Hyperopia in schoolchildren: Investigating the impact on vision and determining appropriate methods for screening.

INTRODUCTION: Hyperopia is associated with reduced vision and educational outcomes in schoolchildren. This study explored the impact of clinically significant hyperopia (≥+2.00 D) on visual function in schoolchildren and compared the ability of different screening tests (alone and in combination) to detect this level of hyperopia. METHODS: Vision testing including monocular logMAR visual acuity (VA) measured to threshold (distance [DVA], near [NVA] and DVA through a plus lens [+2.50 D]), stereoacuity and cycloplegic autorefraction (tropicamide 1%) were undertaken on 263 schoolchildren (mean age: 11.76 years ± 3.38) in Queensland, Australia. Vision measures were compared between children with clinically significant hyperopia in at least one meridian (≥+2.00 D) and emmetropia/low hyperopia (>0.00 and <+2.00 D). Receiver operating curve (ROC) analysis was performed to identify optimal pass/fail criteria for each test and the diagnostic accuracy of individual and combinations of tests. RESULTS: Thirty-two children had clinically significant hyperopia and 225 had emmetropia/low hyperopia. DVA and NVA were worse (p < 0.01), while the difference in DVA through a plus lens was less in children with clinically significant hyperopia (p < 0.01). ROC analysis for individual tests resulted in areas under the curve (AUCs) ranging from 0.65 to 0.85. Combining screening tests revealed that failing one or more of the following tests was most effective for detecting hyperopia: DVA, NVA and difference in DVA through a plus lens, resulting in a sensitivity and specificity of 72% and 81%, respectively. CONCLUSION: Significant differences in visual function existed between schoolchildren with clinically significant hyperopia and emmetropia/low hyperopia. Combining measures of DVA and NVA and the difference in DVA through a plus lens demonstrated good discriminative ability for detecting clinically significant hyperopia in this population.

Assessment of optical quality of ready-made reading spectacles for presbyopic correction.

PURPOSE: Many presbyopic patients in both developed and developing countries use ready-made reading spectacles for their near vision correction even though the quality of these spectacles cannot always be assured. This study assessed the optical quality of ready-made reading spectacles for presbyopic correction in comparison with relevant international standards. METHODS: A total of 105 ready-made reading spectacles with powers ranging from +1.50 to +3.50 dioptres (D) in +0.50 D steps were randomly procured from open markets in Ghana and assessed for their optical quality, including induced prisms and safety markings. These assessments were done in line with the International Organization for Standardization (ISO 16034:2002 [BS EN 14139:2010]) as well as the standards used in low-resource countries. RESULTS: All lenses (100%) had significant induced horizontal prism that exceeded the tolerance levels stipulated by the ISO standards, while 30% had vertical prism greater than the specified tolerances. The highest prevalence of induced vertical prism was seen in the +2.50 and +3.50 D lenses (48% and 43%, respectively). When compared with less conservative standards, as suggested for use in low-resource countries, the prevalence of induced horizontal and vertical prism reduced to 88% and 14%, respectively. While only 15% of spectacles had a labelled centration distance, none had any safety markings per the ISO standards. CONCLUSION: The high prevalence of ready-made reading spectacles in Ghana that fail to meet optical quality standards indicates the need for more robust, rigorous and standardised protocols for assessing their optical quality before they are sold on the market. This will alleviate unwanted side effects including asthenopia associated with their use. There is also the need to intensify public health awareness on the use of ready-made reading spectacles, especially by patients with significant refractive errors and ocular pathologies.

Toward the Fourth Industrial Revolution among E7 Economies: Assessment of the Combined Impact of Institutional Quality, Bank Funding, and Foreign Direct Investment.

