Category: Nevin Manimala

Orphanet J Rare Dis. 2024 May 3;19(1):186. doi: 10.1186/s13023-024-03178-x.

ABSTRACT

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare blood disorder, leading to various complications and impairments in patients’ health-related quality of life (HRQOL). Limited research has been conducted to evaluate the HRQOL of Chinese patients with PNH. Understanding the HRQOL in this specific population is crucial for providing effective healthcare interventions and improving patient’ health outcomes. This study aimed to assess HRQOL of Chinese patients with PNH, and identify key determinants.

METHODS: A cross-sectional study was conducted during 2022 to recruit patients with PNH in China. The study population was recruited from PNH China, one of the largest public welfare PNH patient mutual aid organization in China. Data were collected via an online questionnaire including the EQ-5D-5L (5L), and social-demographic and clinical characteristics. Descriptive statistics were employed to summarize the characteristics of the participants and their HRQOL. Multiple linear and logistic regression analyses were adopted to explore key factors affecting HRQOL.

RESULTS: A total of 329 valid questionnaires were collected. The mean (SD) age of the patients was 35.3 (10.0) years, with 52.3% of them being male. The patients reported more problems in Anxiety/Depression (81.5%) and Pain/Discomfort (69.9%) dimensions compared to the other three 5L dimensions. The mean (SD) of 5L health utility score (HUS) and EQ-VAS score were 0.76 (0.21) and 62.61 (19.20), respectively. According to multiple linear regression, initial symptoms (i.e., Anemia [fatigue, tachycardia, shortness of breath, headache] and back pain) and complication of thrombosis were significant influencing factors affecting 5L HUS. Total personal income of the past year, initial symptom of hemoglobinuria and complication of thrombosis were significantly influencing factors of VAS score. Social-demographic and clinical characteristics, such as gender, income, and thrombosis, were also found to be significantly related to certain 5L health problems as well.

CONCLUSION: Our study manifested the HRQOL of PNH patients in China was markedly compromised, especially in two mental-health related dimensions, and revealed several socio-demographic and clinical factors of their HRQOL. These findings could be used as empirical evidence for enhancing the HRQOL of PNH patients in China.

PMID:38702811 | DOI:10.1186/s13023-024-03178-x

Virol J. 2024 May 3;21(1):104. doi: 10.1186/s12985-024-02377-z.

ABSTRACT

BACKGROUND: Rift Valley fever (RVF) is a zoonotic viral disease of increasing intensity among humans in Africa and the Arabian Peninsula. In Uganda, cases reported prior to 2016 were mild or not fully documented. We report in this paper on the severe morbidity and hospital-based mortality of human cases in Uganda.

METHODS: Between November 2017 and March 2020 human cases reported to the Uganda Virus Research Institute (UVRI) were confirmed by polymerase chain reaction (PCR). Ethical and regulatory approvals were obtained to enrol survivors into a one-year follow-up study. Data were collected on socio-demographics, medical history, laboratory tests, potential risk factors, and analysed using Stata software.

RESULTS: Overall, 40 cases were confirmed with acute RVF during this period. Cases were not geographically clustered and nearly all were male (39/40; 98%), median age 32 (range 11-63). The median definitive diagnosis time was 7 days and a delay of three days between presumptive and definitive diagnosis. Most patients (31/40; 78%) presented with fever and bleeding at case detection. Twenty-eight (70%) cases were hospitalised, out of whom 18 (64%) died. Mortality was highest among admissions in regional referral (11/16; 69%) and district (4/5; 80%) hospitals, hospitalized patients with bleeding at case detection (17/27; 63%), and patients older than 44 years (9/9; 100%). Survivors mostly manifested a mild gastro-intestinal syndrome with nausea (83%), anorexia (75%), vomiting (75%), abdominal pain (50%), and diarrhoea (42%), and prolonged symptoms of severe disease including jaundice (67%), visual difficulties (67%), epistaxis (50%), haemoptysis (42%), and dysentery (25%). Symptom duration varied between two to 120 days.

CONCLUSION: RVF is associated with high hospital-based mortality, severe and prolonged morbidity among humans that present to the health care system and are confirmed by PCR. One-health composite interventions should be developed to improve environmental and livestock surveillance, prevent infections, promptly detect outbreaks, and improve patient outcomes.

PMID:38702807 | DOI:10.1186/s12985-024-02377-z

Arthritis Res Ther. 2024 May 3;26(1):93. doi: 10.1186/s13075-024-03325-6.

