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Abstract Background Real-world studies of the burden of severe haemophilia B in the context of recent therapeutic advances such as extended half-life (EHL) factor IX (FIX) products are limited. We analysed data from the recent CHESS II study to better understand the clinical, humanistic, and economic burden of severe haemophilia B in Europe. Data from male adults with severe haemophilia B receiving prophylaxis were analysed from the retrospective cross-sectional CHESS II study conducted in Germany, France, Italy, Spain and the United Kingdom. Inhibitors were exclusionary. Patients and physicians completed questionnaires on bleeding, joint status, quality of life, and haemophilia-related direct and indirect costs (2019–2020). All outcomes were summarised using descriptive statistics. Results A total of 75 CHESS II patients were eligible and included; 40 patients (53%) provided self-reported outcomes. Mean age was 36.2 years. Approximately half the patients were receiving EHL versus standard half-life (SHL) prophylaxis (44% vs 56%). Most patients reported mild or moderate chronic pain (76%) and had ≥ 2 bleeding events per year (70%), with a mean annualised bleed rate of 2.4. Mean annual total haemophilia-related direct medical cost per patient was €235,723, driven by FIX costs (€232,328 overall, n = 40; €186,528 for SHL, €290,620 for EHL). Mean annual indirect costs (€8,973) were driven by early retirement or work stoppage due to haemophilia. Mean quality of life (EQ-5D) score was 0.67. Conclusions These data document a substantial, persistent real-world burden of severe haemophilia B in Europe. Unmet needs persist for these patients, their caregivers, and society.

Bernard Soulier Syndrome is a genetically inherited platelet disorder that commonly presents with symptoms of impaired blood coagulation, such as epistaxis, menorrhagia, and petechiae formation. Here we present a case of Bernard Soulier Syndrome in which the individual has presented with melena, which is the appearance of black tarry stools due to bleeding from the upper gastrointestinal tract. This presentation is rare and should be discussed so that appearance of the less common symptoms can be caught early, leading to an early diagnosis and consequently earlier and more effective management options.

Abstract Background Hemophilia B is a rare congenital bleeding disorder that has a significant negative impact on patients’ functionality and health-related quality of life. The standard of care for severe hemophilia B in the United States is prophylactic factor IX replacement therapy, which incurs substantial costs for this lifelong condition. Accurate estimates of the burden of hemophilia B are important for population health management and policy decisions, but have only recently accounted for current management strategies. The ‘Cost of Severe Hemophilia across the US: a Socioeconomic Survey’ (CHESS US) is a cross-sectional database of medical record abstractions and physician-reported information, completed by hematologists and care providers. CHESS US+ is a complementary database of completed questionnaires from patients with hemophilia. Together, CHESS US and CHESS US+ provide contemporary, comprehensive information on the burden of severe hemophilia from the provider and patient perspectives. We used the CHESS US and CHESS US+ data to analyze the clinical, humanistic, and economic burden of hemophilia B for patients treated with factor IX prophylaxis between 2017 and 2019 in the US. Results We conducted analysis to assess clinical burden and direct medical costs from 44 patient records in CHESS US, and of direct non-medical costs, indirect costs, and humanistic burden (using the EQ-5D-5L) from 57 patients in CHESS US+. The mean annual bleed rate was 1.73 (standard deviation, 1.39); approximately 9% of patients experienced a bleed-related hospitalization during the 12-month study period. Nearly all patients (85%) reported chronic pain, and the mean EQ-5D-5L utility value was 0.76 (0.24). The mean annual direct medical cost was $614,886, driven by factor IX treatment (mean annual cost, $611,971). Subgroup analyses showed mean annual costs of $397,491 and $788,491 for standard and extended half-life factor IX treatment, respectively. The mean annual non-medical direct costs and indirect costs of hemophilia B were $2,371 and $6,931. Conclusions This analysis of patient records and patient-reported outcomes from CHESS US and CHESS US+ provides updated information on the considerable clinical, humanistic, and economic burden of hemophilia B in the US. Substantial unmet needs remain to improve patient care with sustainable population health strategies.

