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Vol. 54. Núm. 4.
Páginas 359-371 (abril 2001)
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Vol. 54. Núm. 4.
Páginas 359-371 (abril 2001)
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Trasplante pulmonar pediátrico
Pediatric lung transplantation
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8128
C.B. Huddlestona,
Autor para correspondencia
huddlestonc@msnotes.wustl.edu

Correspondencia: Children's Place, Suite 5550. Children's Hospital. St. Louis, MO USA 63110.
, E. Mendeloffa, G.B. Malloryb
a Division of Cardiothoracic Surgey. Washington University School of Medicine. St. Louis Children's Hospital, St. Louis, Estados Unidos.
b Hospital for Sick Children, Great Ormond Street, Londres, Inglaterra.
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Se han efectuado aproximadamente 700 trasplantes en niños menores de 18 años de edad en todo el mundo; en cambio, se han efectuado más de 11.000 en adultos. El principal grupo diagnóstico es el de fibrosis quística. Un grupo emergente de pacientes está constituido por los niños que nacen con enfermedades pulmonares de base congénita, como la deficiencia de la proteína B del surfactante. La supervivencia de los niños es muy similar a la observada en adultos, aunque, por lo general, los niños pertenecen a una clase de riesgo más elevado. Por ejemplo, no hay niños que hayan sido sometidos a trasplante por enfermedad pulmonar obstructiva crónica (un grupo diagnóstico de bajo riesgo), en tanto que dicho diagnóstico comprende alrededor del 40 % de todos los adultos sometidosa trasplante de pulmón. La inmunosupresión consiste por lo general en ciclosporina, azatioprina y prednisona. Las complicaciones postrasplante son similares a las observadas en adultos. Alrededor del 40 % de los pacientes desarrollan una bronquiolitis obliterante hacia los 3 años postrasplante y ello sigue constituyendo el principal impedimento para la supervivencia largo plazo. La escasez de donantes también es un obstáculo significativo, sobre todo en el grupo de pacientes adolescentes. Como solución parcial a dicho problema, el trasplante pulmonar de donante vivo ha asumido mayor trascendencia en el programa. Aunque es una estrategia terapéutica compleja y costosa, el trasplante pulmonar sigue siendo el tratamiento más eficaz de las neumopatías parenquimatosas y vasculares en estadios terminales, incluso en los niños de menor edad.

Palabras clave:
Trasplante pulmonar
Niños
Lactantes
Fibrosis quística
Hipertensión pulmonar
Deficiencia de proteína B del surfactante

Approximately 700 transplants world-wide have been performed in patients aged less than 18 years; in contrast, over 11,000 lung transplants have been performed in adults. The major diagnostic group is cystic fibrosis. An emerging group of patients are infants born with congenitally based pulmonary diseases such as surfactant protein B deficiency. Survival in children is very similar to that in adults, although it is generally perceived that children are at higher risk. For instance, no children have been transplanted for chronic obstructive lung disease (a low-risk diagnostic group) while this disease comprises approximately 40 % of all adult lung transplantations. Immunosuppression generally consists of cyclosporine, azathioprine and prednisone. Post-transplant complications in children are similar to those observed in adults. Around 40 % of patients will develop bronchiolitis obliterans approximately 3 years after transplantation and this remains the major impediment to long term survival. Donor shortage is also a significant obstacle, especially in adolescents. As a partial solution to this problem, living donor lung transplantation has taken on a greater role in our program. Although this is a complex, expensive treatment strategy, lung transplantation remains the most effective therapy for end-stage pulmonary parenchymal and vascular diseases even in children.

Key words:
Lung transplantation
Children
Cystic fibrosis
Pulmonary hypertension
Surfactant protein B deficiency
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