Objective To explore the role and mechanism of peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α) in remifentanil (REM) resistance to hepatic ischemia-reperfusion injury (HIRI). Methods Forty SD rats were randomly divided into sham surgery group, HIRI group, HIRI+REM group, HIRI+PGC-1α inhibitor SR-18292 (HIRI+SR-18292) group and HIRI+REM+SR-18292 group, 8 rats in each group. HIRI rat models were constructed using non-invasive arterial clip occlusion method, and REM or SR-18292 were intravenously injected before surgery. The liver function indicators and liver tissue adenosine triphosphate (ATP) levels in the serum of rats were detected by assay kits. The activity levels of mitochondrial respiratory chain complexes Ⅲ and Ⅳ (COX-Ⅲ, COX-Ⅳ) in rat liver tissue were assessed by colorimetric methods. The pathological changes in rat liver tissue were observed by hematoxylin-eosin staining. Reactive oxygen species (ROS) and oxidative stress-related indicators in rat liver tissue were measured using the fluorescent probe (DCFH-DA) method and colorimetric methods. The mitochondrial DNA (mtDNA) copies and the expression levels of PGC-1α, nuclear respiratory factor-1 (NRF-1) and mitochondrial transcription factor A (TFAM) messenger RNA (mRNA) in rat liver tissue were quantified by real-time fluorescent quantitative polymerase chain reaction (RT-qPCR). And the protein expression levels of PGC-1α, NRF-1 and TFAM in rat liver tissue were assessed by Western blotting. Results Compared with the sham group, rats in the HIRI group showed increased pathological scores and hepatic cell necrosis in liver tissue, elevated levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) in serum, and increased levels of ROS and malondialdehyde (MDA) in liver tissue. Additionally, there was a decrease in ATP content and the activity levels of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), COX-Ⅲ and COX-Ⅳ in liver tissue, as well as a decrease in mtDNA copies and the expression levels of PGC-1α, NRF-1 and TFAM mRNA and protein (all P<0.05). Compared with the HIRI group, rats in the HIRI+REM group exhibited decreased pathological scores and hepatic cell necrosis, reduced levels of serum ALT and AST, and decreased levels of ROS and MDA in liver tissue. There was also an increase in ATP content and the activity levels of SOD, GSH-Px, COX-Ⅲ and COX-Ⅳ in liver tissue, as well as an increase in mtDNA copies and the expression levels of PGC-1α, NRF-1 and TFAM mRNA and protein (all P<0.05). In contrast, rats in the HIRI+SR-18292 group showed increased pathological scores and hepatic cell necrosis, elevated levels of serum ALT and AST, and increased levels of ROS and MDA in liver tissue. There was a decrease in ATP content and the activity levels of SOD, GSH-Px, COX-Ⅲ and COX-Ⅳ in liver tissue, as well as a decrease in mtDNA copies and the expression levels of PGC-1α, NRF-1 and TFAM mRNA and protein (all P<0.05). Compared with the HIRI+REM group, rats in the HIRI+REM+SR-18292 group had increased pathological scores and hepatic cell necrosis, elevated levels of serum ALT and AST, and increased levels of ROS and MDA in liver tissue. There was a decrease in ATP content and the activity levels of SOD, GSH-Px, COX-Ⅲ and COX-Ⅳ in liver tissue, as well as a decrease in mtDNA copies and the expression levels of PGC-1α, NRF-1 and TFAM mRNA and protein (all P<0.05). Conclusions PGC-1α plays a role in regulating the process of REM resistance to HIRI by promoting mitochondrial biogenesis and reducing the levels of oxidative stress.

