Objective To investigate the current status of nurses'knowledge,attitude and practice regarding sleep management of critically ill children in pediatric ICU,and to analyze its impact factors.Methods A self-designed questionnaire on general information and a questionnaire on knowledge and practical behaviors of pediatric ICU nurses on child's sleep management were used.In March 2023,902 pediatric ICU nurses from 24 hospitals in China were surveyed using a convenient sampling method,and the impact factors were analyzed using multiple stepwise linear regression.Results 893 valid questionnaires were collected and the recovery rate of valid questionnaires was 99.00％.Nurses in pediatric ICU scored(33.71±7.76)in knowledge dimension,(37.38±4.86)in attitude dimension and(80.60±16.78)in practice dimension,with a total score of(151.78±24.27).The scores of knowledge and attitude,knowledge and practice,attitude and practice are all positively correlated(r=0.393,P＜0.001;r=0.495,P＜0.001;r=0.320,P＜0.001).The results of multiple stepwise linear regression analysis showed that gender,region,whether they had received sleep management training were the influencing factors of pediatric ICU nurses'total score of knowledge,attitude and practice towards children's sleep management(P＜0.05).Conclusion Nurses in pediatric ICU are positive about sleep management for critically ill children,but their knowledge and practice levels need to improve.Nursing managers should strengthen the theoretical knowledge and practical behavioral training of pediatric ICU nurses on child sleep management,develop scientific sleep management plans,and guide nurses to make reasonable evaluation and interventions to improve children's sleep quality.

Background: Insulin resistance (IR) is the key pathological basis of many metabolic disorders. Lack of asialoglycoprotein receptor 1 (ASGR1) decreased the serum lipid levels and reduced the risk of coronary artery disease. However, whether ASGR1 also participates in the regulatory network of insulin sensitivity and glucose metabolism remains unknown. Methods: The constructed ASGR1 knockout mice and ASGR1-/- HepG2 cell lines were used to establish the animal model of metabolic syndrome and the IR cell model by high-fat diet (HFD) or drug induction, respectively. Then we evaluated the glucose metabolism and insulin signaling in vivo and in vitro. Results: ASGR1 deficiency ameliorated systemic IR in mice fed with HFD, evidenced by improved insulin intolerance, serum insulin, and homeostasis model assessment of IR index, mainly contributed from increased insulin signaling in the liver, but not in muscle or adipose tissues. Meanwhile, the insulin signal transduction was significantly enhanced in ASGR1-/- HepG2 cells. By transcriptome analyses and comparison, those differentially expressed genes between ASGR1 null and wild type were enriched in the insulin signal pathway, particularly in phosphoinositide 3-kinase-AKT signaling. Notably, ASGR1 deficiency significantly reduced hepatic gluconeogenesis and glycogenolysis. Conclusion The ASGR1 deficiency was consequentially linked with improved hepatic insulin sensitivity under metabolic stress, hepatic IR was the core factor of systemic IR, and overcoming hepatic IR significantly relieved the systemic IR. It suggests that ASGR1 is a potential intervention target for improving systemic IR in metabolic disorders.

