Background Comorbid attention-deficit/hyperactivity disorder(ADHD)and emotional dysregulation may represent a distinct subtype of ADHD,which is characterized by an increased risk of anxiety or depressive disorder and a poor clinical prognosis,so research is urgently required to explore its unique executive functioning profile and clinical characteristics.However,there is limited research comparing the clinical symptoms and executive function in children with ADHD in terms of the presence or absence of emotional dysregulation.Objective To explore the executive function and clinical characteristics of ADHD children with emotional dysregulation.Methods From June 2020 to December 2023,118 children aged 7 to 12 with ADHD attending the Mental Health Center of West China Hospital,Sichuan University and fulfilling the Diagnostic and Statistical Manual of Mental Disorders,fifth edition(DSM-5)diagnostic criteria were enrolled.Children were classified into emotional dysregulation group(n=68)and non-emotional dysregulation group(n=50)based on the standard T-scores of Achenbach's Child Behavior Checklist(CBCL)-anxious/depressed,aggressive behavior and attention problems subscales.All children were then subjected to complete the Chinese version of Swanson Nolan and Pelham,Version IV Scale-parent form(SNAP-IV),Chinese Wechsler Intelligence Scale for Children(C-WISC),Weiss Functional Impairment Scale-Parent form(WFIRS-P)and 4 tests of the Cambridge Neuropsychological Test Automated Battery(CANTAB):①Stockings of Cambridge(SOC)testing spatial planning.②Intradimensional-extradimensional Set Shifting(IED)testing cognitive/attentional flexibility,adjusting the total errors across the task.③Spatial Working Memory(SWM)testing spatial working memory.④Rapid Visual Information Processing(RVP)testing sustained attention.Results The SNAP-IV Inattention,Hyperactivity/Impulsivity and Oppositional Defiant Disorder domain scores and total score were all higher in emotional dysregulation group compared with non-emotional dysregulation group(t=3.206,5.088,6.316,6.553,P<0.01).The WFIRS-P family,school learning,life skills,self-concept,social activities and risky activities domain scores and total score were all higher in emotional dysregulation group compared with non-emotional dysregulation group(t=6.074,4.406,4.143,3.984,6.575,6.662,8.254,P<0.01).In CANTAB,emotional dysregulation group made more total adjusted errors across the IED task compared with non-emotional dysregulation group(t=2.168,P<0.05).Conclusion Children with ADHD who exhibit emotional dysregulation have been observed to experience more severe core symptoms,impaired social functioning and poorer performance on tests assessing executive function,particularly in the area of cognitive flexibility.

Objective To investigate the effect of ultrasound-guided stellate ganglion block(SGB)on intraoperative fentanyl dosage in the patients undergoing open thyroidectomy.Methods A total of 70 patients with elective open thyroidectomy under general anesthesia in the Affiliated Hospital of North Sichuan Medical College from November 2021 to April 2022 were selected as the study subjects and divided into the SGB group(group S,n=35)and the control group(group C,n=33).The group S conducted ultrasound-guided SGB at 15 min before anesthetic induction(injection of 0.25％marcaine 6-8 mL),and group C conducted the stellate ganglion recognition under the ultrasound guidance in 15 min before anesthetic induction without conducting other operations.All patients all received the anesthesia induction and maintenance under the same BIS moni-toring.The fentanyl dosage,recovery time,anesthetic drugs dosage,fluid infusion amounts,bleeding volume,use rate of atropine and ephedrine,operation time and postoperative complications as well as the VAS scores in PACU 30 min,at postoperative 3,6,12,24 h were recorded.Results Compared with group C,the intraopera-tive amount of fentanyl in group S was significantly decreased[(247.9±65.4)μg vs.(295.7±61.5)μg,P=0.003].The propofol dosage,cisatracurium dosage,fluid infusion amounts,bleeding amounts,use rate of atro-pine and ephedrine,recovery time and incidence rate of complications had no statistical differences between the two groups(P＞0.05).The VAS scores at various time points in group S all were lower than those in group C(P＜0.05).Conclusion Ultrasound-guided SGB could reduce the fentanyl use amounts during operation in the patients with open thyroidectomy.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy, there is no standard treatment and the prognosis is very poor. Affiliated Zhongshan Hospital of Dalian University report a case of 85-year-old BPDCN male patient treated with DVT regimen (decitabine combined with Venetoclax and thalidomide) and achieved complete remission. The patient with skin nodules and the pathology diagnosed BPDCN, the next generation sequencing of skin nodules showed mutations of IDH2 and ASXL1. DVT (decitabine combined with Venetoclax and thalidomide) has significant efficacy with rapid and deep remission for BPDCN, and the adverse effects is less, especially suitable for elderly patients who cannot tolerate intense chemotherapy.