Technological innovation and its paradigm, that is, the Fourth Industrial Revolution-4IR, have shown strong impact on income levels of adopters across the globe. To this end, this analysis examines the impact of bank funding and institutional quality on technological advancement. This study adds additional variables such as high-technology exports and foreign direct investment (FDI) as control variable. Our study period spans from 2000 to 2018 on an annual frequency for E7 economies (Brazil, Indonesia, Mexico, India, Turkey, Russia, and China). This study leverages on cross-sectional ARDL, Augmented Mean Group (AMG), and Common Correlated Effects Mean Group Estimates (CCEMG) estimation techniques to examine long-run relationship between the outlined variables. Empirical findings show that institution quality, bank finance, income, high-technology exports, and foreign direct investments exert a positive effect on advancements in technology. Furthermore, the interaction between bank finance and institution quality on technological advancement is also positive and statistically significant. Based on the findings, it is concluded that large-scale funding is crucial for businesses to leverage revolutionary technology. Likewise, access to large capital sources if made easier encourages technology affordance as well as innovation and operational excellence. Thus, economies with established legal and financial systems stand to offer businesses such security, which encourages business innovation. Consequently, E7 economies ought to improve their financial and legal systems to boost financial security, creativity, and competitiveness of businesses.

Building Critical Infrastructures: Evaluating the Roles of Governance and Institutions in Infrastructural Developments in Sub-Sahara African Countries.

BACKGROUND: The Sub-Saharan African (SSA) region has notably been in the limelight of infrastructural deficit discussions over the decades. Although the region's infrastructural development is gradually improving, the levels and pace of development remain generally poor compared to the rest of the world. OBJECTIVES: This study thus aims to empirically examine the roles of governance and institutions in infrastructural developments in the Sub-Sahara African (SSA) region toward addressing the pressing needs for critical infrastructures for the region. RESEARCH DESIGNS: The empirical strategies utilized in the study include the Common Correlated Efficient Mean Group (CCEMG) and Dynamic CCEMG methods among others. These empirical approaches were applied to analyze data on governance and institutional quality proxies for the SSA region to achieve the study's objectives while controlling for the effects of industrial value-added, foreign capital inflow (FDI), and overall economic growth for the understudied period (1990-2019). RESULTS: The results reflect the essence of governance and institutional quality as these variables significantly boost infrastructural development in SSA. In addition, industrialization and growth also show a favorable impact on the development of infrastructure thus reflecting that the transition from agrarian to industrial economies occurs in parallel with infrastructure development in the SSA. However, FDI inflows were not found to be significantly instrumental to infrastructural development in the region. CONCLUSIONS: Hence, the SSA must strive to strengthen institutions and harmonize their industrial and economic push with infrastructural developments while encouraging potential foreign investors to diversify investments to infrastructural projects beyond the usual primary sector/resource-based activities.

Hydroxocobalamin Administration After Inhalation Injury Is Not Associated With Mesenteric Ischemia.

Hydroxocobalamin has been administered in the United States since 2006 by first responders and burn centers as a safe antidote for cyanide toxicity, a serious complication of smoke inhalation. There are no current contraindications to the use of this rescue measure. A recent retrospective French publication reported a potential correlation between hydroxcobalamin administration and acute mesenteric ischemia (AMI) in critically ill burn and inhalation injury patients. The purpose of this study is to characterize the risk of AMI related to hydroxocobalamin. A retrospective review of hydroxocobalamin administration among adult burn patients was conducted at a regional burn center over a 2-year period. Injury characteristics, demographics, and outcomes including presence of mesenteric ischemia (defined as presence of pneumatosis or perforation on CT scan or necrotic bowel on laparotomy) were recorded. Of 17 confirmed inhalation injuries, patients had a median (interquartile range) age, total body surface area (TBSA), and abbreviated burn severity (ABSI) index as follows: 60 (45, 65 IQR), 8.5 (1.8, 39 IQR), and 6 (5,7 IQR). Inhalation injury was diagnosed with bronchoscopy, carboxyhemoglobin, or clinical suspicion. Eighty-two percent of those with bronchoscopy had an inhalation grade of 2 or greater. None (0%) of the patients showed signs of mesenteric ischemia, tube feeding intolerance, pneumatosis/perforation on CT, or necrotic bowel on laparotomy. Aware of the study limitations, we conclude that hydroxocobalamin does not increase risk for AMI.

