Coagulation system activated in Duchenne muscular dystrophy patients with cardiac dysfunction.
We investigated basic abnormalities of coagulation and fibrinolysis in Duchenne muscular dystrophy (DMD) patients with cardiac dysfunction. Forty seven patients with DMD, aged 13-37 years old, were enrolled. Based on left ventricular ejection fraction (LVEF) results determined by echocardiography, patients were divided into 3 groups: LVEF less than 30% (markedly depressed group), LVEF between 30 and 50% (slightly depressed), and LVEF greater than 50% (normal). We measured serum levels of total fibrin and fibrinogen degradation products (FDP), as well as plasma fibrinogen, thrombin-antithrombin complex (TAT), prothrombin fragment (F1+2), and D-dimer. The levels of TAT and F1+2 in the markedly depressed group were significantly elevated compared with the other groups, whereas FDP, fibrinogen, and D-dimer levels did not differ among the groups. We concluded that activated coagulation is associated with cardiac dysfunction in patients with DMD.
Saito
Toshio
T
Division of Neurology, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan. saitot@toneyama.hosp.go.jp
Yamamoto
Yuko
Y
Matsumura
Tsuyoshi
T
Nozaki
Sonoko
S
Fujimura
Harutoshi
H
Shinno
Susumu
S
D000293:Adolescent; D000328:Adult; D000990:Antithrombin III; D001778:Blood Coagulation Disorders / Q000097:blood / Q000150:complications*; D002311:Cardiomyopathy, Dilated / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging; D003328:Coronary Thrombosis / Q000097:blood / Q000150:complications*; D004452:Echocardiography; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism; D005340:Fibrinogen / Q000378:metabolism; D005342:Fibrinolysis; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000097:blood / Q000150:complications*; D010446:Peptide Fragments / Q000097:blood; D010447:Peptide Hydrolases / Q000097:blood; D011498:Protein Precursors / Q000097:blood; D011516:Prothrombin
D005338:Fibrin Fibrinogen Degradation Products; D010446:Peptide Fragments; D011498:Protein Precursors; C046193:antithrombin III-protease complex; C036309:fibrin fragment D; C029733:prothrombin fragment 1; C044183:prothrombin fragment 2; D000990:Antithrombin III; D011516:Prothrombin; D005340:Fibrinogen; D010447:Peptide Hydrolases