Phenanthroline Derived N-Doped Carbon Dots as Robust Metal-Free Photocatalysts for PET-RAFT Polymerization and Polymerization-Induced Self-Assembly.

Metal-free organic photocatalysts for photo-mediated reversible deactivation radical polymerization (photo-RDRP) are witnessed to make increasing advancement in the precise synthesis of polymers. However, challenges still exist in the development of high-efficiency and environmentally sustainable carbon dots (CDs)-based organocatalysts. Herein, N-doped CDs derived from phenanthroline derivative (Aphen) are prepared as metal-free photocatalysts for photoinduced electron transfer reversible addition-fragmentation chain transfer (PET-RAFT) polymerization. The introduction of phenanthroline structure enhances the excited state lifetime of CDs and expands the conjugated length of their internal structure to enable the light-absorption to reach green light region, thereby enhancing photocatalytic activity. The as-designed CDs exhibit unprecedented photocatalytic capacity in photopolymerization even in large-volume reaction (100 mL) with high monomer conversion and narrow polymer dispersity (Mw/Mn < 1.20) under green light. The photocatalytic system is compatible with PET-RAFT polymerization of numerous monomers and the production of high molecular weight polyacrylate (Mn >250 000) with exquisite spatiotemporal control. Above results confirm the potential of CDs as photocatalyst, which has not been achieved with other CDs catalysts used in photo-RDRP. In addition, the construction of fluorescent polymer nanoparticles using CDs as both photocatalyst and phosphor through photoinitiated polymerization-induced self-assembly (Photo-PISA) technology is successfully demonstrated for the first time.

Two-Dimensional Cu-Porphyrin Metal-Organic Framework Nanosheet-Supported Flaky TiO2 as an Efficient Visible-Light-Driven Photocatalyst for Dye Degradation and Cr(VI) Reduction.

A series of 2D M(Cu, Zn, Co, and Mn)-TCPP MOFs/TiO2 binary nanocomposites (TCPP = tetrakis(4-carboxyphenyl)porphyrin) were constructed by solvothermal in situ loading of flaky TiO2 on the surface of 2D metal-organic frameworks (MOFs). The influence of different coordination metals on the catalytic activity was studied, and it was found that the 2D Cu-TCPP MOFs/TiO2 nanocomposite exhibited the best photo-Fenton performance. The superior property can be attributed to the high absorption coefficient and ultrathin two-dimensional structure of the 2D Cu-TCPP MOFs nanosheets. Meanwhile, the 2D Cu-TCPP MOFs/TiO2 II heterostructure can effectively promote the separation and transfer of photoformed carriers. Moreover, under visible irradiation, the optimized 2D Cu-TCPP MOFs/TiO2 composite can convert 99.9% of Cr(VI) to Cr(III) within 60 min with methanol as the hole scavenger at pH 3.14. Also, the photocatalytic performance of 2D Cu-TCPP MOFs/TiO2 was maintained after five reaction cycles. Furthermore, the proposed visible-light-driven photocatalysis mechanism of the 2D Cu-MOFs/TiO2 composite was reasonably derived according to experimental results. This study demonstrates the potential of building efficient TiO2-based visible light photocatalysts with 2D metal-porphyrin MOFs.

Brahma-Related Gene 1 Inhibition Prevents Liver Fibrosis and Cholangiocarcinoma by Attenuating Progenitor Expansion.

