Objective:To analyze the clinical features, imaging features and diagnosis and treatment process of a female pelvic epithelioid inflammatory myofibroblastic sarcoma (EIMS) with peritoneal metastasis, so as to improve the clinical understanding and diagnostic ability of the disease and avoid misdiagnosis and missed diagnosis.Methods:The clinical data of a female patient with pelvic EIMS combined with peritoneal metastasis in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.Results:The triad examination of the patient involved an irregular solid mass in the pelvic cavity about 8.0 cm×9.0 cm in size. Laboratory examination revealed human epididymal protein 4(HE4)154.00 pmol/L. The ultrasonography showed multiple low-echo masses in deep pelvic cavity with unclear boundary and uneven internal echo, and color doppler flow imaging (CDFI) showed abundant internal blood flow signals. Enhanced CT showed uneven and obvious enhancement of the lesion. Pathological examination showed the infiltration of inflammatory cells in the mucous interstitial background. The tumor cells were round and epithelioid, with large nuclei, deep staining and obvious nucleolus. Immunohistochemistry showed anaplastic lymphoma kinase (ALK) positive, and molecular pathology fluorescence in situ hybridization showed ALK gene amplification (positive). Combined with pathological, immunohistochemical and genetic tests, EIMS was diagnosed. Conclusions:EIMS should be considered when there are single or multiple solid or cystic nodules or masses in the pelvic cavity with obvious enhancement, invasive growth and peritoneal implantation metastasis, and the correct diagnosis can be made according to the pathological findings, immunohistochemistry and genetic test results.

Methods:This study was a cross-sectional study. A prospective cohort study enrolled 42 patients with clinically suspected acute cerebrovascular disease and those undergoing follow-up examinations after intracranial vascular stenting at the First Affiliated Hospital of Zhengzhou University from June 2024 to May 2025. All patients underwent UHR PCD-CT examinations of the head and neck. Reconstructions were performed based on raw data, yielding conventional standard resolution (SR group) reconstructions and UHR images reconstructed using four distinct convolution kernels (Hv40, Hv48, Hv56, Hv64) in separate groups (Hv40 UHR group, Hv48 UHR group, Hv56 UHR group, Hv64 UHR group). Regions of interest were selected in the anterior cerebral artery, middle cerebral artery, posterior cerebral artery, posterior communicating artery, and anterior communicating artery. CT values and standard deviation (SD) values were measured for each artery, and signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Additionally, the sharpness of the vessel edges and the full-width-half-maximum (FWHM) of each artery were measured. One-way analysis of variance or the nonparametric Kruskal-Wallis test was used to compare the subjective and objective image quality metrics across the five groups. Pairwise comparisons were performed using the LSD test or Dunn method.Results:Statistically significant differences were observed in the overall comparison of vascular imaging SD, SNR, CNR, vascular edge sharpness, and FWHM among the SR group, Hv40 UHR group, Hv48 UHR group, Hv56 UHR group, and Hv64 UHR group ( P<0.05). No statistically significant differences in CT values were found ( P>0.05). Pairwise comparisons revealed statistically significant differences between all groups ( P<0.05), except that no significant differences were observed in image SD, SNR, CNR, vascular edge sharpness, or FWHM between the Hv56 UHR and Hv64 UHR groups ( P>0.05). Conclusions:UHR PCD-CT provides better image quality for neurovascular imaging. For the display of small intracranial vessels, the Hv64 provides sharper vessel walls and better subjective image quality compared to the less sharp convolutional cores.Objective:To explore the value of ultra-high resolution (UHR) photon-counting detector CT (PCD-CT) to improve the quality of neurovascular images.

Neurofibromatosis type 1(NF1)is a neurocutaneous syndrome and a rare autosomal dominant hereditary disorder able to affect multiple systems and organs,and the age of initial diagnosis crossing neonates to elderly.NF1 has more age-dependent clinical manifestations with changeable onset and severity.With the increased attention to rare diseases and revisions of diagnostic criteria,more and more NF1 were timely diagnosed in childhood.The status and advancements in imaging research of NF1 in children were reviewed in this article.

