罗马尼亚硬皮病患者内皮功能障碍的临床表型
Clinical Phenotype of Endothelial Dysfunction in Romanian Scleroderma Patients.
作者信息
Groseanu Laura, Berghea Florian, Balanescu Andra, Predeteanu Denisa, Bojinca Violeta, Saulescu Ioana, Constantinescu Cosmin, Opris Daniela, Abobului Mihai, Borangiu Andreea, Negru Maria-Magdalena, Vlad Violeta, Ionescu Ruxandra
机构信息
"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
出版信息
Maedica (Bucur). 2016 Mar;11(1):26-31.
OBJECTIVE
To identify the particularities of the clinical phenotype of endothelial dysfunction in a lot of Romanian patients from a reference center and compare it to data reported by international registries.
MATERIAL AND METHODS
51 patients were included in a cross-sectional study. The patients were evaluated for the pattern of disease, main visceral involvement, serum markers of disease.
RESULTS
41.2% patients had history of digital ulcers, 27.45% had pulmonary arterial hypertension; cardiovascular involvement also included: diastolic dysfunction in 31.1% of the patients, global systolic dysfunction in 9.8%, rhythm and conduction disturbances in 19.6%, peripheral vascular disease in 19.6%. Scleroderma renal crisis was identified in 2 patients.
CONCLUSION
Vascular complications are a major cause of morbidity and mortality in systemic sclerosis. Earlier therapeutic intervention demands improved screening and diagnosis in all cases.
目的
确定来自一家参考中心的众多罗马尼亚患者内皮功能障碍的临床表型特点，并将其与国际登记处报告的数据进行比较。
材料与方法
51例患者纳入一项横断面研究。对患者进行疾病模式、主要内脏受累情况、疾病血清标志物的评估。
结果
41.2%的患者有指端溃疡病史，27.45%有肺动脉高压；心血管受累还包括：31.1%的患者有舒张功能障碍，9.8%有整体收缩功能障碍，19.6%有节律和传导紊乱，19.6%有外周血管疾病。2例患者被诊断为硬皮病肾危象。
结论
血管并发症是系统性硬化症发病和死亡的主要原因。早期治疗干预需要在所有病例中改进筛查和诊断。
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