PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

Mimics of Guillain-Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital-A Case Series.

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

Expanding the spectrum on brain-heart interactions in cerebellopontine angle surgeries.

Lesions at the cerebellopontine angle (CP angle) are associated with various brain-heart interactions, which can include those from stimulation of the fifth cranial nerve along the scalp incision in a retrosigmoid suboccipital surgical approach. A 27-year-old male patient with recently diagnosed hypertension (on calcium channel blocker) underwent left CP angle lesion decompression. Transient episodes of bradycardia, hypotension, and bradypnea were observed from the skin incision onward, exacerbated during tumor manipulation. Most episodes subsided with cessation of the surgical stimulus while some required intervention. Postoperatively, blood pressure decreased below the pre-operative levels. Thus, trigeminocardiac reflex can occur as early as the skin incision even in a retrosigmoid approach due to stimulation of the mandibular division, when specific risk factors exist. Such episodes may serve as early warning signs for subsequent intraoperative occurrences. Brainstem compression can be a possible etiology of hypertension in young patients. It underscores the importance of considering brain-heart interactions in surgical interventions involving the CP angle.

Glucose-6-phosphate dehydrogenase enzyme deficiency as a diagnostic factor of diabetes mellitus: An original study.

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common genetic disorders affecting approximately 400 million people worldwide. Several recent studies have reported a relationship between G6PD deficiency and the incidence of diabetes. Objectives: The aim of the present study was to evaluate and compare levels of G6PD deficiency in diabetes mellitus patients. Materials and Methods: G6PD activity and fasting glucose levels were measured in blood samples of 49 diabetic patients and 21 healthy controls. Results: G6PD activity was decreased in patients with diabetes mellitus as compared to healthy controls and showed that overall G6PD deficiency was significantly associated with diabetes mellitus as compared to nondiabetics. Conclusion: The study concluded that G6PD deficiency is noted in diabetics than in nondiabetics and can be a biomarker of oxidative stress and poor glycemic control in diabetes mellitus.

Spinal Muscular Atrophy With Severe Hyperlordosis: A Case Report.

Spinal muscular atrophy (SMA) indicates a set of inherited autosomal recessive genetic disorders, where, specifically, the anterior horn cell motor neurons in the brain and spinal cord are affected, leading to a severe form of hypotonia and muscle weakness. The incidence is exceptionally rare, commonly manifesting as slowly progressive muscular weakness and atrophy of lower limbs. As per our existing knowledge, this is the first case of SMA associated with hyperlordosis in a patient. Hyperlordosis is a deformity in spinal curvature characterized by an excessive forward spinal curve in the region of the lower back, forming the characteristic C-shape curvature in the lumbar region, just above the buttocks. Parents brought an 11-year-old male child with complaints of inability to get up from a sitting position along with difficulty in walking for the past six months. Upon physical examination, deep tendon reflexes were absent; there was severe hyperlordosis, proximal limb weakness, and notable hypotonia. In our study, we aim to understand the clinical presentation, impact, and association of hyperlordosis in a child diagnosed with SMA. This case report describes the complaints and successful diagnosis of a patient of survivor motor neuron (SMN) gene-related SMA along with severe hyperlordosis backed by evidences of electrophysiology and neuropathology. However, a complete cure and normal lifestyle are not possible due to the lack of affordable and easily accessible therapies.

Rehabilitation Engineering: A Narrative Review on Recent Advances in Mobility Aids in India.

Mobility has been characterized as the capacity to move across an environment safely, pleasantly, elegantly, and autonomously. India's current population is 1.4 billion, out of which 2.3%, i.e., 32 million people, are suffering from some kind of disability. With the rise in the geriatric population, the incidence of non-communicable and communicable diseases also rises and presents the risk of disorders that may progress to disability. People often neglect their disability and learn to live with it, even when most of them can use rehabilitation programs in conjunction with various mobility aids. Affordable access to adequate healthcare and assistive devices is limited, contributing to the challenges faced by disabled adults. Despite the potential for many disabled individuals to engage in productive work, their employment rates remain significantly lower. Mobility aids can provide significant benefits to individuals affected by a range of medical conditions, including arthritis, cerebral palsy, developmental disabilities, diabetic ulcers and wounds, fractures or broken bones, injuries, and walking impairments resulting from brain injury or stroke. Each person is different and may require help in a certain way for their disability, so choosing the most appropriate aid is crucial for the individual's well-being. Commonly used mobility aids are canes, walking sticks, walkers, and wheelchairs, with prostheses being used less commonly. With the advent of techniques such as state-of-the-art 3D printing and challenging surgeries, various Indian tech companies, along with non-governmental organizations (NGOs), have brought about many significant changes in the world of prosthesis by making it better, affordable, and accessible.

