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Síndrome de Bassen-Kornzweig - Treatment

Nombres alternativos

Abetalipoproteinemia; Acantocitosis; Deficiencia de apolipoproteína B

Tratamiento:

El tratamiento involucra grandes dosis de suplementos vitamínicos que contengan vitaminas liposolubles (vitamina A, vitamina D, vitamina E y vitamina K).

Igualmente, se recomiendan suplementos de ácido linoleico.

Las personas con esta afección deben hablar con un nutricionista. Se necesitan cambios en la alimentación para prevenir problemas estomacales. Esto implica:

  • Limitar la ingesta de grasa de 5 a 20 gramos por día.
  • No comer más de 140 gramos (5 onzas) de carne magra, pescado o aves por día.
  • Usar leche descremada en vez de leche entera.

Los suplementos de triglicéridos de cadena media se toman bajo la supervisión de un médico o un nutricionista, pero se deben usar con cautela, debido a que pueden ocasionar daño hepático.

Pronóstico:

El pronóstico para el paciente depende de la cantidad de problemas del sistema nervioso y el cerebro.

Posibles complicaciones:

  • Ceguera
  • Deterioro mental
  • Pérdida de la función de los nervios periféricos, movimiento descoordinado (ataxia)

Cuándo contactar a un profesional médico:

Consulte con el médico si su bebé o niño presenta síntomas de esta enfermedad. La asesoría genética le puede ayudar a las familias a entender la enfermedad y los riesgos de heredarla y aprender cómo cuidar al paciente.

  • Reviewed last on: 8/4/2011
  • Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Rodriguez-Oquendo A, Kwiterovich Jr PO. Dyslipidemias. In: Fernandes J, Saudubray J-M, van den Berghe G, Walter JH, eds. Inborn metabolic diseases: diagnosis and treatment. Germany: Springer; 2006:400-401.

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