Hippophae rhamnoides is a traditional Chinese medicine with homology of medicine and food， which has the effects of relieving cough and resolving phlegm， strengthening the stomach and digestion， and promoting blood circulation and resolving blood stasis. H. rhamnoides contains not only flavonoids， phenols， proteins， vitamins， and amino acids but also abundant polysaccharides. In order to explore the functional value and current research status of H. rhamnoides polysaccharides， this study systematically summarized the extraction process， structural characteristics， pharmacological effects， and mechanism of action of H. rhamnoides polysaccharides by reviewing Chinese and foreign literature. The results showed that H. rhamnoides polysaccharides have anti-tumor， hypoglycemic， antioxidant， immune regulation， anti-inflammatory， and anti-hyperlipidemia functions and could improve intestinal microbiota. There were various extraction processes for polysaccharides， including hot water extraction， microwave extraction， ultrasonic extraction， enzymatic extraction， flash extraction， ultrasonic-microwave synergistic extraction， emerald hot water extraction， enzymatic-ultrasonic synergistic extraction， etc. Based on comprehensive analysis， hot water extraction is suitable for industrial development and application. However， multiple homogeneous polysaccharides have been isolated and purified from H. rhamnoides polysaccharides， but their efficacy， structure， and structure-activity relationship still need to be further explored and studied. This study can provide a reference for the research and development of H. rhamnoides polysaccharides.

Objective:To investigate the clinical phenotype and genetic variation of Basel-Vanagaite-Smirin-Yosef syndrome (BVSYS), and to enhance clinicians′ knowledge of the disease.Methods:The clinical data of a child with BVSYS admitted to the Department of Neurological Rehabilitation, Qingdao Women and Children′s Hospital Affiliated to Qingdao University in February 2023 were collected. Whole genome sequencing was used to analyze the pathogenic genes of the child, and Sanger sequencing was used to verify the suspected mutation sites of the family members. The clinical phenotype and genetic variation characteristics were analyzed, and the clinical characteristics of BVSYS were summarized in combination with relevant literature.Results:The patient, a female aged 3 years and 1 month, presented with global developmental delay, speech disorder, distinctive facial features, esotropia, epilepsy, hypotonia and atrial septal defect. Brain magnetic resonance imaging revealed bilateral ventriculomegaly with abnormal signal intensity in the posterior bodies of both lateral ventricles and thinning of the corpus callosum. The whole genome sequencing revealed a homozygous missense mutation c.518 (exon5) T>C (p.IIe173Thr) in the MED25 gene of the child, and Sanger sequencing confirmed that her parents and elder brother carried the aforementioned heterozygous mutation, which was classified as a likely pathogenic mutation according to the guidelines of the American College of Medical Genetics and Genomics. A total of 22 cases from 6 literature sources were retrieved, with no reported cases in China so far. Conclusions:BVSYS is clinically rare. For patients presenting with unexplained global developmental delay or intellectual disability combined with craniofacial, neurological, cardiac, and eye abnormalities, targeted genetic testing can facilitate a definite diagnosis.

Background:At present,domestic guidelines and consensus recommend the use of nutritional risk screening 2002(NRS 2002)and patient-generated subjective global assessment(PG-SGA)for nutritional risk screening and assessment of patients with gastrointestinal cancer during the perioperative period.However,PG-SGA has higher professional requirements,complex content and time-consuming.In the current busy situation of medical staff,NRS 2002 is more used for screening alone.Aims:To explore the consistency of NRS 2002 and PG-SGA in the assessment of nutritional status and clinical outcomes in patients with gastrointestinal malignancies,and to explore the accuracy of screening using NRS 2002 alone,so as to provide guidance for the establishment of clinical nutritional screening and assessment standards.Methods:A retrospective analysis was conducted on 157 patients with gastrointestinal malignancies who underwent radical operation in the Department of Gastrointestinal Surgery of Xiangya Hospital,Central South University from January 2020 to October 2022.Nutritional screening and evaluation were performed by NRS 2002 and PG-SGA scales and demographic data and nutrition-related laboratory indicators were collected to observe short-term postoperative clinical outcomes.Results:Patients with nutritional risk or malnutrition had lower body mass index(BMI),lymphocytes and prealbumin(P<0.05).The correlation and consistency of NRS 2002 and PG-SGA scales were good(r=0.728,κ=0.46)and the areas under the curve(AUC)for predicting postoperative complications were 0.691 and 0.702,respectively.In addition,nutritional risk and postoperative complications were significantly increased in patients with malnutrition(P<0.05).Conclusions:Therefore,gastrointestinal surgeons can only use NRS2002 to perform nutritional screening of patients and make corresponding nutritional treatment according to the screening results in the case of busy clinical work.

