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Vol. 53. Núm. 1.
Páginas 12-16 (Julio 2000)
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Vol. 53. Núm. 1.
Páginas 12-16 (Julio 2000)
Acceso a texto completo
¿Trombofilia y enfermedad de Perthes?
Thrombophilia and perthes' disease¿
Visitas
9089
J.M. Tusell Puigberta,*, C. Aulesa Martíneza, M. Aguirre Canyadella, I. Nicolau Fustéb, P. Valentín Valentínb, J.J. Ortega Aramburua
a Hospital Materno-Infantil
b Hospital General Vall d'Hebron. Barcelona.
Este artículo ha recibido
Información del artículo
Fundamento

Se ha postulado que la causa de la enfermedad de Perthespueda ser una trombosis intravascular condicionadapor un potencial trastorno congénito de la hemostasia queconduciría a situaciones de trombofilia o hipofibrinólisis.

Pacientes y método

Un estudio completo de la hemostasia, que incluya factoresde coagulación, estudio de la trombofilia y de la fibrinólisisen un grupo de niños con enfermedad de Perthes,podría determinar la prevalencia de trastornos de lahemostasia y fibrinólisis en este colectivo. Un grupo de25 pacientes con edades comprendidas entre 5 y 25 añosen el momento del estudio, anteriormente diagnosticadosde enfermedad de Perthes, fueron sometidos a análisis dela hemostasia y trombofilia, comparando los resultadoscon los de un grupo control.

Resultados

Se detectaron alteraciones en la trombofilia en un solopaciente con un déficit moderado-leve de proteína S,siendo los demás casos considerados dentro de la normalidaden relación a la edad de los pacientes.

Conclusiones

Los datos clínicos, epidemiológicos y de laboratorio deeste grupo de pacientes pediátricos, comparados con losde un grupo control estudiado, no abonan la hipótesis deun defecto en la trombogénesis, como causante de la necrosisavascular de la cabeza del fémur en nuestro medio.

Palabras clave:
Enfermedad de Perthes
Niños
Trombofilia
Background

It has been suggested that the cause of Perthes diseasemay be intravascular thrombosis induced by a potentialcongenital hemostatic disorder leading to conditions ofthrombophilia or hyperfibrinolysis.

Patients and method

Complete study of hemostasis with coagulation and antithrombinfactors as well as study of thrombophilia andfibrinolysis in these patients could determine the prevalenceof hemostasis and fibrinolysis in this group of patients.

Results

Alterations in hemostasis and thrombosis were detectedin one patient who had moderate-to-light protein Sdeficiency. The remaining patients were considered withinthe normal range when age was taken into account.

Conclusions

Epidemiological and laboratory data from this group ofpediatric patients and from the control group do not supportthe hypothesis that a thrombogenesis defect couldbe the underlying cause of avascular necrosis of the hipjoint.