ObjectiveThrough qualitatively and quantitatively analysis of the differences in chemical composition between the combined decoction and single decoction of Huaganjian and comparison of their core efficacy， to explore the rationality of the flexible clinical application of Huaganjian compound preparations and single-flavored dispensing granules. MethodsUltra performance liquid chromatography-quadrupole-electrostatic field orbitrap high resolution mass spectrometry（UPLC-Q-Exactive Orbitrap MS） was used to qualitatively analyze the combined decoction and single decoction samples of Huaganjian， and meanwhile， the contents of four index components（geniposide， paeoniflorin， hesperidin and paeonol） were quantitatively analyzed by high performance liquid chromatography（HPLC）. Nonalcoholic fatty liver disease（NAFLD） rat model induced by high-fat diet was applied to compare the efficacy of combined decoction and single decoction of Huaganjian. A total of 30 male SD rats were randomly divided into the control group， model group， lovastatin group（1.8 mg·kg^-1）， combined decoction group（1.26 g·kg^-1） and single decoction group（1.18 g·kg^-1）. After successful modeling， lovastatin group， combined decoction group and single decoction group were given corresponding doses of drugs by intragastric administration every day， and the control group and model group were given equal amounts of normal saline by intragastric administration， after 4 weeks of administration， the serum and liver tissues were collected， and the contents of alanine aminotransferase（ALT）， aspartate aminotransferase（AST）， total cholesterol（TC）， triglyceride（TG）， low-density lipoprotein cholesterol（LDL-C） and high-density lipoprotein cholesterol（HDL-C） in serum of rats were detected， and the liver pathological examination was carried out by hematoxylin-eosin（HE） staining and oil red O staining， so as to compare differences of their efficacy. ResultsSeventy chemical components were initially identified and attributed from the lyophilized powder of the combined decoction and single decoction samples of Huaganjian， and there was no obvious difference in composition between the two. Further quantitative analysis showed that the contents of geniposide， paeoniflorin， hesperidin and paeonol in the combined decoction samples were significantly increased when compared with those of the single decoction samples（P<0.01）. The pharmacodynamic results showed that compared with the model group， both the combined and single decoction groups of Huaganjian could improve the liver index of NAFLD rats， reduce the serum levels of AST， ALT， TC， TG and LDL-C， increase the serum level of HDL-C， and ameliorate the pathological changes of liver cell steatosis and fat accumulation. However， there was no significant difference in pharmacodynamic effects between the combined decoction group and the single decoction group. ConclusionThere is no significant difference between the combined decoction and single decoction of Huaganjian in terms of chemical composition， but the contents of the four index components show significantly difference. Both of them can significantly improve the fat accumulation and liver function in NAFLD rats. This study provides a reference basis for the rational clinical application and evaluation of famous classical formula compound preparations and single-flavored dispensing granules.

ObjectiveTo establish a geographical origin identification model for Foeniculi Fructus from Haiyuan， providing a new technical reference for the protection of Haiyuan's geo-authentic medicinal materials and its designation as a national geographical indication agricultural product. MethodsSamples of Foeniculi Fructus were collected from eight producing areas， including Minqin （Gansu）， Bozhou （Anhui）， Qingdao （Shandong）， Dezhou （Shandong）， Urumqi （Xinjiang）， Nujiang （Yunnan）， Gutuo （Inner Mongolia）， and Haiyuan （Ningxia）. Gas chromatography-ion mobility spectrometry （GC-IMS） was used to detect the volatile organic compounds （VOCs） in samples from these geographic origins. VOCs were qualitatively analyzed through dual matching with the National Institute of Standards and Technology （NIST） mass spectral database and the IMS drift time database. Using the Reporter module and Gallery Plot visualization tools within the LAV analytical platform， VOC fingerprint profiles characterizing geographic origins were constructed. A non-targeted analytical strategy was adopted， and 97 VOCs detected via GC-IMS were subjected to principal component analysis （PCA） and partial least squares discriminant analysis （PLS-DA） based on their differential distribution patterns to construct an origin identification model for Foeniculi Fructus from Haiyuan region. Key discriminative markers were screened using variable importance in projection （VIP） values greater than 1. ResultsA total of 97 VOCs were identified， including alcohols， aldehydes， ketones， esters， organic acids， terpenoids， ethers， alkenes， and benzenes. The PLS-DA model， based on VOCs data obtained by GC-IMS， effectively distinguished Foeniculi Fructus in Haiyuan region from those of other origins. During cross-validation， the model achieved a prediction parameter （Q²） of 0.976 and a goodness-of-fit parameter （R²） of 0.936， with no overfitting observed in permutation testing. Twelve key flavor markers with VIP > 1 were identified as characteristic indicators of Haiyuan origin. ConclusionA stable and highly predictive origin identification model for Foeniculi Fructus from Haiyuan was successfully established using GC-IMS technology， PLS-DA， and VIP-based marker screening. This model provides a novel technical strategy for accurately distinguishing Foeniculi Fructus in Haiyuan region from other regional varieties and offers new technical support for its protection as a geo-authentic medicinal material and a nationally designated geographical indication agricultural product in China.

