Abstract Background: People with haemophilia (PwH) are living longer. Therefore, they can develop atherosclerotic cardiovascular disease (ASCVD). Electrocardiogram (ECG) alterations may be a sign of initial ASCVD before the occurrence of symptoms. Objective: To describe the prevalence of resting ECG alterations among PwH adults asymptomatic for ASCVD. Methods: PwH aged ≥ 30 years without previous ASCVD events were considered for the analysis. Resting ECG traces were analysed according to international reference values and the Brazilian Longitudinal Adult Health Study (ELSA-Brasil) results for asymptomatic Brazilian men. Based on the established normal values and using the QT index, we further described the altered ECGs as minor or major changes, according to the Minnesota Code. Differences between prevalences were evaluated by Pearson's χ2 test. Differences between medians were evaluated by the Mann-Whitney U test. A p-value < 0.05 was accepted as statistically significant. Results: A total of 64 PwH were included in the study. Median age was 44 years (interquartile range 35-52). Most patients had haemophilia A (81%) and 47% were severe. The prevalence of obesity, systemic arterial hypertension (SAH), diabetes mellitus (DM), and dyslipidaemia were 16%, 56%, 14%, and 72%, respectively. All the PwH had sinus rhythm, except for one, who had an implanted pacemaker due to idiopathic third-degree atrioventricular block. Altered ECGs were found in 25% and 30% of PwH, according to established criteria and ELSA-Brasil criteria, respectively. Major changes were found in eight (13%) PwH according to the Minnesota Code, including two ECGs with ischaemia-like wall inactivity. Conclusions: The prevalence of altered ECG varied from 25% to 30% among asymptomatic PwH. Background (PwH longer Therefore . (ASCVD) (ECG symptoms Objective Methods 3 analysis ELSABrasil ELSA Brasil (ELSA-Brasil men index Code Pearsons Pearson s χ test MannWhitney Mann Whitney pvalue p value 005 0 05 0.0 significant Results 6 study 4 interquartile 3552. 3552 35 52 35-52) 81% 81 (81% 47 severe obesity SAH, SAH , (SAH) DM, DM (DM) 16 16% 56 56% 14 14% 72 72% respectively rhythm one thirddegree third degree block 25 13% 13 (13% ischaemialike ischaemia like inactivity Conclusions (ASCVD 00 0. 355 5 35-52 8 (81 (SAH (DM 1 7 2 (13 35-5 (8 (1 35- (

ABSTRACT BACKGROUND: Until recently, the treatment of people with hemophilia A and inhibitors (PwHAi) was based on the use of bypassing agents (BPA). However, the advent of emicizumab as prophylaxis has demonstrated promising results. OBJECTIVES: We aimed to compare the bleeding endpoints between PwHAi on BPA and those on emicizumab prophylaxis. DESIGN AND SETTING: Systematic review of interventions and meta-analysis conducted at the Universidade Federal de Goiás, Goiânia, Goiás, Brazil. METHODS: The CENTRAL, MEDLINE, Scopus, and LILACS databases were searched on February 21, 2023. Two authors conducted the literature search, publication selection, and data extraction. The selected publications evaluated the bleeding endpoints between PwHAi on emicizumab prophylaxis and those on BPA prophylaxis. The risk of bias was evaluated according to the Joanna Briggs Institute criteria. A meta-analysis was performed to determine the annualized bleeding rate (ABR) for treated bleeds. RESULTS: Five publications (56 PwHAi) were selected from the 543 retrieved records. Overall, bleeding endpoints were lower during emicizumab prophylaxis than during BPA prophylaxis. All the publications had at least one risk of bias. The only common parameter for the meta-analysis was the ABR for treated bleeds. During emicizumab prophylaxis, the ABR for treated bleeds was lower than during BPA