Combined devascularization and splenorenal shunt (PCDV + PSRS) significantly decreases portal venous flow and portal pressure, as well as maintaining hepatopedal flow, thus entailing fewer complications compared to either PCDV or PSRS. We aimed to determine the rationality of pericardial devascularization (PCDV) plus proximal splenorenal shunt (PSRS) for cirrhotic patients with portal hypertension with variceal bleeding, using a duplex sonography study of the effects of the different surgical procedures (PCDV, PSRS, and PCDV + PSRS) on the hemodynamics of the portal system.

Marfan syndrome is a common autosomal dominant hereditary connective tissue disorder. There is no cure for Marfan syndrome currently. Next-generation sequencing (NGS) technology is efficient to identify genetic lesions at the exome level. Here we carried out exome sequencing of two Marfan syndrome patients. Further Sanger sequencing validation in other five members from the same family was also implemented to confirm new variants which may contribute to the pathogenesis of the disease. Two new variants, including one nonsense SNP in the Marfan syndrome gene FBN1 and one missense mutation in exon 15 of LRP1, which may be related to the phenotype of the patients were identified. The exome sequencing analysis provides us a new insight into the molecular events governing pathogenesis of Marfan syndrome.Virtual slidehttp://www.diagnosticpathology.diagnomx.eu/vs/1229110069114125.

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