• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

镰状细胞贫血患者的临床和实验室特征

Clinical and laboratory profile of patients with sickle cell anemia.

作者信息

Sant'Ana Phelipe Gabriel Dos Santos, Araujo Ariane Moreira, Pimenta Cynthia Teixeira, Bezerra Mário Lúcio Pacheco Ker, Junior Sílvio Pereira Borges, Neto Viviana Martins, Dias Janaina Sousa, Lopes Aline de Freitas, Rios Danyelle Romana Alves, Pinheiro Melina de Barros

机构信息

Universidade Federal de São João Del Rei (UFSJ), Divinópolis, MG, Brazil.

Fundação Centro de Hematologia e Hemoterapia do Estado de Minas Gerais (HEMOMINAS), Divinópolis, MG, Brazil.

出版信息

Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):40-45. doi: 10.1016/j.bjhh.2016.09.007. Epub 2016 Oct 19.

DOI:10.1016/j.bjhh.2016.09.007
PMID:28270345
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5339391/
Abstract

OBJECTIVE

This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea.

METHODS

Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia.

RESULTS

Data from the medical records of 50 patients were analyzed. Most of the patients were female (56%), aged between 20 and 29 years old. Infections, transfusions, cholecystectomy, splenectomy and systemic arterial hypertension were the most common clinical adverse events of the patients. The most frequent cause of hospitalization was painful crisis. The majority of patients had reduced values of hemoglobin and hematocrit (8.55±1.33g/dL and 25.7±4.4%, respectively) and increased fetal hemoglobin levels (12±7%). None of the clinical variables was statistically significant on comparing the two groups of patients. Among hematological variables only hemoglobin and hematocrit levels were statistically different between patients treated with hydroxyurea and untreated patients (p-value=0.005 and p-value=0.001, respectively).

CONCLUSION

Sickle cell anemia requires treatment and follow-up by a multiprofessional team. A current therapeutic option is hydroxyurea. This drug reduces complications and improves laboratorial parameters of patients. In this study, the use of the drug increased the hemoglobin and hematocrit levels of patients.

摘要

目的

本研究旨在描述和分析在巴西迪维诺波利斯的Hemominas基金会接受治疗的镰状细胞贫血患者的临床和实验室特征。此外,本研究旨在比较接受羟基脲治疗的患者组与未接受羟基脲治疗的患者的临床和实验室结果。

方法

通过分析镰状细胞贫血患者的病历获得临床和实验室数据。

结果

分析了50例患者的病历数据。大多数患者为女性(56%),年龄在20至29岁之间。感染、输血、胆囊切除术、脾切除术和系统性动脉高血压是患者最常见的临床不良事件。住院最常见的原因是疼痛性危机。大多数患者的血红蛋白和血细胞比容值降低(分别为8.55±1.33g/dL和25.7±4.4%),胎儿血红蛋白水平升高(12±7%)。在比较两组患者时,没有临床变量具有统计学意义。在血液学变量中,只有接受羟基脲治疗的患者和未接受治疗的患者之间的血红蛋白和血细胞比容水平存在统计学差异(p值分别为0.005和0.001)。

结论

镰状细胞贫血需要多专业团队进行治疗和随访。目前的一种治疗选择是羟基脲。这种药物可减少并发症并改善患者的实验室指标。在本研究中,使用该药物提高了患者的血红蛋白和血细胞比容水平。