BACKGROUND AND AIMS: Intrahepatic cholangiocarcinoma (iCCA) is closely correlated with hepatic progenitor cell (HPC) expansion and liver fibrosis. Brahma-related gene 1 (Brg1), an enzymatic subunit of the switch/sucrose nonfermentable complex that is critical in stem cell maintenance and tumor promotion, is prominently up-regulated in both HPCs and iCCA; however, its role in this correlation remains undefined. APPROACH AND RESULTS: A retrospective cohort study indicated that high Brg1 expression suggests poor prognosis in patients with iCCA. In chronically injured livers induced by a 0.1% 3,5-diethoxycarbonyl-1,4-dihydrocollidine diet or bile duct ligation surgery, HPCs were dramatically activated, as indicated by their enhanced expression of Brg1 and a subset of stem cell markers; however, Brg1 ablation in HPCs strongly suppressed HPC expansion and liver fibrosis. Furthermore, in a chemically induced iCCA model, inhibition of Brg1 by a specific inhibitor or inducible gene ablation markedly improved histology and suppressed iCCA growth. Mechanistically, in addition to transcriptionally promoting both Wnt receptor genes and target genes, Brg1 was found to bind to the ß-catenin/transcription factor 4 transcription complex, suggesting a possible approach for regulation of Wnt/ß-catenin signaling. CONCLUSIONS: We have demonstrated the function of Brg1 in promoting HPC expansion, liver cirrhosis, and, ultimately, iCCA development in chronically injured livers, which is largely dependent on Wnt/ß-catenin signaling. Our data suggest that therapies targeting Brg1-expressing HPCs are promising for the treatment of liver cirrhosis and iCCA.

Mussel-inspired polydopamine-encapsulated carbon dots with dual emission for detection of 4-nitrophenol and Fe3.

Carbon dots (CDs) with excellent optical properties are widely used in biomedicine, fluorescence sensing, and light-emitting diodes (LEDs). However, it is still a challenge to prepare CDs that can stably emit red fluorescence in the water environment. In this study, polydopamine-encapsulated luminescent carbon dots (CDs@PDA) with an encapsulating structure were synthesized at room temperature from p-phenylenediamine-derived red-light CDs as the core and using mussel-inspired chemical properties of polydopamine (PDA). In the binary system of water:ethanol = 1: 3 (volume ratio), the as-prepared CDs@PDA had a dual emission of ultraviolet light (330 nm) and red light (640 nm) with the fluorescence quantum yields of 8.0 and 15.5%, respectively, at the same time under 285 nm light excitation. The as-prepared CDs@PDA could be directly used for fluorescence selective sensing of 4-nitrophenol (4-NP) and Fe3+ through simultaneously quenching of ultraviolet and red fluorescence based on the internal filtration effect mechanism with detection limits of 3.44 and 3.75 µM, respectively. This research showed that PDA-coated CDs can significantly improve the photoluminescence stability of CDs with new optical features. This means that the encapsulated structure of mussel chemistry is very helpful for expanding the application range of CDs in the water environment.

Enhanced SMARCD1, a subunit of the SWI/SNF complex, promotes liver cancer growth through the mTOR pathway.

The chromatin remodeling complex SWI/SNF regulates the accessibility of target genes to transcription factors and plays a critical role in the tumorigenesis of hepatocellular carcinoma (HCC). The SWI/SNF complex is assembled from approximately 15 subunits, and most of these subunits have distinct roles and are often aberrantly expressed in HCC. A comprehensive exploration of the expression and clinical significance of these subunits would be of great value. In the present study, we obtained the gene expression profile of each SWI/SNF subunit and the corresponding clinical information from The Cancer Genome Atlas (TCGA). We found that 14 out of the 15 SWI/SNF subunits were significantly increased in HCC tissues compared with paired normal liver tissues, and 11 subunits were significantly associated with overall survival (OS). We identified a four-gene prognostic signature including actin-like 6A (ACTL6A), AT-rich interaction domain 1A (ARID1A), SWI/SNF related, matrix associated, actin dependent regulator of chromatin subfamily C member 1 (SMARCC1) and SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily D, member 1 (SMARCD1) that could effectively predict OS in HCC patients. Among the genes, SMARCD1 has the most prognostic value. We further conducted in vitro and in vivo experiments and revealed that SMARCD1 promotes liver cancer growth by activating the mTOR signaling pathway. In conclusion, our study has revealed that the expression of SWI/SNF complex subunits, especially SMARCD1, is highly associated with HCC development and acts as a promising prognostic predictor.

A facile synthesis of multifunctional carbon dots as fluorescence 'turn on' and 'turn off' probes for selective detection of Al3+ and 2,4,6-trinitrophenol.

