A Rare Case of Benign Recurrent Intrahepatic Cholestasis Initially Diagnosed in Middle-age.

Background: Repeated episodes of jaundice and pruritus are common in a group of autosomal recessive liver diseases known as benign recurrent intrahepatic cholestasis. Benign recurrent intrahepatic cholestasis (BRIC) is divided into two types, type 1 and type 2, and is caused by mutations in the ATP8B1 and ABCB11 genes. Here, we report a rare case of BRIC type 2 mutation. Case presentation: A 45-year-old Chinese man had three frequent episodes of jaundice marked by extensive excoriation and severe pruritis, although he had no prior history of jaundice. Laboratory investigations showed no evidence of liver damage caused by viral, autoimmune, or acquired metabolic etiologies. The CT scan revealed an enlarged gallbladder with numerous punctate high-density shadows, while no wall thickening was observed. Endoscopic ultrasonography showed no evidence of dilation of the intrahepatic and extrahepatic bile duct, as well as the absence of gallstone. Diagnostic evaluation: Immunohistochemical examinations of liver biopsy samples showed cytokeratin-7 positive hepatocytes, suggesting chronic intrahepatic cholestasis. The reticulin fiberstaining demonstrated that the portions of the hepatic plate in the center of the lobule were asymmetrically organized,and somewhat enlarged, with collapsed areas indicating intralobular inflammation. Moreover, there were areas of collapse that indicated the presence of intralobular inflammation. Whole exome sequencing revealed mutations in the ABCB11 gene; c.3084A>G, p.A1028A homozygous mutation (chr2-169789016), and c.2594C>T, p.A865V heterozygous mutation (chr2-169801131). Based on these findings, the final diagnosis of the patient was metabolism-related jaundice. Treatment: Apart from receiving tapering dosage of prednisone to lower bilirubin levels, the patient received no extra care. Conclusion: The comprehensive diagnosis of a middle-aged male patient with BRIC-2, which involved extensive radiological, hematological, and genetic investigations, informed a tailored tapering prednisone regimen, highlighting the importance of personalized medicine in managing atypical presentations of this rare cholestatic disorder.

Human activities have opposing effects on distributions of narrow-ranged and widespread plant species in China.

Human activities have shaped large-scale distributions of many species, driving both range contractions and expansions. Species differ naturally in range size, with small-range species concentrated in particular geographic areas and potentially deviating ecologically from widespread species. Hence, species' responses to human activities may be influenced by their geographic range sizes, but if and how this happens are poorly understood. Here, we use a comprehensive distribution database and species distribution modeling to examine if and how human activities have affected the extent to which 9,701 vascular plants fill their climatic potential ranges in China. We find that narrow-ranged species have lower range filling and widespread species have higher range filling in the human-dominated southeastern part of China, compared with their counterparts distributed in the less human-influenced northwestern part. Variations in range filling across species and space are strongly associated with indicators of human activities (human population density, human footprint, and proportion of cropland) even after controlling for alternative drivers. Importantly, narrow-ranged and widespread species show negative and positive range-filling relationships to these human indicators, respectively. Our results illustrate that floras risk biotic homogenization as a consequence of anthropogenic activities, with narrow-ranged species becoming replaced by widespread species. Because narrow-ranged species are more numerous than widespread species in nature, negative impacts of human activities will be prevalent. Our findings highlight the importance of establishing more protected areas and zones of reduced human activities to safeguard the rich flora of China.

GIS assessment of the risk of gene flow from Brassica napus to its wild relatives in China.

Risk of gene flow from canola (Brassica napus) to species of wild relatives was used as an example to evaluate the risk of gene flow of transgenic crops. B. juncea and B. rapa were the most common weedy Brassica species in China, which were both sexually compatible with canola. Data on canola cultivation in China were collected and analyzed using geographic information system (GIS), and the distribution of its wild relatives was predicted by MaxEnt species distribution model. Based on biological and phenological evidence, our results showed that gene flow risk exists in most parts of the country, especially in places with higher richness of wild Brassica species. However, risk in dominant canola cultivation regions is relatively low owing to the reduced distribution density of wild species in these regions. Three regions of higher risk of gene flow had been identified. Risk of gene flow is relatively high in certain areas. China has been assumed to be the original center of B. juncea and B. rapa, and gene flow may lead to negative effects on the conservation of biodiversity of local species. Strategies had been proposed to reduce the possibility of gene flow either by monitoring introgression from crops to wild relatives in the areas of high adoption of the crop or by taking measures to limit the releasing of new crops or varieties in the areas with abundant wild relatives.

