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β-地中海贫血杂合子状态对健康预期有不利影响。

β-Thalassemia heterozygote state detrimentally affects health expectation.

机构信息

Campus of Hematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, Palermo, Italy.

Dipartimento Attività Sanitarie e Osservatorio Epidemiologico Assessorato Salute Regione Sicilia, Palermo, Italy.

出版信息

Eur J Intern Med. 2018 Aug;54:76-80. doi: 10.1016/j.ejim.2018.06.009. Epub 2018 Jun 19.

Abstract

BACKGROUND

Thalassemia minor (Tm) individuals, are generally considered healthy. However, the prognosis of Tm individuals has not been extensively studied. The aim of this study was to evaluate the prognosis of Tm versus controls without β-thalassemia carrier state.

METHODS

A total of 26,006 individuals seeking thalassemia screening at the AOOR Villa Sofia-V. Cervello, Palermo (Italy) were retrospectively studied. Logistic penalised regression model was used to estimate risk of potential complications and survival techniques were used to study mortality.

RESULTS

We identified a total of 4943 Tm and 21,063 controls. Tm was associated with significantly higher risks of hospitalisation for cirrhosis (OR 1·94, 95% CI 1·30 to 2·90, p = 0·001), kidney disorders (OR 2·11, 95% CI 1·27 to 3·51, p = 0·004), cholelithiatis (OR 1·39, 95% CI 1·08 to 1·79, p = 0·010), and mood disorders (OR 2·08, 95% CI 1·15 to 3·75, p = 0·015). No statistically difference in life expectancy between thalassemia minor and control group was found (HR 1·090, 95% CI 0·777 to 1·555, p < 0·590; log-rank test p = .426).

CONCLUSION

This study shows that Tm affects the prognosis of Tm carriers regarding health expectation. Probably, iron overload and anaemia for several years may be at the basis of these effects.

摘要

背景

轻度地中海贫血(Tm)个体通常被认为是健康的。然而,Tm 个体的预后尚未得到广泛研究。本研究旨在评估 Tm 个体与无β-地中海贫血携带者状态的对照组的预后。

方法

回顾性研究了在意大利巴勒莫 AOOR Villa Sofia-V. Cervello 接受地中海贫血筛查的 26006 名个体。使用逻辑惩罚回归模型估计潜在并发症的风险,使用生存技术研究死亡率。

结果

我们共确定了 4943 名 Tm 和 21063 名对照组。Tm 与肝硬化(OR 1.94,95%CI 1.30-2.90,p=0.001)、肾脏疾病(OR 2.11,95%CI 1.27-3.51,p=0.004)、胆结石(OR 1.39,95%CI 1.08-1.79,p=0.010)和情绪障碍(OR 2.08,95%CI 1.15-3.75,p=0.015)的住院风险显著增加相关。在预期寿命方面,轻度地中海贫血组和对照组之间没有统计学差异(HR 1.090,95%CI 0.777-1.555,p<0.590;对数秩检验 p=0.426)。

结论

本研究表明,Tm 影响 Tm 携带者的健康预期预后。可能是多年的铁过载和贫血导致了这些影响。

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