Crystal-chemical origins of the ultrahigh conductivity of metallic delafossites.

Despite their highly anisotropic complex-oxidic nature, certain delafossite compounds (e.g., PdCoO2, PtCoO2) are the most conductive oxides known, for reasons that remain poorly understood. Their room-temperature conductivity can exceed that of Au, while their low-temperature electronic mean-free-paths reach an astonishing 20 µm. It is widely accepted that these materials must be ultrapure to achieve this, although the methods for their growth (which produce only small crystals) are not typically capable of such. Here, we report a different approach to PdCoO2 crystal growth, using chemical vapor transport methods to achieve order-of-magnitude gains in size, the highest structural qualities yet reported, and record residual resistivity ratios ( > 440). Nevertheless, detailed mass spectrometry measurements on these materials reveal that they are not ultrapure in a general sense, typically harboring 100s-of-parts-per-million impurity levels. Through quantitative crystal-chemical analyses, we resolve this apparent dichotomy, showing that the vast majority of impurities are forced to reside in the Co-O octahedral layers, leaving the conductive Pd sheets highly pure (∼1 ppm impurity concentrations). These purities are shown to be in quantitative agreement with measured residual resistivities. We thus conclude that a sublattice purification mechanism is essential to the ultrahigh low-temperature conductivity and mean-free-path of metallic delafossites.

A novel tool for risk assessment, screening, diagnosis, assessment, and therapy in postpartum depression.

Postpartum depression (PPD), as a common condition occurring in women in the postnatal period, is underdiagnosed, seldom identified, and infrequently treated. Undiagnosed PPD can have negative consequences for overall quality-of-life outcomes in the mother, psychological growth of the infant, and bonding between the infant and its mother. However, early diagnosis and prompt treatment of PPD can lead to an improvement in postpartum maternal health and a healthier relationship between the mother and her infant. Additionally, early identification of risk factors in the prenatal and antenatal period can enable preventive treatment and ensure that the expectant or pregnant woman remains free of disease after giving birth. Therefore, we propose a protocol for the risk assessment, screening, diagnosis, assessment, and therapy of postnatal depression (RASDAT). The RASDAT protocol recommends assessment of risk factors for PPD in the perinatal period as well as early diagnosis and treatment in the early-to-mid postpartum period. It also recognizes, for the first time, insomnia and anxiety as important predictors, comorbidities, and poor prognostic factors in postpartum depression. Further studies are necessary to determine the feasibility and applicability of this novel tool, including costs of availing health facilities, burden on the health system, and knowledge, attitudes, and practices among physicians and clinical pharmacists regarding PPD and its resolution.

A case report of left atrial myxoma presenting as embolic stroke.

Key Clinical Message: Embolic stroke may rarely be the first presenting symptom of atrial myxoma. Multiple infarcts should be evaluated for embolic causes. Correct etiological diagnosis in cardio-embolic stroke guides proper management strategy. It reinforces on the importance of early echocardiogram in the initial evaluation of patients presenting with ischemic stroke. Abstract: Atrial myxoma is a benign cardiac tumor found most commonly in the left atrium in 80% of the cases. Almost 1 in 10 myxomas are familial and are most common in women. Cardiac myxoma mostly present with dyspnea, fatigue, or palpitations. Previously undiagnosed left atrial myxoma (LA) presenting as stroke is extremely rare. Authors describe the case of a middle-aged man with LA myxoma presenting with acute ischemic embolic stroke that was surgically excised. This case report emphasizes on the rare presentation as embolic stroke and the role of cardiac imaging in patients presenting with ischemic stroke. Early and coordinated teamwork among the neurologist, cardiologist, and cardiothoracic surgeon help establish the etiology and provide appropriate treatment.

Naturally acquired functional antibody responses to group A Streptococcus differ between major strain types.