OBJECTIVE: To explore the clinical characteristics and genetic variants in a patient with adult ceroid lipofuscinosis neuronal type 7 (ACLN7). METHODS: A female patient diagnosed with ACLN7 in Henan Provincial People's Hospital in June 2021 was selected as the study subject. Clinical data, auxiliary examination and result of genetic testing were retrospectively analyzed. RESULTS: The patient, a 39-year-old female, has mainly presented progressive visual loss, epilepsy, cerebellar ataxia and mild cognitive decline. Neuroimaging analysis has revealed generalized brain atrophy, prominently cerebellum. Fundus photography has revealed retinitis pigmentosa. Ultrastructural skin examination has revealed granular lipofuscin deposits in the periglandular interstitial cells. Whole exome sequencing revealed that she has harbored compound heterozygous variants of the MSFD8 gene, namely c.1444C>T (p.R482*) and c.104G>A (p.R35Q). Among these, c.1444C>T (p.R482*) was a well established pathogenic variant, while c.104G>A (p.R35Q) was a missense variant unreported previously. Sanger sequencing confirmed that the daughter, son and elder brother of the proband have respectively carried heterozygous c.1444C>T (p.R482*), c.104G>A (p.R35Q), and c.104G>A (p.R35Q) variants of the same gene. The family has therefore fit with the autosomal recessive inheritance pattern of the CLN7. CONCLUSION Compared with previously reported cases, this patient has the latest onset of the disease with a non-lethal phenotype. Her clinical features have involved multiple systems. Cerebellar atrophy and fundus photography may be indicative of the diagnosis. The c.1444C>T (p.R482*) and c.104G>A (p.R35Q) compound heterozygous variants of the MFSD8 gene probably underlay the pathogenesis in this patient.
Male ; Female ; Humans ; Membrane Transport Proteins/genetics* ; Neuronal Ceroid-Lipofuscinoses/diagnosis* ; Retrospective Studies ; Atrophy ; Mutation

Objective To retrieve,appraise and integrate the relevant evidence of non-pharmacological sleep management measures in critically ill children.Methods We conducted a systematical search on the topic of non-pharmacological sleep management measures in critically ill children across multiple databases,including BMJ best practice,UpToDate,International Guidelines International Network,National Institute for Health and Care Excellence,Scottish Intercollegiate Guidelines Network,Registered Nurses'Association of Ontario,Joanna Briggs Institute Library,the American Academy of Sleep Medicine,International Pediatric Sleep Association,CNKI,WanFang database,VIP database,SinoMed,Cochrane Library,PubMed,Web of Science,and CINAHL.The literature retrieval time limit was from the establishment of the database to February 2023.The study design includes clinical practice guideline,evidence summary,clinical decision-making,expert consensus,and systematic review.The evidence was extracted and summarized according to the subject after the independent literature quality evaluation by 2 researchers.Results 10 relevant pieces of literature were identified,including 2 guidelines,1 consensus statement,4 systematic reviews,2 evidence summaries,and 1 top clinical decision.These sources provided a total of 28 pieces of evidence across 5 key themes,including sleep assessment,management principles and education,environmental management and sleep promotion.Conclusion This study summarizes the best available evidence on non-pharmacological sleep management measures in critically ill children.It is recommended that healthcare professionals should consider the clinical context when implementing evidence-based interventions,aiming to reduce nocturnal awakenings,extend sleep duration,and improve sleep quality in critically ill children.

Objective:To explore the effect of repetitive transcranial magnetic stimulation (rTMS) combined with cognitive behavioral therapies (CBT) on the cognitive function and alcohol craving in patients with alcohol dependence.Methods:From March 2019 to September 2021, a total of 150 patients with alcohol dependence were enrolled and randomly divided into rTMS treatment group (rTMS+ sham CBT, n=41), CBT treatment group (CBT+ sham rTMS, n=34), rTMS+ CBT treatment group( n=36) and control group (sham rTMS+ sham CBT, n=39). At baseline (before treatment), 2nd week, 8th week, 12th week and 24th week, alcohol dependence scale (ADS) was used to evaluate the degree of alcohol dependence, the obsessive compulsive drinking scale (OCDS) was used to assess patients' drinking craving, and Montreal cognitive assessment scale (MoCA) was used to assess the overall cognitive level of patients.SPSS 23.0 statistical software was used to compare the differences of ADS, OCDS and MoCA scale scores of the four groups by repeated measure ANOVA and simple effect analysis. Results:(1)The patients in the four groups were evaluated with ADS scale at baseline, 12th week and 24th week respectively.The interaction of group×time( F=1.279, P=0.279) and the main effect of group were not significant ( F=0.882, P=0.454), and the main effect of time was significant ( F=12.925, P<0.001) .Further simple effect analysis showed that the ADS score of rTMS+ CBT group was lower than that of baseline(14.48±5.70, 10.00±6.51) ( P=0.01) at 24th week.(2)Patients in the four groups were assessed with OCDs scale at baseline, 2nd week, 8th week, 12th week and 24th week, and the interaction of group×time was significant ( F=2.015, P=0.042). Further simple effect analysis showed that the OCDs scores of rTMS group and rTMS+ CBT group at each follow-up time node were lower than those at baseline period (all P<0.05). (3)Patients in the four groups were assessed with MoCA scale at baseline, 8th week, 12th week and 24th week, and the interaction of group×time was not significant ( F=1.660, P=0.106), and the main effect of group and the main effect of time were significant ( F=2.964, P=0.038; F=14.239, P<0.001). Further simple effect analysis showed that the score of MoCA scale in CBT group at the 24th week was higher than that at baseline (21.73±5.81, 24.60±3.98)( P=0.029), the score of MoCA scale in rTMS+ CBT group at the 24th week was higher than that at the 8th week (23.50±6.01, 25.95±2.87) ( P=0.006), and the score of MoCA scale in rTMS group at the 12th week was higher than that in control group (22.08±6.64, 26.64±2.46)( P=0.009). Conclusion:rTMS combined with CBT can be effective in improving alcohol craving and cognitive function in patients with alcohol dependence, and has a good long-term effect.