ABSTRACT

BACKGROUND: Anti-SS-A/Ro antibody (anti-SSA), the diagnostic marker of Sjögren’s syndrome (SS), is often detected in systemic sclerosis (SSc). Some patients are diagnosed with SSc/SS overlap syndromes, while there are anti-SSA-positive SSc cases without SS. In this study, we investigated the clinical characteristics of SSc with anti-SSA and clarified the clinical impact of this antibody in SSc.

METHODS: A retrospective chart review was conducted of 156 patients with SSc at Yokohama City University Hospital from 2018 to 2021. Clinical data, laboratory data, imaging, and autoantibody positivity status were collected and analysed to assess the association between these variables and anti-SSA using multivariable logistic regression analysis.

RESULTS: This cohort included 18 men and 138 women with SSc (median age, 69.0 years). Thirty-nine patients had diffuse cutaneous SSc (dcSSc) (25%), and 117 patients had limited cutaneous SSc (75%). Forty-four patients were anti-SSA-positive. Among them, 24 fulfilled the SS criteria. Multivariable logistic regression revealed that anti-SSA was statistically associated with interstitial lung disease (ILD; odds ratio [OR] = 2.67; 95% confidence interval [CI], 1.14-6.3; P = 0.024). Meanwhile, anti-SSA positivity tended to increase the development of digital ulcer (OR = 2.18; 95% CI, 0.99-4.82, P = 0.054). In the comparative analysis of the autoantibody single-positive and anti-SSA/SSc-specific autoantibody double-positive groups, the anti-SSA single-positive group showed a significantly increased risk of ILD (OR = 12.1; 95% CI, 2.13-140.57; P = 0.003). Furthermore, patients with SSc and anti-SSA indicated that anti-SSA-positive SSc without SS was strongly associated with dcSSc when compared to that in patients with SS (OR = 6.45; 95% CI, 1.23-32.60; P = 0.024).

CONCLUSIONS: Anti-SSA positivity increases the risk of organ involvement, such as ILD, in patients with SSc. Additionally, the anti-SSA-positive SSc without SS population may have more severe skin fibrosis than others. Anti-SSA may be a potential marker of ILD and skin severity in SSc.

PMID:38702799 | DOI:10.1186/s13075-024-03325-6

Global Health. 2024 May 3;20(1):37. doi: 10.1186/s12992-024-01040-0.

ABSTRACT

BACKGROUND: Cardiovascular diseases (CVDs) are estimated to be the leading cause of global death. Air pollution is the biggest environmental threat to public health worldwide. It is considered a potentially modifiable environmental risk factor for CVDs because it can be prevented by adopting the right national and international policies. The present study was conducted to synthesize the results of existing studies on the burden of CVDs attributed to air pollution, namely prevalence, hospitalization, disability, mortality, and cost characteristics.

METHODS: A systematic search was performed in the Scopus, PubMed, and Web of Science databases to identify studies, without time limitations, up to June 13, 2023. Exclusion criteria included prenatal exposure, exposure to indoor air pollution, review studies, conferences, books, letters to editors, and animal and laboratory studies. The quality of the articles was evaluated based on the Agency for Healthcare Research and Quality Assessment Form, the Newcastle-Ottawa Scale, and Drummond Criteria using a self-established scale. The articles that achieved categories A and B were included in the study.

RESULTS: Of the 566 studies obtained, based on the inclusion/exclusion criteria, 92 studies were defined as eligible in the present systematic review. The results of these investigations supported that chronic exposure to various concentrations of air pollutants, increased the prevalence, hospitalization, disability, mortality, and costs of CVDs attributed to air pollution, even at relatively low levels. According to the results, the main pollutant investigated closely associated with hypertension was PM_2.5. Furthermore, the global DALY related to stroke during 2016-2019 has increased by 1.8 times and hospitalization related to CVDs in 2023 has increased by 8.5 times compared to 2014.

CONCLUSION: Ambient air pollution is an underestimated but significant and modifiable contributor to CVDs burden and public health costs. This should not only be considered an environmental problem but also as an important risk factor for a significant increase in CVD cases and mortality. The findings of the systematic review highlighted the opportunity to apply more preventive measures in the public health sector to reduce the footprint of CVDs in human society.

PMID:38702798 | DOI:10.1186/s12992-024-01040-0

BMC Psychol. 2024 May 3;12(1):247. doi: 10.1186/s40359-024-01747-0.

