Romiplostim therapy as a second-line treatment before splenectomy for refractory immune thrombocytopenia in a cirrhotic patient with iatrogenic Cushing syndrome secondary to corticosteroids.

Our case report discusses the usefulness of administering romiplostim as a second-line treatment before splenectomy in a cirrhotic patient with immune thrombocytopenia who developed corticosteroid-induced Cushing's syndrome. Corticosteroids were tapered and consequently withdrawn. The patient made a full recovery postsplenectomy.

Validación prospectiva de la versión corta en español del test específico de calidad de vida Short form-liver disease quality of life (SF-LDQOL) para hepatopatías crónicas y trasplante hepático / Prospective validation of the spanish version of the Short Form-Liver Disease Quality of life

La calidad de vida relacionada con la salud (CVRS) evaluada con un test específico, validado y breve es una medida importante del estado de salud percibida por los pacientes diagnosticados de hepatopatía crónica. OBJETIVO: Validar de forma prospectiva el SF-LDQOL (Short Form-Liver Disease Quality of Life) en lengua española, en pacientes con hepatopatías de diversa etiología y gravedad, atendidos en el Hospital Universitari de Bellvitge. MÉTODOS: Estudio observacional y longitudinal, en pacientes ambulatorios con hepatopatía crónica. Se administró el SF-LDQOL, que contiene el test genérico SF-36 y el test específico SF-LDQOL. Igualmente se evaluaron las características sociodemográficas, el número de respuestas en blanco, así como la fiabilidad de la consistencia interna (alpha de Cronbach) y la correlación de Pearson entre las puntuaciones del SF-36 y las del SF-LDQOL mediante la técnica de multi-rasgo multi-método. La muestra fue de 340 pacientes. RESULTADOS: 6 de las 9 dimensiones específicas de enfermedad hepática obtuvieron coeficientes de fiabilidad alfa para la consistencia interna superiores a 0,7; la validez convergente de estos ítems fue aceptable en 8 de las 9 dimensiones, con un éxito de escalaje del 100%. El porcentaje de ítems en blanco fue inferior al 1,5% en todas las dimensiones excepto Funcionamiento Sexual. CONCLUSIONES: El SF-LDQOL en lengua española cuenta con buenas propiedades psicométricas y se convierte en un instrumento útil para la práctica clínica diaria en pacientes diagnosticados de hepatopatía crónica, con o sin trasplante hepático

Health-Related Quality of Life (HRQL) assessed by a specific, validated, brief test is an important measure of the health status perceived by patients diagnosed with chronic liver disease. AIM: To prospectively validate the SF-LDQOL (Short Form-Liver Disease Quality of Life) instrument in Spanish, in patients diagnosed with liver disease of diverse etiologies and distinct severity levels, attended at the Hospital Universitari de Bellvitge (Barcelona). METHODS: This observational, longitudinal study was conducted by using the SF-LDQOL in outpatients diagnosed with chronic liver disease. This instrument contains the generic SF-36 test, and 9 liver disease-specific dimensions. We also evaluated socio-demographic features, the number of missing responses, and internal consistency (Cronbach's alpha), as well as Pearson's correlation between SF-36 and SF-LDQOL scores on specific dimensions by means of a multi-trait multi-method technique. The sample consisted of 340 patients. RESULTS: In 6 out of 9 liver disease-specific dimensions, reliability coefficients for internal consistency exceeded 0.70. The convergent validity of these items was acceptable in 8 out of 9 dimensions, with a scaling success of 100% in each item. Missing items were under 1.5% in all dimensions, except for Sexual Functioning. CONCLUSIONS: The Spanish version of the SF-LDQOL has, in general, good psychometric properties, making it a useful instrument for clinical practice in a population of patients diagnosed with chronic liver disease, with or without liver transplantation

[Prospective validation of the Spanish version of the Short Form-Liver Disease Quality of life]. / Validación prospectiva de la versión corta en español del test específico de calidad de vida Short form-liver disease quality of life (SF-LDQOL) para hepatopatías crónicas y trasplante hepático.