IntroductionThe present study examined the moderating effects of sleep quality in the relationship between coping and distress among medical college students. Present study was conducted to ensure the mental health of medical students and to dig out the reasons behind their disturbed health which can directly impact their performance at work.MethodsThe study utilized a cross-sectional survey and was distributed to students at various medical institutions in the Punjab province of Pakistan from October 2019 to June 2020. The sample comprised 369 participants (120 males; 32.5%). The survey included the Pittsburgh Sleep Quality Index (PSQI), Kessler Scale of Psychological Distress (K10), Brief Cope Scale, and Wong and Law Emotional Intelligence Scale.ResultsThe results showed there was a significant relationship between coping and distress. More specifically, adaptive coping and distress were negatively associated (r=-.24), and maladaptive coping and distress were positively associated (r=.46). Moreover, the present study found that poor sleep quality was a significant positive predictor of distress. Moderation analysis showed that sleep quality was a significant moderator in the relationship between adaptive coping and distress (ΔR2=.011, β=-.36, p

BackgroundMigraine is a neurological condition marked by frequent headaches, which tends to be accompanied by nausea and vomiting in severe instances. Injectable therapies for migraine, such as monoclonal antibodies that target calcitonin gene-related peptide (CGRP), have proven to be effective and safe. While various oral drugs are available, none have been developed for migraines. Patients prefer oral therapies because they are easier to use, making atogepant, an orally accessible small-molecule CGRP receptor antagonist, a possible alternative.ObjectivesThis systematic review and meta-analysis compared the safety and effectiveness of atogepant with placebo in treating migraine.MethodsAdhering to the PRISMA guidelines, we meticulously gathered randomized controlled trials (RCTs) from databases including the Cochrane Library, PubMed, Science Direct, and ClinicalTrials.gov. Studies comparing atogepant with placebo and reporting monthly migraine days (MMDs) as the primary outcome along with secondary outcomes such as monthly headache days and acute medication use days were included. Two independent reviewers conducted the data extraction and quality assessment. Statistical analyses were carried out using RevMan, utilizing risk ratios for dichotomous outcomes and mean differences for continuous outcomes, and a random-effects model.ResultsOur primary outcome was the change in MMDs over 12 weeks, which showed a significant reduction with atogepant at dosages of 10, 30, and 60 mg. Secondary outcomes, such as monthly headache days, proportion of patients achieving a ≥ 50% reduction in MMDs, acute medication use days, and patient-reported outcomes, consistently showed that atogepant outperformed placebo, highlighting its effectiveness in reducing the migraine burden.ConclusionHigher doses of atogepant are more effective in lowering migraine and headache-related days and increasing quality of life metrics. However, this is accompanied by an increased incidence of adverse events, suggesting the need for careful dose optimization to balance the benefits and risks.Systematic review registrationhttps://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=563395. Unique Identifier: CRD42024563395.

Background: Valvular heart disease (VHD) represents a spectrum of cardiac conditions, including valvular stenosis, valvular regurgitation, or mixed lesions affecting single or multiple valves. The severity of VHD has emerged as a major cause of cardiovascular (CV) morbidity and mortality among the older population in the United States (U.S). Objective: To evaluate temporal trends in mortality associated with VHD in the elderly U.S population between 1999 and 2019. Methods: We utilized the CDC WONDER database for VHD mortality in adults ≥75 from 1999 to 2019, using ICD-10 codes. Age-adjusted mortality rates (AAMR) per 100,000 people with associated annual percentage change (APC) were calculated. Joinpoint regression was used to assess the overall trends and trends for demographic, geographic, and type of valvular disease subgroups. Results: A total of 666,765 VHD deaths in older adults from 1999 to 2019 was identified, with an initial decline in AAMR until 2007 with an APC: 0.62, 95 % CI (−1.66-0.33), stability until 2014, and a significant decrease until 2019 (APC: 1.47, 95 % CI [-2.24-1.04], P