prophylaxis (standard mean difference: −1.58; 95% confidence interval −2.50, −0.66, P = 0.0008; I2 = 68.4%, P = 0.0031). CONCLUSION: Emicizumab was superior to BPA in bleeding prophylaxis in PwHAi. However, both the small population size and potential risk of bias should be considered when evaluating these results. SYSTEMATIC REVIEW REGISTRATION: CRD42021278726, https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=278726. BACKGROUND recently (PwHAi BPA. . (BPA) However results OBJECTIVES SETTING metaanalysis meta analysis Goiás Goiânia Brazil METHODS CENTRAL MEDLINE Scopus 21 2023 search selection extraction criteria (ABR RESULTS 56 (5 54 records Overall standard difference −1.58 158 1 58 95 250 2 50 −2.50 066 0 66 −0.66 0.0008 00008 0008 I 684 68 4 68.4% 0.0031. 00031 0.0031 0031 0.0031) CONCLUSION REGISTRATION CRD42021278726 CRD https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=278726. httpswwwcrdyorkacukprosperodisplayrecordphpRecordID278726 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID https //www.crd.york.ac.uk/prospero/display_record.php RecordID=278726. www crd york ac uk prospero display record php RecordID 278726 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=278726 (BPA 202 5 ( −1.5 15 9 25 −2.5 06 6 −0.6 0.000 0000 000 68.4 0003 0.003 003 CRD4202127872 phpRecordID https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=278726 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID27872 wwwcrdyorkacukprosperodisplayrecordphp RecordID278726 RecordID=278726 27872 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=27872 20 −1. −2. −0. 0.00 00 68. CRD420212787 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=27872 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID2787 RecordID27872 RecordID=27872 2787 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=2787 −1 −2 −0 0.0 CRD42021278 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=2787 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID278 RecordID2787 RecordID=2787 278 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=278 − 0. CRD4202127 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=278 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID27 RecordID278 RecordID=278 27 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=27 CRD420212 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=27 httpswwwcrdyorkacukprosperodisplayrecordphpRecordID2 RecordID27 RecordID=27 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=2 CRD42021 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID=2 RecordID2 RecordID=2 https://www.crd.york.ac.uk/prospero/display_record.php?RecordID= CRD4202 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID= RecordID= https://www.crd.york.ac.uk/prospero/display_record.php?RecordID CRD420 https//www.crd.york.ac.uk/prospero/display_record.phpRecordID CRD42 CRD4

Resumo Fundamento A taxa de mortalidade de pessoas com hemofilia (PCH) no Brasil está diminuindo, mas a incidência relativa de mortes associadas a doenças cardiovasculares (DCV) tem aumentado. Objetivos Nosso objetivo foi descrever o escore de risco de DCV de PCHs de acordo com a ferramenta Pooled Cohort Equations Risk (PCER) Calculator e suas recomendações de tratamento. Além disso, foram comparadas as estimativas da PCER com o respectivo escore de risco de Framingham (FRS). Métodos Este estudo transversal incluiu PCHs do sexo masculino, com idade igual ou superior a 40 anos, tratados no Centro de Tratamento Integral de Hemofilia de Pernambuco (Recife/Brasil). PCHs com um evento cardiovascular prévio ou colesterol lipídico de baixa densidade ≥ 5,0 mmol/L foram excluídas. Entrevistas, revisões de prontuários médicos e exames de sangue foram realizados. A