Neonatal hyperinsulinism/hyperammonemia syndrome is a genetic disease result from glutamate dehydrogenase gene mutations.The clinical manifestations are hypoglycemia,hyperinsulinemia and mild hyperammonemia.Hypoglycemia may occur quickly due to eating protein.It is a rare neonatal disease that was easily ignored or delayed diagnosis and treatment causing serious sequelae of nervous system.This review summarized pathogenesis,clinical manifestation and diagnosis of the disease.

Objective To observe the effects of epidural anesthesia and subarachnoid anesthesia on puerperants and fetus perioperatively undergoing cesarean delivery after epidural labor analgesia.Methods A retrospective cohort study was conducted to select and analyze the relevant clinical data of women who had epidural labor anal-gesia and were converted to cesarean delivery at Beijing Tiantan Hospital.According to the anesthesia method of cesarean section,the participants were divided into the epidural anesthesia(EA)group and the subarachnoid an-esthesia(SA)group.That were aimed to compare the effects of the two anesthesia methods on the mother and fe-tus perioperatively.Results Compared with women in the epidural anesthesia group,women in the subarachnoid anesthesia group had a better anesthesia outcome,visual analogue scale(VAS)scores,frequency(%)[VAS scores 0-3:5(25%)vs.14(70%);VAS scores 4-6:12(60%)vs.6(30%);and VAS scores 7-10:3(15%)vs.6(30%)],and higher perioperative complication rate.There was nonsignificance difference in perio-perative maternal bleeding,neonatal Apgar score,and neonatal umbilical artery blood gas analysis between the two groups.Conclusions In women with epidural analgesia undergoing cesarean section,the anesthesia effect of subarachnoid anesthesia is better than that of the epidural anesthesia.The anesthesiologist must pay attention to prevent complications in the perioperative period.

Objective:To explore the perceived stress and coping experience of spouses of elderly people with long-term disability and empty nest.Methods:Using phenomenological research methods, semi-structured interviews were conducted with 19 spouses of disabled elderly people in a community in Beijing from February to June 2023, and data analysis was conducted using Colaizzi's 7-step analysis method.Results:The perceived stress and coping experience of spouses of long-term disabled empty nest elderly people were summarized into three themes and ten sub-themes, namely, the dynamic and variable stress perception of spouses (stress changed synchronously with the state of disabled elderly people, stress changed in a "U" shape, and pressure increased in a progressive manner), the challenges for spouses in long-term care of disabled elderly people (difficult in unifying opinions and views, incomprehensible inner emotions, difficulty in ensuring their elderly care, and coexistence of multiple roles and identities) and positive coping experiences and strategies of spouses (actively seeking external support, respecting the subjective wishes of disabled elderly people, and improving dyadic coping abilities) .Conclusions:Community nursing staff should support and guide spouses who undertake care work based on changes in perceived stress characteristics of long-term disabled empty nest elderly people. At the same time, it is necessary to understand the various challenges they face in long-term care, draw on positive coping experiences of spouses, so as to provide a basis for building a friendly family support system for home-based elderly care.

Four compounds were isolated from the lateral roots of Aconitum carmichaeli by Al2O3,silica gel and preparative HPLC.Their structures were identified as:(13R,15S,19S)-13,15,19-triol-hetisan (1),fuziline (2),neoline (3) and beiwutine (4),by methods of NMR,MS spectroscopy as well as single crystal X-ray crystallography.Among them,compound 1 was considered as a new diterpenoid alkaloid.

Objective To develop and validate a prediction model for severe disability or death(SDD)in acute ischemic stroke(AIS)patients who underwent endovascular treatment(EVT).Methods Based on the dataset of ANGEL-ACT study who received EVT for AIS between november 2017 and march 2019,a retrospective analysis was performed on 1 677 patients,including 1 111 males and 566 females,aged ≥ 18 years.Patients were divided into two groups according to whether SDD occurred(mRS 5-6 scores 90 days after surgery):SDD group(n=478)and non-SDD group(n=1 199).Risk factors that might influence SDD after EVT in AIS patients were screened and analyzed by multifactorial analysis,LAS-SO regression,and RF-RFE methods.A nomogram was developed after evaluating the model performance and the execution of internal validation.Results SDD occurred in 380(28.1％)patients in the develop-ment cohort and 98(30.2％)patients in the validation cohort.Combining the three variable screening meth-ods,10 risk factors were selected for inclusion in the final model:age,NIHSS score,whether successful re-canalization,glucose level,hemoglobin,hematocrit,onset to puncture time,systolic blood pressure,AS-PECT score,and whether have treatment-related serious adverse events.A two-stage model means that model 1 contains pre-treatment variables(7 in total)and model 2 contains pre-treatment and post-treatment variables(10 in total).The area under the curve(AUC)of model 1 in the development cohort was 0.705(95％CI 0.674-0.736)and 0.731(95％CI 0.701-0.760)in model 2.Both models had good calibration with aslope of 1.000,and the decision curve analysis showed good clinical applicability.The results of the validation cohort were similar to those of the development cohort.Conclusion Age,admission NIHSS score,whether successful recanalization,admission glucose level,hemoglobin content,erythrocyte pressure volume,onset to puncture time,admission systolic blood pressure,ASPECT score,and whether have treat-ment-related serious adverse events are risk factors for SDD in patients with acute ischemic stroke.The two prediction models based on the above factors were used before and after endovascular treatment to predict SDD occurrence better.