BackgroundPatients with prior cardiac surgery undergoing acute type A aortic dissection (ATAAD) are thought to have worse clinical outcomes as compared to the patients without prior cardiac surgery.AimTo compare the safety and efficacy of ATAAD in patients with prior cardiac surgery.MethodsWe systematically searched PubMed, Cochrane Library and Google Scholar from database inception until April 2024. We included nine studies which consisted of a population of 524 in the prior surgery group and 5,249 in the non-prior surgery group. Our primary outcome was mortality. Secondary outcomes included reoperation for bleeding, myocardial infarction, stroke, renal failure, sternal wound infection, cardiopulmonary bypass (CPB) time, cross-clamp time, hospital stay, and ICU stay.ResultsOur pooled estimate shows a significantly lower rate of mortality in the non-prior cardiac surgery group compared to the prior cardiac surgery group (RR = 0.60, 95% CI = 0.48–0.74). Among the secondary outcomes, the rate of reoperation for bleeding was significantly lower in the non-prior cardiac surgery group (RR = 0.66, 95% CI = 0.50–0.88). Additionally, the non-prior cardiac surgery group had significantly shorter CPB time (MD = −31.06, 95% CI = −52.20 to −9.93) and cross-clamp time (MD = −21.95, 95% CI = −42.65 to −1.24). All other secondary outcomes were statistically insignificant.ConclusionPatients with prior cardiac surgery have a higher mortality rate as compared to patients who have not undergone cardiac surgery previously. Patients with prior cardiac surgery have higher mortality and longer CPB and cross-clamp times. Tailored strategies are needed to improve outcomes in this high-risk group.

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder with risk of sudden cardiac death (SCD) in children and adolescents. Mavacamten, also referred to as MYK-461, a myosin inhibitor of cardiac myocytes is studied in symptomatic HCM. The safety and efficacy of this medication is not well studied in pooled meta-analysis. Online database search was performed from inception to September 2023. We selected randomized clinical trials that compared Mavacamten with placebo/guideline medical treatment for HCM. We studied safety outcomes (Serious adverse events (SAEs), treatment emergent adverse events (TEAs) and Atrial fibrillation). Functional status of patients was assessed as New York Heart Association (NYHA) Classification improvement of at least + 1 grade, Kansas City Cardiomyopathy Questionnaire Clinical Summary Score (KCCQ-CSS) change from baseline). Relative risk ratios were used in randomized model using Review Manager Version 5.4 statistical software. A total of 4 RCTs comprising 503 patients were included in meta-analysis. On random effect model, we found that HCM patients that received Mavacamten had significant symptomatic improvement as depicted by improvement in NYHA class by at least + 1 grade (RR = 2.15; P

IntroductionMajor depressive disorder (MDD), postpartum depression (PPD), and insomnia are neuropsychological conditions in which zuranolone is used to improve symptoms and prognosis of the disorder. This meta-analysis aimed to determine the efficacy of zuranolone in comparison to other drugs used for treating these conditions.MethodsThis meta-analysis included patients aged between 18 and 75 years who were diagnosed with major depressive disorder and postpartum depression with or without insomnia and were administered zuranolone for treatment. Only randomized controlled trials (RCTs) were included, and animal studies were excluded. The databases used were PubMed, Scopus, Cochrane, and Clinicaltrials.gov, with MeSH terms and relevant keywords for (Zuranolone) and (Depression). The Cochrane risk of bias tool was used for quality assessment.ResultsThe meta-analysis included eight RCTs that analyzed data from 2031 patients. The meta-analysis revealed statistically significant changes in the Hamilton Depression Rating Scale (HAM-D), Montgomery-Åsberg Depression Rating Scale (MADRS), Hamilton Anxiety Rating Scale (HAM-A), and treatment-emergent adverse effects (TEAE) scores in the PPD subgroup. HAM-D and TEAEs scores were also significant in the MDD subgroup, but the changes in the MADRS, HAM-A, and Bech-6 scores were insignificant. Serious adverse events were insignificant in all subgroups.ConclusionMeta-analysis found a significant improvement in depressive symptoms with zuranolone treatment, especially on day 15. This suggests that zuranolone is a promising therapeutic option for patients with MDD and PPD with or without insomnia.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=459554, identifier CRD42023459554.