Carbon dots (CDs) have drawn increasing interests due to their unique optical properties and promising application in various fields. In this study, citric acid (CA) and 5-chloromethyl-8-hydroxyquinoline (LQ) were used to synthesize nitrogen-doped CDs as novel fluorescent probes using a one-step solvothermal route. The as-prepared CDs had strong blue-white fluorescence emission when excited at 405 nm wavelength with a quantum yield (QY) of 25%, behaving with high ion concentration stability. Water-soluble CDs with a 8-hydroxyquinoline structure on their surface could be used to detect Al3+ using a 'turn on' mechanism and trinitrophenol (TNP) using a 'turn off' mechanism with detection limits of 229 nM and 44.4 nM, respectively. Al3+ enhances the fluorescence of CDs by forming a coordination complex to generate a fluorescence synergistic role and limit CD nonradiative transition. TNP quenched the fluorescence with high selectivity and sensitivity, which was attributed to the inner filter effect and static quenching. These results indicated that these CDs with their unique 'turn on' and 'turn off' nature have potential application in the environmental protection field and in prevention of terrorist threats.

The prognostic value of microvascular invasion in early-intermediate stage hepatocelluar carcinoma: a propensity score matching analysis.

BACKGROUND: Microvascular invasion (MVI) is well established as a negative prognostic factor for hepatocelluar carcinoma (HCC). However, its prognostic value in different subgroups of Barcelona Clinical Liver Cancer (BCLC) stages remains to be elucidated. METHODS: Four hundred fifty-eight MVI-negative and 204 MVI-positive patients who underwent hepatectomy were retrospectively analyzed. After propensity score matching (PSM) analysis, 187 pairs of matched patients were generated. Long-term survival was compared by the Kaplan-Meier method. RESULTS: Patients with MVI commonly had more advanced tumors. All the patients with MVI had significantly worse survival rate compared to the patients without MVI before and after PSM(p < 0.001). In the subgroup analysis, BCLC stage A HCC patients without MVI had better prognosis than those with MVI before and after PSM (p < 0.001 and p = 0.024). For BCLC stage B HCCs, long-term survival was significantly better for patients without MVI before PSM(p = 0.001). However, the overall survival (OS) rate was comparable between both groups after PSM (p = 0.682), although MVI-positive group had a higher rate of recurrence (p = 0.011).. Surgery type, satellite lesions, tumor size, and serum ALT level were statistically significant factors associated with survival in MVI-positive group. Tumor number, tumor size and neutrophil to lymphocyte ratio (NLR) were predictors of survival in MVI-negative group. CONCLUSIONS: Its prognostic value in different subgroups of BCLC stages differed. MVI is an independent predictor of prognosis in patients with BCLC stage A. For BCLC stage B HCCs, MVI-positive group had poor prognosis through more advanced HCCs.

Thermoresponsive Amphiphilic Block Copolymer-Stablilized Gold Nanoparticles: Synthesis and High Catalytic Properties.

A series of novel well-defined 8-hydroxyquinoline (HQ)-containing thermoresponsive amphiphilic diblock copolymers {poly(styrene- co-5-(2-methacryloylethyloxy-methyl)-8-quinolinol)- b-poly( N-isopropylacrylamide) P(St- co-MQ)- b-PNIPAm (P1,2), P(NIPAm- co-MQ)- b-PSt (P3,4)} and triblock copolymer poly( N-isopropylacrylamide)- b-poly(methyl-methacrylate- co-5-(2-methacryloylethyloxymethyl)-8-quinolinol)- b-polystyrene PNIPAm- b-P(MMA- co-MQ)- b-PSt (P5) were prepared by reversible addition-fragmentation chain-transfer (RAFT) polymerization, and their self-assembly behaviors were studied. Block copolymer P1-P5-stabilized gold nanoparticles (Au@P1-Au@P5) with a small size and a narrow distribution were obtained through the in situ reduction of gold precursors in an aqueous solution of polymer micelles with HQ as the coordination groups. The resulting Au@P nanohybrids possessed excellent catalytic activity for the reduction of nitrophenols using NaBH4. The size, morphology, and surface chemistry of Au NPs could be controlled by adjusting the structure of block polymers with HQ in different block positions, which plays an important role in the catalytic properties. It was found that longer chain lengths of hydrophilic or hydrophobic segments of block copolymers were beneficial to elevating the catalytic activity of Au NPs for the reduction of nitrophenols, and the spherical nanoparticles (Au@P5) stabilized with triblock copolymers exhibit higher catalytic performance. Surprisingly, the gold nanowires (Au@P4) produced with P4 have the highest catalytic activity due to a large abundance of grain boundaries. Excellent thermoresponsive behavior for catalytic reaction makes the as-prepared Au@P hybrids an environmentally responsive nanocatalytic material.

