Marie-Claude Becker

A twenty-year history of alveolar echinococcosis: analysis of a series of 117 patients from eastern France.

Objectives Alveolar echinococcosis of the liver is a very rare and severe parasitic disease due to the growth of the larva of Echinococcus multilocularis. The aim of this paper was to describe a 20‐year study of the epidemiological, clinical and therapeutic aspects of alveolar echinococcosis in eastern France. Design One hundred and seventeen consecutive cases, diagnosed and followed in our liver unit, were studied from 1972 to 1993. Methods Data from 85 patients followed since 1983 (period B) were compared to data from a first series of 32 patients (period A) collected from 1972 to 1982; 1983 was chosen as the cut‐off year because of the numerous changes that occurred in the diagnosis, follow‐up and treatment of the disease at this time, in particular the introduction of parasitostatic benzimidazoles. Results The results of patient follow‐up were evaluated in December 1997. The cumulative prevalence was 2.5 per 100 000 persons in period A whereas it reached 6.6 per 100 000 in period B. The annual incidence in period B was 7.3 on average, compared with 2.7 in period A. Twenty‐nine per cent of patients from period B were asymptomatic at the time of diagnosis compared with 10% in period A. This change was correlated with less advanced liver lesions, and was related to the extensive use of abdominal ultrasound, and from 1987, serological screening. Curative resections were performed in 24% of the cases in period B versus only 3% in period A. From 1986, liver transplantations were performed in eight patients from period A and 13 patients from period B. In period B, palliative surgery was frequently replaced by radiological non‐operative procedures to treat abscesses and jaundice. From 1982, 73 patients received benzimidazoles for a period of time ranging from 4 to 138 months. Stabilization of the lesions was observed in two‐thirds of the patients. Episodes of jaundice or digestive haemorrhage due to portal hypertension were 31.5 and 11 times less frequent respectively in patients from period B compared with period A. Actuarial survival at 5 years improved from 67% in period A to 88% in period B in patients of similar age. Conclusions Radical changes in the diagnosis and the management of alveolar echinococcosis have occurred during the last decade. Together they have contributed to an improvement in the status of the patients affected by this very severe parasitic disease. Eur J Gastroenterol Hepatol 12:327‐336

Infliximab treatment for steroid-refractory acute graft-versus-host disease after orthotopic liver transplantation: a case report.

Acute graft‐versus‐host disease (GVHD) following orthotopic liver transplantation is a rare but severe disease with a 75% death rate in adults. Various therapeutic strategies have been proposed for steroid‐refractory GVHD, but there is still no consensus. Tumor necrosis factor‐alpha is a key inflammatory cytokine involved in acute GVHD physiopathology, and infliximab has shown encouraging results for the treatment of acute GVHD following hematopoietic stem cell transplantation. We report the first case of acute GVHD following liver transplantation that was refractory to steroids and anti‐lymphocyte globulin but was successfully treated with infliximab. Liver Transpl 15:682–685, 2009.

Liver transplantation for cardiac failure in patients with hereditary hemorrhagic telangiectasia

Liver involvement in hereditary hemorrhagic telangiectasia may lead to high‐output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we review literature on this subject. Our two patients resumed normal cardiac function after OLT. This procedure appears to be a promising therapy with good long‐term results despite dissection difficulties encountered due to the collateral arterial network reorganization. (Liver Transpl 2005;11:834–838.)

Traitements complémentaires de l’hépatite chronique virale C

Resume La bitherapie associant l’interferon pegylee et la ribavirine permet de guerir plus de la moitie des malades atteints d’hepatite chronique virale C. Cependant, ces progres therapeutiques ne doivent pas faire oublier les nombreux effets secondaires, notamment hematologiques et psychiatriques, rencontres avec ces molecules. Cette mise au point est consacree aux differents traitements complementaires (erythropoietine, G-CSF, vitamine E, glutathion, acide ursodesoxycholique et antidepresseurs) susceptibles d’apporter un benefice dans le maintien des doses optimales et dans l’amelioration de la tolerance de la bitherapie anti-virale C. La recherche bibliographique etait realisee a l’aide de la base de donnees Medline® et des differents laboratoires ayant commercialises ces molecules. En l’absence d’autres molecules innovantes disponibles sur le marche dirigees contre le virus de l’hepatite C, l’erythropoietine, le G-CSF et les antidepresseurs sont les traitement complementaires les plus interessants pour optimiser la bitherapie actuelle dans sa dose et sa duree tout en privilegiant la qualite de vie des malades.

Acute recurrent biliary pancreatitis associated with the ABCB4 gene mutation.

The ABCB4 gene codes for a protein involved in the transport of phosphatidylcholine across the canalicular membrane of the hepatocyte. ABCB4 gene defects have been associated with progressive familial intrahepatic cholestasis type 3, intrahepatic cholestasis of pregnancy, adult biliary cirrhosis and the more recently described low phospholipid associated cholelithiasis syndrome. The present paper describes 2 probands with a long history of recurrent pancreatitis and cholelithiasis and the same heterozygous, as yet undescribed del 3683>3688 within exon 28 of the ABCB4 gene resulting in a loss of function. This report shows that ABCB4 mutations may cause acute recurrent biliary pancreatitis.