Pesquisa | SciELO

Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety. date disease reallife real life attacks vasoocclusive vaso occlusive events syndrome transfusion which part physicians physician s report safety

https://doi.org/10.1016/j.htct.2024.01.001

Blood concentrations of α-Klotho and FGF-23 exhibit no correlation with bone mineral density in elderly individuals αKlotho α Klotho FGF23 FGF 23 FGF-2 FGF2 2 FGF-
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Sawada, Karina Moura

; de Moraes, Niele Silva

; Araújo, Lara Miguel Quirino

; Gazoni, Fernanda Martins

; Lazaretti-Castro, Marise

; Cendoroglo, Maysa Seabra

; Bilezikian, John P.

; Figueiredo, Maria Stella

; dos Santos, Fania Cristina

einstein (São Paulo)

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2024, Volume 22 elocation eAO0412

Resumo: En | Texto: En | PDF: En

Objective: To investigating the relationship between α-Klotho and FGF-23 with bone biochemical markers and bone density findings in extremely aged individuals. Methods: A total of 55 individuals with a mean age of 85.6 years were subjected to clinical, biochemical, and bone mineral density analyses and the enzyme-linked immunosorbent assay-based detection of α-Klotho and FGF-23. The mean, standard deviation, median, and interquartile ranges of the sample values were determined, and Spearman's test for association assessments was used for statistical analysis. Results: The study participants expressed median FGF-23 and α-Klotho levels of 69.81 RU/mL (51.43 RU/mL) and 733.43 pg/mL (360.83 pg/mL), respectively. The majority of the participants possessed osteopenia (54.5%) and a vitamin D deficiency (57%). The 25-hydroxyvitamin D concentrations ranged between 7.1 and 47.5ng/mL, with a median of 18.1ng/mL. Conclusion: No substantial associations were discovered between α-Klotho and FGF-23 levels and bone density in the study participants. Objective αKlotho α Klotho FGF23 FGF 23 FGF-2 Methods 5 856 85 6 85. clinical enzymelinked enzyme linked assaybased assay based FGF23. 23. deviation determined Spearmans Spearman s analysis Results 6981 69 81 69.8 RUmL RU mL 51.43 5143 51 43 (51.4 73343 733 733.4 pgmL pg 360.83 36083 360 83 (360.8 pg/mL, , pg/mL) respectively 54.5% 545 54 (54.5% 57%. 57 57% . (57%) 25hydroxyvitamin hydroxyvitamin 25 71 7 1 7. 475ngmL ngmL 47 5ng ng 47.5ng/mL 181ngmL 18 1ng 18.1ng/mL Conclusion FGF2 2 FGF- 8 698 69. 51.4 514 4 (51. 7334 73 733. 360.8 3608 36 (360. 54.5 (54.5 (57% 51. (51 360. 3 (360 54. (54. (57 (5 (36 (54 ( (3

https://doi.org/10.31744/einstein_journal/2024ao0412

Patient Blood Management Program Implementation: Comprehensive Recommendations and Practical Strategies Implementation
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Céspedes, Isabel Cristina

; Figueiredo, Maria Stella

; Hossne Junior, Nelson Americo

; Suriano, Ítalo Capraro

; Rodrigues, Rita de Cássia

; Barros, Melca Maria Oliveira

; Paiva Neto, Manoel Antonio de

; Atallah, Fernanda Chohfi

; Benini, Bárbara Burza

; Gonzalez, Adriano Miziara

; Sparapani, Fábio Veiga de Castro

; Barros Júnior, Newton de

; Carneiro, Ieda Aparecida

; Sarto, Celina Mayumi Morita

; Motoyama, Caio Sussumu de Macedo

; Sacchi, Leonardo

; Piovezan, Victor

; Almeida, Simone Luna de

; Pereira-Rufino, Laís da Silva

; Guizilini, Solange

; Rocco, Isadora Salvador

; Mansur, Nacime Salomão

; Arakaki, Jaquelina Sonoe Ota

; Santos, Antonio Alceu dos

; Panfilio, Carlos Eduardo

Brazilian Journal of Cardiovascular Surgery

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2024, Volume 39 Nº 5 elocation e20240205