Group A Streptococcus (GAS) is a leading human pathogen for which there is no licensed vaccine. Infections are most common in young children and the elderly suggesting immunity accumulates with exposure until immune senescence in older age. Though protection has been postulated to be strain type specific, based on the M-protein (emm-type), the antigenic basis of population-level immunity remains poorly understood. Naturally acquired GAS antibody responses were investigated using intravenous immunoglobulin (IVIG), which contains pooled immunoglobulins from thousands of healthy human donors, as a surrogate for population immunity. Functional opsonophagocytic killing assays were conducted with GAS strains (n = 6) representing the three major emm-pattern types (emm12, A-C pattern; emm53, D-pattern; and emm75, E-pattern). While IVIG induced opsonophagocytic killing of all GAS strains tested, specificity assays showed the profile of protective antibodies differed considerably between emm-types. Antibodies targeting the M-protein were a major component of the functional IVIG antibody response for emm12 and emm53 strains but not for emm75 strains. The striking differences in the contribution of M-protein specific antibodies to killing suggest naturally acquired immunity differs between strains from the major emm-patterns. This challenges the dogma that M-protein is the primary protective antigen across all GAS straintypes. IMPORTANCE Group A Streptococcus (GAS) is a globally important pathogen. With the surge of invasive GAS infections that have occurred in multiple countries, contemporaneous with the relaxation of COVID-19 pandemic restrictions, there is increased interest in the mechanisms underpinning GAS immunity. We utilized intravenous immunoglobulin (IVIG), pooled immunoglobulins from thousands of healthy donors, as a surrogate for population-level immunity to GAS, and explored the contribution of strain-specific (M-type specific) antibodies to GAS immunity using functional killing assays. This revealed striking differences between major strain types as to the contribution of strain specific antibodies to killing. For GAS strains belonging to the E pattern group, M-type specific antibodies do not mediate killing and immunity, which contrasts with strains belonging to pattern A-C and D groups. This challenges the historical dogma, originally proposed by Rebecca Lancefield in the 1950-1960s, that the M-protein is the major protective antigen across all GAS strain types.

Genetic analysis of iron, zinc and grain yield in wheat-Aegilops derivatives using multi-locus GWAS.

BACKGROUND: Wheat is a major staple crop and helps to reduce worldwide micronutrient deficiency. Investigating the genetics that control the concentrations of iron (Fe) and zinc (Zn) in wheat is crucial. Hence, we undertook a comprehensive study aimed at elucidating the genomic regions linked to the contents of Fe and Zn in the grain. METHODS AND RESULTS: We performed the multi-locus genome-wide association (ML-GWAS) using a panel of 161 wheat-Aegilops substitution and addition lines to dissect the genomic regions controlling grain iron (GFeC), and grain zinc (GZnC) contents. The wheat panel was genotyped using 10,825 high-quality SNPs and phenotyped in three different environments (E1-E3) during 2017-2019. A total of 111 marker-trait associations (MTAs) (at p-value < 0.001) were detected that belong to all three sub-genomes of wheat. The highest number of MTAs were identified for GFeC (58), followed by GZnC (44) and yield (9). Further, six stable MTAs were identified for these three traits and also two pleiotropic MTAs were identified for GFeC and GZnC. A total of 1291 putative candidate genes (CGs) were also identified for all three traits. These CGs encode a diverse set of proteins, including heavy metal-associated (HMA), bZIP family protein, AP2/ERF, and protein previously associated with GFeC, GZnC, and grain yield. CONCLUSIONS: The significant MTAs and CGs pinpointed in this current study are poised to play a pivotal role in enhancing both the nutritional quality and yield of wheat, utilizing marker-assisted selection (MAS) techniques.

Microenvironment-dependent growth of Sezary cells in humanized IL-15 mice.

