Spastic paraplegia type 4 (SPG4) is the most common type of autosomally inherited spastic paraplegia. Its main clinical features include typical simple hereditary spastic paraplegia, with neurological impairments limited to lower limb spasticity, hypertonic bladder dysfunction, and mild weakening of lower limb vibration sensation, without accompanying features such as nerve atrophy, ataxia, cognitive impairment, seizures, and muscle tone disorders. SPAST is the main pathogenic gene underlying SPG4, and various pathogenic SPAST variants have been discovered. This disease has featured a high degree of clinical heterogeneity, and the same pathogenic variant can have different age of onset and severity among patients and even within the same family. There is a lack of systematic research on the correlation between the genotype and phenotype of SPG4, and the pathogenic mechanism has remained controversial. This article has provided a review for the clinical characteristics, pathogenic gene characteristics, correlation between the genotype and phenotype, and pathogenic mechanism of this disease, with an aim to provide reference for its clinical diagnosis and treatment.

Objective:To analyze the early warning value of laboratory examination on admission of patients with hemorrhagic fever with renal syndrome to critically ill patients.Meetods:In this study, a retrospective case-control study was used to analyze the clinical data and laboratory examination results of patients with hemorrhagic fever with renal syndrome admitted to the emergency department of Tangdu Hospital of Air Force Medical University from January 2021 to January 2022. According to the patient's laboratory indexes and clinical symptoms, the patients were divided into mild, moderate, severe and critical groups. The general data of the two groups were compared, and the independent risk factors of critically ill patients were screened by multi-factor logistic regression analysis, the predictive model of severe HFRS patients was constructed, and the ROC curve was drawn. .Results:Of the 164 patients with HFRS, 50 were in the severe group and 114 in the mild group. The serum levels of WBC, AST, ALT, Cr, BUN, DD and PCT in the severe group were higher than those in the mild group, while the levels of PLT, ALB and PTA in the severe group were lower than those in the mild group. Multiple logistic regression analysis showed that WBC, PLT and PCT were independent influencing factors for the progression of critically ill patients. The predictive model of severe HFRS was established as follows: logit (P) = -0.321 + 0.040 WBC (×10 9/L) -0.045 PLT (×10 9/L) + 0.086 PCT(ng/mL). The early warning ef?cacy of WBC, PLT, And PCT for severe HFRS was further analyzed. The area under the ROC curve (area under curve, AUC) was 0.779, 0.842, 0.862, and the optimal threshold was 10.435×109/L, 41.5 ×109/Land 2.97 ng/mL, respectively. The AUC of joint detection is 0.900, the sensitivity is 88.0%, and the speci?city is 82.5%, which is better than that of a single laboratory. . Conclusions:HFRS laboratory indexes have certain clinical signi?cance for the identi?cation of critically ill patients, in which serum WBC, PLT and PCT indexes are the risk factors of severe HFRS, which provides a theoretical basis for clinical diagnosis, treatment and prognosis of severe HFRS patients.

Peripheral nerve block is a kind of analgesia with few adverse reactions and high safety.It has been widely used in anesthesia and postoperative analgesia in various kinds of operations.The rebound pain after nerve block is gradually recognized.This article reviews the definition,mechanism of occurrence,and intervention measures of rebound pain after peripheral nerve block,aiming to enhance the clinical doctors'attention to rebound pain after peripheral nerve block,and provide reference for the comprehensive management of rebound pain after peripheral nerve block.

