Clinical practice guideline adaptation (hereinafter referred to as "guideline adaptation") is the consolidation and revision of existing high-quality guidelines so that the recommendations are better suited to the specific needs of different regions, thereby guiding optimal clinical practice. Currently, the guideline adaptations is increasing in number internationally, but their reporting quality still needs to be improved. In 2022, the RIGHT-Ad@pt guideline adaptation reporting checklist was released. It provides a detailed description of the guideline adaptation process and reporting content, which will significantly enhance the rigor, transparency, and standardization of guideline adaptations. This paper interprets and analyzes the 34 items on the checklist, with the aim of providing reference for guideline adapters to standardize the reporting process.

Liver transplantation, the only effective treatment for end stage liver disease, is characterized by complicated surgery, long surgery time, and high trauma. Patients may experience a variety of difficulties following surgery, including infection, abdominal bleeding and rejection, all of which directly affect the quality of rehabilitation. Enhanced Recovery After Surgery (ERAS), a novel perioperative management strategy, can effectively promote postoperative recovery of patients and has been extensively implemented in various fields of surgery. However, there are no scientific and universal ERAS protocols in the fields of liver transplantation in China. The first Consensus Recommendations of Enhanced Recovery for Liver Transplantation was issued by the International Liver Transplantation Society in December 2022, offering recommendations about ERAS strategies for liver transplantation recipients who receive deceased and living organ donations, and for living donors of liver transplantation. This paper provides a detailed interpretation of the key points to offer a practical reference for domestic liver transplantation perioperative ERAS management.

One of the critical pathogenic factors contributing to non-healing chronic wounds is the formation of bacterial biofilms. In light of this, exploring effective strategies for the prevention and control of biofilms to improve the prognosis of patients with chronic wounds has become a research focus in the field of wound care and treatment. This review aimed to provide an in-depth exploration of the concept of bacterial biofilms, their complex formation mechanisms, and how they promote persistent infection states within the context of chronic wounds, offering valuable references for the detection and treatment of these biofilms in chronic wounds.

A large proportion of patients with non-small cell lung cancer (NSCLC) are diagnosed with metastatic and incurable advanced lung cancer at the time of discovery, so these patients are given no surgical opportunity and have a low 5-year survival rate. In the era of immunotherapy, many kinds of immune checkpoint inhibitors (ICIs) have been approved as the first/second-line treatment for patients with advanced NSCLC with negative driving genes and have been combined with radiotherapy as an important treatment strategy for patients with advanced NSCLC. The innovative strategy of combining radiotherapy and immunotherapy has shown feasibility as supported by practical evidence in clinical research. A preclinical experiment of observing the immune mechanism at the molecular and cellular levels preliminarily revealed the interaction among tumor, radiation, and immune system. This paper briefly reviews the progress of combined radiotherapy and immunotherapy in the treatment of advanced NSCLC with negative drvier genes.

Objective:To investigate the clinical features, treatment, and prognosis of transient hyperammonemia of the newborn (THAN).Methods:Data of two infants with severe THAN admitted to the Department of Neonatology of Shanghai Children's Hospital in September 2021 and August 2022 were retrospectively investigated. Clinical data of confirmed THAN cases (blood ammonia>400 μmol/L) were collected from relevant literature retrieved from the Wanfang Database, China National Knowledge Infrastructure, Chinese Medical Journal Database, and PubMed up to July 2022. A descriptive method was used for statistical analysis.Results:A total of 24 cases were involved (two in the present study, and 22 in 12 retrieved articles), including 19 (79.2%) premature newborns and five term infants. The average birth weight was (2 237±608) g and the average onset time was 27 h (4-55 h) after birth. The early clinical symptoms included respiratory distress and hyporesponsiveness (drowsiness, lethargy, coma or hypotonia) in 18 cases (75.0%), metabolic acidosis in 11 cases (45.8%), hypocalcemia in seven cases (29.2%), pupil fixation/dilation in six cases (25.0%), convulsion in five cases (20.8%), apnea in three cases (12.5%) and sinus bradycardia in one case (4.2%). The serum ammonia levels were 1 422.8 μmol/L (547.2-4 494.1 μmol/L). Treatments included peritoneal dialysis plus exchange transfusion in eight cases (33.3%), exchange transfusion in seven cases (29.2%), continuous renal replacement therapy (CRRT) in four cases (16.7%), arginine in two cases (8.3%), peritoneal dialysis in two cases (8.3%), and CRRT+peritoneal dialysis in one case (4.2%). During follow-ups of four months (one month to six years), 13 cases (54.2%) showed no abnormalities in development; two (8.3%) had a neurodevelopmental delay, and six (25.0%) died. The follow-up of the other three cases (12.5%) were not reported in the literature.Conclusions:The early clinical manifestation of severe THAN is atypical. A good prognosis can be expected through early exclusion of possible hyperammonemia-related genetic metabolic diseases and lowering the serum ammonia level. Long-term follow-up is needed for neurological evaluation.

