Objective To explore the correlation between serum levels of retinol-binding protein(RBP)and β2-microglobulin(β2-MG)levels with the disease and disease outcome in patients with septic shock.Methods A total of 120 patients with sepsis admitted to Qinzhou Hospital of Traditional Chinese Medicine from November 2020 to November 2022 were selected as research objects,and divided into sepsis group(76 cases)and septic shock group(44 cases)according to the severity of the disease.A total of 96 healthy subjects were selected as control group.According to the 28-day disease outcome of sepsis shock group,10 patients died(worsening group)and 34 patients survived(outcome group).Serum RBP and β2-MG levels were detected by immunoturbidimetry within 24 h after admission.Serum RBP and β2-MG levels were compared among all groups.Receiver operating characteristic curve(ROC)was used to evaluate the predictive value of serum RBP and β2-MG on the disease outcome of patients with septic shock.Multivariate Logistic regression was used to analyze the related factors affecting the disease outcome of patients with septic shock.Results WBC count,procalcitonin,C reactive protein level,acute physiology and chronic health status score system Ⅱ score,se-quential organ failure assessment(SOFA)score in sepsis shock group were higher than those in sepsis group and control group,and platelet count was lower than those in sepsis group and control group,the difference was statistically significant(P＜0.05).Serum RBP and β2-MG levels in sepsis shock group were higher than those in sepsis group and control group,and the difference was statistically significant(P＜0.05).The levels of serum RBP and β2-MG in the worsening group were higher than those in the outcome group,and the differ-ence was statistically significant(P＜0.05).ROC curve analysis showed that the area under the curve of ser-um RBP and β2-MG combined to evaluate the outcome of septic shock disease was 0.910(95％CI:0.865-0.955).Multivariate Logistic regression analysis showed that serum RBP,β2-MG,C reactive protein and SO-FA score were all risk factors affecting the disease outcome of septic shock patients(P＜0.05).Conclusion The serum levels of RBP and β2-MG in patients with septic shock are increased,and the changes of both levels are related to the disease outcome.The combined application has a better predictive value for the disease outcome in patients with septic shock.

OBJECTIVE To analyze the chemical components that were the absorbed in blood and liver tissue of rats after intragastric administration of aqueous extract from Abrus cantoniensis， and to speculate its possible metabolic pathways， providing reference for basic analysis of pharmacological substance in A. cantoniensis. METHODS Male SD rats were randomly divided into A. cantoniensis group （0.63 g/kg， calculated by crude drug） and blank group； they were given relevant drug solution/ultrapure water intragastrically. After a single dose， plasma and liver samples of rats in each group were collected. UPLC-Q-TOF/MS technology was used to identify chemical components that were absorbed in the blood and liver tissue of rats. RESULTS Totally， 30 chemical constituents were identified from the water extracts of A. cantoniensis， including alkaloids， flavonoids， organic acids， iridoids （such as L-abrine， schaftoside， isoshaftoside）. Ten prototype components and nine metabolites （such as decarboxylation and sulfation metabolites of protocatechuic acid， reduced sulfated metabolites of p-hydroxybenzoic acid） were identified from plasma samples； six prototype components and five metabolites （such as sulfated metabolites of p-hydroxybenzoic acid， decarboxylation and sulfation metabolites of p-hydroxybenzoic acid） were identified from liver samples. The main metabolic pathways included hydroxylation， demethylation， methylation， sulfation， glucuronidation， etc. CONCLUSIONS Alkaloids， flavonoids and organic acids are the main components of the aqueous extract from A. cantoniensis that are absorbed into the blood and liver， their metabolism mainly involves hydroxylation，demethylation， and sulfation.

