Objective To analyze the relationship of lipoprotein(a)[Lp(a)]with poor prognosis in chronic heart failure(CHF)patients aged over 80 years,and explore the influencing factors for poor prognosis in these patients.Methods A total of 135 over-80-year-old patients with acute de-compensated CHF admitted in Tianjin University Chest Hospital from June 2018 to August 2021 were enrolled in this study.With Lp(a)300 mg/L as the cut-off value,they were divided into higher Lp(a)group(73 cases)and lower Lp(a)group(62 cases).Their baseline clinical data and outcomes after 12 months of follow-up were collected.The primary endpoint was composite end-point including re-hospitalization due to CHF and/or all-cause mortality.Kaplan-Meier survival curve were plotted to compare the survival rates between the two groups.Logistic regression anal-ysis was employed to analyze the risk factors of poor prognosis in these patients.Results The higher Lp(a)group had larger proportion of chronic kidney disease and higher levels of homocys-teine,total cholesterol and low-density lipoprotein-cholesterol than the lower Lp(a)group(P＜0.05).Kaplan-Meier survival analysis showed that the lower group obtained significantly longer 1-year survival time without primary endpoint events than the higher group[9.8 months(95％CI:8.884-10.665)vs 8.2 months(95％CI:7.057-9.272),P＜0.05].Multivariate logistic regression analysis revealed that Lp(a)≥300 mg/L(OR=2.841,95％CI:1.133-7.092,P=0.026),female(OR=2.809,95％CI:1.111-7.092,P=0.029)and use of diuretic(OR=4.631,95％CI:1.103-19.443,P=0.036)were independent risk factors for primary endpoint events within 1 year.Con-clusion Lp(a)≥300 mg/L is an independent risk factor for re-hospitalization due to CHF and/or all-cause mortality in elderly CHF patients within 1 year after discharge.

Objective:To explore the possible effects of Bushen Huoxue Formula (the kidney tonifying and blood activating prescription) on the proliferation and extracellular matrix synthesis of nucleus pulposus cells by regulating the circ_0036763/miR-583 axis.Methods:Real time fluorescence quantitative polymerase chain reaction (qRT-PCR) was used to detect the expression levels of circ0036763 and miR-583 in normal and intervertebral disc degeneration (IDD) nucleus pulposus cells; IDD nucleus pulposus cells were divided into pcDNA group, pcDNA circ_0036763 group, pcDNA circ_0036763+ mimic NC group, and pcDNA circ_0036763+ miR-583 mimic group. qRT-PCR was used to detect the expression levels of circ_0036763 and miR-583 in nucleus pulposus cells in each group, methyl thiazolyl tetrazolium (MTT) was used to detect cell proliferation (A value), and Western blot was used to detect the expression of proliferating cell nuclear antigen (PCNA), collagen Ⅰ, and collagen Ⅱ proteins in nucleus pulposus cells, The dual luciferase assay reported experimental validation of the targeting relationship between circ_0036763 and miR-583. 27 mice were divided into sham surgery group, IDD group, and kidney tonifying and blood activating formula group. IDD models were established in all groups except for the sham surgery group. After successful modeling, the sham surgery group and IDD group were given physiological saline by gavage, while the kidney tonifying and blood activating formula group was given 1.5 g/ml of kidney tonifying and blood activating formula by gavage for 3 consecutive weeks. QRT-PCR was used to detect the expression levels of circ0036763 and miR-583 in the nucleus pulposus cells of mice in each group, MTT was used to detect cell proliferation, and Western blot was used to detect the expression of PCNA, collagen Ⅰ, and collagen Ⅱ proteins.Results:The expression level of circ_0036763 in IDD nucleus pulposus cells decreased, while the expression level of miR-583 increased (all P<0.05); Overexpression of circ_0036763 can promote proliferation and extracellular matrix synthesis of nucleus pulposus cells (all P<0.05); Circ_0036763 targets miR-583 and upregulates miR-583 reversible overexpression. Circ_0036763 enhances the proliferation and extracellular matrix synthesis ability of IDD nucleus pulposus cells. Compared with the sham surgery group, the IDD group showed an increase in collagen Ⅰ protein expression and miR-583 expression levels (all P<0.05), while the cell A value, PCNA and collagen Ⅱ protein expression, and circ_0036763 expression levels decreased (all P<0.05); Compared with the IDD group, the Kidney Tonifying and Blood Activating Formula group showed a decrease in collagen Ⅰ protein expression and miR-583 expression levels (all P<0.05), while the cell A value, PCNA and collagen Ⅱ protein expression, and circ_0036763 expression levels increased (all P<0.05). Conclusions:The kidney tonifying and blood activating formula (Bushen Huoxue) may induce proliferation and extracellular matrix synthesis of nucleus pulposus cells by regulating the circ_0036763/miR-583 axis.