L'hydroxocobalamine (OHB12), antidote peu dangereux en cas d'intoxication cyanhydrique (complication grave de l'inhalation de fumées), est administrée depuis 2006 par les premiers secours étatsuniens. Il n'existe actuellement pas de contre- indication à son utilisation. Une étude rétrospective française récente a rapporté la possible association entre OHB12 et ischémie mésentérique (IM) chez des brûlés graves ayant inhalé des fumées. Cette étude se propose d'étudier cette éventuelle corrélation. Il s'agit d'une étude rétrospective conduite pendant 2 ans dans un CTB régional. Les caractéristiques démographiques, celles de la brûlure et l'évolution (en recherchant particulièrement une IM) ont été relevées. Le diagnostic d'IM était posé devant des images scannographiques évocatrices (pneumopéritoine, perforation digestive) ou la constatation per- opératoire d'une nécrose digestive. Dix- sept patients avaient une inhalation de fumées confirmée par endoscopie (dans ce cas, 87% des patients avec inhalation de fumées avaient des lésions de grade 2 ou plus) ou carboxyhémoglobine ou simple suspicion clinique. Leur âge médian était de 65 ans (Q1: 45, Q3 : 35), leur surface brûlés de 8,5% (Q1: 1,8, Q3 : 39), leur ABSI de 6 (Q1 : 5 ; Q3 : 7). Aucun patient n'a développé une IM ni une intolérance digestive. Bien que cette étude ait des limitations, nous concluons que OHB12 n'est pas un facteur de risque d'IM.

How do trade and economic growth impact environmental degradation? New evidence and policy implications from the ARDL approach.

Due to various environmental degradation and natural resource depletion around the world, researchers' and policymakers' attention has turned to what causes environmental degradation. The pursuit of a healthy environment has become a global challenge, a problem that affects more than one nation. Climate change is causing severe weather conditions in every world, disrupting economies and affecting the lives of many people. Hence, the study analyzes how trade and economic growth impact environmental degradation in Belgium, the USA, and Canada using panel data from 1995 to 2016. The study utilized the autoregressive distributed lag approach to provide new evidence and policy implications. The outcome confirmed the presence of cointegration among the selected variables. However, it was observed that economic growth decreases environmental degradation in the long run while trade openness shows a positively insignificant relationship with carbon emission. Nevertheless, a positive short-run relationship was observed between economic growth and carbon emissions whereas a negatively insignificant relationship was observed for trade and carbon emission. The findings prompted policy implications that more trading could be done between the countries. When countries trade more, their economies will flourish, ensuring global prosperity and minimizing environmental degradation.

Metabolic features of recurrent major depressive disorder in remission, and the risk of future recurrence.

Recurrent major depressive disorder (rMDD) is a relapsing-remitting disease with high morbidity and a 5-year risk of recurrence of up to 80%. This was a prospective pilot study to examine the potential diagnostic and prognostic value of targeted plasma metabolomics in the care of patients with rMDD in remission. We used an established LC-MS/MS platform to measure 399 metabolites in 68 subjects with rMDD (n = 45 females and 23 males) in antidepressant-free remission and 59 age- and sex-matched controls (n = 40 females and 19 males). Patients were then followed prospectively for 2.5 years. Metabolomics explained up to 43% of the phenotypic variance. The strongest biomarkers were gender specific. 80% of the metabolic predictors of recurrence in both males and females belonged to 6 pathways: (1) phospholipids, (2) sphingomyelins, (3) glycosphingolipids, (4) eicosanoids, (5) microbiome, and (6) purines. These changes traced to altered mitochondrial regulation of cellular redox, signaling, energy, and lipid metabolism. Metabolomics identified a chemical endophenotype that could be used to stratify rrMDD patients at greatest risk for recurrence with an accuracy over 0.90 (95%CI = 0.69-1.0). Power calculations suggest that a validation study of at least 198 females and 198 males (99 cases and 99 controls each) will be needed to confirm these results. Although a small study, these results are the first to show the potential utility of metabolomics in assisting with the important clinical challenge of prospectively identifying the patients at greatest risk of recurrence of a depressive episode and those who are at lower risk.

