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T1  - Abnormal coagulation and enhanced fibrinolysis due to lysinuric protein intolerance associates with bleeds and renal impairment 
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UR  - http://hdl.handle.net/10138/308858 
T3  -  
A1  - Pitkänen, H. H.; Kärki, M.; Niinikoski, H.; Tanner, L.; Näntö-Salonen, K.; Pikta, M.; Kopatz, W. F.; Zuurveld, M.; Meijers, J. C. M.; Brinkman, H. J. M.; Lassila, R. 
A2  -  
PB  -  
Y1  - 2018 
LA  - eng 
AB  - Introduction: Lysinuric protein intolerance (LPI), a rare autosomal recessive transport disorder of cationic amino acids lysine, arginine and ornithine, affects intestines, lungs, liver and kidneys. LPI patients may display potentially life-threatening bleeding events, which are poorly understood. Aims: To characterize alterations in haemostatic and fibrinolytic variables associated with LPI. Methods: We enrolled 15 adult patients (8 female) and assessed the clinical ISTH/ SSC-BAT bleeding score... 
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KW  - coagulation disorder; fibrinolysis; lysinuric protein intolerance; thrombin generation; PULMONARY; DISEASE; SLC7A7; HYPERCOAGULABILITY; IDENTIFICATION; COMPLICATIONS; INVOLVEMENT; PROTEASES; GENE; 3121 General medicine, internal medicine and other clinical medicine; 3122 Cancers 
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