Sirolimus plus sorafenib in treating HCC recurrence after liver transplantation: A case report

A case of hepatocellular carcinoma (HCC) with pulmonary recurrence after liver transplantation for HCC is presented in this report. The patient showed disease progression on sorafenib therapy demonstrated by computed tomography scans as well as serial serum α-fetoprotein (AFP) elevation. After his immunosuppression therapy was successfully transitioned to sirolimus and a continuation of sorafenib, he achieved partial remission based on RECIST criteria and normalization of AFP. Mammalian target of rapamycin inhibitors including sirolimus alone or in conjunction with sorafenib may be useful in the treatment of post transplant HCC

Phosphotyrosyl-protein phosphatase of TCRC-2 cells

Homogenization of TCRC-2 cells yielded a phosphotyrosyl-protein phosphatase with a specific activity approximately 10-=fold higher in particulate than in soluble fractions. Over 90% of the phosphotyrosyl-protein phosphatase associated with the particles was solubilized with 1.0% Nonidet P-40. Chromatography of the detergent-solubilized particulate fraction on either wheat germ lectin-Sepharose or histone-Sepharose columns separated two major components of phosphatase activity. One peak (eluted with 200 mM NaCl from histone-Sepharose or with N-acetylglucosamine from the lectin column) contained both phosphotyrosyl-and phosphoseryl-protein phosphatase as well as p-nitrophenyl phosphatase activities. The other peak (eluted with 1.0 M NaCl from histone-Sepharose or not bound to the lectin column) contained essentially only phosphoseryl-protein phosphatase activity. Various agents (EDTA, p-nitrophenyl phosphate, fluoride) showed considerable differences in their ability to inhibit the two phosphatase fractions; of these, the most potent and selective inhibitor was orthovanadate. At micromolar concentrations, vanadate inhibited the fraction containing phosphotyrosyl-protein phosphatase and failed to inhibit the fraction containing only phosphoseryl-protein phosphatase activity. These data show that the particulate forms of phosphotyrosyl-protein phosphatase and p-nitrophenyl phosphatase represent the activities of very similar or identical proteins

Scintigraphic study of the lymphatic drainage of the anterior chamber of the mouse eye and its pathophysiological implications

Pendant de nombreuses années, le réseau lymphatique intraoculaire et notamment le drainage de l'humeur aqueuse par ce réseau ont été considérés comme inexistants. Notre étude démontre de fac¸on dynamique et in vivo la présence d'un drainage lymphatique de l'oeil de souris. Cela a été permis grâce à la lymphoscintigraphie après injection de nanomolécules de sulfure de rhénium marquées au technétium 99 m en chambre antérieure de l'oeil de souris. Les acquisitions ont été faites par un tomographe à émission monophotonique expérimental dédié au petit animal. L'hypothèse d'une voie de drainage " uvéolymphatique ", présente dans le corps ciliaire, qui contribuerait à l'écoulement de l'humeur aqueuse a été confortée par les récents progrès de la microbiologie (découverte de marqueurs spécifiques de l'endothélium lymphatique) et de l'imagerie. Cette voie de drainage ouvre des perspectives nouvelles : le développement de techniques de visualisation et de quantification de ce flux lymphatique in viv

A novel nonsense B3GALTL mutation confirms Peters plus syndrome in a patient with multiple malformations and Peters anomaly

Peters plus syndrome is an autosomal recessive rare congenital disorder defined by corneal Peters anomaly with short disproportionate stature, development delay and dysmorphic facial features. In addition, cardiac, genito-urinary and/or central nervous system malformations can be present. Mutations in the beta-1,3-galactosyltransferase-like glycosyltransferase gene (B3GALTL) have been reported in patients with Peters plus syndrome prompting phenotype-genotype studies because of the variable clinical spectrum related to the syndrome. A 20 month old boy presenting with bilateral Peters anomaly in association with multiple developmental anomalies including cerebral malformations was found to carry a novel homozygous B3GALTL nonsense mutation [p.Tyr366X]. This is the first stop mutation described in association with this gene. The present report confirms the wide clinical spectrum of Peters plus syndrome, underlines the major clinical criteria of the syndrome and the major implication of B3GALTL gene in this condition. Ophthalmologic examination in multiple developmental anomalies remains an important clinical issue that may lead to specific gene screening. In Peters plus syndrome B3GALTL molecular test provides diagnosis confirmation and improves dramatically genetic counselling for the families.Genetics of disease, diagnosis and treatmen