Resumo: En | Texto: En | PDF: En

ABSTRACT Introduction: Blood transfusion is one of the most common medical practices worldwide. However, current scientific literature has shown that the immunomodulatory effects of blood transfusion are associated with an increased likelihood of infection, prolonged hospitalization, and morbimortality. Also, it means high costs for healthcare systems. Methods: In this context, acknowledging that blood transfusions are essentially heterologous cell transplantations, the use of therapeutic options has gained strength and is collectively known as the patient blood management (PBM) program. PBM is an approach based on three main pillars: (1) treating anemias and coagulopathies in an optimized manner, especially in the preoperative period; (2) optimizing perioperative hemostasis and the use of blood recovery systems to avoid the loss of the patient's blood; (3) anemia tolerance, with improved oxygen delivery and reduced oxygen demand, particularly in the postoperative period. Results: Current scientific evidence supports the effectiveness of PBM by reducing the need for blood transfusions, decreasing associated complications, and promoting more efficient and safer blood management. Thus, PBM not only improves clinical outcomes for patients but also contributes to the economic sustainability of healthcare systems. Conclusion: The aim of this review was to summarize PBM strategies in a comprehensive, evidence-based approach through a systematic and structured model for PBM implementation in tertiary hospitals. The recommendations proposed herein are from researchers and experts of a high-complexity university hospital in the network of the Sistema Único de Saúde, presenting itself as a strategy that can be followed as a guideline for PBM implementation in other settings. Introduction worldwide However infection hospitalization morbimortality Also Methods context transplantations (PBM program pillars 1 (1 manner period 2 (2 s 3 (3 tolerance demand Results complications Thus Conclusion comprehensive evidencebased hospitals highcomplexity complexity Saúde settings (

https://doi.org/10.21470/1678-9741-2024-0205

Patient Blood Management in Cardiovascular Surgery
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Céspedes, Isabel Cristina

; Figueiredo, Maria Stella

; Santos, Antonio Alceu dos

; Hossne Junior, Nelson Americo

Brazilian Journal of Cardiovascular Surgery

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2024, Volume 39 Nº 5 elocation e20240994

Resumo: En | Texto: En | PDF: En

https://doi.org/10.21470/1678-9741-2024-0994

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics
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Carrara, Fernanda Souza Angotti

; Piotto, Daniela Gerent Petry

; Silva, Ilana Izidoro

; Len, Claudio Arnaldo

; Russo, Gleice Clemente Souza

; Chiba, Sonia Mayumi

; Sdepanian, Vera Lucia

; Braga, Josefina Aparecida Pellegrini

; Figueiredo, Maria Stella

; Andrade, Maria Cristina

; de Almeida Maia, Marta Liliane

; Abreu, Ana Lúcia

; Silva, Celia Maria Camelo

; Terreri, Maria Teresa

Jornal de Pediatria

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Jun 2023, Volume 99 Nº 3 Páginas 254 - 262

Resumo: En | Texto: En | PDF: En

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores. illnesses grown Brazil crosssectional cross sectional 30 1 2 followup follow up specialties (TRAQ domains data socioeconomic socio economic adherence functionality evaluated 37 3 7 3. 3.2 32 (3. 4.2. 42 4.2 . 4 4.2) AB, AB B, B A-B employment education year alone medications age alphaCronbach alpha Cronbach 0.86. 086 0.86 0 86 0.86) regression gender approach system scores (3 4. 08 0.8 8 ( 0.

https://doi.org/10.1016/j.jped.2022.10.006

Sickle cell anemia: hierarchical cluster analysis and clinical profile in a cohort in Brazil anemia
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Dutra, Valéria de Freitas

