A global systematic review and meta-analysis on the emerging evidence on risk factors, clinical characteristics, and prognosis of multisystem inflammatory syndrome in adults (MIS-A).

Background: The multisystem inflammatory syndrome in adults (MIS-A) has emerged, similar to those in children associated with SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) [multisystem inflammatory syndrome in children (MIS-C)]. This review aimed to analyze the risk factors, clinical course, and prognosis of MIS-A. Methods: A comprehensive literature search was conducted using several databases for cases reporting MIS-A from 1 December 2019 till 9 September 2021. The case definitions used to identify potential cases were those recommended by the World Health Organization, Center for Disease Control, and individual country/physician classification. The meta-analysis was performed using Comprehensive Meta-Analysis (CMA) 2.2.027 and Review Manager (RevMan) 5.4.1, employing 95% confidence intervals (CI). Results: Seventy studies were assessed for full-text eligibility, out of which 37 were included. The mean age of the study population was 32.52±10.29 years. The most common symptoms were fever (89.8%, 95% CI: 77.7-95.7%) and diarrhea (49%, 95% CI: 35.4-62.7%). Ventricular tachycardia (57.1%, 95% CI: 43.1-70.1%) was the most common electro-cardiac abnormality. The most common inflammatory marker was elevated C-reactive protein (89.8%, 95% CI: 77.7-95.7%). Abnormal echocardiogram was the most common imaging test result (commonly, ventricular dysfunction and arrhythmias), while steroids were the most administered treatment. Severe cases had a higher need for vasopressor and inotropic support and antibiotic therapy compared to the non-severe cases. One death was reported due to cardiovascular failure. Conclusion: Our collated findings will help clinicians identify the typical presenting symptoms and optimal management of MIS-A. Further research is required to understand the long-term prognosis and the correlation between coronavirus disease 2019 (COVID-19) and MIS-A to understand its pathogenesis and clinical spectrum.

Epidemiology, Clinical Features, and Outcomes of Multisystem Inflammatory Syndrome in Children (MIS-C) and Adolescents-a Live Systematic Review and Meta-analysis.

Purpose of Review: A multisystem inflammatory condition occurring in children and adolescents with COVID-19 has become increasingly recognized and widely studied globally. This review aims to investigate and synthesize evolving evidence on its clinical characteristics, management, and outcomes in pediatric patients. Recent Findings: We retrieved data from PubMed, EMBASE, Cochrane Library, WHO COVID-19 Database, Google Scholar, and preprint databases, covering a timeline from December 1, 2019, to July 31, 2021. A total of 123 eligible studies were included in the final descriptive and risk factor analyses. We comprehensively reviewed reported multisystem inflammatory syndrome in children (MIS-C) cases from published and preprint studies of various designs to provide an updated evidence on epidemiology, clinical, laboratory and imaging findings, management, and short-term outcomes. Latest evidence suggests that African black and non-Hispanic white are the two most common ethnic groups, constituting 24.89% (95% CI 23.30-26.48%) and 25.18% (95% CI 23.51-26.85%) of the MIS-C population, respectively. Typical symptoms of MIS-C include fever (90.85%, 95% CI 89.86-91.84%), not-specified gastrointestinal symptoms (51.98%, 95% CI 50.13-53.83%), rash (49.63%, 95% CI 47.80-51.47%), abdominal pain (48.97%, 95% CI 47.09-50.85%), conjunctivitis (46.93%, 95% CI 45.17-48.69%), vomiting (43.79%, 95% CI 41.90-45.68%), respiratory symptoms (41.75%, 95% CI 40.01-43.49%), and diarrhea (40.10%, 95% CI 38.23-41.97%). MIS-C patients are less likely to develop conjunctivitis (OR 0.27, 95% CI 0.11-0.67), cervical adenopathy (OR 0.21, 95% CI 0.07-0.68), and rash (OR 0.44, 95% CI 0.26-0.77), in comparison with Kawasaki disease patients. Our review revealed that the majority of MIS-C cases (95.21%) to be full recovered while only 2.41% died from this syndrome. We found significant disparity between low- and middle-income countries and high-income countries in terms of clinical outcomes. Summary: MIS-C, which appears to be linked to COVID-19, may cause severe inflammation in organs and tissues. Although there is emerging new evidence about the characteristics of this syndrome, its risk factors, and clinical prognosis, much remains unknown about the causality, the optimal prevention and treatment interventions, and long-term outcomes of the MIS-C patients. Supplementary Information: The online version contains supplementary material available at 10.1007/s40124-022-00264-1.

