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2005, Número 6

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Acta Pediatr Mex 2005; 26 (6)


Síndrome de Budd-Chiari (SBC) y su asociación con síndrome antifosfolípidos (SAF)

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

Idioma: Español
Referencias bibliográficas: 47
Paginas: 302-307
Archivo PDF: 47.53 Kb.


RESUMEN

Antecedentes: El Síndrome de Budd-Chiari (SBC) es infrecuente. Se debe a la obstrucción venosa intrahepática por trombosis o sin ella. Puede desencadenarla el síndrome antifosfolipidos (SAF) que se manifiesta por trombosis, a lo cual se agrega la presencia de anticuerpos anticardiolipina (AACL) y anticoagulante lúpico (ACL). Este se ha dividido en primario y secundario, (autoinmunidad, infecciones, neoplasias y medicamentos).
Material y métodos: Estudio retrospectivo, observacional y transversal de 24 pacientes con SAF; siete de los cuales tenían SBC (29%). Se estudiaron las variables: edad, género, tipos, complicaciones, sitios de trombosis, estudios de laboratorio y gabinete.
Resultados: De los siete pacientes con SBC, cinco fueron primarios y dos secundarios. Edad 10 a 15 años, (5 primarios 2 secundarios). Género, cuatro femeninos (tres primarios, uno secundario), tres masculinos (dos primarios, uno secundario). Tuvieron hepatomegalia siete (cinco primarios y dos secundarios). Un paciente con SBC primario tuvo esplenomegalia, ascitis y sangrado. Siete tuvieron trombosis en la vena porta. Se determinaron TgO, TgP, glutamino-amino transferasa (GGT) en los siete (cinco primarios y dos secundarios), DNAn; anticuerpos antinucleares (ANA) y complemento hemolítico (CH50) en dos pacientes (secundarios); AACL en siete pacientes (cinco primarios y dos secundarios). ACL en siete casos (seis primarios, uno secundario); Beta 2 glucoproteina 1 (B2GP1) en cinco casos primarios.
Conclusiones: Las manifestaciones clínicas fueron las mismas en ambos tipos de SAF. Los secundarios fueron debidos a LEG, los AACL, ACL y B2GP1 predominaron en los casos primarios.


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