Purpose To analyze the clinical and CT features of hepatoid adenocarcinoma of the stomach.Materials and Methods A total of 38 patients with hepatoid adenocarcinoma of the stomach from September 2012 to April 2023 in the First Affiliated Hospital of Soochow University were retrospectively reviewed,and the clinicopathological data,laboratory examinations,and CT characteristics of the tumor size,morphology,density,border,enhancement pattern,metastasis and invasion were recorded and analyzed.Results Among 38 patients,there were 24 cases with elevated serum alpha-fetoprotein levels and 32 cases with positive immunohistochemical alpha-fetoprotein expression.The maximum diameter was 2.38-11.95 cm in the portal phase,with the median(interquartile range)of 5.200(3.365,7.215)cm.On contrast-enhanced CT scan,there were 23 cases with ulceration,20 cases with internal necrosis,25 cases with peripheral invasion,14 cases with liver metastases,and 5 cases with carcinoma emboli in the portal system.Conclusion Hepatoid adenocarcinoma of the stomach is a rare tumor.Serum alpha-fetoprotein often increases.Size of the tumor is often large on contrast-enhanced CT scan,necrosis,progressive or continuous enhancement,metastasis and invasion of the portal vein are commonly manifested.Understanding these characteristics is helpful to improve the diagnostic level.

OBJECTIVE To observe the changes of static functional connectivity(sFC) and dynamic functional connectivity(dFC) of amygdala subregions in patients with bilateral idiopathic tinnitus and hearing loss(TINHL). METHODS The resting-state functional magnetic resonance imaging(fMRI) data of 30 patients with bilateral tinnitus and hearing loss and 37 normal controls(NCs) were collected to analyze the intensity changes of sFC and dFC in 8 subregions of amygdala and the whole brain in TINHL patients. RESULTS There were no significant differences in age,sex and education between the two groups. Compared with the NCs group,the sFC intensity of the right basolateral subregion and right cerebellar peduncle 1 region,the left basolateral subregion and left orbital medial frontal gyrus and left angular gyrus in TINHL group was significantly decreased. The dFC intensity of left amygdalostriatal subregion and left precuneus in TINHL group was increased,but the dFC intensity was reduced in the left basolateral subregion and right angular gyrus as well as between the right superficial subregion and right medial orbital of superior frontal gyrus. CONCLUSION Among the 8 subregions of amygdala,the bilateral basolateral subregions,the left amygdalostriatal subregion and the right superficial region shown abnormal functional connectivity with other regions of the brain,which are the important parts of emotional abnormalities in TINHL.

Background::Genetic variants of dopaminergic transcription factor-encoding genes are suggested to be Parkinson’s disease (PD) risk factors; however, no comprehensive analyses of these genes in patients with PD have been undertaken. Therefore, we aimed to genetically analyze 16 dopaminergic transcription factor genes in Chinese patients with PD.Methods::Whole-exome sequencing (WES) was performed using a Chinese cohort comprising 1917 unrelated patients with familial or sporadic early-onset PD and 1652 controls. Additionally, whole-genome sequencing (WGS) was performed using another Chinese cohort comprising 1962 unrelated patients with sporadic late-onset PD and 1279 controls.Results::We detected 308 rare and 208 rare protein-altering variants in the WES and WGS cohorts, respectively. Gene-based association analyses of rare variants suggested that MSX1 is enriched in sporadic late-onset PD. However, the significance did not pass the Bonferroni correction. Meanwhile, 72 and 1730 common variants were found in the WES and WGS cohorts, respectively. Unfortunately, single-variant logistic association analyses did not identify significant associations between common variants and PD. Conclusions::Variants of 16 typical dopaminergic transcription factors might not be major genetic risk factors for PD in Chinese patients. However, we highlight the complexity of PD and the need for extensive research elucidating its etiology.