ObjectiveTo establish a geographical origin identification model for Foeniculi Fructus from Haiyuan， providing a new technical reference for the protection of Haiyuan's geo-authentic medicinal materials and its designation as a national geographical indication agricultural product. MethodsSamples of Foeniculi Fructus were collected from eight producing areas， including Minqin （Gansu）， Bozhou （Anhui）， Qingdao （Shandong）， Dezhou （Shandong）， Urumqi （Xinjiang）， Nujiang （Yunnan）， Gutuo （Inner Mongolia）， and Haiyuan （Ningxia）. Gas chromatography-ion mobility spectrometry （GC-IMS） was used to detect the volatile organic compounds （VOCs） in samples from these geographic origins. VOCs were qualitatively analyzed through dual matching with the National Institute of Standards and Technology （NIST） mass spectral database and the IMS drift time database. Using the Reporter module and Gallery Plot visualization tools within the LAV analytical platform， VOC fingerprint profiles characterizing geographic origins were constructed. A non-targeted analytical strategy was adopted， and 97 VOCs detected via GC-IMS were subjected to principal component analysis （PCA） and partial least squares discriminant analysis （PLS-DA） based on their differential distribution patterns to construct an origin identification model for Foeniculi Fructus from Haiyuan region. Key discriminative markers were screened using variable importance in projection （VIP） values greater than 1. ResultsA total of 97 VOCs were identified， including alcohols， aldehydes， ketones， esters， organic acids， terpenoids， ethers， alkenes， and benzenes. The PLS-DA model， based on VOCs data obtained by GC-IMS， effectively distinguished Foeniculi Fructus in Haiyuan region from those of other origins. During cross-validation， the model achieved a prediction parameter （Q²） of 0.976 and a goodness-of-fit parameter （R²） of 0.936， with no overfitting observed in permutation testing. Twelve key flavor markers with VIP > 1 were identified as characteristic indicators of Haiyuan origin. ConclusionA stable and highly predictive origin identification model for Foeniculi Fructus from Haiyuan was successfully established using GC-IMS technology， PLS-DA， and VIP-based marker screening. This model provides a novel technical strategy for accurately distinguishing Foeniculi Fructus in Haiyuan region from other regional varieties and offers new technical support for its protection as a geo-authentic medicinal material and a nationally designated geographical indication agricultural product in China.