ABSTRACT

BACKGROUND, OBJECTIVE AND HYPOTHESES: During emerging adulthood, vocational indecision (i.e., the inability to make coherent career choices) develops in a heterogeneous fashion, with three distinct patterns: low; decreasing (i.e., developmental or adaptative); high and stable or increasing (i.e., chronic or maladaptive). Among the determinants of vocational indecision that have been identified in past research, academic motivation is a crucial an excellent choice, since it is at school that students’ vocational choices are validated or not. According to SDT, this motivation can vary both in quantity and quality, and students tend to experience more positive academic outcomes when their motivational profile is optimal (high quantity, high quality) as opposed to suboptimal (e.g., low quantity, low quality). Thus, the purpose of this longitudinal study was to verify if the patterns found with emerging adulthood students characterized vocational indecision in adolescent students, and if supported, to predict the belonging to the most problematic trajectory by using students’ academic motivational profiles. We expected several distinct trajectories of vocational indecision that would differ in shape and magnitude, and several motivational profiles that vary in quality as well as in quantity. We also expected students in high-quality or quantity motivational profiles to be less likely to follow a chronic indecision trajectory.

METHOD AND RESULTS: Using data from 384 students (56% female; Mage = 13.52 years; SD = .52 at Secondary 2) surveyed annually from Secondary 2 to 5, person-centered analyses enabled estimation of motivational profile in Secondary 2 and vocational indecision trajectories during the 4-year period. Results revealed four distinct patterns of vocational indecision during adolescence labelled Low and Stable, Moderate and Stable, Developmental and Chronic Intermittent. Four motivational profiles were also identified in Secondary 2, ranging from poor (Highly Amotivated) to moderate (Autonomous-Introjected) quality of self-determination level. Also, in reference to the most self-determined profile, students in the Mixed profile were at greatest risk of following Chronically-Intermittently Undecided trajectory. Finally, the most self-determined students were at greatest probability of following the Developmentally Undecided trajectory.

CONCLUSION: Overall, the findings suggest that the student motivational functioning in early secondary school years could be used to identify students at risk of experiencing the negative indecision patterns across secondary school. Several theoretical and practical implications are suggested.

PMID:38702790 | DOI:10.1186/s40359-024-01747-0

J Neurosurg. 2024 May 3:1-8. doi: 10.3171/2024.2.JNS232752. Online ahead of print.

ABSTRACT

OBJECTIVE: Evaluation of the demographic and academic characteristics of current neurosurgery residents may provide prospective students with insight into factors that affect research output. Therefore, this study aimed to evaluate the research output among neurosurgery residents.

METHODS: US neurosurgery residency programs were abstracted from the American Association of Neurological Surgeons website. Demographic data on 1690 current residents across 119 programs were collected using publicly available institutional websites, Doximity, and LinkedIn. The h-index of each resident was recorded using Scopus and exported into the NIH iCite tool to determine the weighted relative citation ratio (w-RCR) and mean relative citation ratio (m-RCR). The total number of publications, h-index, and w-RCR were used as a proxy for research output, while m-RCR was used to measure research impact. One-way ANOVA and Kruskal-Wallis H-tests were used to assess the statistical significance of relationships between demographic data and measures of research activity.

RESULTS: A total of 1690 residents (25.4% female), representing 119 programs, were evaluated. Neurosurgery residents had an average of 17 publications, h-index of 5.5, m-RCR of 1.4, and w-RCR of 16.9, with an upward trend of research activity by postgraduate year (PGY) class. Male residents on average had a greater total number of publications (p < 0.001), higher h-index (p < 0.001), and higher w-RCR (p = 0.002) compared with their female peers. Significant differences in research activity were also observed by degree (Doctor of Medicine [MD], Doctor of Osteopathy [DO], or other), where those with MD and other degrees had higher metrics than those with DO degrees. International medical graduates (IMGs) also had higher research output than American medical graduates (AMGs) (p < 0.001). Differences in all measures of research activity except impact were also observed in research activity when pre-residency medical school ranks were compared.

CONCLUSIONS: The authors observed overall high research activity among neurosurgery residents. Factors such as gender, degree, PGY, IMG/AMG status, and medical school rank may therefore be related to the success of matching within neurological surgery. Although large disparities in gender representation have been identified in neurosurgery, newer classes are trending toward shrinking the gap. These data may be used by prospective residents to gauge changes and progress occurring in the neurosurgery match.

PMID:38701523 | DOI:10.3171/2024.2.JNS232752

Hepatol Commun. 2024 May 3;8(5):e0443. doi: 10.1097/HC9.0000000000000443. eCollection 2024 May 1.