Objective:To observe the clinical efficacy of herb-insulated moxibustion on the Governor Vessel in dog days of summer for postpartum pantalgia. Methods:A total of 64 patients with postpartum pantalgia were randomized into an observation group and a control group,with 32 cases in each group.Both groups were treated with herb-insulated moxibustion on the Governor Vessel.The control group was treated in non-dog days,and the observation group was treated in dog days of summer.Both groups were treated with moxibustion once every 3 d,3 times as one course of treatment,for a total of 3 courses.The range and degree of pain were evaluated by the 45-region body surface area score and visual analog scale(VAS)score before treatment,after treatment,1 month after treatment,and around the Winter Solstice.The revised fibromyalgia impact questionnaire(FIQR)was used to evaluate the quality of life of the patients.The clinical efficacy was compared between the two groups after treatment. Results:During the trial,there were 2 dropout cases in the observation group,and 2 cases were eliminated from the control group.After treatment,the total effective rate in the observation group was 93.3%,which was higher than 76.6%in the control group(P<0.05).Compared with before treatment,the number of pain sites and pain areas of the patients in both groups decreased after treatment,1 month after treatment,and around the Winter Solstice(P<0.05),and the FIQR score decreased(P<0.05).The score of each item in the observation group was lower than that in the control group(P<0.05).Compared with before treatment,the VAS score in the observation group decreased after treatment,1 month after treatment,and around the Winter Solstice(P<0.01),and was lower than that in the control group at the same time point(P<0.05). Conclusion:Herb-insulated moxibustion on the Governor Vessel can improve the range and degree of pain in patients with postpartum pantalgia and improve their quality of life.The total efficacy of the moxibustion performed in dog days is superior to that in non-dog days,and the effect lasts longer.

Hepatic alveolar echinococcosis (HAE) is a parasitic disease caused by Echinococcus multilocularis infection and has wide distribution and great harm in China. At present, ultrasound, CT, and MRI are the main radiological examination methods for HAE, with certain limitations in preoperative diagnosis and evaluation. This article introduces the guiding effect of three-dimensional visualization technique and its derivative technologies in the accurate diagnosis and preoperative evaluation of HAE, so as to provide help for the clinical diagnosis and treatment of HAE in the future.

Hepatocellular carcinoma is a common malignant disease in clinical practice, and portal vein tumor thrombosis (PVTT) is one of the important factors affecting the prognosis of hepatocellular carcinoma. PVTT has strong oncologic characteristics and is highly susceptible to extrahepatic metastasis, complicating portal hypertension, leading to gastrointestinal bleeding or liver failure and causing death. In this paper, we review the formation mechanism of hepatocellular carcinoma combined with PVTT in terms of local anatomy, hemodynamics, molecular biology and tumor microenvironment to provide effective reference for clinical treatment.