Residual symptoms and the quality of life in individuals recovered from COVID-19 infection: A survey from Pakistan.

BACKGROUND: There remains scarcity of literature regarding the patient's health status post-COVID-19 infection. This study analyzes the prevalence of residual symptoms and quality of life (QoL) after COVID-19. METHODS: An anonymous online survey was administrated in Pakistan from November 2020 to April 2021 in COVID-19 survivors. The questionnaire used the 12-Item Short Form Health Survey (SF-12) to assess mental and physical QoL. Multivariate linear regression was used to explore factors associated with mental and physical QoL scores. RESULTS: A total of 331 COVID-19 survivors participated in our survey. Around 42.0% of the cohort reported within 1-3 months of diagnosis of COVID-19. The common residual symptoms were body aches (39.9%), low mood (32.6%), and cough (30.2%). Better physical QoL was associated with being male (adjusted beta: 3.328) and having no residual symptoms (6.955). However, suffering from nausea/vomiting during initial COVID-19 infection (-4.026), being admitted to the ICU during COVID-19 infection (-9.164), and suffering from residual body aches (-5.209) and low mood (-2.959) was associated with poorer QoL. Better mental QoL was associated with being asymptomatic during initial COVID-19 infection (6.149) and post-COVID (6.685), while experiencing low mood post-COVID was associated with poorer mental QoL (-8.253 [-10.914, -5.592]). CONCLUSION: Despite presumed "recovery" from COVID-19, patients still face a wide range of residual symptoms months after initial infection, which contributes towards poorer QoL. Healthcare professionals must remain alert to the long-lasting effects of COVID-19 infection and aim to address them appropriately to improve patients' QoL.

The mystery of COVID-19 reinfections: A global systematic review and meta-analysis.

BACKGROUND: As the COVID-19 pandemic rages on, reports on disparities in vaccine roll out alongside COVID-19 reinfection have been emerging. We conducted a systematic review to assess the determinants and disease spectrum of COVID-19 reinfection. MATERIALS AND METHODS: A comprehensive search covering relevant databases was conducted for observational studies reporting Polymerase Chain Reaction (PCR) confirmed infection and reinfection cases. A quality assessment tool developed by the National Institute of Health (NIH) for the assessment of case series was utilized. Meta-analyses were performed using RevMan 5.3 for pooled proportions of findings in first infection and reinfection with a 95% confidence interval (CI). RESULTS: Eighty-one studies reporting 577 cases were included from 22 countries. The mean age of patients was 46.2 ± 18.9 years and 179 (31.0%) cases of comorbidities were reported. The average time duration between first infection and reinfection was 63.6 ± 48.9 days. During first infection and reinfection, fever was the most common symptom (41.4% and 36.4%, respectively) whilst anti-viral therapy was the most common treatment regimen administered (44.5% and 43.0%, respectively). Comparable odds of symptomatic presentation and management were reported for the two infections. However, a higher Intensive Care Unit (ICU) admission rate was observed in reinfection compared to first infection (10 vs 3). Ten deaths were reported with respiratory failure being the most common cause of death (7/10 deaths). CONCLUSION: Our findings support immunization practices given increased ICU admissions and mortality in reinfections. Our cohort serves as a guide for clinicians and authorities in devising an optimal strategy for controlling the pandemic. (249 words).

Granulomatosis With Polyangiitis: A 17 Year Experience From A Tertiary Care Hospital In Pakistan.

Granulomatosis with Polyangitis (GPA) is an uncommon immunologically mediated necrotizing vasculitis affecting the small and medium sized systemic blood vessels. We previously reported our experience with this condition and herein, we document our study findings and compare them to the clinical and radiological findings of various studies from around the world. By doing so we hope to further create awareness of this condition afflicting not only our part of the population but is part of a larger global phenomenon.

Polycythaemia Vera Presenting As A Porta Hepatis Mass.