Abstract Introduction An unusual association between thyroid dysfunction and autoimmune encephalitis (AE) was noticed when patients presented with low free triiodothyronine (fT3) levels and antithyroid antibodies. We conducted a meta‐analysis to investigate whether thyroid dysfunction, that is, lower fT3 levels are associated with worsening clinical manifestations and prognosis in patients with AE. Methods Literature search of five electronic databases was performed till April 5, 2023. Inclusion criteria were as follows: Observational studies reporting patients with all subtypes of AE and assessing thyroid dysfunction categorized as low fT3 and non‐low fT3. Primary endpoints included modified Rankin scale (mRS) at admission, abnormal magnetic resonance imaging, length of stay, seizures, and consciousness declination. Results Comprehensive literature search resulted in 5127 studies. After duplicate removal and full‐text screening, six observational studies were included in this analysis. Patients with low fT3 were 2.95 times more likely to experience consciousness declination (p = .0003), had higher mRS at admission (p

IntroductionSalmonella typhi, a gram-negative bacterium responsible for typhoid fever, can infect the inner lining or valves of the heart and cause endocarditis. This systematic review aimed to report cases of S. typhi-associated endocarditis and its clinical features.MethodsThis systematic review was reported as per the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) checklist. Only case reports and case series of endocarditis caused by S. typhi, irrespective of age, gender, and demographics, were considered eligible for inclusion. To identify relevant studies, a literature search was conducted using relevant keywords on PubMed, Google Scholar, and the Cochrane Library from inception to 31 December 2023. After selecting the studies, the relevant data were extracted and pooled in terms of frequencies and percentages. A quality assessment was performed using the Joanna Briggs Institute Critical Appraisal Checklist for Case Reports.ResultsThis review included seven case reports, comprising 22.2% female and 77.8% male patients. The mean age of patients was 27.9 + 12.0 years. Regarding past medical history, 33.3% (3/9) of patients had a previous cardiac pathology. Fever remained the most common complaint, occurring in 88.9% of cases. Transthoracic and transesophageal echocardiography were used to diagnose all cases, with 33.3% identifying vegetation on the mitral, aortic, and tricuspid valves. Ceftriaxone, with or without gentamycin, remained the choice of antibiotic for 88.9% of cases, and all patients responded to the offered treatment.ConclusionS. typhi-associated endocarditis, though rare, presents unique challenges and requires timely diagnosis. This systematic review of seven cases highlights a predominantly male population affected, with a mean age in the third decade, suggesting a higher invasiveness than other causes. The findings from this study underscore the importance of early recognition and appropriate management, primarily with antibiotic therapy. Further research with larger cohorts is crucial to refine understanding and guide policymaking for this rare but life-threatening condition.

Abstract The current conflict in Sudan severely hinders the accessibility of health services across the country. To address this, several initiatives were proposed including offering services using teleconsultations. This study aimed to assess Sudanese doctors' teleconsultation experience, perception, and concerns during the recent conflict. This cross-sectional survey focused on Sudanese medical officers, residents, specialists, and consultants living inside or outside the country having a practice license from the Sudan Medical Council and conducting teleconsultations with Sudanese patients during the conflict period. The questionnaire was distributed to personal and professional contacts and via social media platforms in the English language among doctors who provided teleconsultation during the conflict. Data analysis was performed using the Statistical Package for Social Sciences software version 26. The study enrolled 2463 clinicians from 17 different specialties, and females represented more than half the sample (56.8%). Internal medicine was the most frequent specialty (36.1%) and the majority (68.7%) of clinicians had less than 5 years of work experience. Voice call was the most frequent platform (50.1%) used for teleconsultation during the conflict and had the highest convenience score (p

Background: The therapeutic effectiveness of interleukin-6 receptor inhibitor in critically ill hospitalized patients with coronavirus disease 2019 (COVID-19) is uncertain. Methods: To evaluate the efficacy and safety of the outcome as recovery or death of tocilizumab for severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection, we conducted a randomized, double-blinded, placebo-controlled phase 2 trial in critically ill COVID-19 adult patients. The patients were randomly assigned in a 4:1 ratio to receive standard medical treatment plus the recommended dose of either tocilizumab or the placebo drug. Randomization was stratified. The primary outcome was the recovery or death after administration of tocilizumab or a placebo drug. The secondary outcomes were clinical recovery or worsening of the patients' symptoms and inflammatory markers and discharge from the hospital. Results: Of 190 patients included in this study, 152 received tocilizumab, and 38 received a placebo. The duration of hospital stay of the interventional group was 12.9 ± 9.2, while the placebo group had a more extended hospital stay (15.6 ± 8.8). The mortality ratio for the primary outcome, ie, mortality or recovery in the tocilizumab group was 17.8%; p = 0.58 by log-rank test). The mortality ratio in the placebo group was 76.3%; p = 0.32 by log-rank test). The inflammatory markers in the tocilizumab group significantly declined by day 16 compared to the placebo group. Conclusions: The use of tocilizumab was associated with decreased mortality, earlier improvement of inflammatory markers, and reduced hospital stay in patients with severe COVID-19.