Objective:To investigate the clinical, genetic, and pathological features of nemaline myopathy type 8 (NEM8) caused by KLHL40 gene variation. Methods:The clinical data, gene sequencing results, and musculoskeletal pathophysiology of two cases (a pair of twins) with NEM8 admitted to our hospital in July 2022 were collected. CNKI, VIP, Wanfang, Yiigle, PubMed, Embase, and Web of Science Database were searched with the English and Chinese terms "nemaline myopathy type 8", "nemaline body myopathy type 8", and " KLHL40" from January 2007 to February 2023. The clinical, genetic, and musculoskeletal characteristics of the NEM8 cases were summarized using the descriptive statistical analysis method. Results:(1) Case report: The mother (G2P2) of the twins (Ⅳ-2 and Ⅳ-3) was conceived by IVF-embryo transfer and delivered at 37 +1 gestational weeks. The two cases were dizygotic twins whose maternal grandmother had lip and palate cleft. The first baby (Ⅳ-1) of the woman exhibited absent left pinna, contracture at the end of both fingers, talipes equinovarus in both feet and died of respiratory failure two hours after birth. Fetal edema and fetal movement delay in the twins and varus right foot in one twin were found during pregnancy. Both cases developed intrapartum asphyxia with the clinical manifestations of generalized muscle weakness, respiratory failure, dysphagia, multiple joint contractures, and fractures. The families withdrew the treatment and the twins died after maintaining life with ventilators for 53 days after birth. Whole exome sequencing of the pedigree found c.1779G>T (p.W593C) homozygous variants of the KLHL40 gene in the twins and c.1779G>T (p.W593C) heterozygous variants of the KLHL40 gene in the parents, both were de novo. Musculoskeletal pathophysiology indicated that muscle fibers are thin and round with a fetal shape and absent rod. (2) Literature review: Among the 29 cases of NEM8, including two current cases and 27 cases retrieved from 15 papers, eight cases terminated the pregnancies, and 21 were live births; seven cases (24.1%) with positive family history; 19 cases (65.5%) were found with abnormalities during pregnancy, including abnormal fetal movements, polyhydramnios, joint contracture, and fetal edema. Of the 21 live births, 20 cases had intrapartum asphyxia, 21 had postnatal respiratory failure, 20 had generalized muscle weakness, and 19 had dysphagia. Among the 29 cases, 17 cases (58.6%) were homozygous variants of the KLHL40 gene, and 12 cases (41.4%) were compound heterozygous variants. The detection rate of c.1516A>C(p.Thr506Pro) was the highest [72.4% (21/29)], followed by c.602G>A(p.Trp201*) [17.2% (5/29)]. Out of the 15 cases who underwent musculoskeletal pathological examination, all had abnormal muscle fiber size and morphology; 10 cases had rods. Conclusions:NEM8 should be considered for those with abnormal fetal movements, polyhydramnios, joint malformation, fetal edema during the fetal period and failure to establish respiration at birth as well as postnatal generalized muscle weakness, respiratory failure, dysphagia, multiple joint contractures, and fracture. Moreover, genetic detection should be performed as soon as possible. NEM8 can be diagnosed in ones with KLHL40 gene homozygous or compound heterozygous variants and musculoskeletal pathological results of abnormal size and shape of muscle fibers, regardless of the presence of rods.

Objective:To explore the effect of early activity based on action research method in severely ill children.Methods:From February 2020 to January 2022, 101 children with severe illness admitted to the Henan Provincial People's Hospital were selected as the study subjects by convenient sampling. The 51 children with severe illness included from February 2020 to January 2021 were set as the control group, and the 50 children with severe illness included from February 2021 to January 2022 were set as the experimental group. The children in the control group were treated with routine rehabilitation in the Pediatric Intensive Care Unit (PICU) , and the experimental group was treated with early activity based on action research method on the basis of the control group. Before and after the intervention, the effect was evaluated by the Medical Research Council Scale (MRC) , the Intensive Care Unit Mobility Scale (IMS) , the length of stay in PICU and hospitalization time, and the incidence of ICU-acquired weakness (ICU-AW) when transferring out of PICU.Results:When transferring out of PICU, the MRC muscle strength score of children in the experimental group was higher than that in the control group, and the incidence of ICU-AW was lower than that in the control group, and the difference was statistically significant ( P<0.05) . After intervention, the IMS score of children in the experimental group was higher than that in the control group, and the difference was statistically significant ( P<0.05) . The length of stay in PICU and hospitalization time of children in the experimental group were shorter than those in the control group with statistical differences ( P<0.05) . Conclusions:Early activity based on action research method can effectively prevent the occurrence of ICU-AW in severely ill children, improve the activity of children, and shorten the hospitalization time, which is worthy of clinical promotion and application.

Objective:To conduct quality evaluation and content analysis of adult postoperative nausea and vomiting (PONV) guidelines, so as to provide reference for management of clinical PONV.Methods:Web of Science, Embase, PubMed, UpToDate, SinoMed, Wanfang Database, China National Knowledge Infrastructure, VIP, domestic and foreign clinical practice guidelines and related professional association websites were systematically searched, and the search period was from database establishment to May 6, 2022. Four researchers independently evaluated the guidelines that met the inclusion and exclusion criteria using a clinical guideline research and evaluation system, and summarized the recommendations of the guidelines.Results:Finally, a total of 15 guidelines were included in this study. The overall quality evaluation of the guide was 3 A-level recommendations and 12 B-level recommendations. The average standardization percentages for 6 areas were 81.57% for scope and purpose, 49.91% for participants, 65.38% for rigor, 89.54% for clarity, 34.86% for applicability and 55.42% for independence. A total of 18 recommendations were summarized from five aspects, such as team and organizational management, PONV risk assessment, baseline risk reduction, multimodal prevention of PONV and effectiveness evaluation and monitoring.Conclusions:The guidelines for PONV management mainly come from foreign countries. It is recommended that clinical personnel should learn from foreign guidelines and combine them with domestic clinical situations to localize the recommended opinions and guide the development of clinical practice.

