法布里病患者中未控制高血压的患病率。

Prevalence of uncontrolled hypertension in patients with Fabry disease.

作者信息

Kleinert Julia, Dehout François, Schwarting Andreas, de Lorenzo Abelardo García, Ricci Roberta, Kampmann Christoph, Beck Michael, Ramaswami Uma, Linhart Ales, Gal Andreas, Houge Gunnar, Widmer Urs, Mehta Atul, Sunder-Plassmann Gere

机构信息

Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria.

出版信息

Am J Hypertens. 2006 Aug;19(8):782-7. doi: 10.1016/j.amjhyper.2006.01.011.

DOI:10.1016/j.amjhyper.2006.01.011

PMID:16876675

Abstract

BACKGROUND

Fabry disease is a rare X-linked disease arising from deficiency of alpha-galactosidase A. It results in early death related to renal, cardiac, and cerebrovascular disease, which are also important outcomes in patients with elevated blood pressure (BP). The prevalence of uncontrolled hypertension, as well as the effect of enzyme replacement therapy on BP, in patients with Fabry disease is unknown.

METHODS

We examined uncontrolled hypertension (systolic BP [SBP] >or=130 mm Hg or diastolic BP [DBP] >or=80 mm Hg) among 391 patients with Fabry disease who were participating in the Fabry Outcome Survey (FOS).

RESULTS

Uncontrolled hypertension was present in 57% of men and 47% of women. In patients with chronic kidney disease (CKD) stage 1 (n100), median SBP was 120 mm Hg and median DBP was 74 mm Hg. In patients with CKD stage 2 (n172), median SBP was 125 mm Hg and median DBP was 75 mm Hg. In patients with CKD stage 3 (n63), median SBP was 130 mm Hg and median DBP was 75 mm Hg. There was a significant decrease in both SBP and DBP during a 2-year course of enzyme replacement therapy.

CONCLUSIONS

This study revealed a high prevalence of uncontrolled hypertension among patients with Fabry disease. Thus there is a need to improve BP control and renoprotection in patients with Fabry disease.

摘要

背景

法布里病是一种罕见的X连锁疾病，由α-半乳糖苷酶A缺乏引起。它会导致与肾脏、心脏和脑血管疾病相关的早期死亡，而这些也是血压（BP）升高患者的重要结局。法布里病患者中未控制高血压的患病率以及酶替代疗法对血压的影响尚不清楚。

方法

我们在参与法布里病结局调查（FOS）的391例法布里病患者中检查了未控制高血压（收缩压[SBP]≥130 mmHg或舒张压[DBP]≥80 mmHg）情况。

结果

57%的男性和47%的女性存在未控制高血压。在1期慢性肾脏病（CKD）患者（n = 100）中，SBP中位数为120 mmHg，DBP中位数为74 mmHg。在2期CKD患者（n = 172）中，SBP中位数为125 mmHg，DBP中位数为75 mmHg。在3期CKD患者（n = 63）中，SBP中位数为130 mmHg，DBP中位数为75 mmHg。在2年的酶替代治疗过程中，SBP和DBP均显著下降。