Objective:To retrieve, evaluate and integrate the evidence related to the preparation process for initiating extracorporeal cardiopulmonary resuscitation in hospital, so as to provide reference for clinical implementation of extracorporeal cardiopulmonary resuscitation.Methods:According to the evidence-based nursing method and the 6S evidence model, guidelines, clinical decisions, expert consensus, systematic review and other literatures related to the preparation process for initiating extracorporeal cardiopulmonary resuscitation in hospital were searched from National Guideline Clearinghouse, Scottish Intercollegiate Guidelines Network, National Institute for Health and Care Excellence, and other websites, UpToDate, The Cochrane Library, PubMed, Embase, CNKI, Wanfang and other databases. The retrieval date limit was from the establishment of the database to May 20, 2023. Researchers assessed the quality of the included articles, and extracted and summarized the evidence that met the quality standards.Results:A total of 11 articles were included, including 2 guidelines, 6 expert consensuses, 1 systematic review and 2 quasi-experimental studies. A total of 18 pieces of evidences were summarized from 6 aspects, including medical conditions, team building, materials management, operation mechanism, pre-initiating treatment and initiating judgment.Conclusions:This study summarizes the evidence of preparation process for initiating extracorporeal cardiopulmonary resuscitation in hospital, which can provide reference for promoting the implementation of extracorporeal cardiopulmonary resuscitation. Future studies still need to focus on team building, personnel training and assessment, and optimisation of the management system, so as to improve the efficiency and readiness of treatment.

Objective To investigate the role and mechanism of silent information regulator 1(SIRT1)-NOD-like receptor protein 3(NLRP3)axis in the effect of remifentanil against ischemia-reperfusion injury(IRI)in rat livers.SD rats were randomly divided into sham operation group(sham group),IRI group,IRI+remifentanil pretreatment group(IRI+RPC group),IRI+SIRT1 inhibitor EX-527 group(IRI+EX-527 group)and IRI+RPC+EX-527 group,with 8 rats in each group.The levels of serum alanine aminotransferase(ALT),aspartate aminotransferase(AST),lactate dehydrogenase(LDH),interleukin(IL)-1β and IL-18 of rats in each group were detected.The liver tissue pathology was observed.The apoptosis rate of hepatocytes in rats was detected.The expressions of SIRT1,NLRP3,cleaved cysteinyl aspartate specific proteinase-1(Cleaved Caspase-1)and Gasdermin D(GSDMD)proteins in rat liver tissue were detected.Results Compared with the sham group,the liver tissue pathological score and hepatocyte apoptosis rate of rats in the IRI group were increased,the serum ALT,AST,LDH,IL-1β,and IL-18 levels were increased,the relative expression of SIRT1 protein in liver tissue was decreased,and the relative expression of NLRP3,Cleaved Caspase-1,and GSDMD proteins were increased(all P<0.05).Compared with the IRI group,the liver tissue pathological score and hepatocyte apoptosis rate of rats in the IRI+RPC group were decreased,the serum ALT,AST,LDH,IL-1β,and IL-18 levels were decreased,the relative expression of SIRT1 protein in liver tissue was increased,and the relative expression of NLRP3,Cleaved Caspase-1,and GSDMD proteins were decreased;the liver tissue pathological score and hepatocyte apoptosis rate of rats in the IRI+EX-527 group were increased,the ALT,AST,LDH,IL-1β,and IL-18 levels were increased,the relative expression of SIRT1 protein in liver tissue was decreased,and the relative expression of NLRP3,Cleaved Caspase-1,and GSDMD proteins were increased(all P<0.05).Compared with the IRI+RPC group,the liver tissue pathological score and hepatocyte apoptosis rate in the IRI+RPC+EX-527 group were increased,the levels of ALT,AST,LDH,IL-1β,and IL-18 were increased,the relative expression of SIRT1 protein in liver tissue was decreased,and the relative expression of NLRP3,Cleaved Caspase-1,and GSDMD proteins were increased(all P<0.05).Conclusions SIRT1 may participate in the regulation of remifentanil against rat liver IRI by inhibiting NLRP3 mediated cell pyroptosis.