Objective:To analyze the positive detection rate, main genotypes of β-thalassemia in western region of Guangxi Zhuang Autonomous Region (referred to as Guangxi).Methods:Retrospective analysis of 26 189 individuals who underwent gene testing for thalassemia at the Affiliated Hospital of Youjiang Medical University for Nationalities from January 2013 to December 2019. Using the crossing breakpoint PCR (Gap-PCR) and reverse dot blot (RDB) techniques to detect Chinese common type of 7 kinds of α-thalassemia and 17 kinds of β-thalassemia genotypes, high-throughput sequencing(Sanger) was performed for suspected rare β-thalassemia. Gap-PCR was used for suspected deletion β-thalassemia types.Results:β-thalassemia was diagnosed in 4 495 (17.16%) of 26 189 samples. A total of 6 177 alleles of 20 types of β-thalassemia were detected, mainly CD17 (2 712 cases, 43.90%) and CD41-42 (2 240 cases, 36.26%), including 7 rare alleles: Gγ +( Aγδβ) 0, SEA-HPFH, Hb New York, Hb G-Taipei, Hb Hezhou, Hb G-Coushatta and IVS-Ⅱ-81. There were 3 903 case (86.83%) heterozygous, 273 case (6.07%) double heterozygous, and 319 case (7.10%) homozygous among 4 495 β-thalassaemia subjects. A total of 48 genotypes were detected. The two most common genotypes were CD17/β N (1 890 cases, 42.05%) and CD41-42/β N (1 212 cases, 26.96%), accounted for 69.01% (3 102/4 495). Seven rare genotypes were detected: Gγ +( Aγδβ) 0/β N in 3 cases, Hb New York/β N in 3 cases, Hb G-Taipei/β N in 2 cases, SEA-HPFH/β N, Hb Hezhou/β N, Hb G-Coushatta/β N and IVS-Ⅱ-81/β N in 1 case each. A total of 1 041 cases (3.97%, 1 041/26 189) of 116 types of αβ-thalassemia were detected, mainly -- SEA/αα composite CD17/β N (144 cases, 13.83%), followed by -α 3.7/αα composite CD17/β N (112 cases, 10.76%). Conclusions:Western region of Guangxi is a high prevalence area of β-thalassemia, CD17/β N and CD41-42/β N are the main genotypes. The variation spectrum of β-thalassemia is complex and diverse, with rich genotype.

Objective To investigate the clinical efficacy of percutaneous closure of foramen ovale guided by transthoracic echocardio-graphy with simply delivery sheath.Methods The clinical data of patients with patent foramen ovale underwent interventional closure and percutaneous closure guided by transthoracic echocardiography with simply delivery sheath in our hospital from January 2020 to December 2022 were analyzed retrospectively,the patients were divided into interventional closure group(40 cases)and simply delivery sheath group(39 cases).The operation time,incidence of surgical complications,and surgical success rates of patients in the two groups were compared.The closure effect were evaluated by ultrasound immediately after surgery.All the patients were followed up for 6 months after surgery to evaluate remission of the symptoms.Results The surgical success rate of simply delivery sheath group(100%)was higher than that of interventional closure group(90.0%),with statistically significant difference(P<0.05).The operation time of simply delivery sheath group was longer than that of interventional closure group,with statistically significant difference(P<0.05).One patient in the interventional closure group had small amount of pericardial effusion during the operation.Two patients had decreased blood pressure and slowed heart rate in simply delivery sheath group,and symptoms disappeared after treatment.There was no significant difference in the incidence of complications between the two groups(P>0.05).After 6-month follow-up,all occluders were in good position and no residual leakage was found.The symptoms of headache or dizziness disappeared in 28 patients in interventional closure group,significantly relieved in 8 patients;the symptoms of headache or dizziness disappeared in 30 patients in simply delivery sheath group,and significantly relieved in 9 patients.Conclusion Percutaneous closure for patent foramen ovale under the guidence of transthoracic echocardiography with simply delivery sheath is safe and feasible with satisfactory efficacy and higher successful rate without radiation hazard.It is worthy of clinical promotion.

Objective To screen Cuproptosis genes and characteristic genes for differential prognosis in acute mye-loid leukemia(AML)and explore their prognosis in AML as well as their biological roles and correlations in the immune and tumor microenvironment.Methods AML clinical,transcriptome,genomic,and copy number data were downloaded from three major databases,TCGA,GEO,and UCSC,and Cuproptosis genes were collected from published studies.From the perspective of multiomics,the effects of Cuproptosis gene and characteristic gene on survival,immunity,tumor microenvironment,stem cell correlation and drug sensitivity were studied by various bioinformatics methods,meta-analysis and secondary typing.Results One Cuproptosis gene was identified as a differential prognostic gene in AML and five characteristic genes were identified as influencing the prognosis of AML patients by influencing Cuproptosis,and a prognostic model was established.The differential genes were mainly concentrated in mitochondrial activity,REDOX enzyme and energy metabolism.In terms of immunity,macrophage M0,neutrophils,activated memory CD4 T cells and Tregs were positively correlated with risk score,while macro-phage M2,resting mast cells,immature CD4 T cells,helper follicular T cells and memory B cells were negatively correlated with risk score.In terms of tumor microenvironment,the immune cell score of the low-risk group was lower than that of the high-risk group,and in the total score,the tumor microenvironment score of the low-risk group was also lower than that of the high-risk group,indicating that the tumor purity of the high-risk group was lower than that of the low-risk group.However,there was no significant association between stem cells in the high-risk and low-risk groups,and a total of 14 drugs were found to be sensitive to treat AML.Conclusion Cuproptosis gene and characteristic gene are closely related to immune and tumor microenvironment in AML by constructing a prognostic model of AML.