Objective:To investigate the relation between rs2298771 genotype in voltage-gated sodium channels 1A ( SCN1A) polymorphism and antiepileptic drug (AED) response in children with epilepsy. Methods:Sixty-two children with epilepsy admitted to Department of Neurology, Zhangjiakou First Hospital from June 2022 to December 2023 were divided into AED response group and AED resistance group ( n=31) according to their response to AED. In addition, 31 children with pharyngitis or mild gastroenteritis admitted to Department of Pediatrics at the same period were selected as control group. Real-time quantitative reverse transcription polymerase chain reaction (qRT-PCR) was used to analyze the rs2298771 genotype in SCN1A polymorphism, and differences in rs2298771 genotype and allele in SCN1A polymorphism were compared among the 3 groups. Relation between rs2298771 genotype in SCN1A polymorphism and AED response was analyzed. Multivariate Logistic regression was used to analyze the influencing factors for AED response in children with epilepsy. Results:(1) Significant differences in type of first seizure and AEDs were noted between AED response group and AED resistance group ( P<0.05); compared with the AED resistance group, the AED response group had significantly lower seizure frequency, significantly longer duration after last seizure, and statistically higher proportions of children with normal EEG or with one kind of AED ( P?0.05). (2) Compared with the control group and AED response group, the AED resistance group had significantly higher rs2298771 GC genotype and G allele, and statistically lower rs2298771 AA genotype and A allele in SCN1A polymorphism ( P?0.05). (3) In the AED response group, rs2298771 AA and AG genotype in SCN1A polymorphism were positively correlated with levetiracetam ( P?0.05); in AED resistance group, rs2298771 AG genotype in SCN1A polymorphism was positively correlated with topiramate and valproic acid ( P<0.05). (4) Multivariate Logistic regression analysis showed that duration after last seizure ( OR=3.249, 95% CI=1.097-9.621, P=0.033), rs2298771 genotype in SCN1A polymorphism ( OR=9.660, 95% CI=4.680-19.970, P=0.011) and seizure frequency ( OR=0.160, 95% CI=0.032-0.804, P=0.026) were independent influencing factors for AED response in children with epilepsy. Conclusion:Epilepsy children with shorter duration after last seizure, rs2298771 GG genotype in SCN1A polymorphism, and high seizure frequency are susceptible to AED resistance; especially, AG genotype is correlated with topiramate and valproic acid.