Loss of Gsα impairs liver regeneration through a defect in the crosstalk between cAMP and growth factor signaling.

BACKGROUND & AIMS: The stimulatory G protein α subunit (Gsα) activates the cAMP-dependent pathway by stimulating the production of cAMP and participates in diverse cell processes. Aberrant expression of Gsα results in various pathophysiological disorders, including tumorigenesis, but little is known about its role in liver regeneration. METHODS: We generated a hepatocyte-specific Gsα gene knockout mouse to demonstrate the essential role of Gsα in liver regeneration using a mouse model with 70% partial hepatectomy (PH) or an intraperitoneal injection of carbon tetrachloride (CCl4). RESULTS: Gsα inactivation dramatically impaired liver regeneration and blocked proliferating hepatocytes in G1/S transition due to the simultaneous depression of cyclin-dependent kinase 2 (CDK2) and cyclin E1. Loss of Gsα led to a fundamental alteration in gene profiles. Among the altered signaling cascades, the MAPK/Erk pathway, which is downstream of growth factor signaling, was disrupted secondary to a defect in phosphorylated Raf1 (pRaf1), resulting in a deficiency in phosphorylated CREB (pCREB) and CDK2 ablation. The lack of pRaf1 also resulted in a failure to phosphorylate retinoblastoma, which releases and activates E2F1, and a decrease in cyclin E1. Although these factors could be phosphorylated through both Gsα and growth factor signaling, the unique function of Raf1 in the growth factor cascade collapsed in response to the lack of Gsα. CONCLUSION: The growth factor signaling pathway that promotes hepatocyte proliferation is dependent on Gsα signaling. Loss of Gsα leads to a breakdown of the crosstalk between cAMP and growth factor signaling and dramatically impairs liver regeneration.

Stiffness Value and Serum Biomarkers in Liver Fibrosis Staging: Study in Large Surgical Specimens in Patients with Chronic Hepatitis B.

Purpose To investigate the capabilities of stiffness value and serum biomarkers in the staging of liver fibrosis in patients with chronic hepatitis B (CHB), with pathologic findings in large surgical specimens serving as the reference standard. Materials and Methods This study was approved by the institutional review board, and informed consent was obtained from all patients. Liver stiffness (determined by means of ultrasonography-based elastography point quantification), aspartate aminotransferase-platelet ratio index (APRI), and fibrosis index (based on the four-factor Fibrosis-4 [FIB-4] calculation) were obtained in 386 patients with CHB. With pathologic fibrosis stages in large surgical specimens as the reference standard, capabilities and cutoffs of stiffness and serum biomarkers were first investigated in a cohort of 284 patients and then validated in an independent cohort of 102 patients by means of area under the receiver operating characteristic curve (AUC) analysis. Results Liver stiffness demonstrated significantly stronger correlation with fibrosis stages than did APRI and FIB-4 (r = 0.738 vs r = 0.477 vs r = 0.427, respectively; P < .05 for all). In the development cohort, liver stiffness had significantly higher AUCs in identifying fibrosis of stage 1 or higher, stage 2 or higher, stage 3 or higher, and stage 4 or higher (0.97, 0.96, 0.91, and 0.87, respectively) than APRI (0.89, 0.84, 0.73, and 0.74, respectively) and FIB-4 (0.82, 0.79, 0.70, and 0.72, respectively). In the validation cohort, liver stiffness was validated as showing significantly higher AUCs in identifying fibrosis of stage 1 or higher, stage 2 or higher, stage 3 or higher, and stage 4 or higher (0.99, 0.95, 0.89, and 0.88, respectively) than APRI (0.83, 0.76, 0.78, and 0.68, respectively) and FIB-4 (0.76, 0.69, 0.75, and 0.67, respectively). Conclusion Liver stiffness demonstrated considerable capability in identifying each stage of liver fibrosis in patients with CHB, whereas serum biomarkers showed limited capabilities. (©) RSNA, 2016 Online supplemental material is available for this article.

