Validation of the Spanish version of the Liver Disease Quality of Life questionnaire in transplant patients.

OBJECTIVE: Our aim was to validate Spanish and Catalan versions of the Liver Disease Quality of Life questionnaire (LDQOL) for use in liver transplant patients. METHODS: The LDQOL consists of the SF-36 generic measure of health-related quality of life (HRQOL) and 12 disease-specific dimensions for liver disease patients. The Spanish and Catalan versions of the questionnaire were administered to 138 patients with a liver transplant. Cronbach's alpha coefficients (CAC) were used to test the internal consistency of disease-specific scales. Test-retest reliability was calculated using the Intraclass Correlation Coefficient (ICC) in a sub-group of 41 patients who completed the questionnaire on two occasions 1 to 2 weeks apart. Validity was analysed by determining the instrument's capacity to discriminate between patient groups classified according to years since transplant, disease etiology, and symptom severity. Ceiling and floor effects were also calculated. RESULTS: Internal consistency in the disease-specific dimensions was acceptable or good (CACs 0.60-0.97), as was test-retest reliability in all dimensions (statistically significant CCIs of 0.62-0.89), except the symptoms dimension (CCI=0.46, P<.05). Few differences were found in disease-specific dimension scores between patients classified according to number of years since transplant or etiology, but differences were found in some dimensions according to symptom severity. Moderate to severe ceiling effects were found in several disease-specific dimensions. CONCLUSIONS: The Spanish and Catalan versions of the LDQOL may be useful for measuring HRQOL in this population, though it will be important to investigate further the instrument's sensitivity to change.

D-penicillamine and plasmapheresis in acute liver failure secondary to Wilson's disease.

We report a case of a 19-year-old woman with acute liver failure, Coombs negative hemolytic anemia, and renal failure as initial manifestations of Wilson disease with recovery following medical treatment. The clinical picture and low serum transaminase and alkaline phosphatase levels gave us a clue to suspect Wilson disease and to initiate plasmapheresis and D-penicillamine soon after admission. The serum and urinary copper levels were elevated with low serum ceruloplasmin. We proceeded to ambulatory follow-up with medical treatment with D-penicillamine. A few months later, during the course of a laparoscopic cholecystectomy because of symptomatic gallstone disease, a liver biopsy sample was obtained that showed histological liver fibrosis and strongly elevated levels of liver tissue copper.

[Transcultural adaptation of the Liver Disease Quality of Life instrument (LDQOL 1.0) for its use in the Spanish population]. / Adaptación transcultural del cuestionario específico de calidad de vida para hepatopatías crónicas (LDQOL 1.0) para su uso en la población española.

Instruments of health-related quality of life (HRQOL) help us to interpret the results of treatments and health interventions. In Spain there is no HRQOL instrument specifically designed for use in patients with liver disease or to measure the effect of interventions such as liver transplantation. The Liver Disease Quality of Life (LDQOL 1.0) questionnaire is an American instrument developed for use in these patients. The aim of this study was to produce an appropriate version of this questionnaire for use in Spain. Cultural adaptation was performed in 3 phases: a) modification for use in Spain of a Hispanic version of this questionnaire supplied by the original authors; b) back-translation to English of a new version of the questionnaire and comparison with the original version in English, and c) a pilot test in a small sample of patients. In the first phase consisting of revision of the Hispanic version, the changes were mainly linguistic due to cultural and idiomatic differences. The validated Spanish version of the SF-36 was directly incorporated and items that could be of interest to local investigators were added. Few changes were made in the second phase of the process: changes involved an item on the appearance of feces and another item on taking naps. In the final phase, various changes suggested by the patients were introduced. Before applying the new version of the LDQOL 1.0 in clinical studies in Spain, its psychometric properties (its reliability, validity and sensitivity to change) must be verified in a subsequent validation study.

Supervivencia con D-penicilamina y plasmaféresis en un caso de insuficiencia hepática aguda secundaria a enfermedad de Wilson / D-pencillamine and plasmapheresis in acute liver failu re secondary to Wilson's disease

Exponemos el caso de una paciente de 19 años que ingresó en nuestro centro con insuficiencia hepática aguda grave, anemia hemolítica Coomb´s negativa e insuficiencia renal. La clínica y los niveles séricos bajos de transaminasas y fosfatasa alcalina nos hicieron sospechar enfermedad de Wilson e iniciar plasmaféresis y D-penicilamina de forma precoz, a los 2 y 5 días respectivamente, con buena evolución posterior. Los niveles de cobre en sangre y orina resultaron ser elevados con niveles bajos de ceruloplasmina sérica. Se procedió a un seguimiento ambulatorio con tratamiento de mantenimiento con D-penicilamina, realizádose una biopsia hepática que demostró fibrosis en el examen anátomo-patológico y una concentración elevada de cobre en tejido hepático seco.En conclusión, en este caso de insuficiencia hepática aguda con hemolisis severa como forma de presentación de la enfermedad de Wilson, la instauración precoz de sesiones de plamaféresis y tratamiento con D-penicilamina fueron medidas eficaces con supervivencia sin necesidad de trasplante hepático ortotópico (AU)

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