An Adjusted Ideal Body Weight Index Formula With Fresh Frozen Plasma (FFP) Rescue Decreases Fluid Creep During Burn Resuscitation.

Severe burn injury requires significant volume resuscitation, but over-resuscitation can be deadly. Accurate resuscitation of obese patients is challenging due to the decreased vascularity of adipose tissue. This study compares an adjusted ideal body weight index formula with fresh frozen plasma rescue to historical controls resuscitated with Parkland-based resuscitation. A retrospective review was conducted of adult patients admitted to our regional burn center with ≥ 20% total body surface area (TBSA) burns from 2010 to 2017 who survived more than 48 hours. Historical controls were resuscitated with Parkland-based resuscitation with occasional albumin. The adjusted ideal body weight (AIBW) patients were resuscitated with 2-4 mL/kg/%TBSA using an adjusted ideal body weight with fresh frozen plasma (FFP) rescue. Outcomes were compared with nonparametric statistics. A total of 161 patients met inclusion criteria: 40 patients received AIBW resuscitation and 121 patients were included as controls. The AIBW group received less fluid (3.30 vs. 4.15 mL/kg/%TBSA, p<0.001). A significant reduction in acute kidney injury requiring dialysis in the AIBW group was appreciated (5% vs. 19%, p=0.03) with improved mortality in AIBW patients as well (5% vs. 20%, p=0.03). Using an adjusted ideal body weight with FFP rescue to resuscitate patients with severe burn injury leads to a significant reduction in fluid administration without increase in acute kidney injury requiring dialysis and with improved mortality.

Les brûlés graves ont besoin d'un remplissage vasculaire majeur, pouvant toutefois être mortel s'il est exagéré. Il est particulièrement difficile chez les obèses, en raison de la faible vascularisation du tissu adipeux. Cette étude compare une formule basée sur le poids idéal (PI) et utilisant du PFC à des séries historiques utilisant la formule de Parkland. Cette étude rétrospective a revu les dossiers des patients admis dans notre CTB, ayant survécu plus de 48 h, entre 2010 et 2017. Les 121 cas historiques recevaient un remplissage selon Parkland (et de l'albumine au besoin). Les 40 patients à l'étude recevaient 2 à 4 mL/kg/% de PFC, le volume étant calculé sur le poids idéal. Ce groupe recevait significativement (p < 0,001) moins (3,3 mL/kg/%) de volume que le groupe contrôle (4,15). Ils avaient aussi moins fréquemment besoin d'épuration extra- rénale (5% VS 19%, p= 0,03) et leur mortalité était moindre (5% VS 20%, p= 0,03).

Metabolic features of Gulf War illness.

BACKGROUND: More than 230,000 veterans-about 1/3 of US personnel deployed in the 1990-1991 Persian Gulf War-developed chronic, multi-symptom health problems now called "Gulf War illness" (GWI), for which mechanisms and objective diagnostic signatures continue to be sought. METHODS: Targeted, broad-spectrum serum metabolomics was used to gain insights into the biology of GWI. 40 male participants, included 20 veterans who met both Kansas and CDC diagnostic criteria for GWI and 20 nonveteran controls without similar symptoms that were 1:1 matched to GWI cases by age, sex, and ethnicity. Serum samples were collected and archived at -80° C prior to testing. 358 metabolites from 46 biochemical pathways were measured by hydrophilic interaction liquid chromatography and tandem mass spectrometry. RESULTS: Veterans with GWI, compared to healthy controls, had abnormalities in 8 of 46 biochemical pathways interrogated. Lipid abnormalities accounted for 78% of the metabolic impact. Fifteen ceramides and sphingomyelins, and four phosphatidylcholine lipids were increased. Five of the 8 pathways were shared with the previously reported metabolic phenotype of males with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS). However, 4 of the 5 shared pathways were regulated in opposite directions; key pathways that were up-regulated in GWI were down-regulated in ME/CFS. The single pathway regulated in the same direction was purines, which were decreased. CONCLUSIONS: Our data show that despite heterogeneous exposure histories, a metabolic phenotype of GWI was clearly distinguished from controls. Metabolomic differences between GWI and ME/CFS show that common clinical symptoms like fatigue can have different chemical mechanisms and different diagnostic implications. Larger studies will be needed to validate these findings.

