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.

Epidemiological evaluation and survival of children with acute myeloid leukemia

Abstract Objective: This study aims to describe the epidemiological characteristics and survival rates of children with acute myeloid leukemia treated in hospitals in southern Brazil and compare them with international data. Methods: A multicenter cohort study was conducted with retrospective data collection of all new patients with acute myeloid leukemia under 18 treated at five referral centers in pediatric hematology-oncology in southern Brazil between January 2005 and December 2015. Results: Of the 149 patients with acute myeloid leukemia, 63.0% (n = 94) were male. The median age at diagnosis was 10.5 years (range 0-18 years) and 40.3% (n = 60) had a white blood cell count below 50,000/mm2. The most common Franco-American-British (FAB) subtype was M3 (n = 43, 28.9%). Nine (6.0%) patients had central nervous system disease. In M3 patients, overall survival (OS) was 69.2% and 3-year event-free survival was 67.7%; in non-M3 patients, these rates were 45.3% and 36.7%, respectively. In non-M3 patients, OS was significantly different between transplanted (61.8%) and non-transplanted (38.2%) patients (p = 0.031). Conclusions: These results show a higher prevalence of the Franco-American-British M3 subtype than that reported in the international literature, as well as a decreased OS compared with that of developed countries. Further multicenter Brazilian studies with a larger sample size are encouraged to better understand the characteristics of acute myeloid leukemia, and to improve the treatment and prognosis in this population.

Epidemiological evaluation and survival of children with acute myeloid leukemia.

OBJECTIVE: This study aims to describe the epidemiological characteristics and survival rates of children with acute myeloid leukemia treated in hospitals in southern Brazil and compare them with international data. METHODS: A multicenter cohort study was conducted with retrospective data collection of all new patients with acute myeloid leukemia under 18 treated at five referral centers in pediatric hematology-oncology in southern Brazil between January 2005 and December 2015. RESULTS: Of the 149 patients with acute myeloid leukemia, 63.0% (n=94) were male. The median age at diagnosis was 10.5 years (range 0-18 years) and 40.3% (n=60) had a white blood cell count below 50,000/mm2. The most common Franco-American-British (FAB) subtype was M3 (n=43, 28.9%). Nine (6.0%) patients had central nervous system disease. In M3 patients, overall survival (OS) was 69.2% and 3-year event-free survival was 67.7%; in non-M3 patients, these rates were 45.3% and 36.7%, respectively. In non-M3 patients, OS was significantly different between transplanted (61.8%) and non-transplanted (38.2%) patients (p=0.031). CONCLUSIONS: These results show a higher prevalence of the Franco-American-British M3 subtype than that reported in the international literature, as well as a decreased OS compared with that of developed countries. Further multicenter Brazilian studies with a larger sample size are encouraged to better understand the characteristics of acute myeloid leukemia, and to improve the treatment and prognosis in this population.

Síndrome da Morte Súbita Infantil em Pelotas de 2006 a 2013: uma análise descritiva / Sudden Infant Death Syndrome in Pelotas from 2006 to 2013: a descriptive analysis

Introdução e objetivo: A Síndrome da Morte Súbita Infantil (SMSI) ocupa a oitava posição entre as causas de anos potenciais de vida perdidos e as primeiras posições como causa de mortalidade infantil pós-neonatal em países desenvolvidos. O presente estudo objetiva conhecer as características socioepidemiológicas das crianças que foram a óbito por SMSI no município de Pelotas. Métodos: Estudo observacional, retrospectivo, descritivo baseado nos dados da Secretaria Municipal de Saúde, coletados através da aplicação de Fichas de Investigação de óbitos padronizadas pelo Ministério da Saúde de todos os casos de SMSI que ocorreram do ano de 2006 a 2013 em Pelotas/RS. Resultados: Houve 37 óbitos registrados no período, o que representa um coefi ciente de mortalidade por SMSI de 1,5 por mil. A média de idade materna foi de 23,5 anos (dp=5,2), 29 (78%) eram fumantes e 23 (62%) concederam aleitamento materno exclusivo até a data do óbito, 28 (76%) tiverem seus bebês nascidos a termo. Dentre os 37 casos, 16 (43%) vieram a falecer com menos de 1 mês de vida, 26 (70%) dormiam junto aos pais e 23 (61%) em decúbito lateral, enquanto que apenas 2 (5%) em decúbito ventral e 16 (43%) dos casos de SMSI ocorreram durante o inverno. Conclusão: O presente estudo é o único que abrange tamanha amostra (37 casos) de SMSI na cidade de Pelotas, a qual apresenta um coefi ciente de mortalidade por essa patologia semelhante aos mais altos encontrados na literatura. Portanto, políticas públicas que visem à prevenção de SMSI em Pelotas são necessárias.

