BDNF, and to address whether specific effects of glucocorticoids arise through perturbation of BDNF signaling. We integrate evidence of glucocorticoid regulation of BDNF at multiple levels, spanning from the well-documented glucocorticoid-induced changes in BDNF mRNA to studies examining alterations in BDNF receptor-mediated signaling. Further, we delineate potential lines of future investigation to address hitherto unexplored aspects of the influence of glucocorticoids on BDNF. Finally, we discuss the current understanding of the contribution of BDNF to the modulation of structural and functional plasticity by glucocorticoids, in particular in the context of the hippocampus. Understanding the mechanistic crosstalk between glucocorticoids and BDNF holds promise for the identification of potential therapeutic targets for disorders associated with the dysfunction of stress hormone pathways. [Copyright &y& Elsevier]

Percutaneous renal biopsy (PRB) is an important diagnostic tool in pediatric nephrology units. But controversy exists whether the procedure can be done in the day care setting. This study was done to document complications of PRB done with automated gun under continuous ultrasonographic guidance and to find whether the procedure can be undertaken as a day care procedure. Retrospective analysis of 67 PRBs is presented. A total of 44% (n = 30) minor and 12% (n = 8) major complications such as gross hematuria, perinephric hematoma, and hemodynamic instability were observed through the study period. All major and 90% of minor complications were detected within four hours in the current study. The procedure may be undertaken in the day care setting with strict pre and postprocedure monitoring up to eight hours in children with normal blood pressures, renal functions, hemoglobin concentrations, and coagulation parameters. [ABSTRACT FROM AUTHOR]

Proposes a homotopy algorithms for solving polynomial equations in mechanism design. Applications of polynomial systems; Details on homotopy method; Equation for path reduction using secant homotopy and side conditions.

Background: The Patient Self-Determination Act of 1991 requires that nursing homes reimbursed by Medicare or Medicaid inform all residents upon admission of their rights to enact care directives in the event of terminal illness. This study investigated the relationship between care directive use and resident functional status.Methods: We analyzed a version of the Minimum Data Set (MDS+) from a single state. We selected residents who were admitted to a nursing home in the first half of 1993 and followed them in the nursing home through the end of 1994. We created logistic models to examine independent correlates associated with having an advance directive or a do-not-resuscitate (DNR) order on admission. We then created similar logistic models to examine independent correlates associated with writing an advance directive or DNR order subsequent to admission.Results: Of the 2,780 residents, 11% (292) had advance directives and 17% (466) had DNR orders upon admission. Of those without care directives upon admission, 6% (143) subsequently had an advance directive and 15% (339) subsequently had a DNR order. Cross-sectionally, older individuals and whites were more likely to have a care directive. Having poor cognitive and physical function was associated with having a DNR order upon admission. Longitudinally, longer stayers and whites were more likely to have an advance directive. Residents who lost physical function were more likely to have an advance directive and those who lost cognitive function were more likely to have a DNR order.Conclusions: Care directive use is influenced by a number of sociodemographic and functional characteristics. [ABSTRACT FROM AUTHOR]

Hypernatremic dehydration in breast fed neonates is a rare but increasingly recognized problem. If not identified early, it may lead to potentially life threatening complications. The authors describe a neonate who developed thrombosis and lower limb ischemia secondary to hypernatremic dehydration. Peripheral gangrene, as a result of hypernatremic dehydration is extremely uncommon and has rarely been reported before. [ABSTRACT FROM AUTHOR]