A case-comparison study of pregnant women with mitochondrial disease - what to expect?

OBJECTIVE: Mitochondrial disease is a disorder of energy metabolism that affects 1 in 4300 adults in the UK. Pregnancy is associated with physiological demands that have implications for energy metabolism. We were interested to know how pregnancy was affected in women with mitochondrial disease, particularly those with the most common pathogenic mutation m.3243A>G. DESIGN: Retrospective case-comparison study. POPULATION/SETTING: Sixty-seven women with genetically confirmed mitochondrial disease from the UK Mitochondrial Diseases Cohort and 69 unaffected women participated. METHODS: Participants answered questionnaires regarding each of their pregnancies. Patients were divided into two groups according to genetic mutation, with those harbouring m.3243A>G comprising a single group. MAIN OUTCOME MEASURES: Pregnancy-related complications, mode of delivery, gestational age and birthweight of newborns. RESULTS: Of 139 live births in the comparison group, 62 were in the m.3243A>G group and 87 were in the 'all other mutations' group. Pregnancies of women with the m.3243A>G mutation had significantly more gestational diabetes (odds ratio [OR] = 8.2, 95% CI 1.3-50.1), breathing difficulties (OR = 7.8, 95% CI 1.0-59.1) and hypertension (OR = 8.2, 95% CI 3.1-21.5) than the comparison group. Only half of the pregnancies in the m.3243A>G group had normal vaginal delivery, with emergency caesarean section accounting for 24.2% of deliveries. Babies were born significantly earlier to mothers harbouring m.3243A>G with 53.3% of them preterm (<37 weeks). These babies were also more likely to require resuscitation and admission. CONCLUSION: Women who carried the m.3243A>G mutation appeared to be at higher risk of complications during pregnancies, caesarean section and preterm delivery than the unaffected women or those with other forms of mitochondrial disease. TWEETABLE ABSTRACT: Pregnant women with mitochondrial disease - m.3243A>G mutation - are at greatly increased risk of complications and preterm delivery.

A robust, single-injection method for targeted, broad-spectrum plasma metabolomics.

BACKGROUND: Metabolomics is a powerful emerging technology for studying the systems biology and chemistry of health and disease. Current targeted methods are often limited by the number of analytes that can be measured, and/or require multiple injections. METHODS: We developed a single-injection, targeted broad-spectrum plasma metabolomic method on a SCIEX Qtrap 5500 LC-ESI-MS/MS platform. Analytical validation was conducted for the reproducibility, linearity, carryover and blood collection tube effects. The method was also clinically validated for its potential utility in the diagnosis of chronic fatigue syndrome (CFS) using a cohort of 22 males CFS and 18 age- and sex-matched controls. RESULTS: Optimization of LC conditions and MS/MS parameters enabled the measurement of 610 key metabolites from 63 biochemical pathways and 95 stable isotope standards in a 45-minute HILIC method using a single injection without sacrificing sensitivity. The total imprecision (CVtotal) of peak area was 12% for both the control and CFS pools. The 8 metabolites selected in our previous study (PMID: 27573827) performed well in a clinical validation analysis even when the case and control samples were analyzed 1.5 years later on a different instrument by a different investigator, yielding a diagnostic accuracy of 95% (95% CI 85-100%) measured by the area under the ROC curve. CONCLUSIONS: A reliable and reproducible, broad-spectrum, targeted metabolomic method was developed, capable of measuring over 600 metabolites in plasma in a single injection. The method might be a useful tool in helping the diagnosis of CFS or other complex diseases.

Low-dose suramin in autism spectrum disorder: a small, phase I/II, randomized clinical trial.

