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Resumen Introduccion La afectacion hepatica en pacientes con enfermedades sistemicas autoinmunes no es infrecuente. Manifestaciones clinicas En la mayoria de los casos se caracteriza por una alteracion bioquimica subclinica en forma de hipertransaminasemia y/o colestasis de grado leve a moderado. El desarrollo de cirrosis o de una hepatitis fulminante es inusual en este grupo de enfermedades. Es de destacar el compromiso vascular cuando se produce trombosis venosa hepatica en forma de sindrome de Budd-Chiari o de pequenas arterias intrahepaticas y su relacion con la hiperplasia nodular regenerativa. Histopatologia Las alteraciones histologicas son diversas y pueden revelar otras enfermedades hepaticas subyacentes como las hepatitis viricas, la esteatosis y la toxicidad por farmacos. Tratamiento El tratamiento de estas alteraciones no es especifico y se basa fundamentalmente en el de la enfermedad de base.

onflict of interest one declared. eral prognostic scores (SAPS II or SAPS 3) were globally higher in the Alegre et al. series whereas the values of liver specific prognostic scores were higher in our study. This data certainly illustrates that the Ci-pro score lacks from universality and is more adapted to situations of decompensated liver disease. Similarly, SAPS 3, although interesting in cases of ImCU admission for general complications as illustrated in Alegre et al.’s study [5], was of less interest in decompensated liver disease in our series. As explained in the discussion of the article [1], the weight of cirrhosis in the calculation of the score could underestimate the discriminating effect of the score in our population of decompensated liver disease, which could not be the case when the reason of the admission in ImCU is not a liver-related cause. Another explanation could come from a difference in the cause of the liver disease. In our study the vast majority of liver diseases (88%) were alcoholic. The cause of the liver disease is not reported by Alegre et al. [5] but we can suspect that the proportion of alcoholic liver diseases was less important. It is admitted that the cause of the liver disease can influence the natural history and prognosis of the cirrhosis and could certainly have influenced the results of these respective analyses [2]. Despite all these explanations, we believe that the relatively limited population (51 patients) in [5] cannot permit to conclude about the real performances of the different studied prognostic scores in cirrhotic patients admitted to ImCU. The population of

We describe a 47-year-old patient who developed cholelithiasis in adolescence, followed by recurrent intrahepatic cholestasis of pregnancy, and finally biliary cirrhosis in adulthood. In our patient, the consecutive presentation of the 3 mentioned disorders raised the suspicion of a defect of MDR3, the canalicular protein involved in the transport of phospatidylcholine to bile. Mutational analysis in our patient showed a heterozygous missense mutation of the MDR3 gene that has not been described previously, which occurs in exon 14 at codon 535, and results in the substitution of glycine for aspartic acid. Further analysis of 7 members of the family showed the same mutation in her daughter who, on follow-up, developed cholestasis of pregnancy and persisting high serum levels of γ-glutamyl transpeptidase and alkaline phosphatase after delivery. Although biliary cirrhosis associated with MDR3 deficiency typically appears before the age of 25 years, in our case, the relatively mild MDR3 dysfunction allowed for a slower progression of the disease with established, well-advanced cirrhosis in the fifth decade of life. The present case, which accumulates the 3 clinical disorders assocaited with MDR3 deficiency, shows that this condition should be suspected not only in children or young people with high γ-glutamyl transpeptidase cholestasis but also in middle-aged or older patients with chronic idiopathic cholestasis, especially when there is a previous history of cholestasis of pregnancy or juvenile cholelithiasis.

In the last 20 years, endovascular procedures have radically altered the treatment of diseases of the aorta. The objective of endovascular treatment of dissections is to close the entry point to redirect blood flow toward the true lumen, thereby achieving thrombosis of the false lumen. In extensive chronic dissections that have evolved with the formation of a large aneurysm, the dissection is maintained from the end of the endoprosthesis due to multiple orifices, or reentries, that communicate with the lumens. In addition, one of the primary limitations of this technique is when the visceral arteries have disease involvement. In this report we present a case where, despite having treated the entire length of the descending thoracic aorta, the dissection was maintained distally, leading to progression of the diameter of the aneurysm. After reviewing the literature, and to the best of our knowledge, we describe the first case in which renal autotransplant was performed to allow for subsequent exclusion of the aorta at the thoracoabdominal level using a fenestrated endoprosthesis for the celiac trunk and the superior mesenteric artery.