Objective:To investigate the clinical features and family gene mutation results of the adult onset Gaucher disease patient.Methods:The diagnosis and treatment of a patient with Gaucher disease who was admitted to the Affiliated Zhongshan Hospital of Dalian University in March 2020 were retrospectively analyzed, and the literature was reviewed.Results:The patient (the proband) was a 34-year-old female, the anemia, thrombocytopenia and splenomegalia were found in 2005. After regular physical examination, the decrease of leukocyte, red blood cell and platelet was gradually aggravated, and the progressive enlargement of the spleen was aggravated. She visited the clinic on March 19, 2020 due to worsening of fatigue. The physical examination revealed hepatomegaly, splenomegaly; blood routine examination showed pancytopenia, and bone marrow morphology showed a large number of Gaucher cells. Peripheral blood β- glucocerebrosidase (GBA) of the proband was 1.8 nmol·mg -1·h -1 (reference value 10-25 nmol·mg -1·h -1), and the GBA of her father, mother, brother, and daughter was normal. The second-generation sequencing results showed that the proband had a mutation in the BCL2 gene at locus c.127G>A p.A43T, with a mutation frequency of 49.53%. GBA gene testing showed that the proband had a heterozygous mutation at c.1448T>C (a pathogenic mutation), heterozygous mutations at c.1026A>G and c.1038C>T (homozygous mutations), and a heterozygous mutation at c.1075G>T (mutation of unknown clinical significance). The proband's father had c.1448T>C heterozygous mutation, and he was a carrier; the proband's mother had c.1075G>T heterozygous mutation; the proband's brother had no gene mutation; the proband's daughter had c.1448T>C heterozygous mutation, and she was a carrier. The diagnosis was type Ⅰ Gaucher disease (non-neuropathic). The patient was proposed to participate in the clinical trial of imiglucerase, and during the follow-up, the blood routine was relatively stable, and the spleen remained progressively enlarged. Conclusions:Gaucher disease is rare, which is easy to be misdiagnosed and underdiagnosed. It is necessary to be alert to the possibility of Gaucher disease when there are patients with unexplained hemocytopenia, liver and spleen enlargement and multiple organs involvement, and enzyme and genetic tests should be performed as soon as possible.

Objective:To assess the efficacy of parent training (PT) on attention deficit hyperactivity disorder (ADHD) symptoms, behavioral problems, and parenting stress in child with ADHD or ADHD symptoms.Methods:Randomized controlled trials of the effect of PT on ADHD symptoms in Chinese children with ADHD or ADHD symptoms were collected by PsychINFO, PubMed, EMBASE, Web of science, Scopus, China National Knowledge database, WanFang database, the China Science and Technology Journal Database and the China Biology Medicine disc. The quality of the included studies was evaluated by version 2 of the Cochrane tool for assessing risk of bias in randomized trial. Forest plots were drawn using Review Manager 5.4.1, sensitivity analyses of results were performed using Stata 18.0 to assess the stability of the results, and publication bias was assessed using funnel plots and Egger's test.Results:A total of 10 studies containing 571 subjects in the experimental group and 562 subjects in the control group met the inclusion exclusion criteria. Significant differences were observed in various measures, including overall ADHD symptoms (MD=-2.43, 95% CI: -3.95--0.91), inattention (MD=-1.10, 95% CI: -1.94--0.26), hyperactivity/impulsivity (SMD=-0.58, 95% CI: -0.87--0.28), overall conduct problems (SMD=-0.80, 95% CI: -1.05--0.55), conduct problem (SMD=-1.34, 95% CI: -2.31--0.37), learning problem (SMD=-0.81, 95% CI: -1.40--0.23), impulsivity/hyperactivity (SMD=-0.53, 95% CI: -0.78--0.29), hyperactivity index (SMD=-1.27, 95% CI: -1.99--0.55), parenting stress index (SMD=-0.69, 95% CI: -1.12--0.26), difficult child (MD=-1.96, 95% CI: -3.44--0.48), dysfunctional interaction (MD=-3.10, 95% CI: -4.43--1.78), and parenting distress (MD=-3.35, 95% CI: -4.59--2.12)(all P<0.05). However, no significant differences were observed in oppositional-defiant disorder, anxiety, or somatic problem. Conclusions:The initial findings suggested that PT was effective for Chinese children with ADHD or ADHD symptoms.The addition of PT appeared to improve core symptoms of ADHD (inattention and hyperactivity/impulsivity), conduct problems, and parental stress more effectively than routine care alone.

