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Órgano Oficial del Instituto Nacional de Pediatría

2005, Number 6

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Acta Pediatr Mex 2005; 26 (6)

Budd-Chiari syndrome (BCS) associated with antiphospholipid syndrome (APS)

Carbajal RL, Vargas QE, Ramírez MJA, Copto GA, Reynés MJN, Rodríguez HR, Barrios FR, Zarco RJ

Language: Spanish
References: 47
Page: 302-307
PDF size: 47.53 Kb.


ABSTRACT

Background: Budd-Chiari syndrome (BCS) is an infrequent condition. It presents with intrahepatic venous thrombosis, and it may be caused by the antiphospolipid symdrome (APHS), which is characterized by the presence of anticardiolipin antibodies and lupic anticoagulant factor. There are two types of BCS: primary and secondary caused by infections, neoplasm, drugs and autoimmunity.
Materials and methods: Retrospective, transversal and observational study of 24 patients with APHS, seven of which had BCS. Age, gender, types, complications, thrombosis and laboratory findings were analyzed.
Results: Seven patients were between 10 and 15 years old, there were four females and three males. Hepatomegaly was present in all; one patient had splenomegaly, ascitis and bleeding problems. Every patient had portal vein thrombosis. TGO, TGP, GGT tests were done in all; CH50 was done in two patients. ACLA were measured in the seven patients; LAC in one; beta 2 glucoprotein in one.
Conclusions: Symptoms were not different in both types of BCS. Secondary BCS ones were caused by LEG, AACL, ACL . The B2GP1 it was most common in the primary BCS cases.


REFERENCES

  1. Dilawari JB, Bambery P, Chawla Y, et al. Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine 1994;73(1):21-36.

  2. Janssen HL, Garcia-Pagan JC, Elias E. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38:364-71.

  3. Mitchell MC, Boitnott JK, Kaufman S, et al. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine 1982;61(4):199-218.

  4. Langlet P, Escolano S, Valla D. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501.

  5. Pati HP, Dayal S, Srivastava A. Spectrum of hemostatic derangements, in Budd-Chiari syndrome. Indian J Gastroenterol 2003;22:59-60.

  6. Simsek S, Verheesen RV, Haagsma EB, Lourens J. Subacute Budd-Chiari syndrome associated with polycythemia vera and factor V Leiden mutation. Neth J Med 2000;57:62-7.

  7. Avenhaus W, Ullerich H, Menzel J, et al. Budd-Chiari syndrome in a patient with factor V Leiden-successful treatment by TIPSS placement followed by liver transplantation. Gastroenterol 1999;37:277-81.

  8. Denniger MH, Chait Y, Casadevall N. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000;31:587-91.

  9. Minnema MC, Janssen HL, Niermeijer P, de Man RA. Budd-Chiari syndrome: combination of genetic defects and the use of oral contraceptives leading to hypercoagulability. J Hepatol 2000;33:509-12

  10. Singh V, Sinha SK, Nain CK, et al. Budd-Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol 2000;15:550-4.

  11. Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology 2003;38:793-803.

  12. Wadhawan M, Kumar N. Budd-Chiari syndrome. Trop Gastroenterol 2003;24:3-7.

  13. Kamashta MA, Asherson RA. Hughes GRV. Syndrome antiphospholipid antibodies move closer to thrombosis in 1994. Br J Rheumatol. 1995;34:493.

  14. Ravelli A, Martini A. Antiphospholipid antibody syndrome in pediatric patients. Rheum Dis Clin North Am 1997;23:657-76.

  15. Martine A, Ravelli A. The clinical significance of antiphospholipid antibodies. Ann Med 1977;29:159-63.

  16. Avcin T, Cimaz R, Moroni PL. Recent advances in antiphospholipid antibodies and antiphospholipid syndromes in pediatric populations. Lupus 2002;11:4-10.

  17. Wilson WA. Criterios de clasificación del síndrome antifosfolípido. Rheum Dis Clin North Am 2001;3:1-8.

  18. Luigi MP, Raschi PE, Testoni PC, Tincani A, Balestieri G. Anticuerpos antifosfolípidos y endotelio. Rheum Dis Clin North Am 2001;3:95-110.

  19. Carbajal RL, Vargas QE, Reynés MJN, Rodríguez HR, Barrios FR, Zarco RJ, Copto GA. Síndrome antifosfolípidos en niños. Bol Med Hosp Infant Mex 2004;61:205-217.

  20. Bernstein ML, Salusinsky-Sternbach M, Bellefleur M, Esseltine DW. Thrombotic and haemorrhagic complications in children with the lupus anticoagulant. Am J Dis Child 1984;138:1132-5.

  21. Powell-Jackson PR, Melia W, Canalese J. Budd-Chiari syndrome: clinical patterns and therapy. QJ Med 1982;51:79-88.

  22. Hughes GRV, Mackworth-Young CG, Harris Gharavi AE. Veno-occlusive disease in systemic lupus erythematosus: possible association with anticardiolipin antibodies? Arthritis Rheum 1984;28:1071.

  23. Espinosa G, Font J, García J, Tassies D, Reverter J, Gaig C, Cervantes F, Cervera R, Bosch J, Ingelmo M. Budd-Chiari syndrome secondary to antiphospholipid syndrome. Medicine 2001;80:345-54.

  24. Archimandritis A, Christopoulou V, Tsirantonaki M, Vlachoyiannopoulos P, Zavos G, Aroni K. Budd-Chiari syndrome in the context of antiphospholipid syndrome: Lupus and therapeutic implications. Diagnosis 1995;4:329-31.