; Biassi, Thais Priscila

; Figueiredo, Maria Stella

Hematology, Transfusion and Cell Therapy

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Mar 2023, Volume 45 Nº 1 Páginas 45 - 51

Resumo: En | Texto: En | PDF: En

Abstract Introduction Sickle cell anemia is a monogenic disorder caused by a mutation in the β-hemoglobin gene, resulting in sickle hemoglobin that can polymerize. Presentation and clinical course have significant inter-individual variability and classifying these patients for severity is a challenge. Methods We applied hierarchical clusters with 10 routine laboratory tests to understand if this grouping could be associated with clinical manifestations. We included 145 adult homozygous patients (SS) at an outpatient clinic in a retrospective study. Results We found five clusters by counting those that had been differentiated by unconjugated bilirubin, reticulocytes, LDH, leukocytes, lymphocytes and monocytes. When comparing groups to clinical findings, the clusters were different only for liver abnormality. Cluster 3 had the lower median of reticulocytes, LDH, leukocytes, lymphocytes and monocytes and a higher percentage of patients under treatment. Clusters 4 and 5 had higher frequencies of liver impairment and higher medians of reticulocytes, LDH, leukocytes, lymphocytes and monocytes. Hemolysis and inflammation seemed to influence the grouping. Conclusion In our study, cluster analysis showed five groups that exhibited different degrees of inflammation and hemolysis. When comparing clinical data, the result was different only for the criteria of liver abnormality. βhemoglobin β gene polymerize interindividual inter individual challenge 1 manifestations 14 SS (SS study bilirubin reticulocytes LDH leukocytes findings abnormality treatment hemolysis data

https://doi.org/10.1016/j.htct.2021.08.015

Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria
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Cançado, Rodolfo D

; Araújo, Aderson da Silva

; Sandes, Alex Freire

; Arrais, Celso

; Lobo, Clarisse Lopes de Castro

; Figueiredo, Maria Stella

; Gualandro, Sandra Fátima Menosi

; Saad, Sara Teresinha Olalla

; Costa, Fernando Ferreira

Hematology, Transfusion and Cell Therapy

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Set 2021, Volume 43 Nº 3 Páginas 341 - 348

Resumo: En | Texto: En | PDF: En

Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and lifethreatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients.

https://doi.org/10.1016/j.htct.2020.06.006 23 downloads

COVID-19 as a trigger of acute chest syndrome in a pregnant woman with sickle cell anemia
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Justino, Caio Cesar

; Campanharo, Felipe Favorette

; Augusto, Marina Nobrega

; Morais, Stela Cezarino de

; Figueiredo, Maria Stella

Hematology, Transfusion and Cell Therapy

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Set 2020, Volume 42 Nº 3 Páginas 212 - 214

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.htct.2020.06.003 577 downloads

Quality of life in adults with sickle cell disease: an integrative review of the literature
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Freitas, Sandra Luzinete Felix de

; Ivo, Maria Lucia

; Figueiredo, Maria Stella

; Gerk, Maria Auxiliadora de Souza

; Nunes, Cristina Brandt

; Monteiro, Fernando de Freitas

Revista Brasileira de Enfermagem

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Fev 2018, Volume 71 Nº 1 Páginas 195 - 205

Resumo: Pt En Es | Texto: Pt En Es | PDF: Pt | PDF: En | PDF: Es

RESUMO Objetivo: Identificar as evidências disponíveis na literatura sobre qualidade de vida relacionada à saúde em adultos com doença falciforme. Método: revisão integrativa nas bases de dados MEDLINE, CUMED, LILACS e SciELO, de artigos provenientes de estudos desenvolvidos nesta temática, publicados entre 2005 a 2015, em inglês, português ou espanhol. Resultados: foram incluídos 22 artigos, sendo utilizadas seis escalas para avaliar os escores de qualidade de vida relacionada à saúde: três genéricas e três específicas. Nenhuma escala específica para adultos com doença falciforme foi adaptada para o português brasileiro até o momento. Pacientes acometidos por crises dolorosas frequentes, com baixa adesão ao tratamento, apresentaram comprometimento da qualidade de vida. Conclusão: Os estudos selecionados evidenciaram que os pacientes com doença falciforme têm piores escores do que a população em geral. Esses indicadores deveriam instrumentalizar o enfermeiro na proposição de intervenções e estratégias assistenciais e socioeducativas, com vistas a melhorar a qualidade de vida desta clientela.

