Objective To summarize the phenotypic features of an unrecognized leukoencephalopathy in infants sharing same clinical features,and to better understand the disease and provide new evidence for identification of new leukoencephalopathy. Methods Clinical and follow-up data of 13 patients with unrecognized infantile leukoen-cephalopathy were collected from Peking University First Hospital from January, 2006 to December, 2014. Results (1) There were 7 male and 6 female. The average age of onset was 11 months (4-25 months). Thirty-eight percent (5/13 cases) of patients had incentives before the onset;all of the cases had acute onset and rapid motor function regression. Fifteen percent (2/13 cases) of the patients suffered from seizures in the course of the disease. Patients′condition became stable,and cognition and motor function improved gradually 1 month after onset. No patient died till the last follow-up. (2) Imaging features:magnetic resonance imaging (MRI) of the patients was characterized by im-plicating deep white matter,presenting T1 hypointense,T2 and fluid attenuated inversion recovery ( FLAIR) hyperin-tense in the periventricular area. All of MRI showed massive and symmetric lesions with heterogeneous signal and cystic degeneration. DWI showed patch or massive hyperintense in some of the lesions. The follow-up MRI showed the original lesions decreased in 88% ( 8/9 cases ) of patients, and white matters atrophied in 55% ( 5/9 cases ) of patients;the cystic degeneration still existed and even expanded;DWI showed regional linear or spot hyperintense in 88% (8/9 cases) of patients,which was smaller than before,and distributed around the original lesions. Conclusions The patients with leukoencephalopathy caused by unknown pathogenic gene were much likely to be mitochondrial leukoencephalopathy. This study provided evidence for further exploration of new pathogenic genes causing leu-koence-phalopathy.

Objective To survey the current situation of core competence and job burnout for nursing professional teachers in college and to analyze the correlation between them.Methods 210 nursing teachers from 16 colleges and universities were investigated with the core competence evaluation questionnaire for nursing professional teachers and Chinese Maslach Burnout Inventory (CMBI).Results The mean score of core competence for college nursing professional teachers was (2.67±0.79) points,which was at the middle low level.The overall level of job burnout for college nursing professional teachers was between none and moderate.There was significant positive correlation between job burnout and core competence,the leadership competence,problem solving competence,professional competence,teaching competence.Conclusions The job burnout level for nursing professional teachers in college is lower than the moderate level.The core competence increases with the increase of job burnout level.Making mild stress for teacher and keeping the job burnout at mild-moderate level can improve the core competence of nursing teachers in College

Objective　To evaluate the efficacy and safety of butylphthalide combined with aspirin in treatment of acute cerebral infarction.Methods　CNKI,Wanfang,VIP,sinomed,PubMed,Cochrane library databases were searched by computer from the building time to October 2019.According to the inclusion and exclusion criteria,the eligible RCT was selected and Meta-analysis was carried out by Revman 5.3 soft ware.Results　A total of 1375 cases were included in 15 articles.The results from Meta-analysis demonstrated that the cure rate of the treatment group was superior to that of the single drug ［RR=1.56,95% CI (1.16,2.10),Z=2.93 (P=0.003)］,additional other treatments［RR=1.99,95% CI(1.30,3.05),Z=3.16(P=0.002)］;the effective rate of the treatment group was superior to that of the single drug［RR=1.41,95%CI(1.22,1.64),Z=4.52(P<0.00001)］,additional other treatments［RR=1.24,95%CI(1.12,1.38),Z=4.07(P<0.0001)］;the NHISS score of the treatment group was lower than that of the single drug［MD=-2.79,95%CI(-3.72,-1.86),Z=5.86(P<0.00001)］,additional other treatments［MD=-2.70,95%CI(-3.54,-1.85),Z=6.26(P<0.00001)］.There was no significant difference in safety between the two groups.Conclusion　Butylphthalide combined with aspirin is effective and safe in the treatment of acute cerebral infarction.

Objective To investigate the clinical and pathological features of children with glycogen storage disease (GSD). Methods A retrospective analysis was performed for ten children with GSD who were admitted to the Third Hospital of Hebei Medical University and The Fifth Medical Center of Chinese PLA General Hospital from January 2002 to January 2022, based on medical history, liver biochemistry, and liver biopsy, and population characteristics, clinical manifestations, biochemical parameters, and liver histopathological characteristics were compared and analyzed. Results All ten children had developmental retardation and a short stature, with the manifestations of abnormal liver function, mild weakness, poor appetite, yellow urine, and yellow eyes, and four children had hepatosplenomegaly. Among the ten children, six had the clinical manifestations of hypoglycemia, and one had bilateral gastrocnemius hypertrophy and positive Gower sign. Two children had positive CMV IgG. Liver histopathological manifestations included diffuse enlargement of hepatocytes, light cytoplasm, and small nucleus in the middle like plant cells, with or without fibrous tissue proliferation. Conclusion Most patients with GSD have developmental retardation and abnormal aminotransferases, and liver pathological examination shows specific pathological features.