OBJECTIVE：To study the inhibi tory effects of genistein on the growth of human nasopharyngeal carcinoma. CNE 1 cells and predict its potential target. METHODS ：CCK-8 method was used to test the effects of 0（blank control ），12.5，25，50， 100，150 µmol/L genistein on the proliferation of CNE 1 cells after treated for 24，48，72 h. Flow cytometry was carried out to detect the effects of 0（blank control ），15，30，60 µmol/L genistein on the cell cycle and ap optosis of CNE 1 cells after treated for 24 h. Scratch test was used to investigate the effects of 0（blank control ）， 10， 20， 30 µmol/L genistein on themigration ability of CNE 1 cells after treated for 24 h. High （No.18210156） throughput sequencing was conducted to discover the differential genes in CNE 1 cells after treated with 0（blankcontrol），30 µmol/L genistein for 24 h. RT-qPCR assay was adopted to verify the mRNA expression of related differential genes in above trials. RESULTS ： Compared with blank control，12.5，25，50，100，150 µmol/L genistein sho wed significant inhibitory effect on the proliferation of CNE 1 cells（P＜ 0.01），in a concentration- time-effect manner ；15，30 µmol/L genistein could arrest CNE 1 cell cycle at G 0/G1 stage（P＜0.05 or P＜ 0.01）；30，60 µmol/L could arrest CNE 1 cell cycle at G 2/M stage and promoted cell apoptosis （P＜0.05 or P＜0.01）. 10，20，30 µmol/L genistein could significantly inhibit the migration ability of CNE 1 cells（padj＜0.01）. High throughput sequencing revealed a total of 2 271 differentialgenes（P＜0.05），1 154 of which were up-regulated while 1 117 of which were down-regulated ；8 potential target genes ，including p53，p21，STC2，FGF2，CDK6，CYCLIN D ，PI3K，AKT，were screened by cell experiment. After validated by RT-qPCR assay ，mRNA expression of p53，p21，STC2，FGF2，CDK6，CYCLIN D and AKT were significantly down-regulated（P＜0.05），which consistent with the sequencing results. CONCLUSIONS ：Genistein can effectively inhibit the growth of human nasopharyngeal carcinoma CNE 1 cells，the mechanism of which may associated with inhibiting the expression of mutant gene p53，restoring the function of wild-type P 53 protein and inhibiting the activity of PI 3K/Akt pathway.