Objective:To summarize the clinical features of intestinal Beh?et′s disease, so as to provide reference for the diagnosis of the disease.Methods:From April 1 2014 to January 31 2019, the clinical data of 47 patients diagnosed as intestinal Beh?et′s disease at the Second Hospital of Hebei Medical University were retrospectively analyzed, which included initial symptoms, gastrointestinal symptoms, complications, erythrocyte sedimentation rate (ESR), the levels of C reactive protein (CRP), hemoglobin, serum albumin, results of acupuncture test, gastrointestinal involved site and ulcer shape. At the same time, gender differences of clinical manifestations were compared. Chi-square test was used for statistical analysis.Results:Among 47 patients with intestinal Beh?et′s disease, the initial symptoms of 26 (55.3%) cases were gastrointestinal symptoms. Abdominal pain was the most common symptom, the others were diarrhea, anorexia, abdominal distension and perianal abscess, and the incidence rate was 80.9%(38/47), 46.8% (22/47), 42.6% (20/47), 36.2% (17/47) and 2.1% (1/47), respectively. The main complications were gastrointestinal bleeding, perforation and obstruction, and the incidence rates was 40.4% (19/47), 4.3% (2/47) and 4.3% (2/47), respectively. Thirty-seven (78.7%) patients had different degrees of hypoalbuminemia (serum albumin<35 g/L). The CRP level of 36(76.6%) patients increased. The ESR of 36 (76.6%) patients increased. Twenty-two (46.8%) patients had mild anemia (hemoglobin<90 g/L). The acupuncture test was positive in 25 (53.2%) patients. The involved sites of gastrointestinal tract were terminal ileum and ileocecal junction, colon, esophagus, duodenum and jejunum, stomach, and rectum, the proportion was 57.4% (27/47), 27.2% (13/47), 23.4% (11/47), 23.4% (11/47), 17.0% (8/47) and 8.5% (4/47), respectively. All 47 (100.0%) patients had oral ulcers. 62.1%(18/29) patients presented with multiple ulcers under endoscope. The shape of ulcer was round ulcer, irregular ulcer, and longitudinal ulcer, the proportion was 48.3% (14/29), 34.5% (10/29) and 17.2 (5/29), respectively. The incidence rate of genital ulcer of female patients with intestinal Beh?et′s disease was higher than that of male patients with intestinal Beh?et′s disease (85.7%, 18/21 vs. 30.8%, 8/26), and the difference was statistically significant ( χ2=14.189, P<0.01). There were no significant differences between the female group and the male group in the incidence rate of oral ulcer, abdominal pain, diarrhea, and positive rate of acupuncture test (100.0%, 21/21 vs. 100.0%, 26/26; 85.7%, 18/21 vs. 76.9%, 20/26; 42.9%, 9/21 vs. 50.0%, 13/26; 52.4%, 11/21 vs. 58.3%, 14/26, all P>0.05). Conclusions:The common clinical symptoms of intestinal Beh?et′s disease are oral ulcers, abdominal pain, diarrhea and genital ulcer. Female patients with intestinal Beh?et′s disease are more likely to develop genital ulcer than male patients with intestinal Beh?et′s disease. Multiple ulcers are more common under endoscopy, which are round ulcer, irregular ulcer and longitudinal ulcer. The most common sites are the terminal ileum and ileocecal junction, followed by colon, esophagus and other parts.