OBJECTIVE: No drug is yet approved to treat the core symptoms of autism spectrum disorder (ASD). Low-dose suramin was effective in the maternal immune activation and Fragile X mouse models of ASD. The Suramin Autism Treatment-1 (SAT-1) trial was a double-blind, placebo-controlled, translational pilot study to examine the safety and activity of low-dose suramin in children with ASD. METHODS: Ten male subjects with ASD, ages 5-14 years, were matched by age, IQ, and autism severity into five pairs, then randomized to receive a single, intravenous infusion of suramin (20 mg/kg) or saline. The primary outcomes were ADOS-2 comparison scores and Expressive One-Word Picture Vocabulary Test (EOWPVT). Secondary outcomes were the aberrant behavior checklist, autism treatment evaluation checklist, repetitive behavior questionnaire, and clinical global impression questionnaire. RESULTS: Blood levels of suramin were 12 ± 1.5 µmol/L (mean ± SD) at 2 days and 1.5 ± 0.5 µmol/L after 6 weeks. The terminal half-life was 14.7 ± 0.7 days. A self-limited, asymptomatic rash was seen, but there were no serious adverse events. ADOS-2 comparison scores improved by -1.6 ± 0.55 points (n = 5; 95% CI = -2.3 to -0.9; Cohen's d = 2.9; P = 0.0028) in the suramin group and did not change in the placebo group. EOWPVT scores did not change. Secondary outcomes also showed improvements in language, social interaction, and decreased restricted or repetitive behaviors. INTERPRETATION: The safety and activity of low-dose suramin showed promise as a novel approach to treatment of ASD in this small study.

Thermal stress exposure, bleaching response, and mortality in the threatened coral Acropora palmata.

Demographic data for Elkhorn coral, Acropora palmata, and in situ water temperature data from seven upper Florida Keys (USA) reefs revealed three warm thermal stress events between 2010 and 2016. During a mild bleaching event in 2011, up to 59% of colonies bleached, but no mortality resulted. In both 2014 and 2015, severe and unprecedented bleaching was observed with up to 100% of colonies bleached. A. palmata live tissue cover declined by one-third following the 2014-2015 events. Colony mortality of mildly- and non-bleached colonies did not differ but increased significantly with more severe bleaching. Increased bleaching prevalence corresponded to maximum daily average water temperatures above 31.3°C. However, the cumulative days with daily average exceeding 31.0°C provided a better predictor of bleaching response. The bleaching response of surviving colonies in 2015 was not consistent with acclimatization as most individual colonies bleached at least as badly as in 2014.

Metabolic features of chronic fatigue syndrome.

More than 2 million people in the United States have myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). We performed targeted, broad-spectrum metabolomics to gain insights into the biology of CFS. We studied a total of 84 subjects using these methods. Forty-five subjects (n = 22 men and 23 women) met diagnostic criteria for ME/CFS by Institute of Medicine, Canadian, and Fukuda criteria. Thirty-nine subjects (n = 18 men and 21 women) were age- and sex-matched normal controls. Males with CFS were 53 (±2.8) y old (mean ± SEM; range, 21-67 y). Females were 52 (±2.5) y old (range, 20-67 y). The Karnofsky performance scores were 62 (±3.2) for males and 54 (±3.3) for females. We targeted 612 metabolites in plasma from 63 biochemical pathways by hydrophilic interaction liquid chromatography, electrospray ionization, and tandem mass spectrometry in a single-injection method. Patients with CFS showed abnormalities in 20 metabolic pathways. Eighty percent of the diagnostic metabolites were decreased, consistent with a hypometabolic syndrome. Pathway abnormalities included sphingolipid, phospholipid, purine, cholesterol, microbiome, pyrroline-5-carboxylate, riboflavin, branch chain amino acid, peroxisomal, and mitochondrial metabolism. Area under the receiver operator characteristic curve analysis showed diagnostic accuracies of 94% [95% confidence interval (CI), 84-100%] in males using eight metabolites and 96% (95% CI, 86-100%) in females using 13 metabolites. Our data show that despite the heterogeneity of factors leading to CFS, the cellular metabolic response in patients was homogeneous, statistically robust, and chemically similar to the evolutionarily conserved persistence response to environmental stress known as dauer.

