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Coagulation system activated in Duchenne muscular dystrophy patients with cardiac dysfunction

      Abstract

      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.

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      References

        • Matsuishi T.
        • Yano E.
        • Terasawa K.
        • Nonaka I.
        • Ishihara O.
        • Yamaguchi Y.
        • et al.
        Basilar artery occlusion in a case of Duchenne muscular dystrophy.
        Brain Dev. 1982; 4: 379-384
        • Biller J.
        • Ionasescu V.
        • Zellweger H.
        • Adams H.P.
        • Schultz D.T.
        Frequency of cerebral infarction in patients with inherited neuromuscular diseases.
        Stroke. 1987; 18: 805-807
        • Gaffney J.F.
        • Kingston W.J.
        • Metlay L.A.
        • Gramiak R.
        Left ventricular thrombus and systemic emboli complicating the cardiomyopathy of Duchenne's muscular dystrophy.
        Arch Neurol. 1989; 46: 1249-1252
        • Riggs T.
        Cardiomyopathy and pulmonary emboli in terminal Duchenne's muscular dystrophy.
        Am Heart J. 1990; 119: 690-693
        • Hanajima R.
        • Kawai M.
        Incidence of cerebral infarction in Duchenne muscular dystrophy.
        Muscle Nerve. 1996; 19: 928
        • Arahata K.
        • Ishiura S.
        • Ishiguro T.
        • Tsukahara T.
        • Suhara Y.
        • Eguchi C.
        • et al.
        Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne muscular dystrophy peptide.
        Nature. 1988; 333: 861-863
        • Dobowitz V.
        Muscle disorders in childhood.
        2nd ed. Saunders, London1995
        • Pelzer H.
        • Schwarz A.
        • Heimburger N.
        Determnation of human thrombin–antithrombin III complex in plasma with an enzyme-linked immunosorbent assay.
        Thromb Haemost. 1988; 59: 101-106
        • Pelzer H.
        • Schwarz A.
        • Stuber W.
        Determination of human prothrombin activation fragment 1+2 in plasma with an antibody against a synthetic peptide.
        Thromb Haemost. 1991; 65: 153-159
        • Nieuwenhuizen W.
        A reference material for harmonisation of D-dimer assays. Fibrinogen Subcommittee of the Scientific and Standardization Committee of the International Society of Thrombosis and Haemostasis.
        Thromb Haemostas. 1997; 77: 1031-1033
        • Ishikawa Y.
        • Bach J.R.
        • Ishikawa Y.
        • Minami R.
        A management trial for Duchenne cardiomyopathy.
        Am J Phys Med Rehabil. 1995; 74: 345-350
        • Saito T.
        • Takenaka M.
        • Miyai I.
        • Yamamoto Y.
        • Matsumura T.
        • Nozaki S.
        • et al.
        Coagulation and fibrinolysis disorder in muscular dystrophy.
        Muscle Nerve. 2001; 24: 399-402
        • Barber M.
        • Langhorne P.
        • Rumley A.
        • Lowe G.D.O.
        • Stott D.J.
        Hemostatic function and progressing ischemic stroke D-dimer predicts early clinical progression.
        Stroke. 2004; 35: 1421-1425
        • Yamamoto K.
        • Ikeda U.
        • Furuhashi K.
        • Irokawa M.
        • Nakayama T.
        • Shimada K.
        The coagulation system is activated in idiopathic cardiomyopathy.
        J Am Coll Cardiol. 1995; 25: 1634-1640
        • Falk R.H.
        • Foster E.
        • Coats M.H.
        Ventricular thrombi and thromboembolism in dilated cardiomyopathy: a prospective follow-up study.
        Am Heart J. 1992; 123: 136-142
        • Dusleag J.
        • Klein W.
        • Eber B.
        • Gasser R.
        • Brussee H.
        • Rotman B.
        • et al.
        Frequency of magnetic resonance signal abnormalities of the brain in patients aged <50 years with idiopathic dilated cardiomyopathy.
        Am J Cardiol. 1992; 69: 1446-1450
        • Sirajuddin R.A.
        • Miller A.B.
        • Geraci S.A.
        Anticoagulation in patients with dilated cardiomyopathy and sinus rhythm: a critical literature review.
        J Card Fail. 2002; 8: 48-53
        • Saito T.
        • Matsumura T.
        • Nozaki S.
        • Shinno S.
        A case of Duchenne muscular dystrophy showing coagulation cascade activation induced by muscle destruction due to convulsion.
        Clinica Neurol. 2003; 43: 274-276
        • Koniaris L.S.
        • Goldhaber S.Z.
        Anticoagulation in dilated cardiomyopathy.
        J Am Coll Cardiol. 1998; 31: 745-748