Polycythaemia Vera (PV) is a myeloproliferative disorder in which bone marrow has increased production of red blood cells, white blood cells and platelets. The hallmarks of the disease are veno-occlusive events, secondary to increased blood viscosity. Polycythaemia Vera rarely presents with portal vein thrombosis below age of 55 years especially in absence of any chronic liver disease. We report a case of 30-years-old South Asian male presenting with abdominal pain, weight loss and vomiting for 3 months. On evaluation, he was found to have oesophageal varices. Furthermore, CT scan showed infiltration at porta-hepatis and portal venous thrombosis. Polycythaemia Vera was diagnosed with a positive JAK2 mutation and increased haemoglobin. Laparoscopy was done to perform biopsy of the porta-hepatis mass. Biopsy showed engorged vessels with no sign of malignancy. Patient underwent repeated sessions of upper GI endoscopy for band ligation and multiple sessions of venous phlebotomy which drastically improved his blood indices. He was started on lifelong aspirin and was advised regular follow-ups. With early recognition and prompt management patients can be prevented from potential complications which can prove to be detrimental.

Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan.

OBJECTIVE: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single centre retrospective single observation study. RESULTS: Study was conducted at the Aga Khan University Hospital, Karachi with records were reviewed from January 2000 to December 2017. Definitive diagnosis was made using a combination of serological anti-neutrophil cytoplasmic antibody (ANCA) testing along with the clinical and radiological presentation. A total of 51 patients met the diagnostic criteria in the time frame of the study. There were 23 males and 28 females with mean age of 44.0 ± 17.8 years on presentation. Arthritis was the most common symptom present in 41.2% of the cases followed by cough in 32.0%. Sixteen patients showed pulmonary infiltrates on chest X-ray. C-ANCA was positive in all of the patients compared with 21.6% p-ANCA positivity. A total of 13 biopsies were done. The median Birmingham Vasculitis Activity Score was 12. We report a 17.6% mortality rate with 5 deaths occurring due to respiratory failure. GPA is a diagnostic challenge leading to late diagnosis which can contribute to significant morbidity and mortality specially in the Third World.

Electronic cigarettes use and perception amongst medical students: a cross sectional survey from Sindh, Pakistan.

OBJECTIVE: The manufacturers of electronic cigarettes (e-cigarettes) are actively marketing their product through electronic and social media. Undergraduate medical students are expected to have better knowledge and awareness as they directly interact with patients in their training, The purpose of this study is therefore, to determine knowledge, use and perception regarding e-cigarettes among medical students from Sindh, Pakistan. RESULTS: A cross-sectional study was conducted between 1st July and 30th September 2016 at five different medical colleges situated in the second largest province of Sindh, Pakistan. The data was collected through a structured, self-administered questionnaire. Of the 500 students, the mean age was 21.5 ± 1.7 years and 58% were females. Over (65.6%) students were aware of e-cigarettes, 31 (6.2%) reported having used e-cigarettes, of whom 6 (1.2%) self-reported daily use. Users of conventional tobacco products were significantly more likely to have heard of e-cigarettes (87.6% vs 51.6%, p < 0.001) and having used them (13.9% vs 1.3%, p < 0.001). On multivariable logistic regression analysis we found a strong association of e-cigarette use with consumption of conventional cigarettes [OR: 10.6, 95% CI 3.6-30.8, p < 0.001], use of smokeless tobacco products [OR: 7.9, 95% CI 2.7-23.4, p < 0.001] however a weak association was observed for Shisha use [OR: 3.05, 95% CI 0.9-9.6, p = 0.05].

Celiac disease associated with aplastic anemia in a 6-year-old girl: a case report and review of the literature.

BACKGROUND: Celiac disease may present with hematological abnormalities including long-standing anemia. Both aplastic anemia and celiac disease have a similar underlying autoimmune process but an association between the two is seldom reported. There have only been three pediatric cases reporting this association and this case is the first reported in a female pediatric patient. CASE PRESENTATION: We report a case of 6-year-old South Asian girl presenting with bruises, petechiae, and recent history of loose stools. On evaluation, she was diagnosed as having celiac disease and was put on a gluten-free diet and further investigations including bone marrow biopsy revealed pancytopenia. She was managed with packed red cells, platelets, and diet restrictions and had improving platelet counts over yearly follow ups. Her parents were counseled regarding the need for bone marrow transplant. CONCLUSIONS: This is the fourth case report suggesting an association between celiac disease and aplastic anemia in the pediatric population and this association could be more common than expected. Timely intervention of either celiac disease through strict gluten-free diet or aplastic anemia through immunosuppressive therapy could potentially reduce the risk for other autoimmune conditions. We can see that all four pediatric cases reported with this potential association are from South East Asia and hence larger studies would be prudent to explore this association.