The key factors for producing the best quality Chinese herbal medicines are high-quality germplasm, suitable cultivation area and the proper processing methods for herbal raw materials. Gentiana crassicaulis in Gentiana (Sect. Cruciata), Gentianaceae is one of the original plants of the Chinese herb Qinjiao (Gentianae Macrophyllae Radix), and its type specimen was collected in Lijiang, Yunnan. There is a long planting history of the herb in this area. In this study a sampling plot was designated in these traditional planting areas. G. crassicaulis was planted and herbal raw materials were harvested from the plot. The raw materials were prepared locally and at a pharmaceutical factory in Shanghai using processing methods such as "sweating" or "no sweating", "slicing" or "no slicing" (whole root), and "stoving" or "no stoving" (air drying). The quality of all processed samples was evaluated. In addition, molecular markers were determined for identifying cultivated and wild samples from Lijiang, Yunnan. The results are as follows: ① Samples from the sampling plot and the field are taxonomically identified as Gentiana crassicaulis. ② A total of 270 sequences of trnC-GCA-petN, atpB-rbcL, psbN, ndhB-rps7 and ycf1 were obtained, and three genotypes were determined from the cultivated samples; the type III was shared by both cultivated and wild plants. Based on the molecular markers, a DNA barcoding method to identify cultivated and wild samples of G. crassicaulis from Lijiang, Yunnan was established. ③ Total content of loganic acid and gentiopicroside in all samples was ≥ 2.5%, and above the Chinese Pharmacopoeia (2020) limit. ④ In HPLC fingerprinting, 9 common peaks were assigned and similarity between all samples was > 0.999; and ⑤ In a PCA score plot all slice samples were clustered, while whole root samples were scattered. Therefore, our studies could provide basic data for optimizing the processing method, producing best quality Gentianae Macrophyllae Radix, and evaluating the quality of different ecotype varieties and the multiple origin of herbal medicines.

Objective: To investigate the gene expression characteristics of peripheral blood mononuclear cells from patients with high altitude pulmonary hypertension (HAPH) in Naxi residents living in Lijiang, Yunnan, and to explore the underlying pathogenesis and value for potential drug selection. Methods: This is a case-control study. Six patients with HPAH (HPAH group) and 4 normal subjects (control group) were selected from the Naxi residents who originally lived in Lijiang, Yunnan Province. The general clinical data of the two groups were collected, and the related indexes of pulmonary artery pressure were collected. Peripheral blood mononuclear cells of the subjects were collected for RNA sequencing. The differences on gene expression, regulatory network of transcription factors and drug similarity between the two groups were compared. The results were compared with the public data of idiopathic pulmonary arterial hypertension (IPAH). Biological processes and signal pathways were analyzed and compared between HPAH and IPAH patients. Results: The age of 6 patients with HAPH was (68.1±8.3) years old, and there were 2 males (2/6). The age of 4 subjects in the control group was (62.3±10.9) years old, and there were 2 males (2/4). Tricuspid regurgitation velocity, tricuspid pressure gradient and pulmonary systolic pressure in HAPH group were significantly higher than those in control group (all P<0.05). The results of RNA sequencing showed that compared with the control group, 174 genes were significantly upregulated and 169 genes were downregulated in peripheral blood mononuclear cells of HAPH group. These differentially expressed genes were associated with 220 biological processes, 52 molecular functions and 23 cell components. A total of 21 biological processes and 2 signal pathways differed between HPAH and IPAH groups, most of which were related to inflammation and immune response. ZNF384, SP1 and STAT3 were selected as highly correlated transcription factors by transcription factor prediction analysis. Trichostatin A and vorinostat were screened out as potential drugs for the treatment of HAPH by drug similarity analysis. Conclusions: There are significant differences in gene expression in peripheral blood monocytes between HAPH patients and normal population, and inflammation and immune dysfunction are the main pathogenic factors. Trichostatin A and Vorinostat are potential drugs for the treatment of HAPH.
Aged ; Altitude ; Altitude Sickness/genetics* ; Case-Control Studies ; China ; Familial Primary Pulmonary Hypertension/genetics* ; Humans ; Hydroxamic Acids/therapeutic use* ; Hypertension, Pulmonary/genetics* ; Inflammation ; Leukocytes, Mononuclear/pathology* ; Male ; Middle Aged ; Transcription Factors ; Transcriptome/genetics* ; Vorinostat/therapeutic use*