Smothering in UK free-range flocks. Part 2: investigating correlations between disease, housing and management practices.

Smothering, when birds group together in a way that results in death from suffocation, is a welfare and economic concern for the egg industry. This questionnaire-based study explored correlations between disease, housing, management practices and smothering on free-range farms. A binomial logistic regression approach was used to test whether question responses predicted occurrence of nest box smothers (NBS) and panic and recurring smothers (PSRS) on farms. Breed (P=0.008) and nest box manufacturer (P=0.014) predicted NBS. Breed and nest box design have been previously reported to affect nesting behaviour. The affect of nest box manufacturer found in this study may illustrate the effect of nest box design features or house layouts. Nest box manufacturer (P=0.009), feeding oyster grit or grain on the litter (P<0.001) and range use on a sunny day (P<0.001) also predicted PSRS. Implementing some management practices to encourage desirable behaviours (eg ranging) may contribute to smothering, whereas some management practices such as those aimed at occupying birds may be beneficial, illustrating the delicate balance of factors involved in free-range egg production. It is hoped that these results will stimulate further work exploring the suitability of housing design and management of laying hens in light of smothering.

Ab initio and density functional theory calculations of molecular structure and vibrational spectra of 4-(2-Hydroxyethyl) piperazine-1-ethanesulfonic acid.

The FTIR and FT-Raman spectra of 4-(2-Hydroxyethyl) piperazine-1-ethanesulfonic acid were recorded and the structural and spectroscopic data of the molecule in the ground state were calculated using Hartree-Fock and Density Functional Method (B3LYP). The most stable conformer was optimized and the structural and vibrational parameters were determined. With the observed FTIR and FT-Raman data, a complete vibrational band assignment and analysis of the fundamental modes of the compound were carried out. Thermodynamic properties, Mulliken and natural atomic charge distribution were calculated using both Hartree-Fock and Density Functional Method and compared. UV-Visible and HOMO-LUMO analysis were carried out. (1)H and (13)C NMR chemical shifts of the molecule were calculated using gauge including atomic orbital method and were compared with experimental results. Stability of the molecule arising from hyperconjugative interactions and charge delocalization has been analyzed using natural bond orbital analysis. The first order hyperpolarizability (ß) and molecular electrostatic potential of the molecule was computed using DFT calculations. The electron density based local reactivity descriptor such as Fukui functions were calculated to explain the chemically reactive site in the molecule.

Early growth and development impairments in patients with ganglioside GM3 synthase deficiency.

Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GSD) causes a complete absence of GM3 and all downstream biosynthetic derivatives. The individuals affected by this disorder manifest severe irritability, intractable seizures and profound intellectual disability. However, we have found that most newborns seem symptom-free for a period of time after birth. In order to further understand the onset of the disease, we investigated the early growth and development of patients with this condition through this study. We compared 37 affected individuals with their normal siblings and revealed that all children with GSD had relatively normal intrauterine growth and development, as their weight, length and head circumference were similar to their normal siblings at birth. However, the disease progresses quickly after birth and causes significant constitutional impairments of growth and development by 6 months of age. Neither breastfeeding nor gastrostomy tube placement made significant difference on growth and development as all groups of patients showed the similar pattern. We conclude that GSD causes significant postnatal growth and developmental impairments and the amount of gangliosides in breast milk and general nutritional intervention do not seem to alter these outcomes.

Developing an Electronic Medical Record for Interlinked Care Services in Haiti.

A large clinical care and research organization in Haiti required an electronic medical record system (EMR) to serve the needs of its 30 interlinked clinical programs. After assessing available open source software, the local team designed and implemented a modular proprietary EMR that is improving data quality and patient care. Despite the many benefits of existing open source medical record systems, clinical centers with complex workflow patterns--even those in resource-limited settings--should consider developing sustainable, local systems that fit their care model.