Objectives. Neuroleptic malignant syndrome (NMS) is a life-threatening adverse reaction of antipsychotic drugs, especially of dopamine receptor antagonists (DRA’s). Excitation, exuberant behavior, violent behavior and lack of insight predispose the patients to the use of depot preparations and high doses of the antipsychotics. These behaviors also make the patients suffer from dehydration leading to cognitive impairment, confusion and incontinence. In addition to clinical and pharmacological risk factors, legal and ethical risk factors may be contributory towards the incidence, diagnosis and prognosis of NMS in Pakistan. Material and methods. This study was conducted as a descriptive, observational study to determine the frequency of deranged pattern of clinical and laboratory feature patients presenting with Neuroleptic Malignant Syndrome at Department of Neurology, Jinnah Postgraduate Medical Center (JPMC), Karachi. Data was prospectively collected from patients after taking a verbal consent. 55 patients who met the diagnostic criteria were included. Quantitative data was presented as simple descriptive statistics giving mean and standard deviation and qualitative variables was presented as frequency and percentages. Effect modifiers were controlled through stratification to see the effect of these on the outcome variable. Post stratification chi square test was applied taking p-value of ≤0.05 as significant. Outcomes. A total of 55 patients who met the inclusion and exclusion criteria were included in this study. Mean age, duration of NMS symptoms, length of hospital stay, SBP and DBP in our study was 39.41±12.67 years, 10.56±7.29 hours, 9.74±3.21 days, 135.87±10.97 mmHg and 83.21±6.42 mmHg. 24 (43.6%) and 31 (56.4%) were male and female. Out of 55 patients, 81.8%, 38.2%, 69.1%, 56.4%, 47.3%, 74.5% and 25.5% had fever, autonomic dysfunction, EPS symptoms, altered GCS, elevated CPK, elevated WBC and abnormal LFT. Conclusions. NMS is an important preventable clinical entity. Early diagnosis and judicious use of antipsychotics is warranted to prevent mortality and heightened morbidity.

Introduction: During the pandemic, sedentary behaviors, anxiety, and boredom caused by confinement at home could affect lifestyle patterns, degrade diet quality, and promote overconsumption. This cross-sectional study aimed to identify the impact of lockdowns on lifestyle behaviors among Pakistan's population and changes in the nutritional and eating patterns. Materials and Methods: This is an online, questionnaire-based, cross-sectional study. The study collected demographic information, dietary habits, and lifestyle patterns. Descriptive and summary statistics were used to describe data, and Chi-square or Fisher's exact tests were used for associations. Results: Most respondents were females (82.1%) aged 18–29 years (98.1%). 90.3% of them were students. 16.0% were overweight pre-pandemic, which increased to 20.0% during the pandemic. The majority ate more during the pandemic (P < 0.001). 47.9% reported a physical activity duration of 0.5–2 h pre-pandemic, and 50.6% reported decreased physical activity during the pandemic, with increased food intake (P = 0.007). Screen time was

Objective: To determine the frequency of skeletal dysplasia in children with short stature presenting to the endocrine clinic of a tertiary care hospital. Methods: This descriptive cross-sectional study was performed in the Outpatient Department of Endocrinology of National Institute of Child Health, Karachi, for 6 months of duration. A total of 200 children coming to endocrine OPD of NICH of either gender, having the age less than 14 years and height more than -2.5 SD below the mean (