Objective:To explore the role of mechanical hemodynamic support (MHS) in mapping and catheter ablation of patients with hemodynamically unstable ventricular tachycardia (VT), report single-center experience in a cohort of consecutive patients receiving VT ablation during MHS therapy, and provide evidence-based medical evidence for clinical practice.Methods:This was a retrospective cohort study. Patients with hemodynamically unstable VT who underwent catheter ablation with MHS at Beijing Anzhen Hospital, Capital Medical University between August 2021 and December 2023 were included. Patients were divided into rescue group and preventive group according to the purpose of treatment. Their demographic data, periprocedural details, and clinical outcomes were collected and analyzed.Results:A total of 15 patients with hemodynamically unstable VT were included (8 patients in the rescue group and 7 patients in the preventive group). The acute procedure was successful in all patients. One patient in the rescue group had surgical left ventricular assist device (LVAD) implantation, remaining 14 patients received extracorporeal membrane oxygenation (ECMO) for circulation support. ECMO decannulation was performed in 12 patients due to clinical and hemodynamic stability, of which 6 patients were decannulation immediately after surgery and the remaining patients were decannulation at 2.0 (2.5) d after surgery. Two patients in the rescue group died during the index admission due to refractory heart failure and cerebral hemorrhage. During a median follow-up of 30 d (1 d to 12 months), one patient with LVAD had one episode of ventricular fibrillation at 6 months after discharge, and no further episodes of ventricular fibrillation and/or VT occurred after treatment with antiarrhythmic drugs. No malignant ventricular arrhythmia occurred in the remaining 12 patients who were followed up.Conclusions:MHS contributes to the successful completion of mapping and catheter ablation in patients with hemodynamically unstable VT, providing desirable hemodynamic status for emergency and elective conditions.

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 effects of thinned anterolateral thigh perforator flaps combined with finger splitting and webplasty in sequential treatment of degloving destructive wound of total hand.Methods:This study was a retrospective observational study. From January 2012 to January 2023, a total of 15 cases who met the inclusion criteria with degloving destructive wound of total hand were admitted to Ningbo No.6 Hospital, including 10 males and 5 females, aged 17-75 years. The wounds were all combined with exposed bones or tendon. Emergency debridement and vacuum sealing drainage were performed in all cases before flap transplantation in stage Ⅰ. After thorough debridement, the wound area was 11.0 cm×3.0 cm-23.0 cm×13.5 cm. One or both anterolateral thigh perforator flaps with size of 12.5 cm×5.0 cm-25.0 cm×15.5 cm were designed, cut, and thinned to repair the skin and soft tissue defects of the hand. The donor site was sutured directly or repaired with medium-thickness skin graft from the opposite thigh. As needed, the flap was reconstructed by finger splitting and webplasty once or more times every 3 months after stage Ⅰoperation. The survival and complications of flap and wound healing at the donor site were observed after stage Ⅰoperation. The appearance of flap, two-point discrimination distance, and hand function were observed during the follow-up. At the final follow-up, the function of the affected hand was evaluated by the trial standards for evaluation of partial function of upper extremity by the Hand Surgery Society of Chinese Medical Association.Results:After the operation of stage Ⅰ, all the flaps of 15 cases of patients survived completely, including 1 case that had arterial crisis of flap but survived completely after exploration and re-anastomosis of blood vessels; all the wounds at the donor site healed. During the follow-up period of 6 to 18 months after stage Ⅰ, the flap was slightly swollen, with a little pigmentation, and the two-point discrimination distance in the finger flap was 8-11 mm. The fingers could complete the basic life actions such as flexion, extension, pinch, and grip. At the final follow-up, 3 cases were excellent, 9 cases were good, and 3 cases were acceptable in function evaluation of the affected hand.Conclusions:For degloving destructive wound of total hand, free transplantation of one or both thinned anterolateral thigh perforator flaps is used for repair in stage Ⅰ, and finger splitting and webplasty are used to reconstruct the flaps in the later stage, which can basically restore the pinch and grip function of the affected hand that is required for daily life, and is worthy of clinical promotion.

In recent years,interest in exploring the potential of Blinatumomab for treatment of B-cell acute lymphoblastic leukemia(B-ALL)at earlier stages has grown.This is because Blinatumomab has shown promising results in the management of refractory/relapsed(R/R)B-ALL and minimal residual disease(MRD).Blinatumomab has shown comparable efficacy to that of conventional chemotherapy as an altern-ative to consolidation or intensive chemotherapy in pediatric B-ALL patients.Moreover,its use as consolidation therapy or in combination with chemotherapy/targeted therapy,especially in combination with third-generation tyrosine kinase inhibitors(TKIs),shows significant promise for improving prognosis for adult B-ALL patients.This may potentially reduce reliance on allogeneic hematopoietic stem cell trans-plantation(allo-HSCT)in Philadelphia chromosome-positive(Ph+)B-ALL patients.Moreover,Blinatumomab is safer,gentler,and more effect-ive than chemotherapy for older adult patients.Effective therapy options are not yet available for infants with KMT2A rearrangement B-ALL;however,preliminary research indicates that Blinatumomab may offer a breakthrough for this subgroup.In this article,we review progress in investigations of Blinatumomab use in newly diagnosed B-ALL patients.