Objective:To assess the feasibility of wavelet index (WLI) in monitoring the depth of sedation with propofol in pediatric patients.Methods:This was a prospective observational trial. One hundred and sixty-five pediatric patients, aged >1-12 yr, of American Society of Anesthesiologists Physical Status classification Ⅰ or Ⅱ, undergoing elective surgery requiring general anesthesia with endotracheal intubation or laryngeal mask airway from July to December 2016 in our hospital, were divided into 11 age groups ( n=15 each): >1-2 yr group, >2-3 yr group, >3-4 yr group, >4-5 yr group, >5-6 yr group, >6-7 yr group, >7-8 yr group, >8-9 yr group, >9-10 yr group, >10-11 yr group, and >11-12 yr group. General anesthesia was induced by injection of propofol 3 mg/kg for more than 30 s. WLI and BIS values were recorded immediately before administration and at 30, 40, 50, 60, 90, 120, 180 and 240 s after the end of administration. If there were differences among age groups, age groups with no statistically significant differences were combined and re-grouped. Pearson linear correlation analysis and Bland-Altman consistency analysis were performed. Results:A total of 149 pediatric patients were actually included. There were no significant differences in BIS values between 4 groups aged > 1-5 yr and between 7 groups aged > 5-12 yr ( P>0.05). Regrouping was performed based on the aforementioned results, 4 groups of children aged > 1-5 yr were divided into > 1-5 yr group ( n=60), and 7 groups of children aged > 5-12 yr were divided into > 5-12 yr group ( n=89). WLI values and BIS values were significantly decreased at each time point after administration compared with immediately before administration in group aged > 1-5 yr and in group aged >5-12 yr ( P<0.05). The BIS values were the lowest at 60 s after the end of the administration, and the WLI values were the lowest at 120 and 180 s after the end of the administration in two groups ( P<0.05). There were no statistically significant differences between WLI values and BIS values at 90 s and 240 s after the end of the administration ( P>0.05), and there was statistically significant difference at the other time points in group aged > 1-5 yr ( P<0.05). There was no significant difference between WLI values and BIS values at 180 s and 240 s after the end of the administration ( P>0.05), but there were significant differences at the other time points in group aged > 5-12 yr ( P<0.05). The Pearson correlation coefficients between WLI values and BIS values were 0.61 and 0.56 in group aged > 1-5 yr and group aged > 5-12 yr, respectively ( P<0.001). Bland-Altman agreement analysis: In group aged > 1-5 yr and group aged > 5-12 yr, the 95% agreement limits were -0.484-0.621 and -0.551-1.015, respectively, and there were 4.6% (23/504) and 5.1% (40/777) of the points outside the 95% agreement limits, respectively, and both limits exceeded the clinically acceptable range. Conclusions:WLI is feasible for monitoring the depth of sedation with propofol in pediatric patients aged > 1-12 yr, but the accuracy is lower than BIS.

Objective:To investigate the distribution of intraocular pressure (IOP) in high-altitude population aged 18 years and over in Xining, Qinghai and establish the reference interval (RI) of IOP.Methods:A cross-sectional study was conducted in Xining, Qinghai Province at 2.271 km above sea level from September 2019 to May 2020.Ophthalmic examinations and IOP measurement were conducted among subjects from Physical Examination Center of Qinghai Provincial People's Hospital.The subjects who had been living in Xining without leaving for three months were enrolled.Ophthalmic examinations included vision examination, IOP measurement, slit-lamp microscopy, fundus photography, anterior and posterior segment optical coherence tomography.IOP was measured using Goldmann applanation tonometry under local anesthesia.Subjects with factors that could cause significant changes in IOP and affect the accuracy of IOP measurement, and those who were unable to receive IOP measurement were excluded.Subjects were grouped according to sex, age and ethnicity, and the distribution and RI of IOP were compared among all groups.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Beijing Tongren Hospital, Capital Medical University (No.TRECKY2017-024). Written informed consent was obtained from each subject.Results:A total of 6 120 subjects (6 120 eyes) aged 18-90 years old were enrolled, including 2 850 males and 3 270 females with average age of (45.54±13.85) years.The average IOP of high-altitude population in Xining, Qinghai Province was (14.32±1.93) mmHg (1 mmHg=0.133 kPa), with the RI of 10.54-18.10 mmHg.The average IOP was (14.42±1.98) mmHg in male with the RI of 10.54-18.30 mmHg, (14.23±1.88) mmHg in female with the RI of 10.55-17.91 mmHg.The IOP of male was higher than that of female ( t=3.71, P<0.001). The IOP of Han, Tibetan, Hui and other nationalities were (14.38±1.91), (13.93±2.06), (14.21±1.87), (13.94±1.95) mmHg, respectively, with a statistically significant overall difference ( F=6.73, P<0.001). The IOP of Han nationality was significantly higher than that of Tibetan, Hui and other nationalities, and the differences were statistically significant (all at P<0.05). Conclusions:RI of IOP in high-altitude population from Xining, Qinghai is lower compared with normal altitude area.

