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        "resumen" => "<span class="elsevierStyleSectionTitle">Objetivos</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">El objetivo de este estudio es evaluar la funci&#243;n respiratoria de 4 pacientes con fibrosis qu&#237;stica sometidos a trasplante hep&#225;tico</p> <span class="elsevierStyleSectionTitle">Pacientes y m&#233;todos</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Entre 1993 y 1997&#44; 4 pacientes con fibrosis qu&#237;stica&#44; 3 varones y 1mujer&#44; de edades comprendidas entre los 12 y los 15 a&#241;os&#44; con cirrosis e hipertensi&#243;n portal&#44; fueron sometidos a un trasplante hep&#225;tico&#46; Tres ten&#237;an antecedentes de sangrado por varices gastroesof&#225;gicas&#46; Antes del trasplante&#44; 3 casos presentaban una capacidad vital forzada &#40;FVC&#41; y un volumen respiratorio m&#225;ximo en el primer segundo &#40;FEV1&#41; superiores al 80 y 75 &#37;&#44; respectivamente&#44; con respecto a los valores te&#243;ricos&#59; el cuarto caso presentaba una FVC del 37 &#37; y un FEV1 del 26 &#37;&#46; Dos pacientes presentaron un brote de aspergilosis broncopulmonar al&#233;rgica en los meses previos al trasplante&#46; S&#243;lo un paciente estaba colonizado de forma cr&#243;nica en esputo por <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> multirresistente y ninguno por <span class="elsevierStyleItalic">Burkholderia cepacia</span>&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Tras el trasplante hep&#225;tico s&#243;lo el paciente colonizado por <span class="elsevierStyleItalic">P&#46;aeruginosa</span> y peor funci&#243;n pulmonar present&#243; una evoluci&#243;n m&#225;s t&#243;rpida precisando ventilaci&#243;n mec&#225;nica durante 43 d&#237;as y ventilaci&#243;n nasal no invasiva durante 8 meses&#44; y falleci&#243; a los 19 meses del trasplante&#46; Los otros 3 casos&#44; con mejor funci&#243;n pulmonar pretrasplante&#44; presentaron una evoluci&#243;n sin complicaciones importantes y en la actualidad llevan una vida normal&#46;</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El trasplante hep&#225;tico es bien tolerado en ni&#241;os con fibrosis qu&#237;stica y afectaci&#243;n pulmonar leve-moderada&#44; pudiendo incluso mejorar su funci&#243;n pulmonar&#46; En los casos de afectaci&#243;n pulmonar grave debe considerarse la realizaci&#243;n de un trasplante combinado hepatopulmonar&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objectives</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The aim of this study was to evaluate pulmonary function in four patients with cystic fibrosis &#40;CF&#41; after liver transplantation&#46;</p> <span class="elsevierStyleSectionTitle">Patients and methods</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">From 1993 to 1997 three males and one female&#44; aged 12 to 15 years&#44; required liver transplantation for CF with cirrhosis and portal hypertension&#46; Three had a history of esophageal variceal bleeding&#46; In three patients&#44; forced vital capacity &#40;FVC&#41; and forced expiratory volume in one second &#40;FEV1&#41; before liver transplantation were over 80 and 75 &#37; of predicted values&#44; respectively&#59; in the fourth patient FVC was 37 &#37; and FEV1 was 26&#37;&#46; Two patients presented allergic bronchopulmonary aspergillosis before transplantation&#46; Only one patient was chronically infected in sputum with multiresistant <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> and none had <span class="elsevierStyleItalic">Burkholderia cepacea</span>&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">After liver transplantation&#44; only the patient with <span class="elsevierStyleItalic">P&#46; aeruginosa</span> in sputum culture and the worst pulmonary function presented a complicated course requiring mechanical ventilation for 43 days followed by non-invasive nasal ventilation for 8months&#46; This patient died 19 months after transplantation&#46; The remaining three patients&#44; with better pulmonary function before transplantation&#44; presented an uncomplicated course and currently lead normal lives&#46;</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">We conclude that liver transplantation can improve pulmonary function and is well tolerated in children with CF and mild or moderate pulmonary involvement&#46; When pulmonary involvement is severe&#44; combined lung and liver transplantation should be considered&#46;</p>"
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Vol. 54. Núm. 3.
Páginas 228-232 (marzo 2001)
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Vol. 54. Núm. 3.
Páginas 228-232 (marzo 2001)
Acceso a texto completo
Evolución respiratoria de 4 pacientes con fibrosis quística receptores de trasplante hepático
Pulmonary function after liver transplantation in cystic fibrosis. Report of four cases
Visitas
6174
E. Urgellés Fajardoa,
Autor para correspondencia
elena_urgelles@hotmail.com

