Carcinosarcoma ; chemistry ; pathology ; Female ; Humans ; Liver Neoplasms ; chemistry ; pathology ; Middle Aged

Animals ; Isoflavones ; pharmacology ; Liver ; metabolism ; pathology ; Liver Diseases ; metabolism ; pathology ; Rats ; Rats, Wistar ; Reperfusion Injury ; pathology ; prevention & control ; Soybeans ; chemistry

Objective: To investigate the clinicopathological and molecular characteristics of hepatic fibrinogen storage disease (FSD) in children. Methods: The clinical, histopathologic, immunophenotypic, ultrastructural and gene sequencing data of 4 FSD cases were collected from September 2019 to January 2021 in the Children's Hospital of Fudan University, Shanghai, China. Retrospective analysis and literature review were conducted. Results: There were 4 cases of FSD, 3 males and 1 female, aged 3 years and 3 months to 6 years (median age, 3 years and 4 months). The clinical manifestations were abnormal liver function and abnormal blood coagulation function, for which 2 cases had family genetic history. Liver biopsies revealed that, besides liver steatosis, fibrosis and inflammation, there were single or multiple eosinophilic inclusion bodies of various sizes and surrounding transparent pale halo in hepatocytes. Immunohistochemistry showed that the inclusion bodies were positive for anti-fibrinogen. Under the electron microscope, they corresponded to the dilated cisternae of the rough endoplasmic reticulum, which were occupied by compactly packed tubular structures and arranged into a fingerprint-like pattern with curved bundles. Gene sequencing revealed that the 2 cases of FGG mutation were located in exon 8 c.1106A>G (p.His369Arg) and c.905T>C (p.Leu302Pro), and 1 case was located in exon 9 c.1201C>T (p.Arg401Trp). No pathogenic variant was detected in the other case. Conclusions: FSD is a rare genetic metabolic disease and clinically manifests as abnormal liver function with hypofibrinogenemia. In the background of liver steatosis, fibrosis and inflammation, there are eosinophilic inclusions with pale halo in the hepatocytic cytoplasm, which can be identified by anti-fibrinogen immunohistochemical staining. The fingerprint-like structures under electron microscope are helpful for the diagnosis, while FGG sequencing detects the pathogenic mutation of exon 8 or 9 that can clearly explain the phenotype. However, the diagnosis of FSD cannot be completely ruled out if the relevant mutations are not detected.
Child ; Child, Preschool ; China ; Female ; Fibrinogen/chemistry* ; Humans ; Liver/pathology* ; Liver Diseases/pathology* ; Male ; Metabolic Diseases/pathology* ; Retrospective Studies

Carcinoma, Hepatocellular ; chemistry ; pathology ; Humans ; Immunohistochemistry ; Liver ; chemistry ; Liver Neoplasms ; chemistry ; pathology ; Receptors, Cytoplasmic and Nuclear ; analysis ; genetics ; Reverse Transcriptase Polymerase Chain Reaction ; Transcription Factors ; analysis ; genetics

OBJECTIVETo study the expression of glucose-regulated protin 78 (GRP78) and glucose-regulated protin 94 (GRP94) in the liver tissues from children with hepatoblastoma (HB) and to investigate the possible clinicopathological values of GRP78 and GRP94 in HB.

METHODSLiver tissue specimens from 15 children with HB and 10 specimens of normal liver tissues were obtained. EnVison immunohistochemistry was used to detect the expression of GRP78 and GRP94 in the conventional paraffin-embedded liver sections.

RESULTSThe positive rates of GRP78 expression (53% vs 10%; P<0.05) and GRP94 expression (60% vs 10%; P<0.05) in HB liver tissues were significantly higher than those in the normal liver tissues. The positive rates of GRP78 expression in the cases without lymphnode metastasis or in clinical stage I-II were significantly lower than those in the cases with lymphnode metastasis or in clinical stage III-IV (P<0.05). GRP94 showed a decreased tendency of positive expression in the cases without lymphnode metastasis or in clinical stage I-II when compared with the cases with lymphnode metastasis or in clinical stage III-IV, although there were no statistical differences between them.

CONCLUSIONSGRP78 and GRP94 expression might play important roles in the pathogenesis and progression of pediatric HB.