Key words:
Perthes' disease
Children
Thrombophilia
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Bibliografía
[1.]
M.J. Manco-Johnson.
Disorders of hemostasis in childhood: risk factors for venous thromboembolism..
Thromb Haemost, 78 (1997), pp. 710-714
[2.]
C.J. Glueck, A. Crawford, D. Roy, R. Freiberg, H. Glueck, D. Stroop.
Association of antithrombotic factor deficiencies and hypofibrinólisis with Legg-Perthes disease.
J Bone Joint Surg, 78 (Supl A) (1996), pp. 3-13
[3.]
C.J. Glueck, G. Brandt, R. Gruppo, A. Crawford, D. Roy, T. Tracy, et al.
Resistance to activated protein C and Legg-Perthes disease.
Clin Orthop, 338 (1997), pp. 139-152
[4.]
R.M. Bertina, B.P. Koeleman, T. Korster, F.R. Rosendaal, R.S. Divan, H. De Ronde, et al.
Mutation in blood coagulation factor V associated with resistance to activated protein C.
Nature, 369 (1994), pp. 64-67
[5.]
D.J. Hall.
Genetics aspects of Perthes disease: a critical review.
Clin Orthop, 209 (1986), pp. 100-114
[6.]
J.S. Greengard, S. Eichinger, J.H. Griffin, K.A. Bauer.
Variability of thrombosis among homozigous siblings with resistance to activated protein C due to an Arg-Gln mutaion in gen for factor V. N Engl.
J Med, 331 (1994), pp. 1559-1562
[7.]
J.H. Griffin, B. Evatt, C. Wideman, J.A. Fernández.
Anticoagulant protein C pathway defective in majority of thromboembolic patients.
Blood, 82 (1993), pp. 1989-1993
[8.]
K.A. Hajjar.
Factor V Leiden•An unselfish geneα A critical review.
N Engl J Med, 331 (1994), pp. 1585-1587
[9.]
P.C. Koeleman, P.H. Reitsma, C. Allaart, R.M. Bertina.
Activated protein C resistance as an additional risk factor for thrombosis in protein C deficient families.
Blood, 84 (1994), pp. 1031-1035
[10.]
P.M. Ridker, C.H. Hennekens, K. Lindpaintner, et al.
Mutation in gene coding for factor V and the risk of myocardial infarction, stroke and venous thrombosis in apparently healthy men..
N Engl J Med, 332 (1995), pp. 912-917
[11.]
F.R. Rosendaal, T. Koster, J.P. Vandenbroucke, P.H. Reitsma.
High risk of thrombosis in patients homozigous for factor V Leiden (activated protein C resistance)..
Blood, 85 (1995), pp. 1504-1508
[12.]
L. Shen, B. Dahlback.
Factor V and protein S as synergistic cofactors to activated protein C in degradation of factor VIIIa..
J Biol Chem, 269 (1994), pp. 18735-18738
[13.]
J. Voorberg, J. Roelse, R. Koopman, H. Buller, F. Berends, J.W. Ten Cate.
Association of idiopathic venous thromboembolism with single point-mutation at Arg 506 of factor.
V. Lancet, 343 (1994), pp. 1535-1536
[14.]
B. Zoller, P.J. Svensson, X. He, B. Dahback.
Identification of the same factor V gene mutation in 47 of 50 thrombosis-prone families with inherited resistance to activated protein C.
J Clin Invest, 94 (1994), pp. 2521-2524
[15.]
W.G. Boettcher, H. Bonfiglio, H.H. Hamilton, R.F. Sheets, K. Smith.
Non traumatic osteonecrosis of the femoral head: 1. Relation of altered hemostasis to etiology.
J Bone Joint Surg, 52 (Supl A) (1970), pp. 312-321
[16.]
C.J. Glueck, R. Freiberg, H.I. Glueck, C. Henderson, M. Welch, T. Tracy.
Hypofibrinolysis: a common, major cause of osteonecrosis.
Am J Hematol, 45 (1994), pp. 156-166
[17.]
C.J. Glueck, R. Freiberg, H.I. Glueck, T. Tracy, D. Stroop, P. Wang.
Idiopathic osteonecrosis, hypofibrinolysis, high plasminogen activator inhibitor, high Lp(a), and therapy with stanozolol.
Am J Hematol, 48 (1995), pp. 213-220
[18.]
C.J. Glueck, R. Freiberg, T. Tracy, D. Stroop, P. Wang.
Thrombophilia and hypofibrinolysis: pathophysiology of osteonecrosis..
Clin Othop, 334 (1997), pp. 43-56
[19.]
C.J. Glueck, R.E. McMahon, J. Bouquot, D. Tripett, R. Gruppo, P. Wang.
Pathophysiology of osteonecrosis of the jaw: thrombophilia and hypofibrinolysis..
Oral Surg Oral Med Oral Pathol, 81 (1996), pp. 557-566
[20.]
R. Gruppo, C.J. Glueck, R.E. McMahon, J. Bouquot, B.A. Rabinovich, A. Beckar, et al.
J Lab Clin Med, 127 (1996), pp. 481-488
[21.]
I.M. Nilsson, H. Krook, H.H. Sternby, U. Hedner.
