Home Isolated COVID-19 Patients Adhering To Recommended Guidelines: A Cross Sectional Survey In Karachi.

Objectives: To assess compliance level of coronavirus disease-2019 patients with recommended isolation guidelines. METHODS: The cross-sectional phone-based survey was conducted in Karachi, from March to October 2020 after approval from the ethics review board of Dow University of Health Sciences, Karachi, and comprised patients of either gender who had been tested positive and were advised home isolation due to mild/asymptomatic nature of their infection. Data was collected using a predesigned 42-item questionnaire in the light of the guidelines issued by the National Institutes of Health, Islamabad, Pakistan. Data was analysed using SPSS 20. RESULTS: Of the 450 patients approached, 305(68%) responded; 176(57.7%) females and 129(41.1%) males. The overall mean age was 35.16±14.15 years (range: 13-78 years). Of the total, 9(2.95%) patients did not isolate themselves at all, 51(16.7%) came into contact with other people, 75(24.6%) broke the home isolation and 69(22.6%) were sharing their rooms with other family members. Overall, 260(85.2%) participants were keeping themselves updated with the changes in the guidelines through conventional and social media. CONCLUSIONS: Coronavirus disease-2019 patients who were advised home isolation adhered to some but not all of the recommendations.

Cystic Fibrosis Transmembrane Conductance Regulator Protein Modulators in Children and Adolescents with different CF Genotypes - Systematic Review and Meta-Analysis.

OBJECTIVE: To determine the efficacy of the first triple CFTR protein modulators in children and adolescents with cystic fibrosis. METHODS: Systematic review and meta-analysis were conducted, following PRISMA guidelines. The following databases were searched extensively: PubMed/Medline, Clinical trials.gov, Google Scholar, Scopus, Embase, and Europe PMC using the keywords: "Ivacaftor," "Elexacaftor," "Tezacaftor," VX_661", VX_770", "VX_445", "cystic fibrosis". A total of ten randomized clinical trials were included in our analysis. Primary outcomes included: Absolute change in predicted FEV1 from baseline, Absolute change in sweat chloride test from baseline, Absolute change in BMI from baseline, Absolute change in CF-QR from baseline, and Adverse Events. RESULTS: Among primary findings, significant absolute change in predictive FEV1 from baseline through 4 weeks favoured the triple CFTR protein modulators. [MD=11.80,95%CI=8.47_15.12, p value=<0.00001]; as well as CF_QR score [MD=0.00,95%CI=-2.50_2.50, p value=1.00], and BMI kg/m² change [MD=16.90,95%CI=12.73_21.06, p value=<0.00001]. No significant change was noted for CFTR channels activity in the treatment group when compared to placebo or VX_770/VX_661 [MD= -12.57,95%CI=-94.46_69.32, p value=0.76]. CONCLUSION: In children aged ≥ 6 y old and adolescents with F508del_CFTR mutation, Elexacaftor-Tezacaftor-Ivacaftor tend to be more effective than first-generation therapy, demonstrating promising results by exhibiting significant improvement in lung function, body weight, and respiratory-related quality of life.

Information Seeking Practices and Availability of Smart Devices Among Healthcare Professionals in a Lower-Middle-Income Country: An Analysis From Pakistan.

Introduction Evidence-based medicine (EBM) is a principle that integrates clinical experience with relevant information available to provide adequate healthcare. It requires access to current medical literature. This paper analyzes the information requirements of a lower-middle-income country (LMIC) and the resources available and preferred by medical professionals. Methods A survey-based cross-sectional study was carried out among 160 participants, ranging in expertise from students to attending physicians in Karachi, Pakistan. The survey comprised questions to assess the clinical background, technology access, need for health-related information, and the preference for resources to obtain that information in different scenarios. They were also asked if they use PubMed and their recommended methods to improve information access. Statistical Package for the Social Sciences (SPSS; IBM, NY, USA) software was used for all analyses. Results A basic mobile phone (with limited internet connectivity) was the most common device used at home (n=159; 99.4%) and work (n=141; 88.1%). No smart devices were available to 28 (17.5%) participants at work. Internet connectivity was available for 155 (96.9%) participants at home but only for 118 (73.7%) participants at work. About one-third (n=49; 30.6%) experienced questions arising in practice two to four times a day, and half of the participants (n=80; 50%) were very likely to look up a reference. The most common resource for the majority of given clinical scenarios was a senior colleague. At the same time, medical websites (Medscape, Up-to-Date, WebMD) were the first choice for a non-specific general medical query. About 68.75% (n=110) of participants claimed to use PubMed in daily practice. The most common reason for not using PubMed was the ease of using other search engines (like Google). Conclusions Improved access to the internet and well-reputed journals can enhance the practice of EBM in Pakistan. Limitations of technological access must be considered while designing information resources in lower-middle-income countries.