Child ; Child, Preschool ; Female ; Heat-Shock Proteins ; analysis ; Hepatoblastoma ; chemistry ; pathology ; Humans ; Immunohistochemistry ; Infant ; Liver ; chemistry ; Liver Neoplasms ; chemistry ; pathology ; Male ; Membrane Glycoproteins ; analysis ; Neoplasm Staging

Objective To investigate the value of gadolinium ethoxybenzyl diethylene-triamine-pentaacetic-acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) in detecting different stages of liver fibrosis in rats.Methods Rat models of liver fibrosis were induced by carbon tetrachloride intraperitoneal injection for 4 - 12 weeks (n=45). The control group was applied with 0.9% saline (n=15). The MRI protocol contained both dynamic contrast-enhanced sequence (60 continuous scans within 3 minutes,including three pre-contrast measurements) and multiple hepatobiliary-phase acquisitions (every 5 minutes after contrast injection,60 minutes in total). METAVIR score was used to grade liver fibrosis:normal (F0),mild fibrosis (F1 - F2),and advanced fibrosis (F3 - F4). Liver perfusion parameters [transfer constant (K ),extravascular extracellular volume fraction (V),initial area under curve (iAUC),maximum relative enhancement (RE),and time of maximum RE (T)] as well as hepatobiliary-phase parameters [RE at different time point,the decrease of RE (RE=RE - RE),and elimination half-life of RE (T)] were measured and compared with ANOVA analysis and Spearman rank correlation.Results Thirty-one rats completed MRI exams and were then divided into normal (n=10),mild fibrosis (n=10),and advanced fibrosis (n=11) groups. K ,V and iAUC decreased as liver fibrosis progressed (r=-0.631,P=0.002;r=-0.503,P=0.017;r=-0.446,P=0.037). K and V showed significant differences among three groups(F=7.011,P=0.005;F=4.656,P=0.023). K and V were significantly lower in advanced fibrosis group than in normal group (P=0.001,P=0.009). There were statistical significant differences of T,T and RE among groups(F=6.633,P=0.005;F=5.493,P=0.010;F=5.343,P=0.014). Compared to normal and mild fibrosis groups,advanced fibrosis group had significantly longer T and T (P=0.005,P=0.004;P=0.008,P=0.008)and significantly lower RE(P=0.007,P=0.012).Conclusion Perfusion and multi-hepatobiliary-phase parameters such as K ,V,T, T and RE obtained from Gd-EOB-DTPA-enhanced MRI,may be valuable for detecting and staging liver fibrosis.
Animals ; Contrast Media ; chemistry ; Gadolinium DTPA ; chemistry ; Liver ; diagnostic imaging ; pathology ; Liver Cirrhosis ; diagnostic imaging ; Magnetic Resonance Imaging ; Rats

OBJECTIVETo investigate the changes of sugar chain structures of alkaline phosphatase (ALP) in hepatoma tissue and its relation to the invasiveness of hepatocellular carcinoma (HCC).

METHODSThe binding ratios of ALP from 9 normal liver tissues, 16 hepatoma tissues and 16 noncancerous tissues surrounding hepatoma were analysed by affinity chromatography on various lectin columns including leukoagglutinating phytohemagglutinin (L-PHA), lentil lectin (LCA), Datura stramonium agglutinin (DSA), erythroagglutinating phytohemagglutinin (E-PHA) and Sambucus nigra bark agglutinin (SNA).

RESULTSThe binding ratios of ALP on L-PHA (22.94%+/-5.30%), DSA (55.97%+/-13.72%), LCA (38.16%+/-8.87%), E-PHA (11.56%+/-4.81%) and SNA (69.80%+/-13.71%) in HCC tissues were significantly increased (P<0.01) compared with that in normal liver tissues (L-PHA 5.89%+/-2.75%, DSA 36.20%+/-11.58%, LCA 17.90%+/-6.71%, E-PHA 5.38%+/-2.20%, SNA 57.32%+/-11.27%), respectively. t values between the two groups were 8.94, 3.64, 5.94, 3.62 and 2.32, respectively. L-PHA-binding ratio (25.84%+/-4.67%) of ALP in HCC with invasiveness was significantly higher than that (18.10%+/-3.64%) without invasiveness (t=3.71, P<0.01).