Objective:To investigate the genetic, clinical, and post-treatment characteristics of patients with glycogen storage disease type Ⅲ (GSD Ⅲ).Methods:A retrospective cohort analysis was performed on the genetic and clinical data of 26 cases with GSD Ⅲ who visited the Children's Hospital affiliated with Fudan University from June 2017 to December 2023. The patients were divided into non-missense variation and missense variation groups according to the types of mutation in the AGL gene.The correlation between genotype and phenotype was analyzed. All patients were treated with uncooked cornstarch after diagnosis. The changes before and after treatment were compared in patients who underwent more than twelve months of follow-up. A P value of <0.05 was used to denote statistical significance. Results:Among the 26 cases enrolled, 13 were female and 13 were male, and the median age of diagnosis was 28 (6 to 134) months. A total of thirty-five different types of AGL gene variation were detected, with c.1735+1G＞T (9/52, 17.3%) as the hotspot variation. The common clinical manifestations were elevated aminotransferases (26/26, 100%), hepatomegaly (25/26, 96.2%), fasting hypoglycemia (25/26, 96.2%), hyperketonemia (16/18, 88.9%), hypertriglyceridemia (TG) (20/26, 76.9%), elevated CK (16/25, 64.0%), and an abnormal electrocardiogram (12/16, 75.0%). Four cases (15.4%) had symptoms of myopathy at diagnosis. Liver biopsy was performed in eighteen cases, among whom 83.3% (15/18) had liver fibrosis≥S2. The number of cases with elevated levels of CK ( P＝0.031) and ALT ( P＝0.038)was pronounced in the non-missense variation group compared to that in the missense variation group. There were no statistically significant differences in age, height, liver size, degree of fibrosis, fasting blood glucose (Glu) and TG ( P>0.05). The median follow-up time of 14 cases was 40.5 (20-73) months, with improvement in body stature, reduced liver size, decreased ALT and TG, and improved Glu. However, four (28.6%) cases had new myopathy symptoms with raised CK ( P<0.05) and with advancing age, increased ALT diminished while CK level elevated ( P<0.05). Conclusions:The common clinical manifestations at the early stage of the GSD Ⅲdiagnosis are elevated aminotransferases, hepatomegaly, fasting hypoglycemia, hyperketonemia, high triglycerides, elevated CK, and fibrotic liver in China. Myopathy symptoms may arise following uncooked cornstarch treatment; however, there is significant improvement in height, liver-related, and metabolic parameters.

Objective To investigate the influence of sodium bicarbonate Ringer's solution and compound Ringer's solution on patients'acid-base balance and liver and kidney function during controlled low central venous pressure(CLCVP)hepatectomy.Methods A total of 60 patients with primary liver cancer who underwent CLCVP hepatectomy in the Affiliated Cancer Hospital of Zhengzhou University from June 2020 to June 2022 were selected and randomly divided into the sodium bicarbonate Ringer's solution group and compound Ringer's solution group,with 30 patients in each group.Patients in the sodium bicarbonate Ringer's solution group were infused with sodium bicarbonate Ringer's solution,while patients in the compound Ringer's solution group were infused with compound Ringer's solution after anesthesia induction and before liver parenchymal disconnection.The pH value,base excess(BE)and blood lactic acid(Lac)levels were detected using the blood gas analyzer in the two groups before anesthesia induction(T,),at the beginning of surgery(T2),2 h during surgery(T3),4 h during surgery(T4),and at the end of surgery(T5),respectively.The levels of serum alanine aminotransferase(ALT),aspartate aminotransferase(AST),blood urea nitrogen(BUN),and serum creatinine(Scr)were detected using the automatic biochemical analyzer 24 h before and 24 h after surgery,respectively.The body mass,surgical time,intraoperative infusion volume,urine volume,changes in body acid-base balance during surgery,and changes in liver and kidney function before and after surgery between the two groups were compared.Results There was no statistically significant difference in body mass,surgical time,intraoperative infusion volume,and urine volume between the two groups of patients(P＞0.05).At T,,there was no statistically significant difference in pH value,BE and Lac levels between the two groups of patients(P＞0.05).Compared to T,,the pH value in the compound Ringer's solution group increased at T2 and decreased at T3,T4 and T5,while BE and Lac levels from T2 to T5 showed an increasing trend(P＜0.05);compared to T,,the pH value in the sodium bicarbonate Ringer's solution group increased at T2 and decreased at T3,BE and Lac levels increased at T2 and T3(P＜0.05),and there was no statistically significant difference in pH value,BE and Lac levels at T4 and T5 compared to T1(P＞0.05).At T5,the pH value in the sodium bicarbonate Ringer's solution group was significantly higher than that in the compound Ringer's solution group,while the BE and Lac levels were significantly lower than those in the compound Ringer's solution group(P＜0.05).There was no statisti-cally significant difference in the levels of ALT,AST,BUN and Scr 24 hours before surgery between the two groups of patients(P＞0.05);compared to 24 hours before surgery,there was no significant change in the levels of ALT,AST,BUN and Scr 24 hours after surgery(P＞0.05);there was no statistically significant difference in the levels of ALT,AST,BUN and Scr 24 hours after surgery between the two groups(P＞0.05).Conclusion Compared to compound Ringer's solution,sodium bicarbonate Ringer's solution has a smaller impact on the internal environment of the body during the CLCVP hepatectomy and can effectively maintain the stability of the body's acid-base balance.Both the two solutions have no significant impact on the patient's liver and kidney function.

