COVID-19 outcomes in patients with sickle cell disease and sickle cell trait compared with individuals without sickle cell disease or trait: a systematic review and meta-analysis.

Background: Clinical manifestations and severity of SARS-CoV-2 infection in individuals with sickle cell disease (SCD) and sickle cell trait (SCT) are not well understood yet. Methods: We performed a systematic review and meta-analysis to assess COVID-19 outcomes in individuals with SCD or SCT compared to individuals without sickle cell disease or trait. An electronic search on PubMed, Embase, and Cochrane Library was performed on August 3, 2023. Two authors (IFM and ISP) independently screened (IFM and ISP) and extracted data (IFM and ILC) from included studies. Main exclusion criterion was the absence of the non-SCD/SCT group. Exposure effects for binary endpoints were compared using pooled odds ratio (OR) with 95% confidence intervals (CI). I2 statistics was used to assess the heterogeneity and DerSimonian and Laird random-effects models were applied for all analyses to minimize the impact of differences in methods and outcomes definitions between studies. The overall quality of evidence was assessed using the GRADE system. Review Manager 5.4 and R software (v4.2.2) were used for statistical analyses. Registered with PROSPERO, CRD42022366015. Findings: Overall, 22 studies were included, with a total of 1892 individuals with SCD, 8677 individuals with SCT, and 1,653,369 individuals without SCD/SCT. No difference in all-cause mortality was seen between SCD/SCT and non-SCD/SCT (OR 1.18; 95% CI 0.78-1.77; p = 0.429; I2 = 82%). When considering only studies adjusted for confounders (8 studies), patients with SCD/SCT were shown to be at increased risk of death (OR 1.86; 95% CI 1.30-2.66; p = 0.0007; I2 = 34%). No significant difference was seen between individuals with SCD and SCT (p = 0.863). The adjusted for confounders analysis for hospitalisation revealed higher rates for the SCD (OR 5.44; 95% CI 1.55-19.13; p = 0.008; I2 = 97%) and the SCT groups (OR 1.31; 95% CI 1.10-1.55; p = 0.002; I2 = 0) compared to the non-SCD/SCT population. Moreover, it was significantly higher for the SCD group (test for subgroup difference; p = 0.028). Interpretation: Our findings suggest that patients with SCD or SCT may present with a higher mortality and hospitalisation rates due to COVID-19 infection. Funding: None.

COVID-19 Impact on Diagnosis and Staging of Colorectal Cancer: A Single Tertiary Canadian Oncology Center Experience.

BACKGROUND: Public health measures have imposed drastic reductions in cancer screening programs at the beginning of the COVID-19 pandemic, with an unknown impact on the diagnosis and staging of colorectal cancer (CRC). METHODS: Newly diagnosed CRC cases at the Centre Hospitalier de l'Université de Montréal (CHUM) were divided into two groups according to the timeline: pre-pandemic (1 January 2018-12 March 2020), and pandemic (13 March 2020-30 June 2021) periods. Colonoscopy, surgery, and staging at diagnosis during the pandemic period were compared to the pre-pandemic period. RESULTS: 254 CRC diagnoses were made during the pre-pandemic period in comparison to 125 during the pandemic period. Mean diagnosis rates were lower in the pandemic period (7.8 vs. 9.8 diagnoses/month, p = 0.048). Colonoscopy deadlines were less respected in the pandemic period (51.7% vs. 38.3%, p = 0.049). The rate of elective surgery did not differ (2.9 vs. 3.5 surgeries/month, p = 0.39) and mean delays were similar (58.6 vs. 60.4 days, p = 0.77). Stages at diagnosis did not differ (p = 0.17). Most of the delayed colonoscopies led to a stage 0 or I CRC (p = 0.2). CONCLUSION: In our center, the COVID-19 pandemic resulted in a decreased rate of CRC diagnosis and increased endoscopic delays without affecting the rate of advanced stage disease. Delays to surgery were quite similar once the CRC diagnosis was established.

COVID-19 and Sickle Cell Disease in the Province of Quebec, Canada: Outcomes after Two Years of the Pandemic.