Correspondencia: 2421 Crystal Bay SW. 55902 Rochester. Minnesota. Estados Unidos
, M.aI. Barrio Gómez de Agüeroa, M.aC. Martinez Carrascoa, P. Jara Vegab, C. Antelo Landeiraa
a Unidades de Neumología. Hospital Infantil La Paz. Madrid
b Unidades de Hepatología. Hospital Infantil La Paz. Madrid
Este artículo ha recibido
Información del artículo
Objetivos

El objetivo de este estudio es evaluar la función respiratoria de 4 pacientes con fibrosis quística sometidos a trasplante hepático

Pacientes y métodos

Entre 1993 y 1997, 4 pacientes con fibrosis quística, 3 varones y 1mujer, de edades comprendidas entre los 12 y los 15 años, con cirrosis e hipertensión portal, fueron sometidos a un trasplante hepático. Tres tenían antecedentes de sangrado por varices gastroesofágicas. Antes del trasplante, 3 casos presentaban una capacidad vital forzada (FVC) y un volumen respiratorio máximo en el primer segundo (FEV1) superiores al 80 y 75 %, respectivamente, con respecto a los valores teóricos; el cuarto caso presentaba una FVC del 37 % y un FEV1 del 26 %. Dos pacientes presentaron un brote de aspergilosis broncopulmonar alérgica en los meses previos al trasplante. Sólo un paciente estaba colonizado de forma crónica en esputo por Pseudomonas aeruginosa multirresistente y ninguno por Burkholderia cepacia.

Resultados

Tras el trasplante hepático sólo el paciente colonizado por P.aeruginosa y peor función pulmonar presentó una evolución más tórpida precisando ventilación mecánica durante 43 días y ventilación nasal no invasiva durante 8 meses, y falleció a los 19 meses del trasplante. Los otros 3 casos, con mejor función pulmonar pretrasplante, presentaron una evolución sin complicaciones importantes y en la actualidad llevan una vida normal.

Conclusiones

El trasplante hepático es bien tolerado en niños con fibrosis quística y afectación pulmonar leve-moderada, pudiendo incluso mejorar su función pulmonar. En los casos de afectación pulmonar grave debe considerarse la realización de un trasplante combinado hepatopulmonar.

Palabras clave:
Trasplante hepático
Función pulmonar
Aspergilosis broncopulmonar alérgica
Fibrosis quística
Objectives

The aim of this study was to evaluate pulmonary function in four patients with cystic fibrosis (CF) after liver transplantation.

Patients and methods

From 1993 to 1997 three males and one female, aged 12 to 15 years, required liver transplantation for CF with cirrhosis and portal hypertension. Three had a history of esophageal variceal bleeding. In three patients, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) before liver transplantation were over 80 and 75 % of predicted values, respectively; in the fourth patient FVC was 37 % and FEV1 was 26%. Two patients presented allergic bronchopulmonary aspergillosis before transplantation. Only one patient was chronically infected in sputum with multiresistant Pseudomonas aeruginosa and none had Burkholderia cepacea.

Results

After liver transplantation, only the patient with P. aeruginosa in sputum culture and the worst pulmonary function presented a complicated course requiring mechanical ventilation for 43 days followed by non-invasive nasal ventilation for 8months. This patient died 19 months after transplantation. The remaining three patients, with better pulmonary function before transplantation, presented an uncomplicated course and currently lead normal lives.

Conclusions

We conclude that liver transplantation can improve pulmonary function and is well tolerated in children with CF and mild or moderate pulmonary involvement. When pulmonary involvement is severe, combined lung and liver transplantation should be considered.

Key words:
Liver transplantation
Pulmonary function
Allergic bronchopulmonary aspergillosis
Cystic fibrosis
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Copyright © 2001. Asociación Española de Pediatría
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