The solute carrier family 12(SLC12)of cation-chloride cotransporters(CCCs)comprises potassium chlo-ride cotransporters(KCCs,e.g.KCC1,KCC2,KCC3,and KCC4)-mediated Cl-extrusion,and sodium po-tassium chloride cotransporters(N[K]CCs,NKCC1,NKCC2,and NCC)-mediated Cl-loading.The CCCs play vital roles in cell volume regulation and ion homeostasis.Gain-of-function or loss-of-function of these ion transporters can cause diseases in many tissues.In recent years,there have been considerable ad-vances in our understanding of CCCs'control mechanisms in cell volume regulations,with many tech-niques developed in studying the functions and activities of CCCs.Classic approaches to directly measure CCC activity involve assays that measure the transport of potassium substitutes through the CCCs.These techniques include the ammonium pulse technique,radioactive or nonradioactive rubidium ion uptake-assay,and thallium ion-uptake assay.CCCs'activity can also be indirectly observed by measuring y-aminobutyric acid(GABA)activity with patch-clamp electrophysiology and intracellular chloride con-centration with sensitive microelectrodes,radiotracer 36Cl-,and fluorescent dyes.Other techniques include directly looking at kinase regulatory sites phosphorylation,flame photometry,22Na+uptake assay,structural biology,molecular modeling,and high-throughput drug screening.This review sum-marizes the role of CCCs in genetic disorders and cell volume regulation,current methods applied in studying CCCs biology,and compounds developed that directly or indirectly target the CCCs for disease treatments.

Objective:The common protocol deviation (PD) in clinical trials of drugs for rheumatic diseases were analyzed to provide reference for improving the quality of clinical trials and protecting the safety and rights of subjects.Methods:Protocol deviation (PD) reports of completed projects conducted by Department of Rheumatology and Immunology, the Second Hospital of Shanxi Medical University, from 2017 to 2021 were collected and sorted out for analysis. Results:The common PD and proportion of deviation were deviation from protocol study procedures (25.4%, 44/173), low medication compliance (23.7%, 41/173), visits out of the scheduled visit dates or the tests or examinations out od the inspection window period (19.1%, 33/173), missed visit or missed laboratory tests (17.9%, 31/174), using of prohibited drugs (9.2%, 16/173) and violation of inclusion criteria or exclusion criteria (4.6%, 8/173). 135 cases were mild and 38 cases severe deviation. PPS was eliminated in 5 subjects during statistical analysis.Conclusion:①Investigator training should be strengthened and the operability should be taken into account in clinical trial design. In the implementation process, the inclusion and exclusion criteria should be strictly followed and the should adherent to study procedures. ②The institution should actively promote the development of hospital information system to reduce investigator associated PD. ③The education and management of subject should be strengthened to ensure their compliance and improve the quality of clinical trials to protect the safety and rights of subjects. ④Institute should strengthen the management of trail supervisors and emphasis on problems identification during inspections.

Objective:To investigate the value of a deep learning method based on MobileNet in classification of bedside chest radiograph and improvement of the work efficiency.Methods:A total of 6, 320 bedside chest radiographs from January 2017 to December 2022 in the Second Peoples′ Hospital of Changzhou were retrospectively collected. The included cases were divided into normal group (885 images), pneumonia group (1 927 images), pleural effusion group (373 images), and pneumonia with pleural effusion group (3 135 images). Three hundred and fifty images were selected as a validation set, while the remaining images were divided into a train set (4 775 images) and a test set (1 195 images) using simple randomization, by 8∶2 ratio. Two lightweight convolutional neural network models (MobileNetV1 and MobileNetV2) were used to construct a bedside chest radiograph classification model, based on which two fine-tuning strategies were designed. Four models were generated namely MobileNetV1_False (V1_False), MobileNetV1_True (V1_True), MobileNetV2_False (V2_False) and MobileNetV2_True (V2_True). In the first stage, a binary classification model was established to divide the images into normal and lesion groups; then a four-class classification model was established in the second stage, with which the images were divided into four groups: normal, pneumonia, pleural effusion and pneumonia with pleural effusion. Metrics for model performance evaluation including accuracy (Ac), precision (Pr), recall rate (Rc), F1 score (F1) and area under the receiver operating characteristic curve (AUC) were calculated.Results:In both the first and second stages, V1_True and V2_True had higher Ac, Pr, Rc, and F1 than V1_False and V2_False in both the training set and validation set; and the V1_True model outperformed the other three models in classification. The classification Ac of the V1_True model in the validation set was higher than that of radiologists in the first stage [95.71% (335/350) vs. 90.29% (316/350)] and in the second stage [93.43% (327/350) vs. 87.14% (305/350)]. The recognition time of V1_True model′s in the validation set of 350 bedside chest radiographs was significantly less than that of the radiologists (mean: 17 s vs. 300 min).Conclusions:V1_True is an optimal MobileNet model for classifying bedside chest radiographs. The application of this model in clinical practice may help to accurately identify the information of lung lesions from bedside chest radiographs in time, and may improve the work efficiency in the radiology department.