Introduction: Diabetes and thyroid disorders are the two most common disorders amongst endocrine diseases. Uncontrolled diabetes mellitus (DM) may disturb thyroid metabolism by disturbing the levels of thyroid hormones in the plasma. This study was undertaken to determine the prevalence and thyroid dysfunction in type 2 diabetic population presenting in a public sector tertiary care teaching hospital. Materials and Methods: This descriptive cross-sectional study was conducted in Civil Hospital Karachi (CHK) in the department of medicine from October 01, 2018, to March 31, 2019. The study population consisted of 317 patients diagnosed with DM type 2 based on American Diabetes Association (ADA) criteria. Patients with fasting blood sugar ≥ 126 mg/dL, or HbA1c >6.5 (%), and 2 h postprandial blood glucose levels >200 mg/dL were included. After a minimum of 8 h of fasting, plasma samples of patients were collected and sent for fasting blood glucose, HbA1c, FT3, FT4, and thyroid-stimulating hormone (TSH). Descriptive statistics were calculated. Post-stratification Chi-squared test was applied, and a P value of ≤ 0.05 was considered significant. Results: Among all enrolled DM type 2 patients, 207 (65.3%) were male with a mean age of 46.54 ± 8.72 years. Mean fasting blood sugar (FBS), random blood sugar (RBS), and HbA1c were 212.76 ± 26.91 (mg/dL), 328.89 ± 52.89 (mg/dL), and 9.43 ± 2.47 (%), respectively. The mean duration of DM was 7.81 ± 3.15 years. Mean FT3, FT4, and TSH were 113.13 ± 44.40 (ng/dL), 7.63 ± 3.11 (mg/dL) and 2.64 ± 2.57 (μIU/mL), respectively. In the present study, 55 (17.4%) patients had subclinical hypothyroidism, 27 (8.5%) had hypothyroidism, 19 (6.0%) had hyperthyroidism, and 16 (5.0%) had subclinical hyperthyroidism. Significant association of thyroid dysfunction was established with age group, female gender, and family history of thyroid dysfunction (P =< 0.001). Conclusion: The prevalence of thyroid dysfunction is higher among patients with type 2 diabetes mellitus (T2DM), in which hypothyroidism was the most common dysfunction with more prevalence among female patients.

This paper focuses on the trends of self-medication practices in treating symptoms that may lead to fatal complications in dengue. As dengue is a viral infection with increasing incidence, decision regarding its treatment is mostly affected by public health believes and practices to self-treat the condition by different home remedies, over-the-counter (OTC) drugs or using outdated prescription drugs that proved beneficial in the past experience. Poverty, lack of education, and poor access to health facilities pave the way for making such decisions. Future complications can be averted by raising awareness, counseling the patients and dispensing of pharmaceuticals with strict monitoring.

Objectives. To determine the frequency of depression in patients with idiopathic parkinsonism presenting to a tertiary care Hospital in Karachi. Material and methods. This case study was conducted at the neurology department Jinnah Postgraduate Medical Centre, Karachi (JPMC). The duration of the study was six months from 22nd January 2019 to 2nd June 2019. A total of 114 patients of parkinsonism (idiopathic Parkinson’s disease) were included in this study. Patients were assured of confidentiality. They were given questionnaire with Beck depression inventory while waiting in the neurology outpatient clinic. Questionnaire was taken back after 25 minutes. Patient score more than 9 was diagnosed as depression. The identified depressed patient was offered treatment. Results. Frequency of depression in patients with idiopathic Parkinson’s disease was observed in 48.25% (55/114) cases. Conclusions. It is concluded that our study indicates the burden of depression in Parkinson disease (PD) patients. However, even with stable or mild deficit in motor function, the wide prevalence of depression indicates that it should be suspected and treated. Over the past several years, systematic studies of depression and its treatment have contributed significantly to this most challenging psychiatric problem in PD. Hence, there is a need of policy for screening and prompt treatment of such patients so they could lead to enhance quality of life.

Abstract Acute fatty liver disease of pregnancy (AFLP) is a rare condition associated with other common liver manifestations such as hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP). We present a 27‐year‐old pregnant woman who developed hepatic encephalopathy and DIC after being diagnosed with Acute fatty liver disease of pregnancy.