[Relationship between mRNA expression of MnSOD and manganese neurotoxicity].

OBJECTIVE: To investigate the relationship between mRNA expression of manganese superoxide dismutase (MnSOD) and manganese neurotoxicity. METHODS: Thirty-one patients with occupational chronic manganese poisoning (case group), as well as 31 controls exposed to the same condition (control group), were included in the study. Whole blood RNA was extracted, and the mRNA expression of MnSOD was measured by RT-PCR; the two groups were compared in terms of the mRNA expression of MnSOD. PC12 cells were treated with 0, 100, 200, 400, 600, 800, and 1000 ümol/L MnCl2 for l, 2, 3, and 4 d; the cell viability was determined by MTT assay, and the mRNA expression of MnSOD was measured by RT-PCR. RESULTS: The case group had significantly lower mRNA expression of MnSOD than the control group (0.390 ± 0.080 vs 0.582 ± 0.219, P < 0.05). MnCl2 had a toxic effect on PC12 cells; the concentration of MnCl2 was positively correlated with the toxic effect but negatively correlated with the mRNA expression of MnSOD. CONCLUSION: MnSOD mRNA may be involved in the manganese-induced damage of nerve cells. It is hypothesized that high mRNA expression of MnSOD may play an inhibitory effect on manganese neurotoxicity.

Tunable hydrogen enhancement of Ce3+ doped CdS with different Poisson's ratio support.

In this article, a 3D photocatalytic support with different Poisson's ratio was used for the first time to control the photocatalytic production rate of hydrogen. It was created by a stereo-lithography method, and the support with the most negative Poisson's ratio got the best result. The Poisson's ratio of the 3D structure influences the rate of hydrogen production, and it is important for the photocatalyst supports to be porous for light to penetrate into them. A series of Ce doped CdS photocatalysts were produced and immobilized on 3D multicellular Al2O3 supports. By changing the proportion of Ce3+ doped into the CdS photocatalysts 1 % of Ce3+ exhibited optimal hydrogen production, which was 222.9 % compared to that of the pure CdS. Using the 3D photocatalytic support with different Poisson's ratio, the photocatalytic production rate of hydrogen increased by 128 %.

Long Non-coding RNA Signature for Liver Metastasis of Colorectal Cancers.

Colorectal cancer ranks within the top three cancers both in terms of incidence as well as deaths. Metastasis is often the major cause of mortality and liver is the primary and most common site to which colorectal cancers metastasize. We tested the prognostic ability of a long non-coding RNA (lncRNA) signature in liver metastatic colorectal cancers. We first evaluated expression levels of several lncRNAs in eight excised liver metastases from primary colorectal cancers and found significantly upregulated lncRNAs HOTAIR and MALAT1 along with significantly downregulated LOC285194. We further compared the expression levels of HOTAIR, MALAT1 and LOC285194 in primary colorectal tumors at the time of initial diagnosis and correlated them with disease progression and liver metastasis. HOTAIR and MALAT1 were significantly upregulated and LOC285194 was significantly downregulated in twelve patients who were diagnosed with liver metastasis within 5 years of initial diagnosis, compared to the five patients with no metastasis. A positive signature comprising of high HOTAIR/MALAT1 and low LOC285194 also correlated with progression to higher grade tumors. Thus, the lncRNA signature comprising of high HOTAIR/MALAT1 and low LOC285194 could be a prognostic signature for liver metastasis as well as overall poor survival.

Using species distribution modeling to delineate the botanical richness patterns and phytogeographical regions of China.

The millions of plant specimens that have been collected and stored in Chinese herbaria over the past ~110 years have recently been digitized and geo-referenced. Here we use this unique collection data set for species distribution modeling exercise aiming at mapping &explaining the botanical richness; delineating China's phytogeographical regions and investigating the environmental drivers of the dissimilarity patterns. We modeled distributions of 6,828 woody plants using MaxEnt and remove the collection bias using null model. The continental China was divided into different phytogeographical regions based on the dissimilarity patterns. An ordination and Getis-Ord Gi* hotspot spatial statistics were used to analysis the environmental drivers of the dissimilarity patterns. We found that the annual precipitation and temperature stability were responsible for observed species diversity. The mechanisms causing dissimilarity pattern seems differ among biogeographical regions. The identified environmental drivers of the dissimilarity patterns for southeast, southwest, northwest and northeast are annual precipitation, topographic &temperature stability, water deficit and temperature instability, respectively. For effective conservation of China's plant diversity, identifying the historical refuge and protection of high diversity areas in each of the identified floristic regions and their subdivisions will be essential.