Objective:To investigate the clinical and molecular genetic features of neonatal congenital Netherton syndrome (NS) caused by mutations in serine protease inhibitor of Kazal type 5 ( SPINK5) gene. Methods:This study retrospectively analyzed the clinical data of an NS neonate admitted to Shanghai Children's Hospital in November 2018. SPINK5 gene was analyzed using high-throughput sequencing and Sanger sequencing. Relevant articles were retrieved from various databases including China National Knowledge Infrastructure, Wanfang and PubMed, and the reported cases who were diagnosed as NS within two months after birth with SPINK5 gene sequencing results were reviewed. Clinical features, gene mutations, treatment and follow-up results of NS were summarized using descriptive statistical analysis. Results:The patient presented with diffuse erythema and desquamation, sparse hair and repeated infections shortly after birth. Laboratory tests revealed elevated IgE (111 IU/ml) and "invagination-like" change in the hair under optical microscope. SPINK5 gene analysis found that there were compound heterozygous mutations of c.2468dup (p.Lys824Glufs*4) and c.377_378del (p.Tys126*) in the child. The pedigree analysis found that the two mutations were respectively inherited from the father and the mother, which supported the diagnosis of NS caused by SPINK5 gene mutation. Though skin rash improved after comprehensive treatments including anti-infection therapy, gamma globulin injection and skincare, the patient suffered from recurrent infection and was discharged from the hospital after giving up treatment and died of infection at two months old. Eleven NS cases were retrieved from literature and altogether 12 cases were analyzed here. The most common clinical manifestations in the 12 patients were early skin diffuse erythema and desquamation (12/12), infection (8/12), dry hair (7/12), hypernatremia dehydration (7/12), high IgE (5/12), growth retardation (4/12), respiratory failure (3/12), atopic constitution (2/12), diarrhea (2/12), dysphagia (1/12), hypothermia (1/12), wheezing (1/12), hypertension (1/12), liver failure (1/12) and metabolic alkalosis (1/12). Conclusions:NS is caused by SPINK5 gene mutation with atypical manifestations in neonates. Neonates with diffuse erythema and desquamation of the skin, repeated infections, dry hair and especially with high blood IgE should be considered the possibility of NS. Genetic testing is conducive to early diagnosis, guiding treatment decisions and providing a basis for genetic counseling.

Objective:To investigate the clinicopathological and molecular features, diagnosis and differential diagnosis of TFE3-rearranged epithelioid hemangioendothelioma (EHE). Methods Two cases of TFE3-rearranged EHE arising from soft tissues, diagnosed by the Pathology Department of the First Affiliated Hospital of Nanjing Medical University from 2013 to 2020 were observed. EnVision method was used for immunophenotyping, fluorescence in situ hybridization (FISH) was used to test TFE3 gene rearrangements and WWTR1-CAMTA1 fusion gene,and next-generation sequencing (NGS) was used to delineate the fusion transcripts.Results:Details of these two cases were as follows: case 1, male, 51 years old, with tumor in the right temporal region; case 2, female, 42 years old, with tumor in the right neck. The tumors showed progressive painless enlargement. Grossly, the tumor of case 1 was multinodular with unclear boundary and grayish red cut surface, while the tumor of case 2, originating from a vein, appeared as a firm, tan mass within vessel wall. Microscopically, both tumors showed moderate cellularity and were consisted of plump, epithelioid, or histiocytoid cells with eosinophilic cytoplasm and mild-to-moderate nuclear pleomorphism. Most of the tumor cells were arranged in solid or alveolar growth patterns, while some tumor cells showed intraluminal papillary growth pattern in case 1 and anastomosing vascular channels and extramedullary hematopoiesis in case 2. Immunohistochemically, the tumor cells showed diffuse positivity for CD31, CD34, ERG, and TFE3. FISH revealed TFE3 break-apart signals in two cases, but WWTR1-CAMTA1 gene fusion was not detected. NGS identified YAP1 (exon1)-TFE3 (exon6) fusion gene in case 2. Clinical follow-up information was available in both cases for a follow-up period of 15 and 59 months respectively. Patient 1 had a relapse 22 months after surgery, and was currently alive with the tumor. Patient 2 remained disease-free.Conclusions:TFE3-rearranged EHE is a rare molecular subtype of EHE, with accompanying characteristic morphologic features. However the morphologic spectrum remains under-recognized, and more experience is needed. Immunohistochemical and molecular examinations are helpful for the diagnosis and differential diagnosis of the disease.

