Objective To explore the value of MSCT in diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Methods 24 patients with CTEPH and 8 patients with pulmonary hypertension by other causes were collected retrospectively.To analyzed the characteristics of CTPA images in patients with CTEPH,summarized the direct and indirect signs,and to compared with non-CTEPH.Statistical analysis was performed with SPSS1 7.0 software.Results According to the CTPA images,744 arteries of 24 patients with CTEPH were evaluated.The direct sign of CTEPH was mural thrombus firstly,accounted for 55.35% of the involved arteries,and then was followed by holo-obstruction,partial filling defect,central filling defect,irregular vessel wall thickening and eccentricity filling defect.There were narrowed lumens in 35.01%,dilated ones in 0.89% and no changes in 45.24%.The common indirect signs of CTEPH were pulmonary broadening (100%),enlargement of right heart (95.83%),mosaic attenuation,pericardial or pleural effusion,ground-glass opacity,infarction and atelectasis or consolidation in order.All patients had different degrees of pulmonary hypertension,and the most common findings of CTPA were widened pulmonary artery and enlarged right ventricle,and then were followed by enhancement of the inferior vena cava and hepatic vein,the expansion of bronchial artery and abnormal septal position.Conclusion CTPA can show the types and direct or indirect signs of CTEPH clearly.The morphological changes of the heart in CT are not enough to differentiate the CTEPH and non-CTEPH,and the severity of CTEPH is not alone decided by the degree of chronic pulmonary embolism.

Objective:To study the clinical and laboratory characteristics of neonatal isolated sulfite oxidase deficiency (ISOD).Methods:An infant with neonatal ISOD admitted to our hospital was retrospectively analyzed. Using key words "isolated sulfite oxidase deficiency", "SUOX gene", "Infant, newborn", databases including CNKI, Wanfang database, National library and literature center of science and technology, China science paper online, PubMed, Web of Science and EMBASE (up to January 2021) were searched and literature review was conducted. The clinical manifestations, laboratory results, treatment and prognosis were analyzed.Results:Our patient was a full-term male infant with eye movement disorder, refractory seizures, feeding difficulties, increased muscle tone, developmental retardation and microcephaly. Urine sulfite paper-strip test was positive. Uric acid was normal. Whole exon sequencing (WES) revealed SUOX c.475G>T and c.1201A>G compound heterozygous mutations. Cranial MRI showed multiple encephalomalacia and brain atrophy at 5-month of age. The infant died at 8-month. In the literature review, a total of 29 articles and 32 cases of neonatal ISOD were found. 87.5% of the cases developed symptoms within 1-week after birth. All had convulsive seizures. Some of them had feeding difficulties, muscle tone changes, developmental retardation, microcephaly and ectopia lentis. Cranial imaging showed white matter cystic lesions and brain atrophy. Laboratory examination showed elevated urinary sulfite and S-sulfocysteine. Uric acid and xanthine/hypoxanthine were normal. Blood homocysteine was decreased. 23 cases received genetic testing and all of them had SUOX mutations. The treatment was mainly symptomatic relief and supportive treatment. During follow-up, 15 cases died, 13 cases survived and 4 cases were unknown. All the surviving children had drug-resistant convulsions and developmental retardation.Conclusions:Neonatal ISOD may present with refractory convulsions, feeding difficulties and developmental retardation. Cystic white matter changes and brain atrophy may be seen on cranial imaging. Elevated urinary sulfites, decreased blood homocysteine and normal uric acid are important clues for diagnosis. Genetic testing is helpful for early diagnosis.