ABSTRACT

BACKGROUND: Frailty is a predictive factor of hospitalization, falls, and mortality in patients with cirrhosis, regardless of the degree of liver failure. The aim was to analyze whether a multifactorial intervention consisting of home-based exercise, branched-chain amino acids, and a multistrain probiotic can improve frailty in these patients.

METHODS: Outpatients with cirrhosis were classified according to the Liver Frailty Index (LFI). Prefrail and frail patients were randomized into 2 groups. The intervention group was assigned to a multifactorial intervention consisting of exercise at home, branched-chain amino acid supplements, and a multistrain probiotic for 12 months. The control group received standard care. All patients were prospectively followed up every 3 months for 1 year to determine LFI, incidence of falls, emergency room visits, hospitalizations, and mortality.

RESULTS: Thirty-two patients were included: 17 patients were assigned to the intervention group and 15 to the control group. In the intervention group, the baseline LFI decreased at 3, 6, 9, and 12 months (p = 0.019 for overall change with respect to the control group). The change in LFI (ΔLFI) at 12 months was -0.71 ± 0.24 in the intervention group and -0.09 ± 0.32 in the control group (p<0.001). During follow-up, patients in the intervention group had a lower 1-year probability of falls (6% vs. 47%, p = 0.03) and emergency room visits (10% vs. 44%, p = 0.04) than patients in the control group.

CONCLUSIONS: A long-term multifactorial intervention that included exercise at home, branched-chain amino acids, and a multistrain probiotic improved frailty in outpatients with cirrhosis and was associated with a decrease in the incidence of clinical events such as falls and emergency room visits.

PMID:38701490 | DOI:10.1097/HC9.0000000000000443

Phys Rev Lett. 2024 Apr 19;132(16):164001. doi: 10.1103/PhysRevLett.132.164001.

ABSTRACT

We study fluctuations of the local energy cascade rate Φ_{ℓ} in turbulent flows at scales (ℓ) in the inertial range. According to the Kolmogorov refined similarity hypothesis (KRSH), relevant statistical properties of Φ_{ℓ} should depend on ε_{ℓ}, the viscous dissipation rate locally averaged over a sphere of size ℓ, rather than on the global average dissipation. However, the validity of KRSH applied to Φ_{ℓ} has not yet been tested from data. Conditional averages such as ⟨Φ_{ℓ}|ε_{ℓ}⟩ as well as of higher-order moments are measured from direct numerical simulations data, and results clearly adhere to the predictions from KRSH. Remarkably, the same is true when considering forward (Φ_{ℓ}>0) and inverse (Φ_{ℓ}<0) cascade events separately. Measured ratios of forward and inverse cascade probability densities conditioned on ε_{ℓ} also confirm the applicability of the KRSH to analysis of the fluctuation relation from nonequilibrium thermodynamics.

PMID:38701479 | DOI:10.1103/PhysRevLett.132.164001

Phys Rev Lett. 2024 Apr 19;132(16):168201. doi: 10.1103/PhysRevLett.132.168201.

ABSTRACT

We study a model chiral fluid in two dimensions composed of Brownian disks interacting via a Lennard-Jones potential and a nonconservative transverse force, mimicking colloids spinning at a given rate. The system exhibits a phase separation between a chiral liquid and a dilute gas phase that can be characterized using a thermodynamic framework. We compute the equations of state and show that the surface tension controls interface corrections to the coexisting pressure predicted from the equal-area construction. Transverse forces increase surface tension and generate edge currents at the liquid-gas interface. The analysis of these currents shows that the rotational viscosity introduced in chiral hydrodynamics is consistent with microscopic bulk mechanical measurements. Chirality can also break the solid phase, giving rise to a dense fluid made of rotating hexatic patches. Our Letter paves the way for the development of the statistical mechanics of chiral particles assemblies.

PMID:38701478 | DOI:10.1103/PhysRevLett.132.168201

Phys Rev Lett. 2024 Apr 19;132(16):160201. doi: 10.1103/PhysRevLett.132.160201.

ABSTRACT

Quantum theory allows information to flow through a single device in a coherent superposition of two opposite directions, resulting into situations where the input-output direction is indefinite. Here we introduce a theoretical method to witness input-output indefiniteness in a single quantum device, and we experimentally demonstrate it by constructing a photonic setup that exhibits input-output indefiniteness with a statistical significance exceeding 69 standard deviations. Our results provide a way to characterize input-output indefiniteness as a resource for quantum information and photonic quantum technologies and enable tabletop simulations of hypothetical scenarios exhibiting quantum indefiniteness in the direction of time.

PMID:38701466 | DOI:10.1103/PhysRevLett.132.160201