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10.1111/bjh.13452

http://scihub22266oqcxt.onion/10.1111/bjh.13452
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C4452408!4452408!25907665
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suck abstract from ncbi


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pmid25907665      Br+J+Haematol 2015 ; 169 (6): 887-98
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  • Dysregulated Arginine Metabolism and Cardiopulmonary Dysfunction in Patients with Thalassaemia #MMPMID25907665
  • Morris CR; Kim HY; Klings ES; Wood J; Porter JB; Trachtenberg F; Sweeters N; Olivieri NF; Kwiatkowski JL; Virzi L; Hassell K; Taher A; Neufeld EJ; Thompson AA; Larkin S; Suh JH; Vichinsky EP; Kuypers FA
  • Br J Haematol 2015[Jun]; 169 (6): 887-98 PMID25907665show ga
  • Pulmonary hypertension (PH) commonly develops in thalassaemia syndromes, but is poorly characterized. The goal of this study was to provide a comprehensive description of the cardiopulmonary and biological profile of patients with thalassaemia at risk for PH. A case-control study of thalassaemia patients at high versus low PH-risk was performed. A single cross-sectional measurement for variables reflecting cardiopulmonary status and biological pathophysiology were obtained, including Doppler-echocardiography, 6-minute-walk-test, Borg Dyspnea Score, New York Heart Association functional class, cardiac magnetic resonance imaging (MRI), chest-computerized tomography, pulmonary function testing and laboratory analyses targeting mechanism of coagulation, inflammation, haemolysis, adhesion and the arginine-nitric oxide pathway. Twenty-seven thalassaemia patients were evaluated, 14 with an elevated tricuspid-regurgitant-jet-velocity (TRV) ?2.5m/s. Patients with increased TRV had a higher frequency of splenectomy, and significantly larger right atrial size, left atrial volume and left septal-wall thickness on echocardiography and/or MRI, with elevated biomarkers of abnormal coagulation, lactate dehydrogenase levels and arginase concentration, and lower arginine-bioavailability compared to low-risk patients. Arginase concentration correlated significantly to several echocardiography/MRI parameters of cardiovascular function in addition to global-arginine-bioavailability and biomarkers of haemolytic rate, including lactate dehydrogenase, haemoglobin and bilirubin. Thalassaemia patients with a TRV ?2.5m/s have additional echocardiography and cardiac-MRI parameters suggestive of right and left-sided cardiac dysfunction. In addition, low arginine bioavailability may contribute to cardiopulmonary dysfunction in ?-thalassaemia.
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