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吉赞地区重型β地中海贫血患者的临床和血液学特征:一项基于医院的研究。

Clinical and hematological features among β-thalassemia major patients in Jazan region: A hospital-based study.

作者信息

Khawaji Mohammed Mansour, Hazzazi Ahmad Ali, Ageeli Mohssen Hassen, Mawkili Yahya Hassan, Darbashi Abdulrahman Hussain, Abo Kathiyah Anwar Mohammed Ali, Humedi Rehab Abdalrhman

机构信息

Faculty of Medicine, Jazan University, Jazan, Saudi Arabia.

出版信息

J Family Med Prim Care. 2020 Jan 28;9(1):412-417. doi: 10.4103/jfmpc.jfmpc_1007_19. eCollection 2020 Jan.

DOI:10.4103/jfmpc.jfmpc_1007_19
PMID:32110628
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7014835/
Abstract

CONTEXT

Beta-thalassemia is a prevalent hereditary blood disorder in Saudi Arabia. Various clinical manifestations and hematological abnormalities are common among these patients.

AIMS

To evaluate the clinical manifestations and hematological parameters of β-thalassemia patients in Jazan, Saudi Arabia.

SETTINGS AND DESIGN

This is a cross-sectional study conducted in Jazan region, Saudi Arabia.

METHODS AND MATERIALS

We included 36 β-Thalassemia major patients who admitted to PMNH during the period from February 1, 2016 to December 31, 2017. Clinical features were obtained via physical examination. The hematological parameters were obtained from the patients' medical records.

STATISTICAL ANALYSIS USED

Descriptive and inferential statistics were conducted using SPSS. Continuous data was evaluated using independent sample -test and Chi-square was used to evaluate categorical variables. < 0.05 indicated statistical significance.

RESULTS

Mean age was 18.56 ± 6.89 years. Males were 23 (63.9%). About 41.4% of patients were underweight. Exposing of the upper teeth was the most prominent feature ( = 19, 59.4%). The majority of patients were splenectomized (62.1%). About two-thirds have moderate anemia. More than half of the patients (58.8%) had high WBCs counts and 35.3% had a high platelet count, especially among those who underwent splenectomy. Approximately, two-thirds had a ferritin level higher than 2500 ng/ml. O blood group was the most frequent blood group among the patients ( = 15, 46.9%).

CONCLUSIONS

Deteriorated clinical and hematological parameters in our study justify the need for more efforts for regular evaluation and follow-up of the βTM patients and reevaluation of the management protocols to be improved or modified.

摘要

背景

β地中海贫血是沙特阿拉伯一种常见的遗传性血液疾病。这些患者中各种临床表现和血液学异常很常见。

目的

评估沙特阿拉伯吉赞地区β地中海贫血患者的临床表现和血液学参数。

设置与设计

这是一项在沙特阿拉伯吉赞地区进行的横断面研究。

方法与材料

我们纳入了2016年2月1日至2017年12月31日期间入住PMNH的36例重型β地中海贫血患者。通过体格检查获得临床特征。血液学参数从患者的病历中获取。

所用统计分析

使用SPSS进行描述性和推断性统计。连续数据使用独立样本t检验进行评估,卡方检验用于评估分类变量。P<0.05表示具有统计学意义。

结果

平均年龄为18.56±6.89岁。男性有23例(63.9%)。约41.4%的患者体重过轻。上牙外露是最突出的特征(n = 19,59.4%)。大多数患者接受了脾切除术(62.1%)。约三分之二的患者有中度贫血。超过一半的患者(58.8%)白细胞计数高,35.3%的患者血小板计数高,尤其是在接受脾切除术的患者中。大约三分之二的患者铁蛋白水平高于2500 ng/ml。O血型是患者中最常见的血型(n = 15,46.9%)。

结论

我们研究中临床和血液学参数的恶化证明需要加大力度对重型β地中海贫血患者进行定期评估和随访,并重新评估管理方案以进行改进或修改。

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本文引用的文献

1
Hematological and biochemical evaluation of β-thalassemia major (βTM) patients in Gaza Strip: A cross-sectional study.加沙地带重型β地中海贫血(βTM)患者的血液学和生化评估:一项横断面研究。
Int J Health Sci (Qassim). 2018 Nov-Dec;12(6):18-24.
2
Distribution of hemoglobinopathy disorders in Saudi Arabia based on data from the premarital screening and genetic counseling program, 2011-2015.基于 2011-2015 年婚前检查和遗传咨询计划的数据,沙特阿拉伯的血红蛋白病分布情况。
J Epidemiol Glob Health. 2018 Mar;7 Suppl 1(Suppl 1):S41-S47. doi: 10.1016/j.jegh.2017.12.001. Epub 2017 Dec 15.
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The new era of chelation treatments: effectiveness and safety of 10 different regimens for controlling iron overloading in thalassaemia major.螯合治疗的新时代:10种不同方案控制重型地中海贫血铁过载的有效性和安全性
Br J Haematol. 2017 Sep;178(5):676-688. doi: 10.1111/bjh.14712. Epub 2017 Apr 25.
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Increasing prevalence of thalassemia in America: Implications for primary care.美国地中海贫血患病率上升:对初级保健的影响。
Ann Med. 2015;47(7):592-604. doi: 10.3109/07853890.2015.1091942. Epub 2015 Nov 5.
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Effectiveness of Prenatal Screening for Hemoglobinopathies in a Developing Country.发展中国家血红蛋白病产前筛查的有效性
Hemoglobin. 2015;39(6):380-3. doi: 10.3109/03630269.2014.1003564. Epub 2015 May 13.
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Association of ABO and Rh Blood Groups to Blood-Borne Infections among Blood Donors in Tehran-Iran.伊朗德黑兰献血者中ABO和Rh血型与血源性感染的关联
Iran J Public Health. 2014 Jul;43(7):981-9.
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J Family Med Prim Care. 2014 Jul;3(3):219-23. doi: 10.4103/2249-4863.141614.
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