相似文献

1
Clinical and laboratory profile of patients with sickle cell anemia.镰状细胞贫血患者的临床和实验室特征
Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):40-45. doi: 10.1016/j.bjhh.2016.09.007. Epub 2016 Oct 19.
2
Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. The Multicenter Study of Hydroxyurea in Sickle Cell Anemia.羟基脲与镰状细胞贫血。一种骨髓抑制性“转换”药物的临床应用。羟基脲治疗镰状细胞贫血多中心研究。
Medicine (Baltimore). 1996 Nov;75(6):300-26. doi: 10.1097/00005792-199611000-00002.
3
Patients with sickle cell disease taking hydroxyurea in the Hemocentro Regional de Montes Claros.在蒙特斯克拉罗斯地区血液中心服用羟基脲的镰状细胞病患者。
Rev Bras Hematol Hemoter. 2011;33(2):105-9. doi: 10.5581/1516-8484.20110029.
4
Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.羟基脲对成年镰状细胞贫血患者死亡率和发病率的影响:长达9年治疗期的风险与益处
JAMA. 2003 Apr 2;289(13):1645-51. doi: 10.1001/jama.289.13.1645.
5
Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia.羟基脲对镰状细胞贫血疼痛性危象发作频率的影响。镰状细胞贫血羟基脲多中心研究的研究者们。
N Engl J Med. 1995 May 18;332(20):1317-22. doi: 10.1056/NEJM199505183322001.
6
Influence of βS-globin haplotypes and hydroxyurea on tumor necrosis factor-alpha levels in sickle cell anemia.βS-珠蛋白单倍型和羟基脲对镰状细胞贫血患者肿瘤坏死因子-α水平的影响。
Rev Bras Hematol Hemoter. 2014 Mar;36(2):121-5. doi: 10.5581/1516-8484.20140028.
7
Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment.接受羟基脲治疗的不同亚型镰状细胞病患者的血液学差异。
Rev Bras Hematol Hemoter. 2012;34(6):426-9. doi: 10.5581/1516-8484.20120107.
8
Analysis of oxidative status and biochemical parameters in adult patients with sickle cell anemia treated with hydroxyurea, Ceará, Brazil.巴西塞阿拉州接受羟基脲治疗的成年镰状细胞贫血患者氧化状态和生化参数分析
Rev Bras Hematol Hemoter. 2011;33(3):207-10. doi: 10.5581/1516-8484.20110055.
9
Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease.促红细胞生成素增强镰状细胞病胎儿血红蛋白对羟基脲的反应
N Engl J Med. 1993 Jan 14;328(2):73-80. doi: 10.1056/NEJM199301143280201.
10
Hydroxyurea for Children with Sickle Cell Anemia in Sub-Saharan Africa.在撒哈拉以南非洲,用羟脲治疗镰状细胞贫血儿童。
N Engl J Med. 2019 Jan 10;380(2):121-131. doi: 10.1056/NEJMoa1813598. Epub 2018 Dec 1.

引用本文的文献

1
Inhibition of sodium-glucose cotransporter-2 improves anaemia in mice and humans with sickle cell disease, and reduces infarct size in a murine stroke model.钠-葡萄糖共转运蛋白 2 抑制剂可改善镰状细胞病小鼠和人类的贫血,并减少小鼠卒中模型中的梗死面积。
J Cell Mol Med. 2024 Sep;28(17):e70091. doi: 10.1111/jcmm.70091.
2
Sickle Cell Anemia Screening in Newborns and Analysis of Haplotypes in Patients from Santiago Island, Cape Verde.佛得角圣地亚哥岛新生儿镰状细胞贫血筛查及患者单倍型分析
Anemia. 2024 Aug 27;2024:1687917. doi: 10.1155/2024/1687917. eCollection 2024.
3
Electrocardiographic Changes and Their Association With Disease Severity in Adults With Sickle Cell Anemia at a Tertiary Care Center: A Cross-Sectional Study.三级护理中心成年镰状细胞贫血患者的心电图变化及其与疾病严重程度的关联:一项横断面研究
Cureus. 2024 May 13;16(5):e60197. doi: 10.7759/cureus.60197. eCollection 2024 May.
4
Clinical Profiles of Children With Sickle Cell Anaemia Presenting With Acute Clinical Events: A Single-Center Study.患有镰状细胞贫血且出现急性临床事件的儿童的临床概况:一项单中心研究。
Cureus. 2023 May 14;15(5):e39008. doi: 10.7759/cureus.39008. eCollection 2023 May.
5
Feature tracking microfluidic analysis reveals differential roles of viscosity and friction in sickle cell blood.特征跟踪微流控分析揭示了粘度和摩擦力在镰状细胞血液中的不同作用。
Lab Chip. 2022 Apr 12;22(8):1565-1575. doi: 10.1039/d1lc01133b.
6
Sickle cell anemia: hierarchical cluster analysis and clinical profile in a cohort in Brazil.镰状细胞贫血:巴西一组人群的层次聚类分析及临床特征
Hematol Transfus Cell Ther. 2023 Jan-Mar;45(1):45-51. doi: 10.1016/j.htct.2021.08.015. Epub 2021 Dec 9.
7
Whole blood viscosity and red blood cell adhesion: Potential biomarkers for targeted and curative therapies in sickle cell disease.全血黏度和红细胞黏附:镰状细胞病靶向和治愈疗法的潜在生物标志物。
Am J Hematol. 2020 Nov;95(11):1246-1256. doi: 10.1002/ajh.25933. Epub 2020 Aug 10.
8
Factors Influencing Motivation and Engagement in Mobile Health Among Patients With Sickle Cell Disease in Low-Prevalence, High-Income Countries: Qualitative Exploration of Patient Requirements.低患病率高收入国家镰状细胞病患者移动健康中的动机与参与影响因素:患者需求的定性探索
JMIR Hum Factors. 2020 Mar 24;7(1):e14599. doi: 10.2196/14599.
9
HAEMATOLOGICAL PROFILE AND BLOOD TRANSFUSION PATTERN OF PATIENTS WITH SICKLE CELL ANAEMIA VARY WITH SPLEEN SIZE.镰状细胞贫血患者的血液学特征和输血模式随脾脏大小而变化。
Ann Ib Postgrad Med. 2019 Jun;17(1):30-38.
10
Geographic Differences in Phenotype and Treatment of Children with Sickle Cell Anemia from the Multinational DOVE Study.来自多国家DOVE研究的镰状细胞贫血患儿的表型和治疗的地理差异。
J Clin Med. 2019 Nov 17;8(11):2009. doi: 10.3390/jcm8112009.