Objective:To explore the clinical application values of radiofrequency thermocoagulation (RF-TC) based on stereotactic electroencephalogram (SEEG) high-frequency oscillations (HFOs) analysis in patients with refractory epilepsy.Methods:Fourteen patients with refractory epilepsy treated with SEEG-guided RF-TC were selected from Department of Neurosurgery, PLA Western Theater Command General Hospital from August 2019 to December 2021. Automatic detection algorithm of Matlab was used to calculate the HFOs incidence in each montage, and the fitting curves of HFOs incidences were used to formulate the threshold of HFOs and delimit the HFOs regions (ripples and fast ripples). These patients were divided into non-seizure group and seizure group according to the prognoses 3 and 6 months after RF-TC. At the last follow-up, these patients were divided into good prognosis group and poor prognosis group according to Engel grading; the differences of ripple thermocoagulation rate and fast ripple thermocoagulation rate between the 2 groups were compared.Results:A total of 7,332 ripples and 1,144 fast ripples were detected in SEEG data from 14 patients. Six months after surgery, neurological dysfunction incidence was 14.3%, without permanent neurological dysfunction, intracranial infection, intracranial hemorrhage, or electrode equipment failure. Within 3 months of RF-TC, seizure-free rate was 71.4% (10/14), and fast ripple thermocoagulation rate in non-seizure group was significantly higher than that in seizure group ( P<0.05); within 6 months of RF-TC, seizure-free rate was 57.1% (8/14), and ripple thermocoagulation rate in non-seizure group was significantly higher than that in seizure group ( P<0.05). At last follow-up, 6 patients had good prognosis and 8 patients had poor prognosis; the ripple thermocoagulation rate in good prognosis group was significantly higher than that in poor prognosis ( P<0.05). Conclusions:HFOs can assist in designating epileptogenic regions. Patients with wider range of thermocoagulation ripples or fast ripples will have better short-term efficacy; patients with wider range ofthermocoagulation ripples will have better prognosis.

Hevin is one of the extracellular matrix proteins secreted by astrocytes. Under physiological conditions, Hevin plays an important role in synaptogenesis in the central nervous system (CNS); secreted protein, acidic and rich in cysteine (SPARC) is its homologue and antagonizes the synaptogenic effects of Hevin. In pathological conditions, the expressions of Hevin and SPARC are altered, suggesting their possible roles at synaptic reorganization in various disease process, such as brain injury, Alzheimer's disease, epilepsy and brain tumors; however, the specific mechanism is not totally understood yet. So this paper reviews the mechanism of Hevin/SPARC in CNS synaptogenesis, reorganization and diseases to provide ideas for further research.

Objective:To investigate the characteristics and change trends of electroencephalogram (EEG) in patients with drug resistant epilepsy (DRE) after vagus nerve stimulation (VNS).Methods:Twenty-five patients with DRE, admitted to our hospital from July 2016 to May 2019, were chosen; all patients accepted VNS and followed up for 12 months. Long range video EEG (VEEG) monitoring was performed before VNS, and 3, 6 and 12 months after VNS, and the tracing time of each monitoring was longer than 12 h. The EEG characteristics of these patients before and different times after VNS were analyzed.Results:In the VEEG monitoring before VNS, 25 patients showed sharp wave, spike wave, sharp slow wave, and compound spike slow wave in the interictal period; 3 patients (12%) could locate the brain region. The interictal EEG of 11 patients 3 months after VNS showed different degrees of improvement as compared with the preoperative one, which manifested as mixed rhythms: mono-spiking as sharp wave, sharp slow wave or spike wave; 8 patients had McHugh grading I-II. The interictal EEG of 18 patients 6 months after VNS showed different degrees of improvement as compared with the preoperative one; 11 patients had McHugh grading I-II. The interictal EEG of 21 patients 12 months after VNS showed different degrees of improvement as compared with the preoperative one; 15 patients had McHugh grading I-II.Conclusion:The EEG improvement effect of DRE patients after VNS is gradually improved with time; in some patients, the EEG improvement is earlier than improvement of clinical symptoms.