Extracellular vesicles (EVs) refer to bilayer membrane transport vesicles secreted by cells. EVs can take macromolecules from cells and transfer them to receptor cells. Among these macromolecular substances, the most studied are microRNAs (miRNAs). miRNA is non-coding RNA involved in the regulation of gene expression. It has been confirmed that there are different non-coding RNAs in mammalian follicular fluid EVs. EVs carrying miRNA can act as an alternative mechanism for autocrine and paracrine, affecting follicular development. This paper systematically introduced the kinds, characteristics and methods of isolation and identification of EVs, focusing on the effects of EVs and miRNAs on follicular development, including early follicular development, oocyte maturation, follicular dominance and effects on granulosa cell function. At the same time, the authors prospected the future research of EVs and microRNAs in follicular fluid, and provided ideas and directions for the research and application of EVs and miRNA functions in follicular fluid.
Animals ; Extracellular Vesicles ; metabolism ; Female ; Follicular Fluid ; chemistry ; Granulosa Cells ; drug effects ; MicroRNAs ; pharmacology ; Oogenesis ; drug effects

Objective To explore the effect of enriched environment on the level of NR2A and NR2B subunits of N-mehyl-D-aspartate (NMDA) receptors which belong to glutamate receptors with excitability at the 17th area of the visual cortex in amblyopia rats after the critical period,and to understand the possible mechanism of synaptic plasticity of the visual cortex in adult amblyopia rats.Methods Eighty Wistar rats were divided into normal group and experimental group by random number table.Right eyelids of all rats were sutured through the whole critical period in order to establish monocular deprivation (MD) amblyopia model.The rats in experimental group were divided into the amblyopia group,standard environment (SE) group and environmental enrichment (EE) group on P45 in random.The sutured right eyelids were opened on P46 in the SE group and EE group.All rats were sacrificed to get the 17th area of the left visual cortex on P60,P75 and P105.Three rats were used at different time points from each group.The Ⅰ-Ⅵ layers of the visual cortex area 17 were observed by using hematoxylin-eosin staining.The expression of NMDA-NR2A and NMDA-NR2B was detected by immunohistochemistry.Integrated optical density of NMDA-NR2A and NMDA-NR2B was detected by using special image analysis software (Image-Pro Plus 6.0).The use of animals complied with Regulation on the Managenment Experimental Ainimals from Shandong Eye Institute and Association for Research in Vision and Ophthalmology (ARVO).Results The positive expression of NMDA-NR2A and NMDA-NR2B were observed in the visual cortex.The positive cells were mostly round or elliptical and mainly expressed in cell membrane.The expression of NMDA-NR2A in P60,P75 and P105 from four groups had statistical differences (all at P＜0.05).There were less positive cells in amblyopia group and EE group than normal control group on P60,P75 and P105,while there were more positive cells in EE group than amblyopia group.Amblyopia can lead to reduced NMDA-NR2A expression in the visual cortex.The expression of NMDA-NR2A was stronger than that in the amblyopia group by intervention 15 days,30 days,and 60 days with the rich environments,but did not reach the normal level (all at P＜0.05).The expression of NMDA-NR2B in P60,P75 and P105 from four groups also had statistical difference (all at P＜0.05).There were more positive cells in amblyopia group than those in normal control group on P60,P75 and P105.There were more positive cells in EE group than normal control group on P60,while there were equal positive cells in EE group and normal control group on P75 and P105.Amblyopia can lead to increase NMDA-NR2B expression in the visual cortex.The expression of NMDA-NR2B was weaker than that in the amblyopia group by intervention 15 days with the rich environments,but did not reach the normal level (all at P＜ 0.05).The expression of NMDA-NR2B after intervention 30 days and 60 days reached the normal levels (all at P＞ 0.05).Conclusions The plasticity of visual cortex exists not only in the critical period but also after the critical period of visual development.EE,as a non-invasion method,can improve and recover the synaptic plasticity in visual cortex of adult rats by the expression of NMDA-NR2A and NMDA-NR2B.