Objective:To develop a modified University of Wisconsin preservation solution (UW solution) containing α 2-adrenergic receptor agonists (dexmedetomidine) and noble gases (argon) and investigate its protective effect on the isolated amputated skeletal muscle of rats. Methods:Sixty male SD rats were selected to establish a hindlimb cold preservation/perfusion model and were divided into blank control group, hypothermic storage group, UW solution perfusion group, and modified UW solution perfusion group using a random number table, with 15 rats in each group. Simultaneously, a cold preservation model of rat skeletal muscle myoblasts (L6 cells) was established and the rats were also divided into four groups in the same way. Animal models were prepared in different ways: In the blank control group, the hindlimbs received no special treatment; In the hypothermic storage group, the amputated hindlimbs were stored in a dry centrifuge tube at 4℃ for 18 hours; In the UW solution perfusion group, the amputated hindlimbs were perfused with UW solution and then stored in a centrifuge tube containing UW solution at 4℃ for 18 hours; In the modified UW solution perfusion group, the amputated hindlimbs were perfused with modified UW solution (containing 0.1 nmol/L dexmedetomidine and 50% volume fraction of argon) and then stored in a centrifuge tube containing the modified UW solution at 4℃ for 18 hours. Cell models were treated as follows: In the blank control group, L6 cells were cultured under standard conditions; In the hypothermic storage group and UW solution group, L6 cells were treated with conventional culture medium or UW solution, stored in argon-filled sealed bags at 4℃ for 8 hours, and then rewarmed and cultured for 6 hours; In the modified UW solution group, L6 cells were treated with the modified solution, stored in argon-filled sealed bags at 4℃ for 8 hours, and then rewarmed and cultured for 6 hours. After sample collection, skeletal muscle morphology, tissue edema and ultrastructure features were assessed by HE staining, wet-to-dry weight ratio, and transmission electron microscopy, respectively. Additionally, L6 cell morphology was examined by light microscopy. L6 cell viability was determined by cell counting kit-8 (CCK-8) assay (expressed as absorbance A value). Expression levels of glutathione peroxidase 4 (GPX4) protein in both skeletal muscle tissue and L6 cells were evaluated by immunofluorescence staining and Western blot, respectively.Results:After 18 hours of in vitro preservation of rat isolated amputated limbs, the following results were obtained: (1) HE staining results showed that the muscle fiber morphology of the modified UW solution perfusion group was close to that of the blank control group. Moreover, the area ratio of skeletal muscle cells in the modified UW solution perfusion group was significantly higher than that in the hypothermic storage group and UW solution perfusion group ( P<0.05). (2) The wet-dry weight ratio results showed that there was no statistically significant difference among the modified UW solution perfusion group, the blank control group and UW solution group ( P>0.05), with significantly lower ratios in all three groups than that in the hypothermic storage group ( P<0.05). (3) Transmission electron microscopy results revealed that the modified UW solution perfusion group showed no statistically significant differences in ultrastructural metrics, including myofiber diameter, sarcomere length, mitochondrial short-axis/long-axis ratio, and mitochondrial cristae count, compared with those in the blank control group ( P>0.05), and performed significantly better than both the hypothermic storage group and UW solution perfusion group ( P<0.05). (4) Morphological observation of L6 cells showed that the cellular morphology was regular in the modified UW solution perfusion group, close to that in the blank control group, while it was severely damaged in the hypothermic storage group. Moreover, the cells were reduced in number and partially damaged in the UW solution group. The sequence of cell viability expressed as absorbance A value was blank control group >modified UW solution perfusion group > UW solution perfusion group > hypothermic storage group, with statistically significant differences among the four groups ( P<0.05). (5) Immunofluorescence staining showed that there was no statistically significant difference in fluorescence intensity of GPX4 protein expression between the modified UW solution perfusion group and blank control group ( P>0.05), while the fluorescence intensity was higher in the modified UW solution perfusion group than that in the hypothermic storage group and UW solution perfusion group ( P<0.05). Western blot analysis showed that the relative expression level of GPX4 in the modified UW solution group was significantly lower than that in the blank control group ( P<0.05), but higher than that in the hypothermic storage group and UW solution perfusion group ( P<0.05). Conclusion:The modified UW solution can stabilize the expression level of GPX4 protein, thereby inhibiting ferroptosis and alleviating cold preservation injury in both rat amputated isolated limb skeletal muscle tissue and L6 cells.