Introduction and aim: Sudden Infant Death Syndrome (SIDS) ranks eighth among the causes of potential years of life lost and is among the leading causes of post-neonatal infant mortality in developed countries. This study aimed to evaluate the social and epidemiological characteristics of children who died of SIDS in the city of Pelotas. Methods: An observational, retrospective, and descriptive study based on Municipal Health Department data collected by applying Research Sheets standardized by the Ministry of Health to all cases of SIDS occurring from 2006 to 2013 in Pelotas, South Brazil. Results: A total of 37 deaths were recorded in the studied period, placing the SIDS mortality rate at 1.5 per thousand. The mean maternal age was 23.5 years (SD = 5.2), 29 (78%) were smokers, 23 (62%) granted exclusive breastfeeding until the date of death, 28 (76%) had term infants. From the 37 cases, 16 (43%) died under 1 month of age, 26 (70%) were sleeping with their parents, and 23 (61%) in the lateral position, while only 2 (5%) in the prone position, and 16 cases (43%) of SIDS occurred during the winter. Conclusion: This study is the only one that covers such sample (37 cases) of SIDS in the city of Pelotas, whose mortality rate from this disorder is close to the highest in the literature. Therefore, public policies for the prevention of SIDS in Pelotas are required.

Expression profile of apoptosis-related genes in childhood adrenocortical tumors: low level of expression of BCL2 and TNF genes suggests a poor prognosis.

BACKGROUND: Impaired apoptosis has been implicated in the development of childhood adrenocortical tumors (ACT), although the expression of apoptosis-related gene expression in such tumors has not been reported. METHODS: The mRNA expression levels of the genes CASP3, CASP8, CASP9, FAS, TNF, NFKB, and BCL2 were analyzed by quantitative real-time PCR in consecutive tumor samples obtained at diagnosis from 60 children with a diagnosis of ACT and in 11 non-neoplastic adrenal samples. BCL2 and TNF protein expression was analyzed by immunohistochemistry. RESULTS: A significant association was observed between tumor size ≥100 g and lower expression levels of the BCL2 (P=0.03) and TNF (P=0.05) genes; between stage IV and lower expression levels of CASP3 (P=0.008), CASP9 (P=0.02), BCL2 (P=0.002), TNF (P=0.05), and NFKB (P=0.03); Weiss score ≥3 and lower expression of TNF (P=0.01); unfavorable event and higher expression values of CASP9 (P=0.01) and lower values of TNF (P=0.02); and death and lower expression of BCL2 (P=0.04). Underexpression of TNF was associated with lower event-free survival in uni- and multivariate analyses (P<0.01). Similar results were observed when patients with Weiss score <3 were excluded. CONCLUSION: This study supports the participation of apoptosis-related genes in the biology and prognosis of childhood ACT and suggests the complex role of these genes in the pathogenesis of this tumor.

Dual diagnosis on "substance abuse".

Three large U.S. epidemiological surveys researching the prevalence of comorbid psychiatric illness and "substance abuse disorders" are reviewed: the Epidemiological Catchment Area Study, (N=20,219); the National Comorbidity Survey (N=8098), and the National Longitudinal Alcohol Epidemiological Survey (N=42,862). Findings suggest that comorbidity is highly prevalent but that longitudinal information remains limited limited--which restricts understanding of its "natural history," stability, common risk factors, and causal relationships.