Elstein Deborah, Nir Amiram, Klutstein Mark, Abrahamov Aya, Zimran Ari
Gaucher Clinic, Shaare Zedek Medical Center, PO Box 3235, 91031 Jerusalem, Israel.
Pulm Pharmacol Ther. 2004;17(5):319-23. doi: 10.1016/j.pupt.2004.07.004.
NT-pro-brain natriuretic peptide (NT-proBNP) values are correlated with right ventricle dysfunction in pulmonary hypertension and have been recommended as a prognostic marker for symptomatic primary pulmonary hypertension. The purpose of this pilot study was to determine whether NT-proBNP values correlate with mild to moderate pulmonary hypertension in Gaucher disease. The NT-proBNP assay was performed on sera from patients with type I (non-neuronopathic) Gaucher disease who were known to have tricuspid insufficiency (TI) gradient values on echocardiography that were normal (TI<20 mmHg), borderline (TI=25-29 mmHg), or abnormal (TI>or= 30 mmHg) over time. There was a statistically significant correlation (p=0.05) between mean NT-proBNP values and TI gradient group: mean NT-proBNP for normal TI gradient (n=20 patients)=133.3 (range: 46-445)pg/ml; mean NT-proBNP for borderline TI gradient (n=17 patients)=288.7 (range: 81-1088)pg/ml; and mean NT-proBNP for elevated TI gradient (n=10 patients)=1034.2 (73-6703)pg/ml. Treatment status was not correlated with TI gradient or NT-proBNP values. Thus, in this pilot study of Gaucher disease that included patients with mild to moderate pulmonary hypertension, NT-proBNP was correlated with echocardiographic findings. Further studies will be required to ascertain if NT-proBNP can be used for diagnosis and monitoring of these patients.
N末端脑钠肽前体(NT-proBNP)值与肺动脉高压中的右心室功能障碍相关,并且已被推荐作为有症状的原发性肺动脉高压的预后标志物。这项初步研究的目的是确定NT-proBNP值是否与戈谢病中的轻度至中度肺动脉高压相关。对I型(非神经元病变型)戈谢病患者的血清进行NT-proBNP检测,这些患者在超声心动图检查中已知三尖瓣反流(TI)梯度值随时间推移正常(TI<20 mmHg)、临界(TI=25-29 mmHg)或异常(TI≥30 mmHg)。平均NT-proBNP值与TI梯度组之间存在统计学显著相关性(p=0.05):正常TI梯度(n=20例患者)的平均NT-proBNP=133.3(范围:46-445)pg/ml;临界TI梯度(n=17例患者)的平均NT-proBNP=288.7(范围:81-1088)pg/ml;TI梯度升高(n=10例患者)的平均NT-proBNP=1034.2(73-6703)pg/ml。治疗状态与TI梯度或NT-proBNP值无关。因此,在这项包括轻度至中度肺动脉高压患者的戈谢病初步研究中,NT-proBNP与超声心动图结果相关。需要进一步研究以确定NT-proBNP是否可用于这些患者的诊断和监测。