Resident training is an important part of after-graduation education for medical students,and is also an important way to cultivate qualified medical talents.With 20 years management experience of resident standardized training,Xuan-Wu Hospital has found 8 aspects,including leaders'attention and implement,process management,base construction,test assessment,24-hour system,guiding teacher system,rewards and punishment system,and scientific research were related to the quality of the resident standardized training.

Objective To investigate the clinical features of glucose transporter 1 deficiency syndrome(GLUT1-DS) and summarize the characteristics of GLUT1-DS through reviewing related references.Methods The clinical data including manifestation,cerebrospinal fluid (CSF) glucose,electroencephalogram,MRI and gene mutation of a patient with GLUT1-DS was collected and the related literatures were reviewed.Results The patient was a 6 years old boy.The patient,whose seizures occurred at the age of 9 month-old and prolonged to 6 year-old,attacked before breakfast.Physical examination showed microcephaly with head circumference 47.5 cm.Laboratory tests showed that CSF glucose decreased (1.87 mmol/L) and CSF-serum ratio was 0.36.And meantime the MRI was normal and electroencephalogram showed general spike and slow wave complex paroxysm.Mutation of SLC2A1 gene,c.350_385del,was found in the patient.There were 219 cases with GLUT1-DS had been reported and the age of onset was 15.69 months.In 219 patients,159 cases (72％) suffered seizures,105 cases (47％) had motor abnormalities,61 cases (27％) suffered intellectual disability.The CSF glucose values were (1.92±0.31) mmol/L,CSF-serum ratio was 0.36±0.07.SLC2A1 gene mutations were detected in 183 patients(96％)in which missense mutation was the most mutation.Conclusion A wide range of phenotypes of GLUT1-DS include seizures,motor abnormalities,mental retardation.The diagnosis is confirmed when CSF glucose and CSF-serum ratio are continuously decreased which in the absence of meningitis.The SLC2A1 gene should be detected in suspicion of GLUTI-DS patients.Early diagnosis and treatment may improve the prognosis of those GLUTI-DS patients.

Objective To evaluate the effectiveness of free highly active antiretroviral therapy (HAART) in adult infected with human immunodeficiency virus (HIV)/ acquired immune deficiency syndrome (AIDS) patients in Dehong area. Methods Clinical data of 1039 adult HIV/AIDS patients from five counties/cities in Dehong area who initiated HAART during the period from July 1st 2004 to June 30th 2008 were retrospectively analyzed to examine their virological and immunological responses to HAART. Data were analyzed by Chi-squared test or F test. Results Among the 1039 HIV/AIDS cases, 611 were males and 428 were females. The mean age was (37.0±9.9) years and the mean treatment duration was (22. 41 ± 12. 69) months. Complete viral suppression (HIV viral load＜50 copy/mL) was achieved in 781 cases (75. 17%). The percentage of patients achieving complete viral suppression rates were 76.95%, 76.49%, 70.65% and 77. 73% in patients treated for 6-12,13-24, 25-36 and more than 37 months, respectively (x2=8.646, P=0.194). The meanCD4+ T cell counts were (164.93±118.05) × 106/L at baseline, and (330.85±201.73) × 106/L, (356.24±205.49) × 106/L, (434.53±250.65) × 106/L and (396.31±202.62) × 106/L in patients treated for 6- 12, 13-24, 25-36 and more than 37 months, respectively. CD4- T cell counts were significantly different in patients treated for 6-12 and 13-24 months (F= 19. 423 , P＜0. 01). Successful immune reconstitution was achieved in 927 ( 90.88 % ) cases. Seven hundred and seventeen (70.29% ) cases achieved both virological suppression and immunological reconstitution with HAART, whereas 40 cases (3. 92%) failed to achieve both virological and immunological responses. Conclusion HIV/AIDS patients in Dehong area show good virological and immunological responses to HAART.

Objective:To summarize the classification of etiology, age of onset, prognosis of children with convulsion, so as to provide experience guidance for clinicians engaged in pediatric emergency department.Methods:The clinical data of children with convulsions received in the emergency department of Children′s Hospital Affiliated to Kunming Medical University from January 2015 to December 2018 were analyzed retrospectively.Results:During the four-year period, 2 957 children with convulsion were received in the emergency department, accounting for 22.20% of the total number of critically ill children in the observation room of the emergency department, and the ratio of male to female was 1.7∶1.The etiological diagnosis of convulsion in emergency are as follows: febrile convulsion(733 cases, 24.79%), central nervous system infection(477 cases, 16.13%), unexplained convulsion(476 cases, 16.09%), epilepsy(371 cases, 12.55%), benign infantile convulsions with mild gastroenteritis(240 cases, 8.12%). The age of onset: 8.25% were in neonatal period, 33.99% were in infant, 34.87% were in toddler′s age, 12.17% were in preschool age, 7.88% were in school age and 2.84% were in adolescence.Destination statistics: 72.00% were admitted to hospital for further treatment, 13.29% were transferred to neurology clinic, 7.85% to pediatric clinic, 1.66% to rehabilitation clinic, and 0.17% died.Inpatient department: 43.64% were admitted to department of neurology, 17.52% to pediatric intensive care unit, 13.71% to department of neonatology, 12.64% to department of gastroenterology and 2.72% to department of rehabilitation.Conclusion:Febrile convulsion is the main cause of convulsion in children who were received emergency treatment in our hospital.Most of the convulsion cases are from birth to preschool age, and the prognosis is good after active treatment.