Objective:To evaluate the anterior expansion of sacral foramen and decompression of sacral plexus via the lateral-rectus approach (LRA) in the surgical treatment of sacral fractures complicated with sacral plexus injury.Methods:From January 2013 to June 2018, 11 patients were treated at Department of Orthopaedics, The Third Hospital Affiliated to Southern Medical University for obsolete sacral fractures complicated with sacral plexus injury. They were 8 males and 3 females, aged from 17 to 54 years (average, 38 years). According to the Denis classification, all the sacral fractures belonged to Denis Zone Ⅱ. According to British Medical Research Council (BMRC) grading system, the nerve injury was complete damage in 2 cases and partial damage in 9. The mean time from injury to surgery was 6 months (range, from 0.7 to 12.0 months). After the sacroiliac joint was exposed via the LRA, the lumbosacral trunk was exposed and released between iliac vessels and the iliopsoas. Next, the S1 foramen was expanded and the S1 nerve root was released after separation of the median sacral artery and the internal iliac artery. Reduction and fixation of the sacroiliac joint was carried out for patients with unstable sacral fracture. X-ray and CT examinations of the pelvis were performed to evaluate fracture healing and neurological function recovery postoperatively.Results:Of this cohort of 11 cases, operation succeeded in 10 but failed in one whose sacral fracture was found to have completely healed with the S1 foramina totally occluded. The surgical time averaged 110 min (range, from 70 to 220 min) and the blood loss 1, 100 mL (range, from 450 to 2, 800 mL). Postoperative X-ray and CT examinations showed that the sacral foramens were expanded significantly without any complications. The follow-up time averaged 18 months (range, from 12 months to 4 years). By the BMRC grading system at the last follow-up, the neural function was completely recovered in 5 cases, partially recovered in 4 cases and not recovered in one.Conclusion:Significant anterior expansion of sacral foramen and decompression of sacral plexus via the LRA is a viable and effective alternative for treatment of sacral fractures complicated with sacral plexus injury.