Background: Novel psychoactive substances (NPSs) are relatively new substances in the illicit drug market, not previously listed in the United Nations Office on Drugs and Crime (UNDOC). Strox and Voodoo are considered some of the most popular blends of NPS in the Egyptian drug market. Objectives: The current study was conducted to assess NPS's use pattern: Voodoo and Strox among acutely intoxicated patients presented to the poison control center of Ain Shams University Hospitals (PCC- ASUH). Methods: A single center based cross-sectional study was carried out in the PCC-ASUH among acutely intoxicated patients presenting to the emergency department (ED) over four months (from January–April 2019. using a previously adopted and validated Fahmy and El-Sherbini socioeconomic scale (SES). Data were presented as mean, median and range as appropriate. Both smoking and crowding indexes were calculated and presented as previously reported. Results: Fifty-one patients were presented to the ED of PCC-ASUH during the study period. A total of 96.1% (n = 49) were males. The mean age was 25 ± 7.5 years. The most common NPS used was Strox: 54.9% (n = 28), followed by Voodoo: 27.4% (n = 14). Neurological and gastrointestinal (GI) symptoms were the most frequent presentations. The most common motive behind NPS use was the desire to give a trial of new psychoactive substances. The mean SES score was 35.1 ± 13.17. Most patients have the preparatory as the highest education 36.0% (n = 18). Conclusions: NPS use is common among young males in preparatory education from different social classes, starting it most commonly as a means to experiencing a new high. Neurological and GI manifestations are the most common presenting symptoms of NPS intoxication.

Abstract. Rationale:. Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barre syndrome, classically diagnosed based on the clinical triad of ataxia, areflexia, and ophthalmoplegia. MFS is usually preceded by viral infections and febrile illness; however, only a few cases have been reported after vaccinations. Patient concerns:. A 53-year-old hypertensive male presented with a 2-day history of progressive ascending paralysis of the lower limbs along with diplopia and ataxia, 8 days after the first dose of the Sinovac–Coronavac coronavirus disease 2019 (COVID-19) vaccination, with no prior history of any predisposing infections or triggers. Diagnoses:. Physical examination showed moderate motor and sensory loss with areflexia in the lower limbs bilaterally. Routine blood investigations and radiological investigations were unremarkable. Cerebrospinal fluid analysis showed albuminocytologic dissociation and nerve conduction studies revealed prolonged latencies with reduced conduction velocities. The diagnosis of MFS was established based on the findings of physical examination, cerebrospinal fluid analysis, and nerve conduction studies. Interventions:. A management plan was devised based on intravenous immunoglobulins, pregabalin, and physiotherapy. However, due to certain socioeconomic factors, the patient was managed conservatively with regular physiotherapy sessions. Outcomes:. Follow-up after 6 weeks showed remarkable improvement, with complete resolution of symptoms 10 weeks after the discharge. Lessons:. This case suggests that MFS is a rare adverse effect after COVID-19 vaccination and additional research is required to substantiate a temporal association. Further studies are needed to understand the pathophysiology behind such complications to enhance the safety of COVID-19 vaccinations in the future.

OBJECTIVES:. To describe the prevalence, associated risk factors, and outcomes of serious neurologic manifestations (encephalopathy, stroke, seizure, and meningitis/encephalitis) among patients hospitalized with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. DESIGN:. Prospective observational study. SETTING:. One hundred seventy-nine hospitals in 24 countries within the Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study COVID-19 Registry. PATIENTS:. Hospitalized adults with laboratory-confirmed SARS-CoV-2 infection. INTERVENTIONS:. None. RESULTS:. Of 16,225 patients enrolled in the registry with hospital discharge status available, 2,092 (12.9%) developed serious neurologic manifestations including 1,656 (10.2%) with encephalopathy at admission, 331 (2.0%) with stroke, 243 (1.5%) with seizure, and 73 (0.5%) with meningitis/encephalitis at admission or during hospitalization. Patients with serious neurologic manifestations of COVID-19 were older with median (interquartile range) age 72 years (61.0–81.0 yr) versus 61 years (48.0–72.0 yr) and had higher prevalence of chronic medical conditions, including vascular risk factors. Adjusting for age, sex, and time since the onset of the pandemic, serious neurologic manifestations were associated with more severe disease (odds ratio [OR], 1.49; p < 0.001) as defined by the World Health Organization ordinal disease severity scale for COVID-19 infection. Patients with neurologic manifestations were more likely to be admitted to the ICU (OR, 1.45; p < 0.001) and require critical care interventions (extracorporeal membrane oxygenation: OR, 1.78; p = 0.009 and renal replacement therapy: OR, 1.99; p < 0.001). Hospital, ICU, and 28-day mortality for patients with neurologic manifestations was higher (OR, 1.51, 1.37, and 1.58; p < 0.001), and patients had fewer ICU-free, hospital-free, and ventilator-free days (estimated difference in days, –0.84, –1.34, and –0.84; p < 0.001). CONCLUSIONS:. Encephalopathy at admission is common in hospitalized patients with SARS-CoV-2 infection and is associated with worse outcomes. While serious neurologic manifestations including stroke, seizure, and meningitis/encephalitis were less common, all were associated with increased ICU support utilization, more severe disease, and worse outcomes.

