Hyperbilirubinemia has been presumed to prevent the process of atherogenesis and cancerogenesis mainly by decreasing oxidative stress.Dubin-Johnson syndrome is a rare,autosomal recessive,inherited disorder characteriz...Hyperbilirubinemia has been presumed to prevent the process of atherogenesis and cancerogenesis mainly by decreasing oxidative stress.Dubin-Johnson syndrome is a rare,autosomal recessive,inherited disorder characterized by biphasic,predominantly conjugatedhyperbilirubinemia with no progression to end-stage liver disease.The molecular basis in Dubin-Johnson syndrome is absence or deficiency of human canalicular multispecific organic anion transporter MRP2/cMOAT caused by homozygous or compound heterozygous mutation(s) in ABCC2 located on chromosome 10q24.Clinical onset of the syndrome is most often seen in the late teens or early adulthood.In this report,we describe a case of previously unrecognized Dubin-Johnson syndrome caused by two novel pathogenic mutations (c.2360_2366delCCCTGTC and c.3258+1G>A),coinciding with cholestatic liver disease in an 82-year-old male patient.The patient,suffering from advanced atherosclerosis with serious involvement of coronary arteries,developed colorectal cancer with nodal metastases.The subsequent findings do not support the protective role of Dubin-Johnson type hyperbilirubinemia.展开更多
This paper presents results of a study on the mechanical properties of sandy and gravely soils within the Cordillera Blanca, Peru. The soils were divided into groups according to their origin(glacial, fluvial, or debr...This paper presents results of a study on the mechanical properties of sandy and gravely soils within the Cordillera Blanca, Peru. The soils were divided into groups according to their origin(glacial, fluvial, or debris flow). The grain size distribution of forty three soil samples was used to classify the soils according to the scheme of the Unified Soil Classification System(USCS). These distributions have then been used to estimate shear strength and hydraulic properties of the soils. There are clear differences between the soils which reflect their divergent origins. The glacial soils normally fit within one of two distinctive groups according to the proportion of fines(Group A, 7%-21.5%; Group B, 21%-65%). The estimation of shear strength at constant volume friction angle and peak shear strength of the glacial sediments with low content of fines was made using published data relating to the measured shear strength characteristics of soils with similar origins and grain size distributions. The estimated values were supported by measurements of the angle of repose taken from fourteen samples from two moraines and by shear tests on samples from one locality. The results of the grain size distribution werealso used to estimate the average hydraulic conductivity using the empirical Hazen formula which results were verified by field infiltration tests at two localities.展开更多
基金Supported by The Project(Ministry of Health,Czech Republic)for Development of Research Organization00023001(IKEM,Prague,Czech Republic)-Institutional supportthe grant SVV-2012-264502
文摘Hyperbilirubinemia has been presumed to prevent the process of atherogenesis and cancerogenesis mainly by decreasing oxidative stress.Dubin-Johnson syndrome is a rare,autosomal recessive,inherited disorder characterized by biphasic,predominantly conjugatedhyperbilirubinemia with no progression to end-stage liver disease.The molecular basis in Dubin-Johnson syndrome is absence or deficiency of human canalicular multispecific organic anion transporter MRP2/cMOAT caused by homozygous or compound heterozygous mutation(s) in ABCC2 located on chromosome 10q24.Clinical onset of the syndrome is most often seen in the late teens or early adulthood.In this report,we describe a case of previously unrecognized Dubin-Johnson syndrome caused by two novel pathogenic mutations (c.2360_2366delCCCTGTC and c.3258+1G>A),coinciding with cholestatic liver disease in an 82-year-old male patient.The patient,suffering from advanced atherosclerosis with serious involvement of coronary arteries,developed colorectal cancer with nodal metastases.The subsequent findings do not support the protective role of Dubin-Johnson type hyperbilirubinemia.
基金Financial support for the contribution was provided by Grant Agency of the Czech Republic (Project No. GACR P209/11/1000)
文摘This paper presents results of a study on the mechanical properties of sandy and gravely soils within the Cordillera Blanca, Peru. The soils were divided into groups according to their origin(glacial, fluvial, or debris flow). The grain size distribution of forty three soil samples was used to classify the soils according to the scheme of the Unified Soil Classification System(USCS). These distributions have then been used to estimate shear strength and hydraulic properties of the soils. There are clear differences between the soils which reflect their divergent origins. The glacial soils normally fit within one of two distinctive groups according to the proportion of fines(Group A, 7%-21.5%; Group B, 21%-65%). The estimation of shear strength at constant volume friction angle and peak shear strength of the glacial sediments with low content of fines was made using published data relating to the measured shear strength characteristics of soils with similar origins and grain size distributions. The estimated values were supported by measurements of the angle of repose taken from fourteen samples from two moraines and by shear tests on samples from one locality. The results of the grain size distribution werealso used to estimate the average hydraulic conductivity using the empirical Hazen formula which results were verified by field infiltration tests at two localities.
