Haemostatic abnormalities, cardiac involvement and serum tumor necrosis factor levels in X-linked dystrophic patients.
Left ventricular thrombosis and systemic emboli have been demonstrated to complicate cardiomyopathy in Duchenne and Becker muscular dystrophy (DMD, BMD). We investigated plasma levels of prothrombin fragment 1+2 (F1+2). thrombin-antithrombin III complex (TAT) and circulating levels of tumor necrosis factor-alpha (TNF-alpha), a procoagulant cytokine that has been shown to be elevated in patients with depressed cardiac function, in 20 patients with DMD and 12 patients with BMD as compared with 30 age-matched control subjects. Significantly elevated levels of F1+2 (DMD: 1.4+/-0.8 nmol/l; BMD: 1.8+/-0.8 nmol/l vs. controls: 0.7+/-0.2 nmol/l, p <0.01 and p <0.001, respectively), TAT complexes (DMD: 4.7+/-2.7 microg/l, BMD: 5+/-2.3 microg/l vs. controls: 1.6+/-0.5 microg/l, p <0.001) and TNF-alpha (54+/-9 vs. 25+/-7 pg/ml, p <0.001) were observed in patients with the dystrophic disease compared to control subjects. A significantly negative correlation was also found between F1+2 and TAT complexes and left ventricular ejection fraction (r = -0.65, p <0.0001; r = -0.80, p < 0.0001, respectively) and a positive correlation between F1+2 and TAT complexes and serum TNF-alpha levels (r = 0.67, p <0.0001; r = 0.70, p <0.0001, respectively). Our results indicate a hypercoagulable state in X-linked dystrophic patients. A possible relationship between haemostatic activation, left ventricular dysfunction and TNF-alpha system upregulation may be suggested.
Porreca
E
E
Department of Internal Medicine, University of Chieti, Medical School, Italy.
Guglielmi
M D
MD
Uncini
A
A
Di Gregorio
P
P
Angelini
A
A
Di Febbo
C
C
Pierdomenico
S D
SD
Baccante
G
G
Cuccurullo
F
F
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D008040:Genetic Linkage; D020141:Hemostatic Disorders / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging; D009130:Muscle, Smooth / Q000000981:diagnostic imaging; D009136:Muscular Dystrophies / Q000097:blood* / Q000000981:diagnostic imaging / Q000235:genetics*; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis*; D018487:Ventricular Dysfunction, Left / Q000097:blood*; D014960:X Chromosome / Q000235:genetics*
D014409:Tumor Necrosis Factor-alpha