This case details a 33-year-old female who presented with a suggestive autoimmune history, arthralgias, and splenomegaly, and tested positive for Sjögren’s syndrome and anti-liver–kidney microsomal antibody. This was further validated by findings from a liver biopsy, confirming a very rare association with Type 2 autoimmune hepatitis. Primary Sjögren’s syndrome is a sporadic disease with a global prevalence of 61 per 100,000 people and a total prevalence of 0.4% for secondary Sjögren’s syndrome. The prevalence of autoimmune hepatitis in association with primary Sjögren’s syndrome is 4–47%. It is divided into two types, associated with characteristic antibodies. Type 2 autoimmune hepatitis is rarely reported with Sjögren’s syndrome; much of the association reported in the literature has been with Type 1 autoimmune hepatitis.

Guillain–Barré syndrome (GBS) is a form of rapidly progressive, acute inflammatory demyelinating polyradiculopathy. Acute inflammatory demyelinating polyradiculopathy is the most common variant of GBS, especially in the western hemisphere. It is diagnosed without hesitation when it presents with its characteristic clinical features of ascending paralysis and areflexia. It is when an atypical presentation appears, with brisk or very brisk reflexes, that diagnosis becomes difficult. In this case, a patient who presented with progressive motor weakness, unilateral facial palsy, and hyperreflexia on examination is described in order to demonstrate a variant of GBS and its management. Keeping in mind that the management of the disease does not change with the variant, the diagnostic challenge that is put forward by those variants needs a focussed approach by physicians.

We present a case of sigmoid volvulus in a young male patient with culture-proven Salmonella Typhi in the blood which was sensitive to Meropenem and Azithromycin only, presented with fever, vomiting, loose stools, hematochezia, abdominal distention and tenderness with no signs of perforation on erect chest x-ray. Further, radiological imaging showed signs of sigmoid volvulus. An urgent colonic decompression with untwisting of the mesentery was performed. In our case, it can be said that sigmoid volvulus was developed as a complication of multiple drug-resistant strains of Salmonella Typhi. The resistance is acquired by alteration in the genome sequence. Currently, it is important to control such an unknown outbreak of multiple drug-resistant strains of Salmonella Typhi as it is a serious health care issue of disease control and prevention in Pakistan.

A 45-year-old Asian woman was presented with fever, easy fatigability, shortness of breath, cervical and axillary lymphadenopathy and other signs and symptoms of anemia. After all the baseline work-up, the patient was investigated for Mono-coombs C3d levels, which were elevated, suggesting the diagnosis of Cold autoimmune hemolytic anemia (Cold AIHA). An Ultrasound-guided true-cut biopsy was done to determine the primary cause associated with it, which showed the presence of tumor cells arranged in cords and clusters. They have dark staining cells with mitotic activity, suggestive of breast carcinoma as an association of Cold AIHA. Estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) were sent, which came out to be positive. So, the patient was diagnosed with Cold AIHA in association with triple-positive breast cancer.

Lupus panniculitis occurs in 1–3% of the patients diagnosed with systemic lupus erythematosus (SLE) and 10% of the patients diagnosed with discoid lupus erythematosus (DLE). It is a disorder of autoimmune origin, manifesting as deep erythematous plaques and nodules involving the trunk, breasts, buttocks, face, and proximal extremities. It does not commonly ulcerate. This report highlights the case of a 22-year-old Asian female with a history of coeliac disease and significant family history of antiphospholipid antibody syndrome (APS) who presented with fever, malaise, weight loss, and subcutaneous non-tender nodules over the forearm, back, bilateral thighs, and feet. Laboratory investigations revealed positive antinuclear antibodies, anti-Ro/SSA antibody, and lupus anticoagulant, resulting in a diagnosis of APS. Biopsies of lesions were consistent with findings of lupus panniculitis. Every case of SLE and DLE with discrete skin lesions should be reviewed for any distinct entity such as lupus panniculitis, as it may be associated with greater risk of flares and systemic involvement. The purpose of this case report is to emphasise that early diagnosis and prompt treatment is crucial to improving the prognosis of such patients.