ABSTRACT Objective: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. Method: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. Results: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Conclusion: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.

RESUMEN Objetivo: Identificar las evidencias disponibles en la literatura sobre cualidad de vida relacionada a la salud en adultos con enfermedad falciforme. Método: revisión integradora en las bases de datos MEDLINE,CUMED,LILACS y SciELO, de artículos provenientes de estudios desarrollados en esta temática, publicados entre 2005 y 2015, en inglés, portugués o español. Resultados: fueron incluidos 22 artículos, siendo utilizadas seis escalas para evaluar las puntuaciones de cualidad de vida relacionada a la salud: tres genéricas y tres específicas. Ninguna escala específica para adultos con enfermedad falciforme fue adaptada para el portugués brasileño hasta el momento. Pacientes acometidos por crisis dolorosas frecuentes, con baja adhesión al tratamiento, presentaron comprometimiento de la cualidad de vida. Conclusión: Los estudios seleccionados evidenciaron que los pacientes con enfermedad falciforme tienen peores puntuaciones que la población en general. Esos indicadores deberían instrumentalizar el enfermero en la proposición de intervenciones y estrategias asistenciales y socioeducativas, con vistas a mejorar la cualidad de vida de esta clientela.

https://doi.org/10.1590/0034-7167-2016-0409 11236 downloads

10.

Quality of life in adults with sickle cell disease: an integrative review of the literature
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Freitas, Sandra Luzinete Felix de

; Ivo, Maria Lucia

; Figueiredo, Maria Stella

; Gerk, Maria Auxiliadora de Souza

; Nunes, Cristina Brandt

; Monteiro, Fernando de Freitas

Revista Brasileira de Enfermagem

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Fev 2018, Volume 71 Nº 1 Páginas 195 - 205

Resumo: En Es Pt | Texto: En Es Pt | PDF: En | PDF: Es | PDF: Pt

https://doi.org/10.1590/0034-7167-2016-0409 40 downloads

11.

Challenges in the diagnosis of iron deficiency anemia in aged people
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Moraes, Niele Silva de

; Figueiredo, Maria Stella

Revista Brasileira de Hematologia e Hemoterapia

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Set 2017, Volume 39 Nº 3 Páginas 191 - 192

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.bjhh.2017.03.005 985 downloads

12.

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence
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Rodrigues, Daniela Laranja Gomes

; Adegoke, Samuel Ademola

; Campos, Rejane de Souza Macedo

; Braga, Josefina Aparecida Pellegrini

; Figueiredo, Maria Stella

; Silva, Gisele Sampaio

Arquivos de Neuro-Psiquiatria

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Jan 2017, Volume 75 Nº 1 Páginas 15 - 19

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

RESUMO Doppler transcraniano (DTC) é um componente estratégico da prevenção primária do acidente vascular cerebral (AVC) em crianças com doença falciforme (DF). Este estudo foi realizado para examinar as características do DTC de crianças com DF em nove centros médicos diferentes no Brasil. Métodos: DTC foi realizado de acordo com o protocolo de Stroke Prevention Trial in Sickle Cell Anemia Protocol (STOP). Resultados: Dos 396 pacientes avaliados, 69,5% eram homozigotos para hemoglobina SS. DTC foi anormal em 4,8%, condicional em 12,6%, inadequado em 5,3% e anormalmente baixo em 1%. As máximas velocidades de fluxo médio foram 121 ± 23,83cm/s e 124 ± 27,21 cm/s nas artérias cerebrais media esquerda e direita, respectivamente. Um total de 28,8% dos pacientes (média de 9,19 ± 5,92 anos) foram avaliados com o DTC pela primeira vez. Conclusões: Pacientes com DF foram avaliados com DTC numa idade considerada avançada, o que representa uma importante oportunidade perdida para a prevenção de AVC nessa população. Uma vez que a triagem com DTC em pacientes com DF é essencial para detectar aqueles com alto risco de AVC, recomenda-se que essa triagem seja amplamente disponível no país.