Objective ::To investigate the role of trehalose in hepatic ischemia-reperfusion injury and its underlying mechanisms.Methods:C57BL/6J mice were randomly divided into no-ischemia group, ischemia-reperfusion group, trehalose-treated group and normal saline control group. After ischemia for 90 minutes, reperfusion immediately or 6h, blood and liver tissues were collected, and serum was separated. The liver function parameters of ALT, AST, the inflammatory factors of TNF-α, IL-1β and IL-2, and the pathological changes of liver were detected to study the role of trehalose during hepatic ischemia-reperfusion injury. Hypoxia-reoxygenation cell model was established by AML12 mouse hepatocyte line, and divided into experimental group and control group. The experimental group was divided into low dose group and high dose group according to the concentration of trehalose administrated. And the control group had no use of trehalose. The level of apoptosis was measured to study the effect of trehalose on apoptosis induced by hepatic ischemia-reperfusion injury with flow cytometry. Western blot was utilized for detecting the levels of Caspase-3, Cleaved Caspase-3 and Bcl-2 protein to understand the molecular mechanisms of trehalose in apoptosis during hepatic ischemia-reperfusion injury.Results:In vivo animal experiments showed that liver function and such inflammatory factors as ALT, AST, TNF-α, IL-1β and IL-2 increased in ischemia-reperfusion group after hepatic ischemia-reperfusion ( P<0.05), and liver tissue became necrotic. After a treatment of trehalose, the levels of ALT, AST, TNF-α, IL-1β and IL-2 were lower than those of normalsaline control group and the area of liver tissue necrosis also decreased ( P<0.05). In vitro cell experiments showed that the apoptosis level of hepatocytes in the experimental group decreased compared with the control group.And the level of activated pro-apoptotic protein Cleaved Caspase-3 decreased, the level of anti-apoptotic protein Bcl-2 increased. Conclusions:Trehalose has protective effects on hepatic ischemia-reperfusion injury in vivo and in vitro. The mechanism may be involved in inhibiting inflammation induced by hepatic ischemia-reperfusion injury, suppressing the activation of Caspase-3 and promoting the expression of Bcl-2, thus played a protective role by extenuation of hepatocyteapoptosis.

Objective:To study the possible effect of soft tissue displacement in the surgical design of primary unilateral cleft lip repair.Methods:Charts of 52 unilateral cleft lip patients who got repaired from January 2014 to December 2015 in Peking Union Medical College Hospital were retrospectively reviewed. The nasolabial region measurements of these 52 cases during the lip surgery were especially collected. The distance between the key points in the upper nasolabial region were directly measured with a ruler and/or caliper. The cleft lips were repaired by rotation-advancement technique. The soft tissue along the cleft edge was sufficiently released, and the muscles were reconstructed.Results:Except the lip height and the distance between crista philtral point and subalar base on the noncleft side, most measurements between the key points on both sides got significant increase postoperatively ( P<0.05). The symmetrical ratio of the preoperative measurements on upper labial region were closing to 1 departing from the cleft region. The symmetrical ratio improved significantly postoperatively ( P<0.01). Conclusions:During the primary unilateral cleft lip surgery, the changes of the distances between the key points of upper nasaolabial region would not always follow the geometry. They are also affected by other factors, like the interaction between each layer and the elasticity of soft tissue, which should be considered and employed during the surgical design.