本文引用的文献

1
Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil.羟基脲疗法对镰状细胞病患者的临床和血液学影响:巴西的单中心经验
Sao Paulo Med J. 2013;131(4):238-43. doi: 10.1590/1516-3180.2013.1314467.
2
The natural history of sickle cell disease.镰状细胞病的自然史。
Cold Spring Harb Perspect Med. 2013 Oct 1;3(10):a011783. doi: 10.1101/cshperspect.a011783.
3
[Ocular and electroretinographic changes in sickle cell disease].[镰状细胞病的眼部及视网膜电图改变]
Arq Bras Oftalmol. 2011 May-Jun;74(3):190-4. doi: 10.1590/s0004-27492011000300009.
4
Pulmonary hypertension and nitric oxide depletion in sickle cell disease.镰状细胞病中的肺动脉高压和一氧化氮耗竭。
Blood. 2010 Aug 5;116(5):687-92. doi: 10.1182/blood-2010-02-268193. Epub 2010 Apr 15.
5
Gallstones in children with sickle cell disease followed up at a Brazilian hematology center.在巴西一家血液学中心接受随访的镰状细胞病患儿的胆结石情况。
Arq Gastroenterol. 2008 Oct-Dec;45(4):313-8. doi: 10.1590/s0004-28032008000400010.
6
[Prevalence of hemoglobin S in the State of Paraná, Brazil, based on neonatal screening].[基于新生儿筛查的巴西巴拉那州血红蛋白S患病率]
Cad Saude Publica. 2008 May;24(5):993-1000. doi: 10.1590/s0102-311x2008000500006.
7
Open splenectomy in Jamaican children with sickle cell disease.牙买加镰状细胞病患儿的开放性脾切除术
West Indian Med J. 2006 Jan;55(1):37-41. doi: 10.1590/s0043-31442006000100009.
8
[Epidemiology of sickle cell disease hospital admissions in Brazil].[巴西镰状细胞病住院情况的流行病学]
Rev Saude Publica. 2005 Dec;39(6):943-9. doi: 10.1590/s0034-89102005000600012. Epub 2005 Dec 7.
9
[Sickle cell disease and infection].[镰状细胞病与感染]
J Pediatr (Rio J). 2004 Sep-Oct;80(5):347-54.
10
Gallstones in sickle cell disease: observations from The Jamaican Cohort study.镰状细胞病中的胆结石:来自牙买加队列研究的观察结果。
J Pediatr. 2000 Jan;136(1):80-5. doi: 10.1016/s0022-3476(00)90054-4.