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Abstract Background This systematic study aims to assess the global epidemiologic, economic, and humanistic burden of illness associated with all types of hereditary angioedema. Methods A systematic search for articles reporting the epidemiologic, economic, and humanistic burden associated with patients with HAE was conducted using English and Chinese literature databases from the inception to May 23, 2022. The selected studies were assessed for their quality and risk of bias. The study was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses and registered with the International Prospective Register of Systematic Reviews (PROSPERO; CRD42022352377). Results In total, 65 articles that met the search inclusion criteria reported 10,310 patients with HAE, of whom 5861 were female patients. Altogether, 4312 patients (81%) and 479 patients (9%) had type 1 and type 2 HAE, respectively, whereas 422 patients (8%) had HAE-normal C1-INH. The overall prevalence of all types of HAE was between 0.13 and 1.6 cases per 100,000. The mean or median delay from the first onset of a symptom of HAE to confirmed diagnosis ranged from 3.9 to 26 years. The estimated risk of death from asphyxiation was 8.6% for patients with HAE. Hospitalization, medication, unnecessary surgeries, doctor visits, specialist services, and nursing costs are direct expenses that contribute to the growing economic burden. The indirect cost accounted mostly due to missing work ($3402/year) and loss of productivity ($5750/year). Furthermore, impairment of QoL as reported by patient-reported outcomes was observed. QoL measures identified depression, anxiety, and stress to be the most common symptoms for adult patients and children. Conclusion This study highlights the importance of early diagnosis and the need for improving awareness among health care professionals to reduce the burden of HAE on patients and society.

Abstract Background Neisseria meningitidis is an encapsulated Gram-negative diplococcus that asymptomatically colonises the upper respiratory tract in up to 25% of the population (mainly adolescents and young adults). Invasive meningococcal disease (IMD) caused by Neisseria meningitidis imposes a substantial public health burden,. The case fatality rate (CFR) of IMD remains high. IMD epidemiology varies markedly by region and over time, and there appears to be a shift in the epidemiology towards older adults. The objective of our review was to assess the published data on the epidemiology of IMD in older adults (those aged ≥ 55 years)in North America and Europe. Such information would assist decision-makers at national and international levels in developing future public health programmes for managing IMD. Methods A comprehensive literature review was undertaken on 11 August 2020 across three databases: EMBASE, Medline and BIOSIS. Papers were included if they met the following criteria: full paper written in the English language; included patients aged ≥ 56 years; were published between 1/1/2009 11/9/2020 and included patients with either suspected or confirmed IMD or infection with N. meningitidis in North America or Europe. Case studies/reports/series were eligible for inclusion if they included persons in the age range of interest. Animal studies and letters to editors were excluded. In addition, the websites of international and national organisations and societies were also checked for relevant information. Results There were 5,364 citations identified in total, of which 76 publications were included in this review. We identified that older adults with IMD were mainly affected by serogroups W and Y, which are generally not the predominant strains in circulation in most countries. Older adults had the highest CFRs, probably linked to underlying comorbidities and more atypical presentations hindering appropriate timely management. In addition, there was some evidence of a shift in the incidence of IMD from younger to older adults. Conclusions The use of meningococcal vaccines that include coverage against serogroups W and Y in immunization programs for older adults needs to be evaluated to inform health authorities’ decisions of the relative benefits of vaccination and the utility of expanding national immunization programmes to this age group.

Abstract Background Untreated hemophilia A patients may experience recurrent bleeding events leading to debilitating joint damages. While RCT and pharmacokinetic data support the value of Kovaltry [an unmodified full-length recombinant factor VIII (FVIII) product], real world evidence in children is lacking. This report describes a descriptive and multivariate analysis of the effectiveness of Kovaltry in children with hemophilia A in the real-world setting, using data from medical chart abstraction and cross-sectional surveys of physicians, patients, and caregivers. Results Male patients aged

Abstract Background The epidemiology of acute kidney injury (AKI) in nonexacerbated chronic obstructive pulmonary disease (NECOPD) patients is unknown. This study investigated the factors associated with AKI and the association between AKI and in-hospital mortality in the hospitalized NECOPD population. Methods The electronic medical records of 2897 patients hospitalized with NECOPD were analyzed retrospectively. Demographic information, medicine used before AKI, diagnosis records and laboratory data were collected. AKI was classified as community-acquired (CA-) or hospital-acquired (HA-) AKI according to the serum creatinine criteria. Risk factors for HA-AKI and in-hospital mortality were analyzed by logistic regression analyses. To avoid an interaction between cor pulmonale and AKI, the association between AKI and in-hospital morality was further analyzed with cor pulmonale stratification. Results The incidence rates of CA- and HA-AKI were 7.1 and 12.0%, respectively. Increased age, female sex, cor pulmonale comorbidity, chronic kidney disease stage, diuretic and glycopeptide use before AKI and iodine-containing contrast medium exposure were independently associated with HA-AKI. A total of 5.7% of the patients died. After adjustment for age, sex, cor pulmonale, chronic kidney disease, Charlson comorbidity index score (without renal disease) and hemoglobin level, HA-AKI was an independent risk factor for in-hospital mortality [OR 13.909 (95% CI 8.699–22.238) in non-cor pulmonale subgroup; OR 26.604 (95% CI 12.166–58.176) in cor pulmonale subgroup], whereas CA-AKI was not. Conclusions AKI is common in the NECOPD population. Diuretics and contrast media are associated with HA-AKI in this population. The patients with HA-AKI have a higher mortality risk than the patients without AKI.

Abstract Background The SNAPSHOT program provides current data on the allergic rhinitis burden in the adult general population of five Middle Eastern countries (Egypt, Turkey, Kuwait, Saudi Arabia and the United Arab Emirates, the latter three grouped into a Gulf cluster). Methods A multi-country, cross-sectional, epidemiological program conducted by telephone in a random sample of the adult general population; quotas were defined per country demographics. Subjects were screened for allergic rhinitis using the Score For Allergic Rhinitis questionnaire. Current prevalence (last 12 months) was estimated. Disease severity and control were assessed using the Allergic Rhinitis and its Impact on Asthma classification and Rhinitis Control Assessment Test respectively. Quality of sleep, impact on daily activities and quality of life were measured using the Epworth Sleepiness Scale, Sheehan Disability Scale and EuroQol Five-Dimension questionnaire respectively. Multivariate logistic regression analyses were used to investigate risk factors and co-morbidities. Results 1808 of 33,486 subjects enrolled in the SNAPSHOT program fulfilled the case definition for allergic rhinitis. Prevalence was 3.6% [95% CI 3.2–4.0%] in Egypt, 6.4% [95% CI 5.9–6.9%] in Turkey and 6.4% [95% CI 6.0–6.9%] in the Gulf cluster. Risk factors identified were country, co-morbid asthma and income. Subjects with allergic rhinitis reported a significantly lower quality of life compared to the general population (p