Advent of Internet hospitals has optimized resource allocation and reduced cost of medical services, yet it also brought along challenges to physicians scheduling. The challenges are found mostly in two aspects.First, diversified service channels of physicians and fragmentation of their service time, result in the difficulty of physicians scheduling, and secondly, massive information as faced by patients causes confusion for them in selection of appropriate physicians. Based on analysis of the present physician management in Internet hospitals and theories of healthcare operations, this paper proposed an improvement path from the perspectives of data-driven, service channel coordination, short-term service capability design and intelligent matching of physician-patient information.

OBJECTIVETo evaluate the short-term outcomes and complication management of submucosal tunneling endoscopic resection(STER) for esophageal submucosal tumors (SMTs) originating from the muscularis propria(MP) layer.

METHODSClinical data of 48 patients with esophageal SMTs from MP layer undergoing STER in the Department of Gastroenterology, the First People's Hospital of Xiangshan, Zhejiang, and the Endoscopy Center of Zhongshan Hospital, Fudan University, Shanghai between September 2013 and August 2014 were retrospectively analyzed. The clinicopathological features, complication management, and short-term outcomes were evaluated.

RESULTSAll the patients underwent STER successfully. The complete resection rate was 100%. The mean maximum diameter of the lesions was (22.9±12.1) mm (range 9.0-60.0 mm), and the mean operation time 41.8 min (range 15.0-140.0 min). Intraoperative mucosal injury occurred in 5 patients (10.4%), which was successfully clipped, pneumoperitoneum in 2 patients (4.2%) and subcutaneous emphysema in 3 patients(6.3%), which were successfully controlled with conservative treatments. Five patients (10.4%) had postoperative severe chest pain. Seven patients (14.6%) developed fever, among them, 5 were managed by conservative therapy, and 2 were submucosal tunnel infection, who were successfully treated after reclosing the ruptured tunnel entry with clips. All the removed tumors had tumor-free resection margins. The average length of postoperative hospital stay was 2.4 days (range 1-13 days). Local recurrence and distant metastasis did not occur during mean 6.8 months (range 2-12 months) follow up.

CONCLUSIONSSTER appears to be a safe and effective option for esophageal SMTs originating from MP layer. Common complications related to STER often can be successfully controlled with conservative treatments.

Obejective To evaluate the short-term outcomes and complication management of submucosal tunneling endoscopic resection (STER) for esophageal submucosal tumors (SMTs) originating from the muscularis propria (MP) layer. Methods Clinical data of 48 patients with esophageal SMTs from MP layer undergoing STER in the Department of Gastroenterology , the First People′s Hospital of Xiangshan, Zhejiang, and the Endoscopy Center of Zhongshan Hospital, Fudan University, Shanghai between September 2013 and August 2014 were retrospectively analyzed. The clinicopathological features, complication management, and short-term outcomes were evaluated. Results All the patients underwent STER successfully. The complete resection rate was 100%. The mean maximum diameter of the lesions was (22.9 ±12.1) mm (range 9.0-60.0 mm), and the mean operation time 41.8 min (range 15.0-140.0 min). Intraoperative mucosal injury occurred in 5 patients (10.4%), which was successfully clipped, pneumoperitoneum in 2 patients (4.2%) and subcutaneous emphysema in 3 patients (6.3%), which were successfully controlled with conservative treatments. Five patients (10.4%) had postoperative severe chest pain. Seven patients (14.6%) developed fever, among them, 5 were managed by conservative therapy, and 2 were submucosal tunnel infection, who were successfully treated after reclosing the ruptured tunnel entry with clips. All the removed tumors had tumor-free resection margins. The average length of postoperative hospital stay was 2.4 days (range 1-13 days). Local recurrence and distant metastasis did not occur during mean 6.8 months (range 2-12 months) follow up. Conclusions STER appears to be a safe and effective option for esophageal SMTs originating from MP layer. Common complications related to STER often can be successfully controlled with conservative treatments.