BACKGROUND: Patients with sickle cell disease (SCD) are considered at higher risk of severe COVID-19 infection. However, morbidity and mortality rates are variable among countries. To date, there are no published reports that document outcomes of SCD patients with COVID-19 in Canada. METHODS: A web-based registry was implemented in June 2020 capturing outcomes of SCD patients with COVID-19 from March 2020 to April 2022 and comparing them to the general population of Quebec, Canada. RESULTS: After 24 months of the pandemic, 185 SCD patients with confirmed SARS-CoV-2 infection were included in the registry. Overall, the population was young (median age 12 years old) and had few comorbidities. No deaths were reported. Risk of hospitalization and admission to intensive care unit (ICU) because of COVID-19 was higher in patients with SCD than in the general population (relative risks (RR) 5.15 (95% confidence interval (95% CI) 3.84-6.91), p ˂ 0.001 and 4.56 (95% CI 2.09-9.93) p ˂ 0.001). A history of arterial hypertension or acute chest syndrome in the past 12 months was associated with a higher risk of severe disease (RR = 3.06 (95% CI 1.85-5.06) p = 0.008 and 2.27 (95% CI 1.35-3.83) p = 0.01). Hospitalized patients had lower hemoglobin F than non-hospitalized patients (12% vs. 17%, p = 0.02). For those who had access to vaccination at the time of infection, 25 out of 26 patients were adequately vaccinated and had mild disease. CONCLUSIONS: The SCD population is at higher risk of severe disease than the general population. However, we report favorable outcomes as no deaths occurred. Registries will continue to be critical to document the impact of novel COVID-19 specific therapy and vaccines for the SCD population.

Screening for Cognitive Dysfunction Using the Rowland Universal Dementia Assessment Scale in Adults With Sickle Cell Disease.

Importance: Adults with sickle cell disease (SCD) disproportionally experience early cognitive decline; however, guidance on the optimal screening strategy for cognitive dysfunction is lacking, and several available tools are biased by language, educational level, socioeconomic status, and race/ethnicity. The Rowland Universal Dementia Assessment Scale (RUDAS) was specifically designed for cognitive screening in multicultural populations. Objective: To ascertain the prevalence of suspected dementia in adults with SCD using the RUDAS, and to identify whether age, sex, educational level, several biological variables, and SCD complications were associated with RUDAS scores. Design, Setting, and Participants: This multicenter, bilingual, cross-sectional study was conducted in 2 SCD comprehensive care centers in Canada (Centre Hospitalier de l'Université Montréal in Montréal and University Health Network in Toronto). Participants were adults aged 18 years or older and were enrolled in the study between July 1, 2018, and July 30, 2019. All outpatients were eligible and offered study participation, unless they had an acute medical condition that required inpatient care or they were unable to follow study instructions. Interventions: The RUDAS was administered by trained personnel in either French or English, according to the patient's language preference. A questionnaire on social determinants of health was also administered, and participants underwent screening for anxiety and depression. Main Outcomes and Measures: Proportion of participants with RUDAS scores that were suggestive of dementia and the RUDAS score. Any score lower than 23 points was suggestive of dementia, a score between 23 and 27 points indicated a possible association with mild neurocognitive disorder, and a score higher than 27 points was normal. Results: A total of 252 adult patients with SCD were included (136 women [54.0%]; mean [range] age, 34.8 [18-75] years). Overall, 29 patients (11.5%) had RUDAS scores that were suggestive of dementia, and this proportion increased with age (15 [8.7%] in the 18-39 years age group, 10 [14.5%] in the 40-59 years age group, and 4 [36.4%] in the ≥60 years age group). The RUDAS scores were not associated with sex, language, SCD genotype, and SCD complications. The highest level of education was significantly associated with the RUDAS score; however, the association was small (η2 = 0.02; 95% CI, 0.00-0.07; P = .02). In a multivariable analysis, lower glomerular filtration rate (r = 0.40; 95% CI, 0.29-0.50; P < .001) and increasing age (r = -0.37; 95% CI, -0.47 to -0.26; P < .001), but not SCD genotype or disease severity, were associated with lower RUDAS scores. Conclusions and Relevance: This study found that using the RUDAS revealed a high prevalence of suspected dementia in adult patients with SCD that was associated with worsening kidney function and age. Cognition should be screened in all adult patients with SCD, regardless of age, disease severity, and SCD genotype; further validation of the RUDAS is ongoing.