Objective:To investigate the clinical characteristics and treatment of patients with perianal necrotizing fasciitis.Methods:This study was a retrospective cohort study. Twenty patients with perianal necrotizing fasciitis who met the inclusion criteria were admitted to the Department of Burn and Plastic Surgery of the First Affiliated Hospital of Guangxi Medical University (hereinafter referred to as our department) from August 2013 to September 2023, including 19 males and 1 female, aged 24-74 (56±11) years. Based on the spreading route of perianal infection to the lower abdomen, the patients were divided into perianal-inguinal-lower abdominal wall group (12 cases) and perianal-pelvic cavity-retroperitoneal group (8 cases). The following clinical data were compared between the two groups of patients: general data, including gender, age, combined underlying diseases, blood glucose level and acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score when admitted to our department, and laboratory risk indicator for necrotizing fasciitis (LRINEC) score when admitted to our department and at 14 d after admitted to our department; infection indicators when admitted to our department, including C-reactive protein level, white blood cell count, lymphocyte count, procalcitonin level, and lactic acid level; clinical outcome-related indicators, including time from onset to definite infection range, number of surgery, treatment in intensive care unit (ICU), length of hospital stay, treatment outcome, and recurrence of necrotizing fasciitis during follow-up; detection of pathogen and bacterial drug resistance in wound necrotic tissue specimen when admitted to our department.Results:Compared with those in perianal-inguinal-lower abdominal wall group, the APACHE Ⅱ score and lactic acid level when admitted to our department and LRINEC score at 14 d after admitted to our department (with t values of -5.98, -5.01, and -2.86, respectively, P<0.05) and ICU treatment ratio ( P<0.05) were significantly increased, the time from onset to definite infection range was significantly prolonged ( Z=-3.75, P<0.05), and the number of surgery was significantly increased ( Z=2.80, P<0.05) in patients in perianal-pelvic cavity-retroperitoneal group. There were no statistically significant differences in other data between the two groups of patients ( P>0.05). Eighteen patients were cured, and no recurrence of perianal necrotizing fasciitis was observed during follow-up of 6 months in 18 cured patients. The main bacteria were Escherichia coliand Klebsiella pneumoniae, and the fungui were Aspergillus and Candida albicans detected in wound necrotic tissue specimens in two groups of patients when admitted to our department. The ratio of multiple drug resistance of bacteria in wound necrotic tissue specimens in perianal-pelvic cavity-retroperitoneal group of patients was significantly higher than that in perianal-inguinal-lower abdominal wall group ( P<0.05). Conclusions:Perianal necrotizing fasciitis can spread to the lower abdomen through two routes: the perianal-inguinal-lower abdominal wall route and the perianal-pelvic cavity-retroperitoneal route. The latter is more insidious in disease progression and more challenging in treatment. Establishing a mechanism of multi-disciplinary team diagnosis and treatment can achieve the goal of early diagnosis and precise treatment of perianal necrotizing fasciitis.

Neurodevelopmental disorders in children have become a significant global public health concern,impacting child health worldwide.In China,the current intervention model for high-risk infants involves early diagnosis and early treatment.However,in recent years,overseas studies have explored novel preventive early intervention strategies for neurodevelopmental disorders in high-risk infants,achieving promising results.This article provides a comprehensive review of the optimal timing,methods,and intervention models of the preventive early intervention strategies for neurodevelopmental disorders in high-risk infants.The aim is to enhance the awareness and knowledge of healthcare professionals regarding preventive early intervention strategies for neurodevelopmental disorders in high-risk infants,facilitate clinical research and application of such interventions in China,and ultimately reduce the incidence of neurodevelopmental disorders in this high-risk population.[Chinese Journal of Contemporary Pediatrics,2024,26(3):297-301]