The Frequency of Refractory Status Epilepticus and Its Outcome in a Tertiary Care Hospital in Pakistan: A Retrospective Study.

Background Refractory status epilepticus (RSE) is a common neurologic emergency with refractory cases leading to increased rates of morbidity and mortality in patients. The lack of previous studies on the incidence, causes, and management of refractory status epilepticus in the pediatric population from our region prompted us to investigate further in this study. Methods We included retrospective data of all patients admitted to the pediatric intensive care unit (PICU) with a provisional diagnosis of RSE at a tertiary care hospital in Karachi from February 2019 to February 2021. No personal identification data was used, and confidentiality of the data was maintained throughout the analysis. The Statistical Package for the Social Sciences (SPSS) software version 22.0 (IBM SPSS Statistics, Armonk, NY, USA) was used to pool data and perform a descriptive analysis. Results Among the 687 patients who presented to the PICU with seizures, 50 (7.27%) patients were eventually diagnosed with RSE during the two-year period. The majority of the patients were male and less than one year of age. Infectious causes predominated our data cohort, and a four-drug regimen consisting of phenytoin, levetiracetam, valproic acid, and midazolam was able to terminate RSE in the majority of the patients in our setting (70%). The mortality rate was noted to be 22% among patients with RSE. Conclusion Morbidity and mortality among pediatric RSE patients are high in our settings. Urgent emergency services and timely cause-directed intervention could improve outcomes.

Sirolimus vs tacrolimus: Which one is the best therapeutic option for patients undergoing liver transplantation for hepatocellular carcinoma?

Liver transplantation (LT) withstands as the most preferred therapeutic option for patients afflicted with hepatocellular carcinoma (HCC) and cirrhosis. To improve prognosis post-transplant, as well as to prevent the occurrence of rejection, a life-long immunosuppression strategy is implemented. The following letter to the editor highlights and provides novel evidence from recently published literature on topics discussed within the review article titled "Trends of rapamycin in survival benefits of liver transplantation for hepatocellular carcinoma" in World J Gastrointest Surg 2021; 13: 953-966. In the recent manuscript, the authors compared immunosuppressive drugs such as the newer option first-generation mammalian target of rapamycin inhibitor, also known as sirolimus, with the most widely used first-generation calcineurin inhibitors, such as tacrolimus (TAC). TAC is commonly known as the most effective immunosuppressive drug after LT, but it has been reported to cause intolerable side effects such as nephrotoxicity, neurotoxicity, diabetes, hypertension, gastrointestinal disturbances, increased risk of infections, and malignancies. It is necessary for physicians to be aware of recent advances in tacrolimus and sirolimus therapies to compare and understand distinctly the effectiveness and tolerability of these drugs. This will assist clinicians in making the best treatment decisions and improve the clinical prognosis of LT recipients with HCC.

Association between sex and Huntington's disease: an updated review on symptomatology and prognosis of neurodegenerative disorders.

Huntington's disease is a rare autosomal dominant disorder presenting with chorea, rigidity, hypo-/akinesia, cognitive decline, and psychiatric disturbances. Numerous risk factors have been defined in the onset of this disease. However, the number of CAG repeats in the genes are the most crucial factor rendering patients susceptible to the disease. Studies have shown significant differences in onset and disease presentation among the sexes, which prompts analysis of the impact of different sexes on disease etiology and progression. This article therefore discusses the evidence-based role of sex in aspects of symptomatology, pathogenesis, biomarkers, progression, and prognosis of Huntington's disease, with a secondary review of sex-linked differences in Alzheimer's and Parkinson's disease.

The Influence of Tomographic Corneal Characteristics on Epithelial Thickness Profile.

Purpose To understand the influence of tomographic corneal characteristics on the epithelium of normal eyes. Methods We scanned a total of 98 eyes of 98 individuals using anterior segment tomography and a spectral-domain optical coherence tomography (OCT) epithelial mapping tool. Only eyes with no previous pathology were included, with a refractive range of +5 diopters (D) to -6 D, intraocular pressure of < 22 mmHg, and no evidence of dry eye (Schirmer's test 2 value > 5 mm). Corneal curvature metrics were statistically correlated with regional epithelial thickness parameters. Results The anterior and posterior corneal surface flat and steep axis, the maximum and minimum curvature, corneal topographic astigmatism, astigmatism polar values, and corneal volume had no statistically significant correlation (p>0.05) with the epithelial thickness. Similarly, anterior corneal surface asphericity had no significant correlation. Posterior surface asphericity had a statistically significant moderate correlation with the epithelium in all areas. Similar results were seen in the multivariate analysis. Conclusions None of the front or back surface parameters had any influence on the corneal epithelium except for the posterior surface asphericity. This statistically significant yet clinically insignificant correlation may be enhanced in diseased populations like keratoconus and could indicate epithelial remodeling with early posterior corneal changes.