Objective:Analyze the clinicopatholocical and imaging characteristics of hepatic epithelioid hemangioendothelioma (HEHE) and the related factors of survival prognosis.Methods:Clinical data of 32 patients diagnosed with HEHE at the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023 retrospectively analyzed, including 11 males and 21 females, aged (46.8±3.6) years. The clinical manifestations, pathological findings, CT and MRI features of the patients were analyzed and the living conditions of the patients were followed up. Kaplan-Meier method was used for survival analysis, and log-rank test was used for survival rate comparison.Results:Among the 32 patients, 17 (53.1%) had no obvious symptoms, 10 (31.3%) had abdominal pain, 6 (18.8%) had abdominal distension, and 4 (12.5%) had lost weight. Under the microscope, HEHE was composed of mucous matrix and epithelioid endothelial cells, with dense surrounding cells and relatively sparse center. Small blood vessels can be seen invading the junction with normal liver tissue. The positive rates of endothelial marker CD34 in immunohistochemically staining was 100%(32/32), the positive rates of CD31 were 100% (32/32). The positive rates of erythroblast transformation specific related gene were 100% (28/28), and the positive rates of Friend leukemia virus integration protein 1 (22/22) were 100%. CT and MRI scan showed that the lesion were mainly multiple nodules and in diffuse formality. In arterial phase, the lesion showed slight homogeneous or ring-like enhancement, while in portal phase, the lesion showed progressive enhancement. Some lesions may exhibit more specific "lollipop sign" ( n=9) and "target ring sign" ( n=15). The median overall survival time of the 32 patients was 47 months, and the cumulative 1-, 3-, and 5-year survival were 100%, 95.0% and 72.7%, respectively. The cumulative survival rate of patients with "target ring sign" ( n=15), extrahepatic organ involvement or metastasis ( n=8) and Ki-67 positive rate >5% ( n=10) were lower than those without the above characteristics (all P<0.05). Conclusion:The clinical manifestations of HEHE were not typical, and the tumor was rich in mucus matrix and epithelioid endothelial cells, expressing endothelial markers. CT and MRI scan mainly showed multiple nodules or diffused lesion, and the "lollipop sign" and "target ring sign" were helpful for diagnosis. HEHE patients with Ki-67 positivity rate >5%, "target ring sign", and extrahepatic organs involvement or metastasis had a poor prognosis.

In recent years, with the endless emergence of meibomian gland dysfunction(MGD)diagnostic equipment, rich treatment methods, and in-depth clinical and basic research on MGD at home and abroad, the understanding of MGD has entered a new stage. MGD-related dry eye is considered to be the main cause of lipid abnormal dry eye, and its occurrence and development is a chronic and multi-factorial pathological process. This article reviews the pathogenesis, imaging analysis and clinical treatment progress of MGD-related dry eye, in order to provide scientific evidence and ideas for clinical diagnosis and therapy of MGD-related dry eye.