ferramenta PCER foi utilizada para estimar o risco de DCV e compará-lo com o respectivo FRS. Um valor de p < 0,05 foi aceito como estatisticamente significativo. Resultados Trinta PCHs foram incluídas. A idade mediana foi de 51,5 [intervalo interquartil-IIQ; 46,0-59,5] anos. A prevalência de obesidade, hipertensão arterial sistêmica, diabetes mellitus, hipertrigliceridemia, hipercolesterolemia e hipoHDLemia foi de 20%, 67%, 24%, 14%, 47% e 23%, respectivamente. O escore mediano da PCER foi de 6,9% [IIQ; 3,1-13,2], com 50% de alto risco (PCER ≥ 7,5%). O uso de estatina foi sugerido para 54% das PCHs. A pressão arterial estava mal controlada em 47% das PCHs. A concordância entre PCER e FRS foi de 80% (κ = 0,60; p = 0,001). Conclusões Metade dos homens com hemofilia, com 40 anos de idade ou mais, teve um alto risco de desenvolver DCV em 10 anos, com fortes recomendações para melhorar o controle da dislipidemia e da pressão arterial. PCH (PCH diminuindo (DCV aumentado tratamento disso . (FRS) masculino 4 Recife/Brasil. RecifeBrasil Recife/Brasil Recife (Recife/Brasil) 50 5 0 5, mmolL mmol L excluídas Entrevistas realizados comparálo compará lo 005 05 0,0 significativo incluídas 515 51 51, intervalo interquartilIIQ interquartil IIQ interquartil-IIQ 46,059,5 460595 46,0 59,5 46 59 46,0-59,5 obesidade sistêmica mellitus hipertrigliceridemia 20 20% 67 67% 24 24% 14 14% 47 23 23% respectivamente 69 6 9 6,9 [IIQ 3,113,2, 31132 3,1 13,2 , 3 1 13 2 3,1-13,2] 7,5%. 75 7,5% 7 7,5%) 54 80 κ 0,60 060 60 0,001. 0001 0,001 001 0,001) mais (FRS (Recife/Brasil 00 0, 059 46,059, 46059 460 46, 595 59, 46,0-59, 6, 113 3,113,2 3113 31 3, 132 13, 3,1-13,2 7,5 8 0,6 06 000 0,00 46,059 4605 46,0-59 11 3,113, 311 3,1-13, 7, 46,05 46,0-5 3,113 3,1-13 46,0- 3,11 3,1-1 3,1-

Abstract Background The mortality rate of Brazilian people with haemophilia (PwH) is decreasing, but the relative incidence of deaths associated with cardiovascular disease (CVD) is increasing. Objectives We aimed to describe the CVD risk score of PwH according to Pooled Cohort Equations Risk (PCER) Calculator tool and its treatment recommendations. We also compared the PCER estimates with the respective Framingham Risk Score (FRS). Methods This cross-sectional study included male PwH ≥ 40 years treated at the Comprehensive Haemophilia Treatment Centre of Pernambuco (Recife/Brazil). PwH with a previous CVD event or a low-density lipid cholesterol ≥ 5.0 mmol/L were excluded. Interviews, medical file reviews, and blood tests were performed. The PCER tool was used to estimate the CVD risk and compare it with the respective FRS. A p-value < 0.05 was accepted as statistically significant. Results Thirty PwH were included. Median age was 51.5 [interquartile range-IQR; 46.0-59.5] years. The prevalence of obesity, systemic arterial hypertension, diabetes mellitus, hypertriglyceridaemia, hypercholesterolaemia, and hypoHDLaemia were 20%, 67%, 24%, 14%, 47%, and 23%, respectively. The median PCER score was 6.9% [IQR; 3.1-13.2], with 50% having a high risk (PCER ≥ 7.5%). Statin use was suggested for 54% of PwH. Blood pressure was poorly controlled in 47% of PwH. The agreement between PCER and FRS was 80% (κ = 0.60; p = 0.001). Conclusions Half of the male people with haemophilia aged 40 years or older had a 10-year high risk of developing CVD with strong recommendations to improve control of dyslipidaemia and blood pressure. (PwH decreasing (CVD increasing . (FRS) crosssectional cross sectional 4 Recife/Brazil. RecifeBrazil Recife/Brazil Recife Brazil (Recife/Brazil) lowdensity low density 50 5 0 5. mmolL mmol L excluded Interviews reviews performed pvalue value 005 05 0.0 significant 515 51 51. interquartile rangeIQR range IQR range-IQR 46.059.5 460595 46.0 59.5 46 59 46.0-59.5 obesity hypertension mellitus hypertriglyceridaemia hypercholesterolaemia 20 20% 67 67% 24 24% 14 14% 47 23 23% respectively 69 6 9 6.9 [IQR 3.113.2, 31132 3.1 13.2 , 3 1 13 2 3.1-13.2] 7.5%. 75 7.5% 7 7.5%) 54 80 κ 0.60 060 60 0.001. 0001 0.001 001 0.001) 10year year 10 (FRS (Recife/Brazil 00 0. 059 46.059. 46059 460 46. 595 59. 46.0-59. 6. 113 3.113.2 3113 31 3. 132 13. 3.1-13.2 7.5 8 0.6 06 000 0.00 46.059 4605 46.0-59 11 3.113. 311 3.1-13. 7. 46.05 46.0-5 3.113 3.1-13 46.0- 3.11 3.1-1 3.1-

