Indications and results of liver transplantation for Echinococcus alveolar infection: an overview

Background: Alveolar echinococcosis (AE) of the liver, caused by the larval stage of the fox tapeworm Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. It is one of the rare parasitic diseases for which a parasitolytic drug is not yet available, and AE is lethal in the absence of appropriate therapeutic management. Complete surgical resection of the parasite at an early stage of infection provides favourable prospects for cure, but, due to a long clinical latency, many cases are diagnosed at an advanced stage, so that partial liver resection can be performed in only 35% of patients. Benzimidazole (BZM) treatment is given in inoperable cases but these compounds are only parasitostatic, and lifelong therapy is required. During the past 20 years some centres have considered liver transplantation (LT) for the treatment of incurable AE. Methods: Our review summarizes the results of this experience based on a series of 47 European patients who received transplants between 1985 and 2002, tries to specify the real place of LT for AE, and underlines the measures that could be undertaken in the future to improve the results. Results: Five-year survival was 71%. Five-year survival without recurrence was 58%. Major technical difficulties related either to previous laparotomies or to the loco-regional involvement were observed. The nine early deaths concerned AE patients with a long past-history of symptomatic AE (iterative cholangitis, secondary biliary cirrhosis). Five late deaths were directly related to ongoing AE, located in the brain in three cases, a very rare AE location that was not investigated before LT in these patients. Conclusions: In general, the pre-LT screening for distant AE metastases appeared insufficient in this series. Heavy immunosuppressive schemes, absence or delayed re-introduction of BZM after LT have clearly played a role in this unfavourable course. This unique experience indicates that, despite major technical difficulties, LT for incurable AE is feasible and could be discussed in very symptomatic cases. Before LT, interventional radiology should be preferred to repeated laparotomies. Pre-LT and post-LT BZM treatment is mandatory. A careful evaluation of possible distant metastases should be done before the decision for LT is made. After LT, the possibility of an ongoing AE must be permanently kept in mind. This could be reduced by lightening the immunosuppressants, carefully following the specific circulating antibodies, and applying a systematic radiological evaluation, not only to the graft but also to the lungs and the brai

Les manifestations extrahépatiques de l'hépatite C (à propos de deux observations)

BESANCON-BU Médecine pharmacie (250562102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

[Alveolar echinococcosis: a disease comparable to a slow growing cancer]

International audienceAlveolar echinococcosis, a parasitic disease due to the larval stage of the cestode Echinococcus multilocularis, is initially located in the liver in 97% of cases. Progression is very slow and the disease remains silent for many years. The developing larva behaves like a slow-growing liver tumor that gradually invades the liver parenchyma, vessels and bile ducts. Marked granulomatosis around the larva, and the subsequent strong reactive fibrosis, contribute to the severity of the disease. Gradual extension to adjacent organs and distant metastases due to haematogenous spread can also occur. Purely extrahepatic alveolar echinococcosis is rare, but physicians in endemic areas should be aware of this possibility. Diagnostic methods have dramatically improved over the past twenty years. The clinical presentation used to be similar to that of liver cancer, with slowly progressivejaundice (due to involvement of the hilum), huge, hard and irregular hepatomegaly, and a chronic Budd-Chiari syndrome due to hepatic vein involvement. Currently, with extensive use of abdominal ultrasonography, alveolar echinococcosis is commonly diagnosed when still asymptomatic. Alveolar echinococcosis may also be revealed by a complication, such as cholangitis due to communication between the parasite mass and the lumen of a bile duct or to pigment stones accumulating above a parasitic biliary stenosis; liver abscess related to centro-parasitic necrosis; or hematemesis due to esophagal varices in case of portal vein involvement. Metastases, especially in the lungs, reveal the disease in 5% of cases

Successful hepatorenal transplantation in hereditary amyloidosis caused by a frame-shift mutation in fibrinogen Aalpha-chain gene.

International audienceHereditary systemic amyloidosis comprises several autosomal dominant diseases caused by mutations in a number of plasma proteins, including the fibrinogen Aalpha-chain. Four mutations in the fibrinogen Aalpha-chain that are able to induce amyloidosis have been identified so far, the most common being the Glu526Val mutation. We have observed a family in which the father and his son reached end-stage renal failure because of renal amyloidosis induced by a frame-shift mutation in the fibrinogen Aalpha-chain gene producing a novel amyloid protein. Two kidney transplantations in the father and one in the son resulted in fast graft loss caused by recurrence of amyloid deposition. We then performed hepatorenal transplantation in the son. Three years later, liver and kidney functions are normal without recurrence of amyloid deposition. This case, together with three others with the Glu526Val mutation in the extensive literature, suggests that liver transplantation can cure hereditary fibrinogen amyloidosis, whatever the mutation may be

Predictive factors of alcohol relapse after orthotopic liver transplantation for alcoholic liver disease

International audienceThe objective of this prospective study was to determine whether sociological and/or alcohol-related behavioral factors could be predictive of relapse after orthotopic liver transplantation for alcoholic liver disease

[Is chronic hepatitis C treatment as efficient in the general population as in randomised trials?].

International audienceThe aim of this population-based study was to determine the effectiveness of antiviral therapy in non selected chronic hepatitis C patients

Granulomatous hepatitis and hemophagocytic syndrome after bacillus Calmette-Guerin bladder instillation.

National audienceIntravesical instillations of bacillus Calmette-Guérin are frequently used for treating superficial bladder carcinoma which is considered a safe treatment. We describe an unusual complication with hemophagocytosis and granulomatous hepatitis. Prompt diagnosis and treatment with corticosteroids, anti-tuberculous agents and intravenous immunoglobulins led to a rapid recovery

Échinococcose alvéolaire en région parisienne…

International audienc

Towards "à la carte" management of patients with inoperable alveolar echinococcosis of the liver: usefullness of a compined follow-up with specific serology, CT-scan and PET-CT

International audienc

[Complementary treatments of chronic viral hepatitis C]

International audiencePegylated interferon and ribavirin combination therapy represent the standard-of-care treatment for chronic hepatitis C, that allows to cure more than half of the patients. However, the success of this bitherapy is in balance with numerous side effects, especially hematologic and psychiatric. This review is focused on complementary treatments (erythropoietin, G-CSF, vitamin E, glutathion, ursodeoxycholic acid and antidepressants) likely to bring a benefit in maintaining adequate interferon and ribavirin dosages and in improving quality of life. This analysis has been performed by using the Medline(R) data base and with data from laboratories which commercialized these molecules. Erythropoietin, G-CSF and antidepressants are the best tools to optimize the bitherapy in its dose and its duration while privileging the quality of life of HCV-infected patients