Severe thrombotic disease in a young man with bone marrow and skeletal changes and with a high content of an inhibitor in the fibrinolytic system.
Acta Med Scand, 169 (1961), pp. 325-327
[22.]
R.I. Van Veldhuizen, J. Neff, M.D. Murphey, D. Bodesteiner, B.S. Skine.
Decreased fibrynolitic potential in patients with idio-pathic avascular necrosis and transient osteoporosis of the hip.
Am J Hematol, 44 (1993), pp. 243-248
[23.]
D. Choi, D.Y. Lee, C.Y. Chung, K.H. Rhyu, S.Y. Parrk.
Changes in coagulation-fibrinolysis system in Legg-Perthes disease: a preliminary report [resumen].
Proc Pediatr Orthop Soc North Am, 2 (1995), pp. 1
[24.]
C.J. Glueck, A. Crawford, D. Roy, R. Freiberg, H. Glueck, D. Stroop.
Association of antithrombotic factor deficiencies and hy-pofibrinolysis with Legg-Perthes disease.
J Bone Joint Surg, 78 (Supl A) (1996), pp. 3-13
[25.]
C.J. Glueck, H.I. Glueck, D. Greenfield, R. Freiberg, A. Kahn, T. Hamer.
Protein C and S deficiency, thrombofilia and hypofibrinolysis: pathophysiologic causes of Legg-Perthes disease.
Pediatr Res, 35 (1994), pp. 383-388
[26.]
C.J. Glueck, H.I. Glueck, L. Mieczkowski, T. Tracy, J. Speirs, D. Stroop.
Familial high plasminogen activator inhibitor with hypofibrinolysis, a new pathophysiologic cause of osteonecrosisα.
Thromb Haemost, 69 (1993), pp. 460-465
[27.]
C.J. Glueck, H.I. Glueck, M. Welch, R. Freiberg, T. Tracy, T. Hamer.
Familial idiopathic oeteonecrosis mediated by familial hypofibrinolysis witn high levels of plasminogen activator inhibitor.
Thromb Haemost, 71 (1994), pp. 195-198
[28.]
J. Petaja, V. Rasi, E. Myllya, Vahtera, H. Hallman.
Familial hypofibrinolysis and venous thrombosis.
Br J Hematol, 71 (1989), pp. 393-398
[29.]
S. Gallistl, T. Reitinguer, W. Linhart, W. Muntean.
The role of inherited thrombotic disorders in the etiology of Legg-CalvéPerthes disease.
J Pediatr Orthop, 19 (1999), pp. 82-83
[30.]
J.T. Guille, G.E. Lipton, G. Szöke, J.R. Bowen, H.T. Harcke, J.J. Glutting.
Legg-CalvéPerthes disease in girls.
J Bone Joint Surg, 80 (Supl A) (1998), pp. 1256-1263
[31.]
V. De Stefano, G. Finazzi, P.M. Mannucci.
Inherited thrombophilia: pathogenesis, clinical syndromes and management..
Blood, 87 (1996), pp. 3531-3544
[32.]
V.R. Arruda, W.D. Belangero, M.C. Ozeto, G.B. Oliveira, R.G. Pagnano, J.B. Volpon, et al.
Inherited risk factors for thrombophilia among children with Legg-CalvéPerthes disease.
J Pediatr Orthop, 19 (1999), pp. 84-87
[33.]
S. Hakey, G. Kenet, A. Lubetsky, N. Rosenberg, S. Gitel, S. Wien-troub.
Does thrombophilia play a role in Legg-CalvéPerthes diseaseα.
J Bone Surg (Br), 81 (Supl B) (1999), pp. 686-690
[34.]
D.P. Thomas, G. Morgan, K. Tayton.
Perthes' disease and the relevance of thrombophilia.
J Bone Surg (Br), 81 (Supl B) (1999), pp. 691-695
[35.]
M. Andrew, P. Verh, M. Johnston.
Maturation of the hemostatic system during childhood.
Blood, 80 (1992), pp. 1998-2005
[36.]
J.S. Dixon, K.A. Musgrove.
Special laboratory evaluation of coagulation..
Clinical hematology, pp. 611-615
[37.]
U. Abildgaard, M. Lie, O.R. Odegard.
Antithrombin heparin cofactor assay with new chromogenic substrates..
Thromb Res, 11 (1977), pp. 549
[38.]
E. Angles Cano.
Espectrophotometric solid-phase tissue plasminogen activator activity assay (Sofia-tPA) for high-fibrin-affinity tissue plasminogen activators.
Anal Biochem, 153 (1989), pp. 201-210
[39.]
J. Chmielewka, B. Wiman.
Determination of plasminogen activator and its fast inhibitor in plasma.
Clin Chem, 32 (1983), pp. 482-485
[40.]
M. Ranby, N. Bergsdorf, T. Nilsson, G. Melbring, B. Winblad, G. Bucht, et al.
Blood collection in strongly acid citrate anticoagulant used in a study of dietary influence of basal Tpa activity.
Thromb Haemost, 62 (1989), pp. 917-922
[41.]
N.E. Harris.
The second international anticardiolipin standardization workshop. The Kinston antiphospholipid group.
Am J Clin Pathol, 94 (1990), pp. 476-484
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