Octreotide prevents liver failure through upregulating 5'-methylthioadenosine in extended hepatectomized rats.

BACKGROUND & AIMS: Insufficient liver regeneration and hepatocyte injury caused by excessive portal perfusion are considered to be responsible for post-hepatectomy liver failure (PLF) or small-for-size syndrome in living-donor liver transplantation. Somatostatin can decrease portal vein pressure (PVP) but simultaneously inhibits liver regeneration. This interesting paradox motivated us to investigate the outcome of PLF in response to somatostatin treatment. METHODS: Rats receiving extended partial hepatectomy (90% PH) were treated with octreotide, a somatostatin analogue, or placebo. Animal survival, serum parameters and hepatic histology were evaluated. Metabolomic analysis was performed to investigate the effect of octreotide on hepatocyte metabolism. RESULTS: Despite significantly inhibiting early regeneration, octreotide application noticeably improved the hepatic histology, liver function and survival after PH but did not decrease the PVP level. Metabolomic analysis exhibited that octreotide profoundly and exclusively altered the levels of five metabolites that participate in or closely associate with the methionine cycle, a biochemical reaction that uniquely produces S-adenosylmethionine (SAMe), an active methyl residual donor for methyltransferase reactions. Among these metabolites, 5'-methylthioadenosine (MTA), a derivate of SAMe, increased three-fold and was found independently improve the hepatic histology and reduce inflammatory cytokines in hepatectomized rats. CONCLUSIONS: Octreotide exclusively regulates the methionine cycle reaction and augments the MTA level in hepatocytes. MTA prominently protects hepatocytes against shear stress injury and reduces the secondary inflammation, thereby protecting rats from PLF.

Hepatocellular carcinoma: stiffness value and ratio to discriminate malignant from benign focal liver lesions.

PURPOSE: To investigate the use of stiffness value and stiffness ratio (ratio of lesion to background liver parenchyma values) to discriminate malignant from benign focal liver lesions by using histologic results as the reference standard. MATERIALS AND METHODS: This study was approved by the institutional review board, and written informed consent was obtained. Three hundred seventy-three patients with focal liver lesions proven at histologic examination underwent measurement of liver stiffness with elastography point quantification. First, stiffness values in two regions of the background liver parenchyma (at 0.5-2 cm and >2 cm from the lesion periphery) near 163 hepatocellular carcinomas were analyzed to determine a reference background liver for calculating the stiffness ratio. Second, the use of the lesion stiffness value and the stiffness ratio for prediction of liver malignancy was investigated in a cohort of patients with 58 benign and 201 malignant lesions. Results were validated in another independent cohort of patients with 25 benign and 89 malignant lesions by using analysis of the area under the receiver operating characteristic (AUC) curve. RESULTS: The coefficient of variation for the background liver at 0.5-2 cm from the lesion was higher (196%) than that at greater than 2 cm from the lesion (66%). In the development phase, diagnostic accuracy with use of the stiffness value was significantly higher than that with use of the stiffness ratio for discrimination of malignant from benign lesions (AUC, 0.86 vs 0.66, respectively; P < .001). Diagnostic performance with the stiffness value was lower than that with the stiffness ratio (AUC, 0.53 vs 0.86, respectively; P < .001) for discrimination of cirrhotic nodules from other benign lesions. Diagnostic performance with the stiffness value was significantly lower than that with the stiffness ratio (AUC, 0.58 vs 0.71 respectively; P = .007) for discrimination of metastasis from primary liver cancers. In the validation phase, similar findings were revealed for the discrimination of malignant from benign lesions (AUC, 0.87 vs 0.67; P < .001) and discrimination between metastasis and primary liver cancers (AUC, 0.49 vs 0.73; P < .001). CONCLUSION: Use of stiffness values measured in the liver parenchyma at more than 2 cm from the lesion allowed better diagnostic performance than did values measured in a region closer to the tumor. Stiffness value was more accurate than stiffness ratio for differentiation of malignant from benign focal liver lesions, but the stiffness ratio might be useful for subclassification of benign and malignant lesions. Online supplemental material is available for this article.