Objective:To explore the surgical method and clinical efficacy of lateral-rectus approach (LRA) in the treatment of fragility fractures of the pelvis (FFP) in the elderly.Methods:A retrospective study was conducted for elderly FFP patients from January 2011 to December 2019. All the pelvic fractures should be exposed and reduced through LRA and fixed with pelvic reconstruction plates and channel screws. Then, a total of 30 elderly patients were included in the study, including 9 males and 21 females, with an average age of 72.5 years (range, 60-87 years). Fractures were caused by car accident in 5 cases, by falling from high places in 8 cases and by tumbling in 17 cases. According to the FFP classification, there were 6 cases of type IIc (6/30, 20%), 22 cases of type III (22/30, 73.33%, of which 13 cases were type IIIa, 7 cases were type IIIb, and 2 cases were type IIIc), 2 cases of type IVa (2/30, 6.67%). Among that, 2 cases suffered ipsilateral acetabular fractures, 17 suffered medical diseases such as hypertension, coronary heart disease, diabetes, etc., and none suffered extremities fractures or other types of injuries. Surgical field was exposed retroperitoneally through LRA, combined with lower limb traction to reduce the anterior and posterior pelvis ring fractures. Then, the reductions were done in direct visions and verified through X-ray fluoroscopy. After that, the posterior ring was fixed with reconstruction plate and/or sacroiliac screws, and the anterior ring was fixed with reconstruction plate or INFIX. The quality of fracture reduction was evaluated by Matta score and the postoperative function was evaluated by Majeed score.Results:All the 30 patients were surgical treated through LRA. The time from injury to operation was 5 to 20 days, with an average of 9.9 days. The operating time ranged from 35 to 150 min (mean, 73.5 min) and the intraoperative bleeding ranged from 220 to 2 400 ml (mean, 586.7 ml). Fractures were successfully reduced under direct vision and the positions of the plates and screws were monitored through X-ray fluoroscopy during the operation. Postoperative X-rays and CT scans showed satisfactory reduction of the pelvic ring. According to Matta criteria, the quality of fracture reduction was evaluated as excellent in 18 cases, good in 6 cases, fair in 4 cases and poor in 2 cases. The overall excellent and good rate was 80% (24/30). All patients were followed up for 6-24 months, the fractures were all healed, and all fractures healed for 2-5 months, with an average of 3.5 months. At the last follow-up, Majeed scores were 86.1±6.11 (range, 66-92), including 21 excellent cases, 7 good cases and 2 fair cases, with an excellent and good rate of 93.3% (28/30). As for complications, 1 patient developed deep venous thrombosis of the affected lower limb, and the thrombosis disappeared after thrombolytic therapy. One patient had fat liquefaction in the incision, and the wound healed after intensive dressing change. After INFIX fixation, 2 patients showed numbness on the lateral thigh of the affected side, and the symptoms were relieved after symptomatic treatment. Four patients were found to have internal fixation screws loosening during follow-up, but still achieved good fracture healing.Conclusion:The entire hemipelvic ring could be exposed through LRA without cutting the peritoneum, so that the sacroiliac joint, ilium wing and anterior ring fractures could be reduced under direct version. In the treatment of pelvis fragility fractures of the elderly, effective reduction and fixation could be achieved using reconstruction plates or channel screws through LRA.

Objective:To investigate the surgical technique and the clinical effects of direct posterior approach (DPA) with the fixation of percutaneous tunnel screw and plate for acetabular posterior comminuted fractures.Methods:Thirty-six cases with acetabular posterior comminuted fractures treated by this technique from January 2016 to July 2020 were retrospectively analyzed in this study. There were 28 males and 8 female, aged 42.0±12.1 (range 19-64) years. According to Letournel-Judet classification, there were 28 cases of transverse associated with posterior wall fractures, 6 cases of posterior column with posterior wall fractures and 2 cases of T shape with posterior wall fractures. DPA was adopted in prone position. The anterior and posterior column fractures of the acetabulum were reduced under direct vision and then fixed with percutaneous tunnel screw. Further, the posterior wall fractures of the acetabulum were reduced and fixed with plate and screws. The operation duration, intraoperative blood loss, incision length, fracture union time, fracture reduction quality, postoperative complications and hip function were recorded.Results:The incision was 9.8±1.2 (range 8-12) cm. The operation duration was 102.9±21.4 (range 65-145) min. Intraoperative bleeding was 214.0±116.9 (range 100-640) ml. Postoperative X-ray and CT examinations showed perfect reduction. All the patients were followed up for 20.9±9.2 (range 10-38) months. The fracture healing time was 4.6±1.0 (range 3-6) months. There was no patient with damaged superior gluteal nerve and blood vessel. There were 2 cases of femoral head cystic changes without pain in walking, 1 case of postoperative infection and bacteremia who was cured at 1 month after debridement and anti-infection treatment, 1 case of sciatic nerve injury but recovered at 3 months after operation, and 1 case of heterotopic ossification at 3 months after surgery without affecting hip motion. According to the Matta's criteria, the reduction quality of the acetabular fracture was rated as excellent in 28 cases, good in 6 cases, fair in 2 cases. According to the modified Merle D'Aubigné and Postel scoring system, hip joint function was excellent in 24 cases, good in 10 cases and fair in 2 cases.Conclusion:DPA approach can directly reduce acetabular posterior comminuted fractures through a minimal incision. Combined with the technique of percutaneous tunnel screw, it displays great advantages of less trauma and with good clinical effects.