RESUMO Objetivo: construir e validar um instrumento de consulta de enfermagem para pessoas com hemofilia. Método: estudo metodológico realizado de fevereiro de 2017 a fevereiro de 2018, em um serviço referência de hematologia do Nordeste do Brasil. Utilizou-se a técnica Delphi para validação, por três grupos de enfermeiras juízas (n=29): Especialistas em hemofilia (n=nove) de nove hemocentros do país; Residência em Hematologia (n=oito); Enfermeiras do Serviço (n=12). A concordância entre as juízas foi verificada por escala Likert e teste exato de Fisher. Resultados: 89,6% não apresentaram dificuldade para compreender o instrumento. Quanto ao grau de relevância, as características ‘credibilidade’ e ‘cientificidade’ apresentaram maiores percentuais de extremamente relevante (90%). O teste exato de Fisher foi significativo no grau de satisfação de ‘clareza das afirmações’ (p<0,05). Conclusão: o instrumento foi considerado válido, proporcionando autonomia, apoio técnico e respaldo ético ao enfermeiro, contribuindo na melhoria da qualidade da assistência.

ABSTRACT Objective: to construct and validate a nursing consultation instrument for people with hemophilia. Method: methodological study conducted from February 2017 to February 2018, in a hematology reference service in Northeast Brazil. The Delphi technique was used for validation, by three groups of nurse judges (n=29): hemophilia specialists (n=nine) from nine blood centers in the country; Hematology Residency (n=eight); Service Nurses (n=12). Inter-rater agreement was checked by Likert scale and Fisher’s exact test. Results: 89.6% had no difficulty in understanding the instrument. As for the degree of relevance, the characteristics ‘credibility’ and ‘scientificity’ showed higher percentages of extremely relevant (90%). Fisher’s exact test was significant in the degree of satisfaction of ‘clarity of statements’ (p<0.05). Conclusion: the instrument was considered valid, providing autonomy, technical support, and ethical support to the nurse, contributing to the improvement of the quality of care.

RESUMEN Objetivo: construir y validar un instrumento de consulta de enfermería para personas con hemofilia. Método: estudio metodológico realizado de febrero de 2017 a febrero de 2018, en un servicio de referencia de hematología del Nordeste de Brasil. Para la validación se utilizó la técnica Delphi, por parte de tres grupos de enfermeras jueces (n=29): Especialistas en hemofilia (n=nueve) de nove hemocentros del país; Residencia en Hematología (n=ocho); Enfermeras del Servicio (n=12). La concordancia entre las jueces se verificó mediante la escala Likert y la prueba exacto de Fisher. Resultados: 89,6% não apresentaram dificuldade para compreender o instrumento. Quanto ao grau de relevância, as características ‘credibilidade’ e ‘cientificidade’ apresentaram maiores percentuais de extremamente relevante (90%). O teste exato de Fisher foi significativo no grau de satisfação de ‘clareza das afirmações’ (p<0,05). Conclusión: o instrumento foi considerado válido, proporcionando autonomia, apoio técnico e respaldo ético ao enfermeiro, contribuindo na melhoria da qualidade da assistência.