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β-重型地中海贫血患者生长并发症的系统评价和荟萃分析。

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients.

机构信息

Social Determination of Health Research Center, Birjand University of Medical Sciences, Birjand, Iran.

Department of Hematology and Blood Banking, School of Paramedical Sciences, Birjand University of Medical Sciences, Birjand, Iran.

出版信息

Ann Glob Health. 2021 Jun 8;87(1):48. doi: 10.5334/aogh.3184.

DOI:10.5334/aogh.3184
PMID:34164261
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8194969/
Abstract

BACKGROUND

Blood transfusion is a traditional treatment for β-thalassemia (β-thal) that improves the patients' anemia and lifespan, but it may lead to iron overload in parenchymal tissue organs and endocrine glands that cause their dysfunctions as the iron regulatory system can't excrete excess iron from the bloodstream.

OBJECTIVE

To evaluate the prevalence of iron-related complications (short stature, growth retardation, and growth hormone deficiency) in β-thalassemia major (TM) patients.

METHODS

We performed an electronic search in PubMed, Scopus, and Web of Sciences to evaluate the prevalence of growth hormone impairment in β-thalassemia major (TM) patients worldwide. Qualities of eligible studies were assessed by the Joanna Briggs Institute checklist for the prevalence study. We used Comprehensive Meta-Analysis (Version 2) to calculate the event rate with 95% CIs, using a random-effects model for all analyses.

FINDINGS

Seventy-four studies were included from five continents between 1978 and 2019; 70.27% (Asia), 16.21% (Europe), 6.75% (Africa), 2.70% (America), 1.35% (Oceania), and 2.70% (Multicenter). The overall mean age of the participants was about 14 years. The pooled prevalence of short stature (ST) was 48.9% (95% CI 35.3-62.6) and in male was higher than female (61.9%, 95% CI 53.4-69.7 vs. 50.9%, CI 41.8-59.9). The pooled prevalence of growth retardation (GR) was 41.1% and in male was higher than in female (51.6%, 95% CI 17.8-84 vs. 33.1%, CI 9.4-70.2). The pooled prevalence of growth hormone deficiency (GHD) was 26.6% (95% CI 16-40.8).

CONCLUSION

Our study revealed that near half of thalassemia patients suffer from growth impairments. However, regular evaluation of serum ferritin levels, close monitoring in a proper institute, suitable and acceptable treatment methods besides regular chelation therapy could significantly reduce the patients' complications.

摘要

背景

输血是治疗β-地中海贫血(β-thal)的传统方法,可改善患者的贫血和寿命,但可能导致实质组织器官和内分泌腺中铁过载,从而导致其功能障碍,因为铁调节系统无法将过量的铁从血液中排出。

目的

评估β-地中海贫血重型(TM)患者中铁相关并发症(身材矮小、生长迟缓、生长激素缺乏)的患病率。

方法

我们在 PubMed、Scopus 和 Web of Sciences 上进行了电子检索,以评估全球范围内β-地中海贫血重型(TM)患者生长激素受损的患病率。使用 Joanna Briggs Institute 患病率研究清单评估合格研究的质量。我们使用 Comprehensive Meta-Analysis(版本 2)计算事件发生率和 95%置信区间(CI),所有分析均采用随机效应模型。

结果

从 1978 年至 2019 年,来自五大洲的 74 项研究被纳入;70.27%(亚洲)、16.21%(欧洲)、6.75%(非洲)、2.70%(美洲)、1.35%(大洋洲)和 2.70%(多中心)。参与者的平均年龄约为 14 岁。身材矮小(ST)的总患病率为 48.9%(95%CI 35.3-62.6),男性高于女性(61.9%,95%CI 53.4-69.7 与 50.9%,CI 41.8-59.9)。生长迟缓(GR)的总患病率为 41.1%,男性高于女性(51.6%,95%CI 17.8-84%与 33.1%,CI 9.4-70.2)。生长激素缺乏(GHD)的总患病率为 26.6%(95%CI 16-40.8)。

结论

我们的研究表明,近一半的地中海贫血患者患有生长障碍。然而,定期评估血清铁蛋白水平、在适当的机构进行密切监测、除了常规螯合治疗外,采用合适和可接受的治疗方法,可显著降低患者的并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d356/8194969/e970d52015fc/agh-87-1-3184-g6.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d356/8194969/e970d52015fc/agh-87-1-3184-g6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d356/8194969/fc7f93ab8259/agh-87-1-3184-g1.jpg
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