Objective:To investigate the surgical techniques and clinical efficacy of periacetabular osteotomy through the lateral-rectus approach (LRA) for obsolete acetabular fractures.Methods:The 22 patients with old acetabular fracture were retrospectively analyzed who had been admitted to Department of Traumatic Surgery, The Third Affiliated Hospital to Southern Medical University from June 2012 to June 2019. They were 16 males and 6 females, aged from 22 to 61 years (average, 45.0 years). By the Judet-letournel classification, there were 4 anterior + posterior hemitransverse fractures, 2 T-shaped fractures and 16 both column fractures. The time from injury to surgery was 3 to 12 weeks in 15 cases, 3 to 6 months in 5 cases, 9 months in one case, and 3 years in one case. After the whole acetabulum was exposed outside the peritoneum through LRA in all patients, periacetabular osteotomy was conducted under direct vision, followed by fixation with multiple reconstruction plates or integrated acetabular airfoil anatomical plates after reduction. Recorded were the operation time, intraoperative blood loss, fracture reduction quality, affected hip function and complications in the patients.Results:In this cohort the operation time ranged from 110 to 205 min, averaging 140.5 min; intraoperative blood loss ranged from 500 to 2,100 mL, averaging 1,250.4 mL. According to the Matta X-ray criteria, the postoperative reduction was assessed as excellent in 7 cases, as good in 11 and as poor in 4, yielding an excellent and good rate of 81.8% (18/22). All the 22 patients were followed up for 12 to 36 months (mean, 22.1 months) and achieved fracture union. The one-year follow-up showed that their modified Merle d'Aubigné & Postel scores ranged from 6 to 18 points, averaging 14.6 points and giving 6 excellent, 10 good, 3 fair and 2 poor cases [an excellent and good rate of 72.7% (16/22)]. Osteonecrosis of the femoral head developed in 2 patients one of whom underwent total hip replacement.Conclusions:As LRA can expose the entire hemipelic ring from the medial side of the pelvis, the periacetabular osteotomy can be performed and the fractures of anterior or posterior column and the quadrilateral area can be reduced under direct vision through the medial side of the acetabulum. Moreover, ideal results can be achieved with the use of multiple reconstruction plates or integrated acetabular airfoil anatomical plates.

Objective:To explore the evaluation dimensions and indicators of research hospital, provide empirical evidence for the construction of research hospital.Methods:Non-probability sampling was adopted, physicians from 9 hospitals in Shanghai that have a National Clinical Medical Research Center or Shanghai Clinical Medical Research Center were invited as survey participants. The physician participants were divided into senior and junior groups. For senior group, a questionnaire including 5 dimensions and 21 indicators was provided. The survey participants were consulted to determine whether the indicators can be used to measure this dimension, and also invited to propose additional dimensions and indicators for improvement.For junior group, two open questions were proposed to explore their needs of support in both clinical service and research.Results:Based on suggestions from the research participants, the research team, and other expert consultant, this article tried to propose 5 dimensions and 21 indicators for evaluating research hospitals. Among them, 11 were indicators compiled by the research team based on the literature review and agreed by more than 2/3 of senior physicians surveyed, while others were proposed based on the survey results. The research team planned to use the entropy method to determine the weights of different indicators, thus, the participants were not required to make judgments on the weights of dimensions and indicators.Conclusions:The evaluation of research hospitals has not yet formed a systematic consensus. Through the exploration and establishment of evaluation dimensions and indicators, identify hospitals that are closer to the development goals of research hospitals can provide a basis for future next-step practices.