Objective:To explore the application effect of humanistic care nursing model in patients with gynecological tumors.Methods:Using the convenient sampling method, a total of 136 gynecological tumor patients who were hospitalized in Henan Cancer Hospital from March 2020 to March 2021 were selected as the research objects. Patients were divided into the observation group and the control group using the random number table method, with 68 patients in each group. The control group received routine nursing intervention during hospitalization, while the observation group received humanistic care based on routine nursing. The intervention effect was compared using Symptom Checklist 90 (SCL-90) , Pittsburgh Sleep Quality Index (PSQI) and Nursing Satisfaction Questionnaire.Results:After intervention, the SCL-90 and PSQI scores of the observation group were lower than those of the control group, and the differences were statistically significant ( P<0.05) . After intervention, the scores of each item in the Nursing Satisfaction Questionnaire of the observation group were higher than those of the control group, and the differences were statistically significant ( P<0.01) . Conclusions:The application of humanistic care model in gynecological tumor patients can effectively improve their psychological status and sleep quality and increase patient satisfaction, which is worthy of clinical promotion.

【Objective】 To investigate the effect of Qingkailing injection on platelet function preserved in vitro. 【Methods】 A total of 15 bags of plateletpheresis (≥250 mL/bag), adding Qingkailing injection(1.25 mL/bag) at the final concentration of 1%, were stored at 22 ℃ with gentle agitation as the experimental group, and samples were collected on day 1, 3, 5, 8, 10 and 14 to detect the thromboelastogram (TEG), CD62p expression rate and mitochondrial membrane potential (JC-1). The control group was set up synchronously, with the same volume and storage conditions as the experimental groups, and samples were taken on day 1, 3 and 5 after preservation to undertake the same test items as the experimental groups. The differences of detection indexes between the two groups were compared. 【Results】 1) In the experimental group, there was no significant change in K and α during day 1 to 14(P>0.05). The R value (min) increased from 6.12±1.58 to 11.02±2.26, and the CI value changed from 0.27±1.24 to -10.47±3.51 (P<0.05). The MA value (mm) had no significant change within 5 days (P>0.05), but decreased to 53.18±2.71 on day 8 and 22.88±3.45 on day 14 (P<0.05). In the control group during day 1 to 5, K(min) and α showed no significant change, while R(min) was significantly prolonged, MA (mm) decreased and CI increased significantly (P<0.05). 2) The expression rate of CD62p (%) was 70.50±9.12 in the experimental group on day 5 (vs 83.16±5.33 in the control groups, P<0.05) and 82.77±7.17 on day 14 (P<0.05). 3) There was no significant change in JC-1 (%) during regular preservation period between the experimental group and control group (P>0.05), but JC-1 (%) was 86.75±3.88 vs 70.36±19.8 on day 5 (P<0.05). In the experimental group, JC-1(%) was 81.04±9.50 vs 71.38±8.77 vs 82.77±7.17 on day 8, 10 and 14, respectively. 【Conclusion】 The activation and aggregation as well as anti-apoptosis function of plateletpheresis, adding Qingkailing injection at the final concentration of 1%, are similar to those of routine preservation.