A retrospective analysis was made on the clinical data of a child with Turnpenny-Fry syndrome who was treated in the Epilepsy Center of Kunming Children′s Hospital in January 2023 for developmental retardation and epileptic spasm.The child, a 1-year-and-4-month-old boy, had developmental retardation since birth and developed epileptic spasm at the age of 5 months.Physical examination and auxiliary examination showed distinct facial features, heart, bone and other developmental malformations.Electroencephalogram indicated hypsarrhythmia and epileptic spasm.The genetic test suggested the presence of c. 194C>T (p.Pro65Leu), a new heterozygous mutation in PCGF2 gene.The seizures were completely controlled with anti-seizure drugs.This child is the only case reported with infantile epileptic spasm as the main manifestation so far, expanding the understanding of the phenotype of the disease.

Objective To investigate the effect evaluation of self-nursing on the stent-tract in the discharged patients with orthopedic external fixation support. Methods A total of 112 patients with orthopedic external fixation support were selected, and designed the self-nursing material. Before discharging guidance, make sure that the patient or patients′ family mastered the operation methods and nursing key points. After discharge, the continuing nursing support was provided. Results The incidence of infection was 4. 46%. All the 112 patients, the times of return visits were 432, and the times of changing the dressing were 3 940, with the mean times of 35. 2;The reducing the costs were 43 340, with the mean reducing of 387. Conclusions The self-nursing on the stent-tract in the discharged patients with orthopedic external fixation support can save the human resource, time and medical costs of the patients and patients′ family, it can also help the doctors with discharged treatment and reduce the work of nurses.

Objective:To analyze the epidemic characteristics and trends of newly reported HIV-infected people among Chinese and Burmese in Dehong Dai and Jingpo Autonomous Prefecture (Dehong Prefecture) of Yunnan Province, China, from 2000 to 2023, and provide evidence for formμlating AIDS prevention and control measures for the Burmese living in Dehong.Methods:The data were obtained from the Chinese Disease Control and Prevention Information System. The distribution of HIV-infected people with different population characteristics was analyzed, and the Joinpoint regression model was used to analyze the temporal trend of crude detection rate in different genders, ethnicities, and ages.Results:From 2000 to 2023, 24 989 newly HIV infections were reported in Dehong Prefecture, of which 14 594 (58.4%) were Chinese and 10 395 (41.6%) were Burmese. Compared with Chinese, Burmese women (32.9%, 3 416/10 395), those aged 20-29 (40.9%, 4 248/10 395), and Jingpo people (26.7%, 2 773/10 395) accounted for a higher proportion. The new diagnosis rate of Chinese nationals increased from 1.0/10 000 in 2000 to 15.4/10 000 in 2004, and then showed a downward trend, falling to 1.2/10 000 in 2023. Among them, compared with other age groups, Dai and other ethnic groups and women, the new diagnosis rate among 20-49 age group, Jingpo and men were relatively higher, at 1.7/10 000, 2.3/10 000 and 1.3/10 000 respectively. Regarding the method of detection, the Chinese HIV-infected people were mainly detected by key population testing (35.7%), while the Burmese HIV-infected people by key population testing (28.9%) and physical examination for entry-exit personnel (25.3%). The transmission routes of both nationalities were mainly heterosexual transmission, but compared with Chinese HIV-infected persons, the proportion of Burmese infected persons through non-marital non-commercial transmission was relatively higher (66.4% vs. 60.6%). The proportion of Chinese nationals with a first CD4 +T lymphocyte (CD4) counts of <200 cells/μl (28.9%) was higher than that of Burmese nationals (19.8%). Conclusions:The rising trend of HIV infection among Chinese and Burmese people in Dehong Prefecture from 2000 to 2023 slowed down. The new diagnosis rate was higher in the 20-49 age group, Jingpo and men. Compared with Burmese HIV-infected people, the proportion of Chinese HIV-infected people with first CD4 counts <200 cells/μl was relatively higher. Comprehensive interventions should be further carried out for Myanmar nationals, and efforts should be made to expand testing for Chinese nationals.