  25. Asherson RA, Thompson RPH, MacLachlan N, Baguley E, Hicks P, Hughes GRV. Budd-Chiari syndrome, visceral arterial occlusions, recurrent fetal loss and the “lupus anticoagulant” in systemic lupus erythematosus. J Rheumatol 1989;16:219-224.

  26. Bernstein ML, Salusinsky-Sternbach M, Bellefleur M, Esseltine DW. Thrombotic and hemorrhagic complications in children with lupus anticoagulant. Am J Dis Child 1984;138:1132-1135.

  27. Docobu J, Schreiber S, Schockert J, Lambert P, Lienart F, Dupont P, van der Henle B. Budd-Chiari syndrome in a patient with systemic lupus erythematosus. Acta Clinica Belg 1987;42:450-456.

  28. Farrant JM, Judge M, Thompson RPH. Thrombotic cutaneous nodules and hepatic vein thrombosis in the anticardiolipin syndrome. Clin Exp Dermatol 1989;14:306-308.

  29. Garrido JA, Diaz Peromingo JA, Martinez-Deben FS, Grandes J. Budd-Chiari syndrome as a presentation form of primary antiphospholipid syndrome. Ann Med Intern 1996;13:355-356.

  30. Ibrahim MB, Kinsara AJ. Primary antiphospholipid syndrome presenting as Budd-Chiari syndrome. Ann Saud Med 1998;18:154-158.

  31. Kitchens CS. Thrombotic storm: When thrombosis begets thrombosis. Am J Med 1998;104:381-385.

  32. Mackworth-Young CG, Melia WM, Harris EN, Gharavi AE, Sivathondan Y, Derue G, Sherlock S, Hughes GRV. The Budd-Chiari syndrome. Possible pathogenetic role of anti-phospholipid antibodies. J Hepatol 1986;3:83-86.

  33. Nakamura H, Uehara H, Okada T, Kambe H, Kimura Y, Ito H, Hayashi E, Yamamoto H, Kishimoto S. Occlusion of the small hepatic veins associated with systemic lupus erythematosus with the lupus anticoagulant and anti-cardiolipin antibody. Hepatogastroenterology 1989;36:393-397.

  34. Ouwendijk RJT, Koster JC, Wilson JHP, Stibbe J, Lameris JS, Visser W, Benhamou JP. Budd-Chiari syndrome in a young patient with anticardiolipin antibodies: Need for prolonged anticoagulant treatment. Gut 1994;35:1004-1006.

  35. Praderio L, Dagna L, Longhi P, Rubin G, Sabbadini MG. Budd-Chiari syndrome in a patient with ulcerative colitis. Association with anticardiolipin antibodies. J Clin Gastroenterol 2000;30:203-204.

  36. Roudot-Thoraval F, Gouault-Heilmann M, Zafrani ES. Barge J, Dhumeaux D. Budd-Chiari syndrome and the lupus anticoagulant. Gastroenterology 1985;88:605.

  37. Saca LF, Szer IS, Henar E, Nanjundiah P, Haddad ZH, Quismorio FP. Budd-Chiari syndrome associated with APL in a child. J Rheumatol 1994;21:545-548.

  38. Segal S, Shenhav S, Segal O, Zohav E, Gemer O. Budd-Chiari syndrome complicating severe preeclampsia in a parturient with primary antiphospholipid syndrome. Eur J Obstet Gynecol 1996;68:227-229.

  39. Shimizu S, Miyata M, Kamiike W, Ito T, Nakamuro M, Nomura M, Negoro S, Nakamura H, Matsuda H. Budd-Chiari syndrome combined with antiphospholipid syndrome: Case report and review of the literature. Vasc Surg 1993;27:501-509,

  40. Ueno T, Takeuchi R, Tsuda M, Nagano T, Tomeoku M, Murashima S, Kuto M, Deguchi K, Shirakawa S. A case of Budd-Chiari syndrome with lupus anticoagulant. Clin Hematol 1985;26:1323-1327.

  41. Van Steenbergen W, Beyls J, Vermylen J, Fevery J, Marchal G, Desmet V, De Groote J. “Lupus” anticoagulant and thrombosis of the hepatic veins (Budd-Chiari syndrome). Report of three patients and review of the literature. J Hepatol 1986;3:87-94.

  42. Yun YY, Yoh KA, Yang HI, Park SH, Lee SH, Cho CS, Kim HY. A case of Budd- Chiari syndrome with high antiphospholipid antibody in a patient with systemic lupus erythematosus. Korean J Intern Med 1996;11:82-86.

  43. Asherson RA, Kamashta MA, Huges GRV. Hepatic complications of the antiphospholipid syndrome. Clin Exp Rheumatol. 1991;9:341.

  44. Derksen RH, Kater L. Lupus anticoagulant: Revival of an old phenomenon. Clin Exp Rheumatol 1986;4:187.

  45. Love PE, Santoro SA. Antiphospholipid antibodies: Anticardiolipin and the lupus anticoagulants in systemic lupus erythematosus (SLE) and in non-SLE disorders. Ann Intern Med 1990;112:682.

  46. Gulko PS, Reveille JD, Koopman WJ, et al. Anticardiolipin antibodies in systemic lupus erythematosus: Clinical correlates, HLA associations and impact on survival. J Rheumatol 1993;20:1684.

  47. Petri M. Thrombosis and systemic lupus erythematosus. The Hopkins Lupus Cohort perspective. Scand J Rheumatol 1996;25:191.




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