Humans ; Aortic Valve ; Aortic Valve Stenosis ; Calcinosis ; Signal Transduction

OBJECTIVE: To investigate the clinicopathological features of blastic plasmacytoid dendritic cell neoplasm (BPDCN). METHODS: A total of 13 cases of BPDCN diagnosed in Peking University First Hospital from January 2013 to March 2022 were collected. The clinical features, histopathological characteristics, immunophenotypes and prognosis of the patients were analyzed retrospectively, and the related literatures was reviewed as well. RESULTS: Among the 13 patients, 11 were male and 2 were female, with a median age of 62 years (ranging from 5 to 78 years). Among them, single organ involvement occurred in 5 cases, all of which presented with skin lesions. Two or more organs were involved in other 8 cases (single organ with bone marrow involved in 3 cases; skin, bone marrow and lymph node involved simultaneously in 3 cases; skin, bone marrow, lymph node and spleen involved simultaneously in 2 cases). Histopathologically, it was characterized by the proliferation of medium to large atypical blastic cells, which infiltrated the whole thickness of dermis. When involved, the bone marrow lesions mainly appeared in a diffuse pattern, while the lymph node structure was usually destroyed, and the red pulp of the affected spleen was diffusely invaded. Immunohistochemical staining showed that all the 13 cases were positive for CD4, CD56, and CD123 (13/13) in varying degrees. All the 9 cases expressed TCL1 (9/9). Variable expression of CD68 (KP1) (8/13), TdT (7/12), CD117 (2/6), and high Ki-67 proliferation index (40%~80%) were showed. The neoplastic cells lacked expressions of CD20, CD3, MPO, CD34, or CD30; EBER in situ hybridization were negative (0/9). After definite diagnosis, 6 cases received chemotherapy, among which 1 received adjuvant radiotherapy, and 2 received subsequent bone marrow transplantation. Another 2 cases only received maintenance treatment. The median follow-up time was 14 months (ranging from 6 to 36 months), 5 patients died of the disease (6 to 18 months), 3 patients survived (7 to 36 months up to now), and the remaining 5 patients lost follow-up. CONCLUSION BPDCN is a rare type of malignant lymphohematopoietic tumor with aggressive behavior and poor prognosis. The diagnosis should be made combining clinical features, histopathology, and immunohistochemical phenotype. Attention should be paid to differentiating BPDCN from other neoplasms with blastoid morphology or CD4+CD56+ tumors.
Male ; Female ; Humans ; Hematologic Neoplasms ; Retrospective Studies ; Dendritic Cells ; Skin Neoplasms/pathology* ; Skin/pathology*

Objective: To evaluate the efficacy and safety of hybutimibe monotherapy or in combination with atorvastatin in the treatment of primary hypercholesterolemia. Methods: This was a multicenter, randomized, double-blind, double-dummy, parallel-controlled phase Ⅲ clinical trial of patients with untreated primary hypercholesterolemia from 41 centers in China between August 2015 and April 2019. Patients were randomly assigned, at a ratio of 1∶1∶1∶1∶1∶1, to the atorvastatin 10 mg group (group A), hybutimibe 20 mg group (group B), hybutimibe 20 mg plus atorvastatin 10 mg group (group C), hybutimibe 10 mg group (group D), hybutimibe 10 mg plus atorvastatin 10 mg group (group E), and placebo group (group F). After a dietary run-in period for at least 4 weeks, all patients were administered orally once a day according to their groups. The treatment period was 12 weeks after the first dose of the study drug, and efficacy and safety were evaluated at weeks 2, 4, 8, and 12. After the treatment period, patients voluntarily entered the long-term safety evaluation period and continued the assigned treatment (those in group F were randomly assigned to group B or D), with 40 weeks' observation. The primary endpoint was the percent change in low density lipoprotein cholesterol (LDL-C) from baseline at week 12. Secondary endpoints included the percent changes in high density lipoprotein cholesterol (HDL-C), triglyceride (TG), apolipoprotein B (Apo B) at week 12 and changes of the four above-mentioned lipid indicators at weeks 18, 24, 38, and 52. Safety was evaluated during the whole treatment period. Results: Totally, 727 patients were included in the treatment period with a mean age of (55.0±9.3) years old, including 253 males. No statistical differences were observed among the groups in demographics, comorbidities, and baseline blood lipid levels. At week 12, the percent changes in LDL-C were significantly different among groups A to F (all P<0.01). Compared to atorvastatin alone, hybutimibe combined with atorvastatin could further improve LDL-C, TG, and Apo B (all P<0.05). Furthermore, there was no significant difference in percent changes in LDL-C at week 12 between group C and group E (P=0.991 7). During the long-term evaluation period, there were intergroup statistical differences in changes of LDL-C, TG and Apo B at 18, 24, 38, and 52 weeks from baseline among the statins group (group A), hybutimibe group (groups B, D, and F), and combination group (groups C and E) (all P<0.01), with the best effect observed in the combination group. The incidence of adverse events was 64.2% in the statins group, 61.7% in the hybutimibe group, and 71.0% in the combination group during the long-term evaluation period. No treatment-related serious adverse events or adverse events leading to death occurred during the 52-week study period. Conclusions: Hybutimibe combined with atorvastatin showed confirmatory efficacy in patients with untreated primary hypercholesterolemia, which could further enhance the efficacy on the basis of atorvastatin monotherapy, with a good overall safety profile.
Male ; Humans ; Middle Aged ; Atorvastatin/therapeutic use* ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use* ; Hypercholesterolemia/drug therapy* ; Cholesterol, LDL/therapeutic use* ; Anticholesteremic Agents/therapeutic use* ; Treatment Outcome ; Triglycerides ; Apolipoproteins B/therapeutic use* ; Double-Blind Method ; Pyrroles/therapeutic use*