Sezary syndrome (SS) is a rare, aggressive leukemic variant of cutaneous T-cell lymphoma (CTCL) that lacks adequate therapeutic options and representative small-animal models. Here, we demonstrate that IL-15 is a critical CTCL growth factor. Importantly, an immunodeficient knock-in mouse model genetically engineered to express human IL-15 uniquely supported the growth of SS patient samples relative to conventional immunodeficient mouse strains. SS patient-derived xenograft (PDX) models recapacitated key pathological features of the human disease, including skin infiltration and spread of leukemic cells to the periphery, and maintained the dependence on human IL-15 upon serial in vivo passaging. Detailed molecular characterization of the engrafted cells by single-cell transcriptomic analysis revealed congruent neoplastic gene expression signatures but distinct clonal engraftment patterns. Overall, we document an important dependence of Sezary cell survival and proliferation on IL-15 signaling and the utility of immunodeficient humanized IL-15 mice as hosts for SS - and potentially other T and NK cell-derived hematologic malignancies - PDX model generation. Furthermore, these studies advocate the thorough molecular understanding of the resultant PDX models to maximize their translational impact.

Accura balloon dilatation catheter for percutaneous balloon pulmonary valvuloplasty among adult patients-its protean use.

BACKGROUND: Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for hemodynamically significant pulmonary stenosis (PS). Currently, the Tyshak balloon is preferred but requires multiple dilatations because of its instability across the valve leading to a watermelon seeding effect. Accura balloon (Vascular Concept, UK) offers an advantage in its self-positioning configuration, variable diameter, and rapid inflation-deflation sequence which shortens the procedural time and valve injury. METHOD: 43 patients with severe pulmonary valve stenosis underwent PBPV using an Accura balloon at LPS Institute of Cardiology, GSVM Medical College, Kanpur, UP, India from March 2018 to February 2022. The procedure was carried out using the standard technique but the metallic straightener was removed when the catheter reached the right atrium to facilitate its delivery across the pulmonary valve. Patients were followed up by 2D echo at 24 hours and 6 months. RESULT: Successful BPV was done in all 43 patients [with mean age 21.9 (range 18-41); 31 males and 12 females] among which 5 patients had dysplastic valves. The mean diameter of the annulus was 18.5 (range 15-21) mm. Immediate hemodynamic improvement was observed in 38 patients (88%) as peak systolic gradient reduced from 84±13 to 22±12 mmHg (P<0.005) while 5 patients (12%) had <50% reduction of resting gradient, though it came down significantly at 6 months. Fluoroscopy and procedural time were 5.2±1.9 min and 22.6±3.4 min respectively. Major complications (death, cardiac perforation, tamponade, tricuspid regurgitation, requirement of blood transfusion) were none. Minor complications (transient hypotension, ventricular premature contraction, transient bradycardia) were reported in all patients. Accura balloon being bulky were delivered over left atrial and super stiff Amplatz wire in 36 and 7 patients respectively. CONCLUSION: PBPV using Accura balloon is safe and effective for both stenosed and dysplastic valves. In a few patients, maximal effect will be observed over a period of 6 months.

TiS3 Nanoribbons: A Novel Material for Ultra-Sensitive Photodetection across Extreme Temperature Ranges.

Photodetectors that can operate over a wide range of temperatures, from cryogenic to elevated temperatures, are crucial for a variety of modern scientific fields, including aerospace, high-energy science, and astro-particle science. In this study, we investigate the temperature-dependent photodetection properties of titanium trisulfide (TiS3)- in order to develop high-performance photodetectors that can operate across a wide range of temperatures (77 K-543 K). We fabricate a solid-state photodetector using the dielectrophoresis technique, which demonstrates a quick response (response/recovery time ~0.093 s) and high performance over a wide range of temperatures. Specifically, the photodetector exhibits a very high photocurrent (6.95 × 10-5 A), photoresponsivity (1.624 × 108 A/W), quantum efficiency (3.3 × 108 A/W·nm), and detectivity (4.328 × 1015 Jones) for a 617 nm wavelength of light with a very weak intensity (~1.0 × 10-5 W/cm2). The developed photodetector also shows a very high device ON/OFF ratio (~32). Prior to fabrication, the TiS3 nanoribbons were synthesized using the chemical vapor technique and characterized according to their morphology, structure, stability, and electronic and optoelectronic properties; this was performed using scanning electron microscopy (SEM), transmission electron microscopy (TEM), Raman spectroscopy, X-ray diffraction (XRD), thermogravimetric analysis (TGA), and a UV-Visible-NIR spectrophotometer. We anticipate that this novel solid-state photodetector will have broad applications in modern optoelectronic devices.

Amplatzer amulet versus watchman device for percutaneous left atrial appendage closure: A systematic review and meta-analysis.

