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Details

Autor(en) / Beteiligte
Titel
A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease
Ist Teil von
  • The New England journal of medicine, 2011-07, Vol.365 (1), p.44-53
Ort / Verlag
Waltham, MA: Massachusetts Medical Society
Erscheinungsjahr
2011
Link zum Volltext
Quelle
MEDLINE
Beschreibungen/Notizen
  • Patients with sickle cell disease underwent ECG with assessment of tricuspid valve regurgitant jet velocity (TRJV) to screen for pulmonary hypertension, followed by right heart catheterization if the TRJV was 2.5 m per second or more. Prevalence was 27% on the basis of ECG criteria but only 6% on the basis of catheterization. In several studies, pulmonary hypertension, particularly pulmonary arterial hypertension, has been reported as a frequent complication of sickle cell disease. 1 – 4 Pulmonary arterial hypertension is characterized by the presence of precapillary pulmonary hypertension in the absence of left-sided heart disease, lung disease, or chronic thromboembolism. On histopathological analysis, pulmonary arterial hypertension is characterized by the proliferation of medial smooth-muscle cells and endothelial cells in the small pulmonary arteries. 5 Pulmonary arterial hypertension may be idiopathic, heritable, or associated with other disorders, such as connective tissue diseases and congenital heart disease. 6 In the updated classification of pulmonary hypertension, sickle cell disease appears . . .

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