Objective:To investigate the prognosis and related factors of ischemic cardiomyopathy.Methods:Clinical data of 271 patients with acute decompensated chronic heart failure admitted to the Cardiac Intensive Care Unit of Tianjin Chest Hospital from December 2019 to June 2022 were enrolled, including 135 cases with ischemic cardiomyopathy (ICM group) and 136 cases with non-ischemic cardiomyopathy (NICM group). Patients were followed up for 48 weeks; and the primary endpoint was all-cause death and/or readmission due to heart failure, the secondary end point was cardiogenic death. Kaplan-Meier survival curve was used to compare the difference in prognosis between the two groups. Univariate and multivariate Cox regression models were used to evaluate the factors influencing the primary and secondary end points of ischemic cardiomyopathy.Results:Compared with the NICM group, ICM group had higher proportion of patients with older age, fast pulse rate, high body mass index, comorbidities of hypertension, diabetes, chronic kidney disease, stroke or peripheral vascular disease; and greater left ventricular end-diastolic diameter, right ventricular end-diastolic anteroposterial diameter, pulmonary artery systolic blood pressure, and left ventricular ejection fraction (all P<0.05). Kaplan-Meier survival analysis showed that survival without primary endpoint (Log-rank P=0.009) and survival without secondary endpoint (Log-rank P=0.037) were lower in the ICM group than in the NICM group. Multivariate Cox regression analysis showed that elevated triglyceride (TG)/high density lipoprotein-cholesterol (HDL-C) ratio and elevated neutrophil percentage were independent risk factors for primary and secondary endpoint events in patients with ischemic cardiomyopathy. Conclusions:Patients with ischemic cardiomyopathy have lower survival rates than those with non-ischemic cardiomyopathy. High levels of TG/HDL-C ratio and neutrophil percentage are independent risk factors for poor prognosis in ischemic cardiomyopathy.

B-cell acute lymphoblastic leukemia(ALL)is a hematological malignancy.Blinatumomab,as a therapeutic target,targeting CD19,has demonstrated notable efficacy as a safe bridge to allogeneic hematopoietic stem cell transplantation(allo-HSCT)in patients with re-lapsed/refractory acute lymphoblastic leukemia(R/R B-ALL).In the treatment of newly diagnosed ALL,blinatumomab,when combined with chemotherapy or other immunotherapies,reduces toxicity while ensuring efficacy.Similarly,combining blinatumomab with second-or third-generation tyrosine kinase inhibitors(TKIs)for the treatment of Philadelphia chromosome--positive(Ph+)ALL holds promise for eliminating the need for subsequent allo-HSCT in patients.Disease relapse and long-term survival of patients are closely related to minimal residual dis-ease(MRD)at the conclusion of treatment,and blinatumomab contributes to enhancing the MRD conversion rate in patients with ALL,thus improving the long-term prognosis.This article comprehensively reviews the clinical trials and current progress of blinatumomab in various B-ALL patient groups.

Occult breast cancer is a rare disease with the primary symptom of enlarged ipsilateral axillary lymph node. At present,it is still difficult to diagnose occult breast cancer. The diagnosis is based on the core needle biopsy and following immunohistochemical staining for the enlarged lymph nodes. If the results support,the diagnosis can be confirmed after the exclusion of other primary tumors. The standard treatment of occult breast cancer is still the mastectomy and axillary lymph node dissection. Neoadjuvant chemotherapy is an independent factor affecting the prognosis,which is also recommended before the oper-ation. Chemotherapy,radiotherapy and endocrine therapy could be chosen based on immunohistochemical results after surgical treatment. The prognosis of occult breast cancer is similar to the other types of breast cancer at the same staging.