CONCLUSIONThe changes of ALP sugar chain structures occur in HCC tissue. b1-6 branching sugar chain structure of ALP is related to the invasiveness of HCC.

Alkaline Phosphatase ; chemistry ; Carbohydrates ; chemistry ; Carcinoma, Hepatocellular ; enzymology ; pathology ; Chromatography, Affinity ; Humans ; Lectins ; metabolism ; Liver Neoplasms ; enzymology ; pathology ; Neoplasm Invasiveness

OBJECTIVESTo seek a better profiling of proteins of hepatoma cells.

METHODSThe homogenate of hepatoma cells QGY-7703 was fractionated into four parts by differential centrifugation: the nuclei, the pellet by 20,000 x g, the pellet by 100,000 x g and the cytosolic supernatant. The four fractions were submitted to two-dimensional gel electrophoresis and their electrophoretic patterns were analyzed.

RESULTSIn comparison with the protein pattern of hepatoma cells not fractionated, the patterns of the four fractions display many more protein spots, and a large number of proteins present in the nuclei and cytosolic supernatant were not shown in the not-fractionated samples.

CONCLUSIONPreparation of subcellular fractions before electrophoretic procedures proves to be very useful; not only can it improve the results of two-dimensional gel electrophoresis, but also can lead to research into the subcellular level.

Carcinoma, Hepatocellular ; chemistry ; pathology ; Electrophoresis, Gel, Two-Dimensional ; Humans ; Liver Neoplasms ; chemistry ; pathology ; Neoplasm Proteins ; analysis ; Proteome ; Tumor Cells, Cultured

A rhabdoid cholangiocarcinoma is a very rare variant of sarcomatous cholangiocarcinomas. Here, we report a vimentin positive cholangiocarcinoma showing rhabdoid features in the entire tumor, with a very aggressive behavior. A 41-year-old woman was admitted to our hospital due to a huge hepatic mass. The resected liver revealed a 17x15cm sized solid mass with extensive necrosis and an infiltrative border. On microscopic examination, the entire tumor was composed of loosely cohesive round to polygonal cells, with rhabdoid features having abundant eosinophilic glassy cytoplasm and eccentrically located vesicular nuclei. Some tumor cells contained intracytoplasmic mucin vacuoles, but definite areas of glandular differentiation or spindle cell were not found. Immunohistochemical staining showed a diffuse strong positive reaction to pan-cytokeratin and vimentin, and focal positivity for the carcinoembryonic antigen. Other immunohistochemical stainings for cytokeratin 7, cytokeratin 20, S-100 protein, HMB-45, desmin, alpha-smooth muscle actin, c-kit, CD34, alpha-fetoprotein, anti-hepatocyte antigen, chromogranin and synaptophysin were all negative. After two months, the patient developed a local recurrence along the resection margin, and multiple hematogenous metastases to the lung and liver were seen on the follow-up CT scan.
Adult ; Cholangiocarcinoma/chemistry/*pathology ; Female ; Human ; Liver Neoplasms/chemistry/*pathology ; Magnetic Resonance Imaging ; Prognosis ; Rhabdoid Tumor/chemistry/*pathology ; Support, Non-U.S. Gov't ; Tomography, X-Ray Computed ; Vimentin/analysis

OBJECTIVETo investigate the change of pathologic morphology in superheat mice.

METHODThe superheat was used in the experinent. The pathologic morphology of thymus gland, spleen, liver and lung was observed by light microscope.

RESULTThe histiocytes of thymus gland, spleen, liver and lung presented no changes in lesion, congestion and morphology.

CONCLUSIONThe Sanxian Xinli capsule have function of protecting the superheat mouse viscera.

Agrimonia ; chemistry ; Animals ; Capsules ; Curculigo ; chemistry ; Drug Combinations ; Drugs, Chinese Herbal ; isolation & purification ; pharmacology ; Epimedium ; chemistry ; Heat Stress Disorders ; pathology ; Liver ; pathology ; Lung ; pathology ; Male ; Mice ; Plants, Medicinal ; chemistry ; Protective Agents ; pharmacology ; Random Allocation ; Spleen ; pathology ; Thymus Gland ; pathology