Water-soluble polymer functionalized CdTe/ZnS quantum dots: a facile ratiometric fluorescent probe for sensitive and selective detection of nitroaromatic explosives.

A ratiometric fluorescent probe based on dual luminescence QD/CPL for selective sensing of the nitroaromatic explosive picric acid (PA) was constructed. The observed ratiometric fluorescence intensity change allows the quantitative detection of PA with a detection limit of 9 nM.

Childhood cystic teratoma of the pancreas: clinical presentation, evaluation and management.

Pancreatic cystic teratoma in children is extremely rare. Here we present a female infant with a mature cystic teratoma of the pancreatic body. The patient was admitted for a palpable abdominal mass and anorexia. Computed tomography (CT) indicated a huge cystic mass in the abdominal cavity. Exploratory laparotomy was performed, and the tumor was excised completely. Pathology confirmed the tumor was a mature cystic teratoma of pancreatic origin. Two months after the initial surgery, a pseudocyst was detected and then cystojejunostomy was performed. Fourteen months after the second surgery, tumor relapse occurred and distal pancreatectomy was performed. The patient remained well without tumor recurrence during the next 24 months of follow-up. Our experience suggests that clinical manifestations of children with pancreatic cystic teratomas might be nonspecific and preoperative diagnosis is difficult. Radical tumor resection and maximized preservation of healthy pancreatic tissue should always be balanced in surgical treatment.

A facile strategy to fabricate thermoresponsive polymer functionalized CdTe/ZnS quantum dots: assemblies and optical properties.

Novel thermoresponsive CdTe/ZnS quantum dots (QDs) decorated with a copolymer ligand (CPL) containing 8-hydroxyquinoline and NIPAM units are prepared through coordinate bonding in aqueous solution. The dependence of the morphology and optical properties of the QDs/CPL assemblies formed via coordinate bonding on the experimental conditions is studied. The coordinate induced self-assemblies are observed by controlling the molar ratio of QDs and CPL. The self-organized structure of QDs/CPL proceeds through a first step of QDs-chains, followed by a necklace-like single annular chain, and subsequently increases its annular chain structure, forming a network. The CPL functionalized QDs can emit multiple colors from the cooperating interaction between the inherent emission (606 nm) of the QDs and the surface-coordinated emission (517 nm) of the CPL complex formed on the QD surface. For QDs-CPL systems, both Förster resonance energy transfer (FRET) and a high rate of photoinduced electron transfer (PET) are simultaneous, the latter mainly contributing to PL quenching. The thermoresponsive QDs/CPL assemblies also exhibit dual reversible PL properties between the inherent emission of QDs and surface-coordinated emission.

The autopsy study of 553 cases of sudden cardiac death in Chinese adults.