ABSTRACT Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19±5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.

https://doi.org/10.1590/0004-282x20160175 2445 downloads

13.

Comments on: "Clinical, hematological and genetic data of a cohort of children with hemoglobin SD"
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Figueiredo, Maria Stella

Revista Brasileira de Hematologia e Hemoterapia

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Set 2016, Volume 38 Nº 3 Páginas 190 - 192

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.bjhh.2016.05.012 735 downloads

14.

The importance of hemoglobin A2 determination
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Figueiredo, Maria Stella

Revista Brasileira de Hematologia e Hemoterapia

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Out 2015, Volume 37 Nº 5 Páginas 287 - 289

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.bjhh.2015.06.002 4839 downloads

15.

Haptoglobin gene polymorphisms and interleukin-6 and -8 levels in patients with sickle cell anemia
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Pierrot-Gallo, Bruna Spinella

; Vicari, Perla

; Matsuda, Sandra Satiko

; Adegoke, Samuel Ademola

; Mecabo, Grazielle

; Figueiredo, Maria Stella

Revista Brasileira de Hematologia e Hemoterapia

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Out 2015, Volume 37 Nº 5 Páginas 329 - 335

Resumo: En | Texto: En | PDF: En

BACKGROUND: Haptoglobin genotypes, and interleukin-6 and -8 participate in the pathophysiology of sickle cell anemia. The expression of cytokines is regulated by genetic mechanisms however the effect of haptoglobin polymorphisms on these cytokines is not fully understood. This study aimed to compare the frequency of haptoglobin genotypes and the interleukin-6 and -8 concentrations in sickle cell anemia patients and controls to investigate the association between haptoglobin genotypes and cytokine levels.METHODS: Sixty sickle cell anemia patients and 74 healthy individuals were analyzed. Haptoglobin genotypes were determined by multiplex polymerase chain reaction, and the interleukin-6 and -8 levels by enzyme linked immunosorbent assay. The association between haptoglobin genotypes and cytokines was investigated by statistical tests.RESULTS:Hp2-1 was the most common genotype in both the cases and controls while Hp1-1 was less frequent among sickle cell anemia patients. Interleukin-6 and -8 levels were higher in patients than controls (p-value <0.0001). There was no significant difference in interleukin-6 and -8 concentrations between the genotypes (p-value >0.05). A similar trend was observed among the controls.CONCLUSION: Although, levels of interleukin-6 and -8 were higher in the sickle cell anemia patients, they appeared not to be related to the haptoglobin genotypes. Further investigations are necessary to identify factors responsible for increased secretion of the interleukin-6 and -8 pro-inflammatory cytokines in patients with sickle cell anemia.

https://doi.org/10.1016/j.bjhh.2015.07.006 1789 downloads

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ab	resumo
ta	título abreviado da revista (ex. Cad. Saúde Pública)
journal_title	título completo da revista (ex. Cadernos de Saúde Pública)
la	código do idioma da publicação (ex. pt - Português, es - Espanhol)
type	tipo do documento
pid	identificador da publicação
publication_year	ano de publicação do artigo
sponsor	financiador
aff_country	código do país de afiliação do autor
aff_institution	instituição de afiliação do autor
volume	volume do artigo
issue	número do artigo
elocation	elocation
doi	número DOI
issn	ISSN da revista
in	código da coleção SciELO (ex. scl - Brasil, col - Colômbia)
use_license	código da licença de uso do artigo

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