Objective To observe the effect of cluster emergency nursing in treating patients with chlorfenapyr poisoning. Methods A retrospective analysis was performed for the treatment and nursing processes of 6 patients with chlorfenapyr poisoning. Results Among the 6 patients with chlorfenapyr poisoning, 5 cases were orally poisoned and one case was poisoned by respiratory tract and skin absorption of toxins. All of the 6 patients with chlorfenapyr poisoning were treated through the green channel for poisoning treatment, and multidisciplinary cooperation cluster treatment and nursing as well as toxicant detection were carried out. One patient was excluded because no chlorfenapyr component was detected in the toxicant detection; the remaining 5 patients had different degrees of fever, fatigue, nausea and vomiting and other symptoms in the early stage, and among them, 4 patients had high fever and aggravated degree of consciousness disturbance in the later stage of the disease, and died of ineffective treatment for 2 to 11 days after poisoning (one patient was out of contact and was expected to die), with a mortality rate of 80%. Conclusion There is no specific antidote for chlorfenapyr poisoning, and the mortality rate is extremely high. After poisoning, blood, urine and other specimens should be collected as soon as possible for toxic examination. At the same time, early gastric lavage (within 6 hours), intestinal adsorption, gastrointestinal catharsis, enema and other symptomatic treatments should be given. Continuous blood purification should be performed as soon as possible to remove blood toxins, and extracorporeal membrane oxygenation can be performed if conditions permit.

OBJECTIVE: To analyze variants of SMN gene in a Chinese pedigree affected with Spinal muscular atrophy (SMA). METHODS: A Chinese pedigree diagnosed at the Nanchang First Hospital in January 2020 was selected as the study subject. Peripheral blood samples were collected for the extraction of DNA. All exons of the SMN gene were detected by multiple ligation-dependent probe amplification (MLPA). Potential variants of the SMN gene were also detected by Whole exome sequencing (WES), and the result was verified by Sanger sequencing. cDNA extracted from fresh blood sample was used as a template to verify the location of variant on the SMN genes. RESULTS: The proband was found to harbor a heterozygous deletion of the SMN1 Exon7+Exon8, and a heterozygous c.81G>A variant. The SMN1 Exon7+Exon8 deletion was inherited from her father and grandmother, whilst the c.81G>A variant was inherited from her mother and maternal grandfather. Her aunt was also a carrier of the heterozygous deletion, while her paternal aunt, her husband, and their daughter were not. cDNA amplification and Sanger sequencing confirmed that the c.81G>A variant was located in the SMN1 gene. CONCLUSION MLPA combined with NGS and Sanger sequencing can identify compound heterozygous variants of the SMN gene in the SMA patients.
Female ; Humans ; Male ; DNA, Complementary ; East Asian People ; Fathers ; Mothers ; Muscular Atrophy, Spinal/diagnosis* ; Pedigree ; Survival of Motor Neuron 1 Protein/genetics*