Despite a recent epidemiological study reporting a lower incidence of sudden cardiac death (SCD) in China as compared with that in Western countries, the exact causes of SCD are still unknown. Using a uniform review protocol and diagnostic criteria, a retrospective autopsy study identified 553 cases of SCD in 14,487 consecutive autopsies from eight regions in China representing different geographic and population features. Their ages ranged from 18 to 80 years (median 43.0 years) with a ratio of 4.3/1.0 for male/female. Out-of-hospital deaths and unwitnessed cases accounted for 74.3 and 22.6 %, respectively. The main causes of death were coronary atherosclerotic disease (CAD 50.3 %), myocarditis (14.8 %), and hypertrophic cardiomyopathy (4.5 %), with unexplained sudden death accounting for 12.1 % of the cases. CAD had a proportion of 10.4 % in victims <35 years, lower as compared with 59.0 and 83.0 % in victims aged 35-54 and in victims ≥55 years. On the other hand, myocarditis and unexplained sudden death were major causes and accounted for 34.7 and 22.5 % in victims <35 years. In order to differentiate the degree of the cause-effect relationship between autopsy findings and sudden death, a grading method was used in this series and characterized 24.3 % of findings as certain, 52.9 % as highly probable, and 22.8 % as uncertain. Our data indicated that there most likely are less CAD but more myocarditis and unexplained sudden death in Chinese youth with SCD than in populations from Western countries. Molecular genetic testing should be conducted in those cases with uncertain findings and unexplained sudden death in routine autopsy.

A case study of a liver transplant-treated patient with glycogen storage disease type Ia presenting with multiple inflammatory hepatic adenomas: an analysis of clinicopathologic and genetic data.

BACKGROUND: Glycogen storage disease (GSD) is a disease caused by excessive deposition of glycogen in tissues due to genetic disorders in glycogen metabolism. Glycogen storage disease type I (GSD-I) is also known as VonGeirk disease and glucose-6-phosphatase deficiency. This disease is inherited in an autosomal recessive manner, and both sexes can be affected. The main symptoms include hypoglycaemia, hepatomegaly, acidosis, hyperlipidaemia, hyperuricaemia, hyperlactataemia, coagulopathy and developmental delay. CASE PRESENTATION: Here, we present the case of a 13-year-old female patient with GSD Ia complicated with multiple inflammatory hepatic adenomas. She presented to the hospital with hepatomegaly, hypoglycaemia, and epistaxis. By clinical manifestations and imaging and laboratory examinations, we suspected that the patient suffered from GSD I. Finally, the diagnosis was confirmed by liver pathology and whole-exome sequencing (WES). WES revealed a synonymous mutation, c.648 G > T (p.L216 = , NM_000151.4), in exon 5 and a frameshift mutation, c.262delG (p.Val88Phefs*14, NM_000151.4), in exon 2 of the G6PC gene. According to the pedigree analysis results of first-generation sequencing, heterozygous mutations of c.648 G > T and c.262delG were obtained from the patient's father and mother. Liver pathology revealed that the solid nodules were hepatocellular hyperplastic lesions, and immunohistochemical (IHC) results revealed positive expression of CD34 (incomplete vascularization), liver fatty acid binding protein (L-FABP) and C-reactive protein (CRP) in nodule hepatocytes and negative expression of ß-catenin and glutamine synthetase (GS). These findings suggest multiple inflammatory hepatocellular adenomas. PAS-stained peripheral hepatocytes that were mostly digested by PAS-D were strongly positive. This patient was finally diagnosed with GSD-Ia complicated with multiple inflammatory hepatic adenomas, briefly treated with nutritional therapy after diagnosis and then underwent living-donor liver allotransplantation. After 14 months of follow-up, the patient recovered well, liver function and blood glucose levels remained normal, and no complications occurred. CONCLUSION: The patient was diagnosed with GSD-Ia combined with multiple inflammatory hepatic adenomas and received liver transplant treatment. For childhood patients who present with hepatomegaly, growth retardation, and laboratory test abnormalities, including hypoglycaemia, hyperuricaemia, and hyperlipidaemia, a diagnosis of GSD should be considered. Gene sequencing and liver pathology play important roles in the diagnosis and typing of GSD.

Integrating Hybrid Perovskite Nanocrystals into Metal-Organic Framework as Efficient S-Scheme Heterojunction Photocatalyst for Synergistically Boosting Controlled Radical Photopolymerization under 980 nm NIR Light.