Objective:To prepare a fluorescent probe Cetuximab-IRDye800CW targeting epidermal growth factor receptor (EGFR) and investigate its application value in surgical navigation of glioblastoma (GBM).Methods:The fluorescence properties of Cetuximab-IRDye800CW were determined by fluorescence spectrophotometer. The specificity of Cetuximab-IRDye800CW bound to GBM cells was verified by Western blot. The competitive binding method of enzyme-linked immunosorbent assay (ELISA) was used to prove whether the probe could achieve tumor targeting by binding to EGFR. Subcutaneous models of 6 nude mice of GBM were divided into experimental group ( n=3; injected with Cetuximab-IRDye800CW) and control group ( n=3; injected with IRDye800CW), and images were obtained at 5 min, 24 h, 48 h and 72 h after injection. Differences of mean fluorescence intensity (MFI) and tumor to background ratio (TBR) between experimental group and control group were compared. In situ models of GBM nude mice were established ( n=6), and MRI and intraoperative navigation were conducted, which were compared with pathological distribution. Independent-sample t test was used to analyze the data. Results:The maximum emission wavelength of Cetuximab-IRDye800CW was 820 nm, which could be received by near infrared fluorescence imaging equipment. Western blot showed that Cetuximab-IRDye800CW was only bound to GBM cells. The competitive binding of ELISA showed that Cetuximab-IRdye800CW could achieve tumor targeting by binding with EGFR. At 5 min, 24 h, 48 h and 72 h after injection of fluorescent materials, the MFI values of experimental group were 109.00±3.81, 73.36±9.93, 55.24±8.82, 37.71±6.11, which were higher than those of control group (91.32±4.17, 42.91±5.39, 25.08±6.05, 8.33±1.00; t values: 4.36-9.40, P values: 0.011-0.049). The TBR of experimental group was higher than that of control group at 24 h and 48 h after injection (24 h: 2.40±0.28 vs 1.57±0.07, t=4.94, P=0.039; 48 h: 2.07±0.12 vs 1.22±0.08, t=9.85, P=0.010). GBM in situ model was successfully constructed and verified by MRI, and the tumor was visualized under the fluorescence device navigation. Pathological distribution of the tumor with HE staining was consistent with fluorescence imaging. Conclusion:Cetuximab-IRDye800CW has fluorescence imaging capability and can identify tumor boundaries in intraoperative navigation of GBM, which has potential clinical application value.

The mechanical microenvironment of cells plays a critical role in regulating the physiological function of cells. Cells in vivo are often subjected to a variety of mechanical forces from their mechanical micro-environment, such as shear, tension, and compression. At the same time, cells can adhere to the extracellular matrix (ECM) through adhesion molecules (such as integrin-ligand binding), and further sense the stiffness of the ECM. Cell mechanics mainly studies the properties and behavior of living cells under mechanical forces, and how they relate to cell functions. This review summarized the advances in cell mechanics in 2022, focusing on integrin-ligand interactions and the effects of matrix stiffness and mechanical forces on cell physiological behavior and morphogenesis.

OBJECTIVE： To investigate current status and quality of domestic pharmacoeconomic literatures， and to provide reference for the standardization of pharmacoeconomic research. METHODS： Retrieved from CNKI， Wanfang， VIP and other database， the pharmacoeconomic literatures published from Jan. 2017 to May 2018 were collected. The qualities of literatures were evaluated with Guidelines for Quality Evaluation of Pharmacoeconomics Evaluation Reports （“PEERs” for short）. RESULTS： Totally 160 domestic pharmacoeconomic research literatures were included. The results of PEERs evaluation showed of which the coincidence rate was 32.5％ （52/160）. The literatureswhich were in full compliance （the report had a certain reference value） accounted for 1.3％ （2/160）， which were in basic compliance （the report had certain reference value after being revised） accounted for 31.3％（50/160），which were in non-conformity （the report did not had reference value） accounted for 67.5％（108/160）. Domestic pharmacoeconomic researches were of high quality in terms of research object， evaluation method and content，research purpose，  research design and design type， etc.； but the researches were of low quality in terms of research angle， incremental cost/incremental output analysis， sensitivity analysis and other aspects， and there was no explanation or unclear elaboration. CONCLUSIONS： The quality of domestic pharmacoeconomic research literatures are uneven， and their research quality needs to be further improved. It is recommended to standardize the evaluation of pharmacoeconomics， making the evaluation of pharmacoeconomics more scientific and objective.

Photorhabdus is a Gram-negative bacterium from the family Enterobacteriaceae that lives in a symbiotic association with nematode or insects. In addition to the role of being insect pathogens, one species called Photorhabdus asymbiotica (Pa) causes human infection around the world. Nevertheless, how does this transkingdom infection occur remains elusive. Here we focus on one pathogenic determinant called Photorhabdus virulence cassette (PVC) that is founded in the Pa genome and many other pathogens. The RNA-seq and qPCR data showed that the NF-κB and MAPK pathways were drastically activated in the PVC-treated mammalian macrophages. Western blotting assays using samples treated with various inhibitors of the affected pathways confirmed the results we have observed for MAPK pathway previously. p65 translocation assays validated the NF-κB activation in the macrophages after PVC treatment. Moreover, the bacterial phagocytosis by macrophage was also promoted by PVC at the early stage, and this phagocytosis was inhibited by cytoskeleton inhibitors. Thus, the results indicated that PVC is involved in the bacterial invasion by activating NF-κB and MAPK signaling pathway, providing a new perspective for analyzing the pathogenicity of Pa in human infections.
Animals ; Humans ; Macrophages ; NF-kappa B/genetics* ; Photorhabdus ; Signal Transduction ; Virulence