BACKGROUND: The Amplatzer Amulet (AA) and Watchman devices (WD) are the 2 most frequently used devices for percutaneous LAA closure globally. OBJECTIVE: To evaluate the safety and clinical outcomes associated with these 2 devices in patients undergoing percutaneous LAA closure. METHOD: We systematically searched all electronic databases from inception until February 21, 2023. The primary endpoint was procedure related complications. Secondary endpoints were device related thrombus, stroke, cardiovascular mortality, peri device leak, systemic embolism, and all-cause mortality. RESULTS: A total of 3 randomized clinical trials with 2150 patients were included in this meta-analysis. The mean age was 75 and 76 years in the Amplatzer group and in the Watchman group, respectively. The odds of procedure-related complications (OR, 1.80 [95% CI: 1.21-2.67], P < .001) were significantly higher among patients with AA compared to the WD. However, the odds of all-cause mortality (OR, 0.75 (95% CI: 0.49-1.16), P = .20), stroke (OR, 0.79 [95% CI: 0.47-1.34], P = .39), systemic/pulmonary embolism (OR, 1.34 [95% CI: 0.30-6.04], P = .70), and major bleeding (OR, 1.10 [95% CI: 0.83-1.48], P = .50) were comparable between the two devices. The odds of device related thrombus (OR, 0.72 [95% CI: 0.46-1.14], P = .17) was comparable between both the group of patients, however the incidence of peri device leak was significantly lower in AA group (OR, 0.41 [95% CI: 0.26-0.66], P < .001) compared with WD group of patients. CONCLUSION: The AA was not superior to the Watchman device in terms of safety and efficacy. However, the Amulet occluder was associated with a higher incidence of procedure-related complications, and lower peri device leak.

Challenges in the treatment and prevention of monkeypox infection; A comprehensive review.

Human monkeypox (HMPX) is a zoonotic disease, literally meaning that it can be passed on from animals (non-primate) to human (primate). All the reported and recorded cases have been traced back either to international travel or import of African animals. In the Unites states, sporadic monkeypox cases have been reported in specific over the past 50 years, starting its first identification in the Democratic Republic of the Congo (D.R.C.) in 1970. Due to its extreme versatility, this disease poses threat as a serious public health issue that needs to be monitored, researched and prevented. Data indicate that prior immunization with the smallpox vaccine is beneficial and may provide protection against the monkeypox virus. JYNNEOSTM is a live viral vaccine that has been approved to improve clinical manifestations of the infection. On the other hand, public ignorance about safety precaution towards monkeypox post-COVID is another challenge that needs to be overcome in tackling HMPX as a possible re-emergent infection. This review is a collation of the epidemiology, etiology, transmission, clinical features and treatment of human monkeypox (HMPX).

Association of antenatal anxiety with adverse pregnancy outcomes: A prospective hospital-based study.

Background: Anxiety is common in pregnant women. Many studies have shown association of antenatal anxiety with adverse pregnancy outcomes, though the results are conflicting. Further, there are very limited studies on the subject reported from India, due to which, the data is limited. Hence this study was undertaken. Materials and Methods: Two hundred randomly selected consenting registered pregnant women reporting for antenatal follow up during third trimester of pregnancy were included in the study. Hindi version of Perinatal Anxiety Screening scale (PASS) was used to assess anxiety. Edinburgh Postnatal Depression rating Scale (EPDS) was used to assess comorbid depression. These women were followed up in the post-natal period to assess pregnancy outcomes. Chi-square test, Analysis of Variance (ANOVA) and correlation coefficients were calculated. Results: Analysis was done for 195 subjects. Most women (48.7%) were between 26 and 30 yrs of age. Primigravidas comprised 11.3 % of total study sample. Mean anxiety score was 23.6 (range 5-80). Adverse pregnancy outcomes were noted in 99 women; however anxiety scores in this group were not different from the group without adverse outcomes. No significant group differences were found with respect to PASS or EPDS scores. None of the women were found to have a syndromal anxiety disorder. Conclusions: Antenatal anxiety was not found to be associated with adverse pregnancy outcomes. This finding is contrary to the results obtained in earlier studies. More enquiry is needed into this area to replicate the results with clarity in larger samples, in Indian context.