S-scheme heterojunction photocatalyst MAPbI3@PCN-222 with light absorption extending to the NIR region is constructed by embedding organic-inorganic hybrid perovskite (MAPbI3) into porphyrinic Zr-MOF (PCN-222). Both in situ X-ray photoelectron spectroscopy, ultraviolet photoelectron spectral characterization, and photocatalytic polymerization experiment prove the formation of S-scheme heterojunction. MAPbI3@PCN-222 with a low dosage (90 ppm) displays an impressive photocatalytic ability for 980 nm light-mediated photoinduced electron/energy-transfer-reversible addition-fragmentation chain-transfer (PET-RAFT) polymerization in air. The well-defined controllable-molecular weight polymers including block copolymers and ultrahigh-molecular weight polymers can be achieved with narrow distributions (Mw/Mn < 1.20) via rapid photopolymerization. The industrial application potential of the photocatalyst also has been proved by scale-up synthesis of polymers with low polydispersity under NIR light-induced photopolymerization in a large-volume reaction system (200 mL) with high monomer conversion up to 99%. The penetration photopolymerization through the 5 mm polytetrafluoroethylene plate and excellent photocontrollable behavior illustrate the existence of long-term photogenerated electron transfer of heterojunction and abundant free radicals in photopolymerization. The photocatalyst still retains high catalytic activity after 10 cycles of photopolymerization in air. It is revealed for the first time that the special PET-RAFT polymerization pathway is initiated by the aldehyde-bearing α-aminoalkyl radical derived from the oxidization of triethanolamine (TEOA) by the heterojunction photocatalyst. This research offers a new insight into understanding the NIR-light-activated PET-RAFT polymerization mechanism in the presence of TEOA.

Clinical features, natural history and outcomes of pseudolymphoma of liver: A case-series and systematic review.

BACKGROUND: Pseudolymphoma is a rare, benign, nonspecific condition that forms a mass-like lesion characterized by the proliferation of non-neoplastic lymphocytes. Lacking of specific clinical symptoms, serological markers, and imaging features, the diagnosis is difficult. We reporte five cases of hepatic pseudolymphoma and provide a systematic review of existing literatures to improve our understanding of this rare liver disease. METHODS: We followed-up five cases of hepatic pseudolymphoma in West China Hospital from January 2002 to January 2022. We also summarized the cases of hepatic pseudolymphoma from January 1981 to December 2021 through the PubMed database and comprehensively analyzed the characteristics of the cases. RESULTS: The pathologic features of the five cases were characterized by benign lymphoid tissue hyperplasia, lymphoid follicle formation, and a polarized germinal center. Immunohistochemistry, in situ hybridization, and gene rearrangement revealed non-malignant lymphoma. Besides, a total of 116 cases have been reported in the PubMed database from 1981 to 2021. The incidence of hepatic pseudolymphoma is higher in middle-aged and elderly women and has been reported more frequently in Asia. All cases were pathologically diagnosed, among which 85.95% of the patients were treated by surgery. CONCLUSIONS: Hepatic pseudolymphoma is an extremely rare benign disease, mainly in middle-aged and elderly women. Without distinctive clinical and imaging characteristics, pathological diagnosis is the highly reliable method at present. Thus, in the absence of risk factors for a primary liver tumor or metastatic tumor in middle-aged and elderly women, the possibility of pseudolymphoma should be considered to avoid extensive treatments.

Expert Consensus on Pathological Diagnosis of Intrahepatic Cholangiocarcinoma (2022 version).

Intrahepatic cholangiocarcinoma (iCCA) can originate from the large bile duct group (segment bile ducts and area bile ducts), small bile duct group (septal bile ducts and interlobular bile ducts), and terminal bile duct group (bile ductules and canals of Hering) of the intrahepatic biliary tree, which can be histopathological corresponding to large duct type iCCA, small duct type iCCA and iCCA with ductal plate malformation pattern, and cholangiolocarcinoma, respectively. The challenge in pathological diagnosis of above subtypes of iCCA falls in the distinction of cellular morphologies, tissue structures, growth patterns, invasive behaviors, immunophenotypes, molecular mutations, and surgical prognoses. For these reasons, this expert consensus provides nine recommendations as a reference for standardizing and refining the diagnosis of pathological subtypes of iCCA, mainly based on the 5th edition of the World Health Organization Classification of Tumours of the Digestive System.