Awareness, use and perceptions about E-cigarettes among adult smokers in Karachi, Pakistan.

OBJECTIVE: To estimate the prevalence of awareness, current use and intention to use of e-cigarettes among adult smokers. METHODS: This cross-sectional survey was carried out at the Aga Khan University Hospital, Karachi, from July to August 2016, and comprised people aged above 18 years who had smoked more than 100 cigarettes in their lifetime. Convenience sampling method was used. A self-administered questionnaire was used to collect data. SPSS 22 was used for data analysis.. RESULTS: Of the 387 participants, 359(92.8%) were male. The overall mean age was 32.4±12.6 years. Moreover, 215(55.5%) respondents belonged to the middle socio-economic class. Besides, 249(64.3%) respondents were aware of e-cigarettes while 39(10.1%) used them, and 81(20.9%) wanted to use them. Socio-economic status was the best predictor for awareness about e-cigarettes (p<0.001), while gender (p=0.001), occupation

Correlation of TP53 Overexpression and Clinical Parameters with Five-Year Survival in Oral Squamous Cell Carcinoma Patients.

Introduction TP53 mutation and overexpression have been correlated with poor survival in many cancers including oral squamous cell carcinoma (OSCC). We aim to understand the role of TP53 overexpression in OSCC in our population and correlate it with five-year survival to test its viability as a prognostic marker for OSCC patients. Materials and methods Patients with biopsy proven OSCC at Aga Khan University Hospital from January 2000 to January 2008 were recruited. Immunohistochemistry was used to establish TP53 status and the results were published. Following up on these patients, five-year data were collected and correlated with TP53 status and other clinicopathologic parameters. Results Overexpression of TP53 was not significantly associated with five-year survival (hazard ratio [HR]: 1.543; 95% CI: 0.911-2.612; p = 0.107). Conclusion Although we had proven statistical relevance when correlated with overall survival in our previous study, we were unable to extend the same relevance to TP53 overexpression when it comes to five-year survival.

Multidisciplinary approach to the prenatal diagnosis and Post natal management of a large suprasellar arachnoid cyst: A case report.

Arachnoid cysts are intra-arachnoid sacs filled with cerebrospinal fluid representing a rare occurence in neonates. We report the case of a suprasellar arachnoid cyst diagnosed prenatally at 21 weeks gestation on routine obstetric ultrasound. A cystic lesion was picked up incidentally at routine antenatal scan. The cyst was noted to be increasing in size over a series of radiological scans. The cyst was diagnosed as a suprasellar arachnoid cyst compressing the third ventricle and bilateral lateral ventricles. A left pteryonal craniotomy for cystocisternostomy of large suprasellar cyst was performed initially. Within a month the cyst recurred with clinical and radiological evidence. An Endoscopic Third Ventriculostomy (ETV) with fenestration was performed.The patient was shifted to the NICU and discharged after 3 days in a stable condition. Patient was followed after 1 week and reassured and advised for one year follow-up.

Quality of Life in Individuals Surgically Treated for Congenital Hydrocephalus During Infancy: A Single-Institution Experience.

BACKGROUND: Congenital hydrocephalus (CH) is a frequently encountered birth anomaly that can hinder long-term neurologic maturity and social well-being of affected children. This study was undertaken to assess quality of life (QOL) 10-15 years after surgical treatment for primary CH during infancy at a tertiary care hospital in a developing country. METHODS: This retrospective cohort study included individuals who presented to Aga Khan University Hospital, Karachi, Pakistan, between 1995 and 2005 at <1 year old and underwent surgery for primary CH. The Hydrocephalus Outcome Questionnaire was used to assess outcomes with respect to QOL. RESULTS: Of 118 patients, 90 patients participated in the study. Mean age at first admission was 6.2 months. Mean length of follow-up was 5.4 years. Of these, 28 patients had died after surgery. Shunt infection (P = 0.012) and delayed milestones (P = 0.003) were found to be statistically significant factors affecting mortality in the patients who died. The mean overall health score was 0.67 ± 0.30. Age <6 months at the time of first surgery was a poor predictor of overall health on the Hydrocephalus Outcome Questionnaire (P = 0.039). CONCLUSIONS: In our analysis, we assessed the QOL associated with CH. We hope that these results will provide insight for future prospective work with the ultimate goal of improving long-term QOL in children with CH.