Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease
作者: Matthieu TurpinChristelle Chantalat-AugerFlorence ParentFrançoise DrissFrançois LionnetAnoosha HabibiBernard MaîtreAlice HuertasXavier JaïsJason WeatheraldDavid MontaniOlivier SitbonGérald SimonneauFréderic GalactérosMarc HumbertPablo BartolucciLaurent Savale
作者单位: 1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
4AP-HP, Service de Médecine Interne, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
5AP-HP, Service de Médecine Interne, Hôpital Tenon, Paris, France
6AP-HP, Unité des Maladies Génétiques du Globule Rouge, Centre de Référence des Pathologies du Globule Rouge, Hôpitaux Universitaire Henri Mondor, Créteil, France
7UPEC, Institut Mondor de Recherche Biomédicale (IMRB), INSERM U955, DHU A-TVB, Créteil, France
8AP-HP, Unité de Pneumologie, Hôpitaux Universitaires Henri Mondor, Créteil, France
9Division of Respirology, Dept of Medicine, University of Calgary, Calgary, AB, Canada
10Libin Cardiovascular Institute of Alberta, Calgary, AB, Canada
11These two authors contributed equally to this work
刊名: European Respiratory Journal, 2018, Vol.52 (4)
来源数据库: European Respiratory Society
DOI: 10.1183/13993003.00272-2018
原始语种摘要: The long-term effects of chronic blood exchange transfusions (BETs) on pre-capillary pulmonary hypertension complicating sickle cell disease (SCD) are unknown.(#br)13 homozygous SS SCD patients suffering from pre-capillary pulmonary hypertension and treated by chronic BETs were evaluated retrospectively. Assessments included haemodynamics, New York Heart Association Functional Class (NYHA FC), 6-min walk distance (6MWD) and blood tests.(#br)Before initiating BETs, all patients were NYHA FC III or IV, median (range) 6MWD was 223 (0–501) m and median (range) pulmonary vascular resistance (PVR) was 3.7 (2–12.5) Wood Units. After a median number of 4 BET sessions, all patients had improved to NYHA FC II or III. Significant improvements in haemodynamics were observed, including a decrease in...
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关键词翻译
关键词翻译
  • hypertension 高血压
  • capillary 毛状的
  • pulmonary 肺的
  • sickle 切割器镰刀
  • blood 血液
  • exchange 交换
  • initiating 启动
  • acute 急尖的
  • range 射程
  • BET 贝塞尔