Department of Pharmacy, The Second Affiliated Hospital of Nanchang University, Nanchang, 330006, China.
Teaching and Research Office of Clinical Pharmacology, College of Pharmacy, Xinxiang Medical University, Xinxiang, 453003, China.
Respir Res. 2020 Feb 6;21(1):44. doi: 10.1186/s12931-020-1309-2.
To investigate the differences in the proportions of BMPR2 mutations in familial hereditary pulmonary arterial hypertension (HPAH) and idiopathic pulmonary arterial hypertension (IPAH) between males and females and the relationship between BMPR2 mutation and PAH severity.
A computer was used to search the electronic Cochrane Library, PubMed/MEDLINE, and EMBASE databases for clinical trials containing information on the relationship between PAH prognosis and BMPR2 mutations through March 2019. After obtaining the data, a meta-analysis was performed using Review Manager Version 5.3 and Stata.
A meta-analysis was performed on 17 clinical trials (2198 total patients: 644 male, 1554 female). The results showed that among patients with HPAH and IPAH, the BMPR2 mutation rate is higher in male than in female patients [male group (224/644, 34.78%), female group (457/1554, 29.41%), OR = 1.30, 95% CI: 1.061.60, P = 0.01, I = 10%]. Furthermore, haemodynamic and functional parameters were more severe in IPAH and HPAH patients with BMPR2 mutations than in those without, and those with BMPR2 mutation were diagnosed at a younger age. The risk of death or transplantation was higher in PAH patients with BMPR2 mutations than in those without (OR = 2.51, 95% CI: 1.293.57, P = 0.003, I = 24%). Furthermore, the difference was significant only in male patients (OR = 5.58, 95% CI: 2.1614.39, P = 0.0004, I = 0%) and not in female patients (OR = 1.41, 95% CI: 0.752.67, P = 0.29, I = 0%).
Among patients with HPAH and IPAH, men are more likely to have BMPR2 mutations, which may predict more severe PAH indications and prognosis.
探讨男性和女性家族性遗传性肺动脉高压（HPAH）和特发性肺动脉高压（IPAH）中 BMPR2 突变比例的差异，以及 BMPR2 突变与 PAH 严重程度的关系。
计算机检索电子 Cochrane 图书馆、PubMed/MEDLINE 和 EMBASE 数据库，检索时间截至 2019 年 3 月，收集关于 PAH 预后与 BMPR2 突变关系的临床试验。获取数据后采用 Review Manager Version 5.3 和 Stata 进行 Meta 分析。
纳入 17 项临床试验（共 2198 例患者：644 例男性，1554 例女性）进行 Meta 分析。结果显示，在 HPAH 和 IPAH 患者中，男性患者的 BMPR2 突变率高于女性[男性组（224/644，34.78%），女性组（457/1554，29.41%），OR=1.30，95%CI：1.061.60，P=0.01，I²=10%]。此外，BMPR2 突变的 IPAH 和 HPAH 患者的血流动力学和功能参数更严重，且发病年龄更小。BMPR2 突变的 PAH 患者的死亡或移植风险高于无突变者（OR=2.51，95%CI：1.293.57，P=0.003，I²=24%）。此外，这种差异仅在男性患者中显著（OR=5.58，95%CI：2.1614.39，P=0.0004，I²=0%），而在女性患者中不显著（OR=1.41，95%CI：0.752.67，P=0.29，I²=0%）。
在 HPAH 和 IPAH 患者中，男性更易发生 BMPR2 突变，这可能预示着更严重的 PAH 指标和预后。