OBJECTIVE: To explore the genetic basis of a child with Chanarin-Dorfman syndrome (CDS) manifesting as ichthyosis. METHODS: A child who had presented at Henan Provincial People's Hospital in June 2023 was selected as study subject. Clinical data of the child was collected. Peripheral blood samples were collected from the child and her parents. Following extraction of genomic DNA, whole-exome sequencing (WES) was carried out. Candidate variants were verified by Sanger sequencing. Relevant literature was searched in databases using key words "Chanarin-Dorfman syndrome" and "ABHD5 gene". The clinical manifestations and variant sites of previously reported cases were compiled and analyzed for correlations. This study was approved by the Medical Ethics Committee of Henan Provincial People's Hospital [Ethics No.: (2019) Jun Shen No. (134)]. RESULTS: WES revealed that the child has harbored compound heterozygous variants of the ABHD5 gene, namely c.99_103del (p.H34*) in exon 2 and c.770C>G (p.P257R) in exon 5, which were inherited from her father and mother, respectively. Bioinformatic analysis suggested that both variants were pathogenic. Literature review indicated that the affected organs in CDS are ranked from most to least including liver, eyes, ears, nervous system, muscles, spleen, and kidneys. The c.594insC and c.594dupC variants are most common. CONCLUSION The identification of the two novel ABHD5 gene variants has enriched the mutation spectrum of CDS. c.594insC or c.594dupC are hotspot mutations of this disease, albeit with no definitive correlation between the genotype and phenotype.
Humans ; Female ; Ichthyosiform Erythroderma, Congenital/genetics* ; Lipid Metabolism, Inborn Errors/genetics* ; Phenotype ; 1-Acylglycerol-3-Phosphate O-Acyltransferase/genetics* ; Mutation ; Muscular Diseases/genetics* ; Exome Sequencing ; Child ; Male ; Child, Preschool

Objective To evaluate the clinical value of loop-mediated isothermal amplification(LAMP)for rapid detection of neonatal infectious pathogens.Methods Biological samples(cord blood and neonatal blood or sputum within 48 hours of birth)of 167 suspected neonates and premature infants delivered in Jinhua Maternal and Child Health Hospital from June 2021 to December 2022 were collected and tested by LAMP method and bacterial culture method respectively.The results of the two methods were compared consistent-ly.Using the bacterial culture method as the standard,the positive rate of LAMP method was evaluated in de-tecting common pathogens in neonatal infections.Results Among the 167 specimens,the positive rate of pathogen detection by LAMP method(24.0％)was higher than that by bacterial culture method(15.0％),and the difference was statistically significant(x2=4.30,P＜0.05).The positive rate of LAMP method for detecting methicillin-resistant Staphylococcus aureus was higher than that of bacterial culture method,and the difference was statistically significant(x2=15.59,P＜0.01).Conclusion Compared with bacterial culture method,LAMP method has a higher positive rate for detecting methicillin-resistant Staphylococcus aureus,and can quickly and accurately diagnose the pathogenic bacteria of neonatal infections,providing a basis for the rapid development of clinical diagnosis and treatment strategies.

[This corrects the article DOI: 10.1016/j.jpha.2023.08.006.].

Objective To evaluate the application of evidence-based perioperative patient-controlled analgesia(PCA)management in patients with liver cancer receiving transcatheter arterial chemoembolization(TACE)treatment.Methods By using the application model of clinical evidence-based practice,the review indicators were formulated based on the best evidence.The baseline assessment was conducted,the barrier factors were analyzed,the best clinical decision was made,the implementation steps of PCA management,including training,monitoring,education,etc.were refined,and two rounds of clinical review were carried out.The knowledge-belief-practice level and the implementation of review indicators in 50 medical and nursing staff engaged in PCA management,as well as the changes in pain scores,the incidence of adverse reactions due to PCA management,and the patient's satisfaction in 159 patients after the application of evidence were compared with their corresponding values determined before the application of evidence.Results After implementing the evidence-based practice plan and applying the evidence,at multiple time points the pain scores and the incidences of adverse reactions were decreased significantly(P＜0.05),the patient's satisfaction increased remarkably(P＜0.01),the execution rate of medical and nursing staff for the review indicators were strikingly increased(P＜0.01),and the knowledge-belief-practice level concerning PCA management was prominently improved(P＜0.01).Conclusion The implementation of perioperative PCA management in patients with liver cancer receiving TACE treatment can help to reduce the perioperative pain level,improve the patient discomfort,increase the patient's satisfaction degree,and improve the ability of medical staff in performing PCA management and evidence-based practices.