Objective:To explore the influence factors of neurodevelopmental disorders in children with SCN8A-related early-onset epilepsy through analyzing their clinical characteristics and following up their neurodeve-lopmental status. Methods:A retrospective analysis was carried out on 21 children (13 males and 8 females, the age ranged from 4 months to 8 years, average 31.6 months)with SCN8A-related early-onset epilepsy treated in Guangzhou Women and Children′s Medical Center and Kunming Children′s Hospital between January 2017 and February 2021.All patients underwent whole-exome sequencing and Sanger sequencing.The pathogenicity was estimated according to the American College of Medical Genetics and Genomics guidelines.The clinical data of all patients were also collected, including the age of onset of the disease, forms of seizures, seizure frequency, neurological development at onset, electroencephalogram (EEG) and brain magnetic resonance imaging (MRI). Besides, the patients were followed up to acquire the effect of sodium channel blockers after the onset of seizures, the process or improvement of neurodeve-lopment, EEG evaluation and neurodevelopmental outcomes.Patients were grouped based on data analysis results.The Fisher′s exact test was conducted to measure the effect of various factors on the neurodevelopmental process and outcome, and corresponding coe-fficients were calculated. Results:The average onset age of 21 patients was 0-9 months.The follow-up duration was 4 months-8 years.Three cases died.Sixteen cases (76.2%) had early infantile epileptic encephalopathy (EIEE), 5 cases (23.8%) had epilepsy without encephalopathy, and 1 case had benign infantile epilepsy.Fourteen cases (66.7%) belonged to drug resistant epilepsy.Only one child showed normal neurodevelopment.Eleven children showed delayed neurodevelopment, but improvement was observed.Nine children were retrogressed and stagnated in terms of neurodevelopment.Small age at onset ( Fisher=9.517, P=0.020, r=0.571), high seizure frequency ( Fisher=10.512, P=0.003, r=0.572), EEG background ( Fisher=10.512, P=0.003, r=0.572), epileptic discharges ( Fisher=8.288, P=0.008, r=0.542), and EEG changes before and after treatment ( Fisher=10.437, P=0.009, r=0.586) were important factors affecting the neurodevelopmental process.Neurodevelopmental outcome was normal in only 1 case, 1 child belonged to mild mental retardation (MR), 7 children belonged to moderate MR, 3 children belonged to severe MR, and 9 children belonged to profound MR.Statistical analysis indicated that the clinical phenotype ( Fisher=10.059, P=0.004, r=0.739) and drug resistance ( Fisher=13.706, P=0.001, r=0.640) were significantly correlated with neurodevelopmental outcomes.However, the forms of seizures, EEG findings at onset and mutation sites were not related to neurodevelopmental disorders. Conclusions:Most children with SCN8A-related early-onset epilepsy are accompanied with neurodevelopmental retardation of varying degrees.Epileptic encephalopathy and poor response to drug treatment will lead to severe neurodevelopmental disorders.

ObjectiveTo investigate the factors that influence the first CD4+T lymphocyte counts in newly reported HIV-infected cases aged 50 and above in Dehong Prefecture of Yunnan Province during 2016 to 2021， and to understand the patient immune status and disease progression so as to provide scientific basis for HIV prevention and control strategies in the future. MethodsData was collected from the national HIV/AIDS information system. Multivariate logistic regression was used for the analysis of factors affecting the first CD4+T lymphocyte counts. ResultsA total of 642 cases of HIV infection were newly reported， among them， 571 cases had CD4+T lymphocyte counts and 200 cases （35.03%） had CD4+T lymphocyte counts <200 cells·μL^-1. Patients who were in the 50-59 age group， male， divorced or widowed， and less educated were more likely to have CD4+T lymphocyte counts <200 cells·μL^-1. Compared with active testing consultants， forced reeducation through labor or drug rehabilitation cases were less likely to have CD4+T lymphocyte counts <200 cells·μL^-1. ConclusionThere is no obvious upward trend in newly reported HIV infected persons aged 50 years and above in Dehong Prefecture during 2016 to 2021. However， the situation of CD4+T lymphocyte counts <200 cells·μL^-1 is still serious. Attention should be paid to the key groups： male， Chinese nationality， farmers， Han nationality， married or divorced， junior high school education or below， and heterosexual transmission. It is necessary to strengthen the intervention in people aged 50 and above and improve the detection efficiency.

ObjectiveTo investigate the demographic characteristics， high-risk behavior and first CD4⁺T cell（CD4 cell） counts among newly reported HIV-infected Burmese in Dehong Dai Jingpo Autonomous Prefecture （Dehong Prefecture） from 2019 to 2020. MethodsA cross-sectional survey was conducted among newly reported HIV-infected Burmese cases in Dehong Prefecture from 2019 to 2020 to collect related information and measure their first CD4 cell counts. ResultsAmong 1 228 HIV-infected Burmese cases， 846 （68.9%） were males and 382 （31.1%） were females； 445 （36.2%） were from Kachin， and 210（17.1%） were from Shan. Of them， 960 cases （78.2%） had participated in the first CD4 cell counts testing， and 405 cases （42.2%） had a first CD4 cell counts <350 cells·μL^-1. In the multivariable logistic regression analysis， age ≥30 years （adjusted OR=1.44， 95%CI： 1.07‒1.92） and history of illicit drug use in Myanmar （adjusted OR=0.41， 95%CI： 0.21‒0.79） were independently associated with first CD4 cell counts <350 cells·μL^-1. ConclusionThe first CD4 cell counts are generally low among newly reported HIV-infected Burmese in Dehong Prefecture， especially those aged ≥ 30 years and with a history of intravenous drug use in Myanmar. Therefore， early detection of HIV infection should be strengthened for timely intervention and treatment management among Burmese.