Objective:To investigate the characteristics and change trends of electroencephalogram (EEG) in patients with drug resistant epilepsy (DRE) after vagus nerve stimulation (VNS).Methods:Twenty-five patients with DRE, admitted to our hospital from July 2016 to May 2019, were chosen; all patients accepted VNS and followed up for 12 months. Long range video EEG (VEEG) monitoring was performed before VNS, and 3, 6 and 12 months after VNS, and the tracing time of each monitoring was longer than 12 h. The EEG characteristics of these patients before and different times after VNS were analyzed.Results:In the VEEG monitoring before VNS, 25 patients showed sharp wave, spike wave, sharp slow wave, and compound spike slow wave in the interictal period; 3 patients (12%) could locate the brain region. The interictal EEG of 11 patients 3 months after VNS showed different degrees of improvement as compared with the preoperative one, which manifested as mixed rhythms: mono-spiking as sharp wave, sharp slow wave or spike wave; 8 patients had McHugh grading I-II. The interictal EEG of 18 patients 6 months after VNS showed different degrees of improvement as compared with the preoperative one; 11 patients had McHugh grading I-II. The interictal EEG of 21 patients 12 months after VNS showed different degrees of improvement as compared with the preoperative one; 15 patients had McHugh grading I-II.Conclusion:The EEG improvement effect of DRE patients after VNS is gradually improved with time; in some patients, the EEG improvement is earlier than improvement of clinical symptoms.

Objective:This study aimed to compare the efficacy and safety of cladribine, smustine, etoposide, cyclophosphamide, and cytarabine (C+SCAV) and smustine, etoposide, cytarabine, and melphalan (SEAM) conditioning regimens in autologous stem cell transplantation (auto-HSCT) for non-Hodgkin’s lymphoma (NHL) .Methods:A retrospective analysis was conducted on 61 NHL patients who received auto-HSCT in the Department of Hematology, the First Affiliated Hospital of Suzhou University, from March 2018 to May 2021. The C + SCAV group and SEAM group had 19 and 42 patients, respectively.Results:① Among the 61 patients with NHL, 37 were male and 24 were female. The median age was 48 (21-66) years old. There were 19 cases in the C+SCAV group and 42 cases in the SEAM group. There was no significant difference in the baseline characteristics between the two groups ( P>0.05) . ② The median time to neutrophil and platelet engraftment in the C+SCAV cohort were 10 (8-15) days and 13 (9-22) days, respectively, which does not differ from the SEAM group ( P=0.103, P=0.403) . ③ No differences existed between the two groups in terms of survival. The 1-year progression-free survival (PFS) was (76.5±10.3) % for patients receiving C+SCAV and (78.4±6.8) % for those who received SEAM ( P=0.841) . The 1-year overall survival was 100.0% for the C+SCAV group and 95.2±3.3% for the SEAM group ( P=0.339) . ④The 1-year PFS of patients with complete remission in the C+SCAV group was similar to those who in the SEAM group [ (92.3±7.4) % vs (82.5±7.2) %, P=0.406]. ⑤ The incidence of non-hematological serious adverse events (≥ grade 3) in the C+SCAV group and SEAM group were 10.5% (2/19) and 40.5% (17/42) ( P=0.013) , the incidence of severe mucositis was 5.3% (1/19) and 31.0% (13/42) ( P=0.015) , and the incidence of severe infection (≥ grade 3) was 10.5% (2/19) and 19.0% (8/42) ( P=0.389) , respectively. Conclusion:C + SCAV conditioning regimen appeared to be no different from the SEAM regimen in terms of survival. It can lower the incidence of SAE and does not increase the risk of severe infection. As a result, it can be used as an alternative conditioning regimen for lymphoma patients undergoing auto-HSCT.