UNLABELLED: Health-Related Quality of Life (HRQL) assessed by a specific, validated, brief test is an important measure of the health status perceived by patients diagnosed with chronic liver disease. AIM: To prospectively validate the SF-LDQOL (Short Form-Liver Disease Quality of Life) instrument in Spanish, in patients diagnosed with liver disease of diverse etiologies and distinct severity levels, attended at the Hospital Universitari de Bellvitge (Barcelona). METHODS: This observational, longitudinal study was conducted by using the SF-LDQOL in outpatients diagnosed with chronic liver disease. This instrument contains the generic SF-36 test, and 9 liver disease-specific dimensions. We also evaluated socio-demographic features, the number of missing responses, and internal consistency (Cronbach's alpha), as well as Pearson's correlation between SF-36 and SF-LDQOL scores on specific dimensions by means of a multi-trait multi-method technique. The sample consisted of 340 patients. RESULTS: In 6 out of 9 liver disease-specific dimensions, reliability coefficients for internal consistency exceeded 0.70. The convergent validity of these items was acceptable in 8 out of 9 dimensions, with a scaling success of 100% in each item. Missing items were under 1.5% in all dimensions, except for Sexual Functioning. CONCLUSIONS: The Spanish version of the SF-LDQOL has, in general, good psychometric properties, making it a useful instrument for clinical practice in a population of patients diagnosed with chronic liver disease, with or without liver transplantation.

Patient with hepatocellular carcinoma related to prior acute arsenic intoxication and occult HBV: epidemiological, clinical and therapeutic results after 14 years of follow-up.

Little is known about the long-term survivors of acute arsenic intoxication. We present here a clinical case report of a man with chronic hepatitis B virus (HBV) infection who developed hepatocellular carcinoma four years after acute arsenic poisoning. HBsAg was detected in serum in 1990 when he voluntarily donated blood. In 1991, the patient suffered from severe psychological depression that led him to attempt suicide by massive ingestion of an arsenic-containing rodenticide. He survived with polyneuropathy and paralysis of the lower limbs, and has been wheelchair-bound since then. During participation in a follow-up study conducted among HBV carriers, abdominal ultrasound detected a two-centimeter liver mass consistent with hepatocellular carcinoma. The tumor was confirmed by computed tomography (CT) and magnetic resonance image (MRI). Because of his significant comorbidity, the patient received palliative treatment with transarterial lipiodol chemoembolization (TACE) on three occasions (1996, 1997 and 1999). At his most recent visit in May 2005, the patient was asymptomatic, liver enzymes were normal and the tumor was in remission on ultrasound.

Long-term immune response after liver transplantation in patients with spontaneous or post-treatment HCV-RNA clearance.

Recurrent HCV infection after liver transplantation is universal and sustained clearance of HCV-RNA rarely occurs. The aim of this study was to characterize cell-mediated immunity and cytokine production in HCV-infected patients after liver transplant. The study included 6 pretransplantation patients (PT) and 15 liver transplanted patients, including 5 with spontaneous HCV-RNA clearance (SC group), 5 with sustained virological response after antiviral treatment (SVR group), and 5 no response (NR group). The control group included 5 HCV-RNA negative, anti-HCV negative healthy individuals. This study examines proliferative T-cell response and cytokine production (gamma-interferon and IL-10) after HCV specific and phytohemagglutinin (PHA) stimulation in cultured peripheral blood mononuclear cells (PBMCs) from each group. Multispecific proliferative responses to HCV antigens (mean Stimulation Index; SI) were higher in the SVR group (mean SI 7.4 +/- 2) and SC group, as compared with the NR group (P <.05, vs SVR) and PT group (P <.05, vs SVR and SC). After PHA stimulation, gamma-interferon levels were similar to controls (4330 +/- 640 pg/ml) in the SC (4474 +/- 300 pg/mL) and SVR groups (3647 +/- 300 pg/mL), but were significantly lower than controls in the PT (401 +/- 331 pg/mL; P <.02) and NR groups (546 +/- 360 pg/mL; P <.01). IL-10 production after PHA stimulation was similar in SC, SVR, and controls (647 +/- 279 pg/mL, 674 +/- 310 pg/mL and 841 +/- 294 pg/mL, respectively), but was lower in PT patients (232 +/- 94 pg/mL). The NR group showed high basal IL-10 production with little increase after stimulation. In conclusion, liver post-transplantation patients with spontaneous clearance of HCV-RNA and those with sustained viral response after therapy showed an immune response despite immunosuppression that might have contributed to their favorable outcome.

Validation and refinement of survival models for liver retransplantation.

Orthotopic liver retransplantation (re-OLT) is highly controversial. The objectives of this study were to determine the validity of a recently developed United Network for Organ Sharing (UNOS) multivariate model using an independent cohort of patients undergoing re-OLT outside the United States, to determine whether incorporation of other variables that were incomplete in the UNOS registry would provide additional prognostic information, to develop new models combining data sets from both cohorts, and to evaluate the validity of the model for end-stage liver disease (MELD) in patients undergoing re-OLT. Two hundred eighty-one adult patients undergoing re-OLT (between 1986 and 1999) at 6 foreign transplant centers comprised the validation cohort. We found good agreement between actual survival and predicted survival in the validation cohort; 1-year patient survival rates in the low-, intermediate-, and high-risk groups (as assigned by the original UNOS model) were 72%, 68%, and 36%, respectively (P <.0001). In the patients for whom the international normalized ratio (INR) of prothrombin time was available, MELD correlated with outcome following re-OLT; the median MELD scores for patients surviving at least 90 days compared with those dying within 90 days were 20.75 versus 25.9, respectively (P =.004). Utilizing both patient cohorts (n = 979), a new model, based on recipient age, total serum bilirubin, creatinine, and interval to re-OLT, was constructed (whole model chi(2) = 105, P <.0001). Using the c-statistic with 30-day, 90-day, 1-year, and 3-year mortality as the end points, the area under the receiver operating characteristic (ROC) curves for 4 different models were compared. In conclusion, prospective validation and use of these models as adjuncts to clinical decision making in the management of patients being considered for re-OLT are warranted.

Pretransplant interferon prevents hepatitis C virus-associated glomerulonephritis in renal allografts by HCV-RNA clearance.

The purpose of this study was to examine the effect of pretransplant interferon administration on the occurrence of post-transplant de novo glomerulonephritis in hepatitis C virus (HCV)-positive renal allografts. From December 1992 to December 2000, 78 HCV-positive patients received a renal allograft in our unit. Fifteen out of 78 received pretransplant interferon for 1 year. Hepatitis C virus was investigated by serology and qualitative polymerase chain reaction (PCR). Hepatitis C virus-related de novo glomerulonephritis (membranoproliferative or membranous) was suggested by proteinuria (>1.5 g/24 h) and/or microhematuria and always diagnosed by renal biopsy. Of 15 HCV-positive recipients who received pretransplant interferon, 10 (67%) became HCV-RNA negative at the time of transplantation and only one out of the 15 (6.7%) developed de novo glomerulonephritis (this patient was HCV-RNA positive at transplantation). Among non-interferon-treated allograft recipients, 28.7% had negative HCV-RNA and 12 out of 63 (19%) developed de novo glomerulonephritis (9, membranoproliferative; 3 membranous), all 12 having positive HCV-RNA at transplantation (p < 0.0001). In conclusion, pretransplant interferon may reduce the occurrence of post-transplant HCV-related de novo glomerulonephritis. Our results suggest that the indication for pretransplant interferon should be extended to treat all HCV-RNA positive candidates for renal transplantation.

Análisis de 500 trasplantes hepáticos en el Hospital de Bellvitge / Analysis of 500 liver transplantations in the Bellvitge Hospital, Spain

Fundamento: Se presenta la experiencia del programa de trasplante hepático del Hospital de Bellvitge en 500 trasplantes realizados durante 15 años, con el objetivo de poner de manifiesto los cambios que se han producido y exponer los resultados a largo plazo de esta terapéutica. Pacientes y método: Se consideraron y compararon 5 grupos de 100 trasplantes consecutivos (I-V). Resultados: Las indicaciones más frecuentes fueron el hepatocarcinoma (23 por ciento), la cirrosis alcohólica (22,8 por ciento) y la hepatopatía crónica por virus C (18,8 por ciento). En 59 pacientes se llevaron a cabo 65 retrasplantes (13 por ciento), cuyas indicaciones más frecuentes fueron la trombosis arterial (13 pacientes) y el fallo primario del injerto (10 pacientes). En 19 enfermos se realizó un trasplante combinado hepatorrenal. La causa más frecuente de muerte del donante en el grupo I fueron los traumatismos craneales (80 por ciento), mientras que en el grupo V fue la enfermedad vascular (52 por ciento). Otras diferencias significativas entre estos grupos se observan en la proporción de pacientes en estadio 2 y 3 de la clasificación UNOS (el 45 frente al 19 por ciento), en el consumo de hemoderivados (29,6 [26] frente a 4,6 [5,3] concentrados de hematíes), en la frecuencia de reintervenciones por hemoperitoneo (el 22 frente al 5 por ciento), en la estancia en UCI (13 [13] frente a 7,4 [11] días) y en el hospital 40 [52] frente a 23,7 [17] días), y en la incidencia de rechazo (el 46 frente al 20 por ciento) y de fallo primario del injerto (el 9 frente al 3 por ciento). Sin embargo, la prevalencia de infección (el 48 frente al 54,5 por ciento) y la incidencia de complicaciones biliares (el 26 frente al 20 por ciento) no han presentado variaciones significativas. La supervivencia actuarial de los pacientes trasplantados desde 1990 es del 83 y del 70 por ciento al año y a los 5 años, respectivamente. Conclusiones: Se observa una mejoría notable y progresiva de los resultados del trasplante hepático. Sin embargo, los tumores de novo, la recidiva de la hepatitis por virus C y el rechazo crónico pueden limitar los resultados a largo plazo. (AU)