Kennedy disease,a motor neuron disease,is an adult onset form of spinal and bulbar amyotrophy characterized by the damage of low motor neuron and uncompleted androgen insensitivity syndrome.So far,there is no effective treatment.This article is intended to provide neurologist with information about Kennedy disease in epidemiology,etiology,pathogenesis,clinical feature,treatment and so on.

Abscess ; pathology ; Adenoma ; metabolism ; pathology ; Adenoma, Sweat Gland ; metabolism ; pathology ; Biomarkers ; metabolism ; Breast Diseases ; pathology ; Breast Neoplasms ; metabolism ; pathology ; Female ; Fistula ; pathology ; Humans ; Keratin-7 ; metabolism ; Keratins ; metabolism ; Mucin-1 ; metabolism ; Nipples ; pathology ; Paget's Disease, Mammary ; metabolism ; pathology ; Receptor, ErbB-2 ; metabolism ; Sweat Gland Neoplasms ; metabolism ; pathology

Adolescent ; Adult ; China ; Dust ; Female ; Health Knowledge, Attitudes, Practice ; Humans ; Industry ; Male ; Middle Aged ; Occupational Exposure ; prevention & control ; Transients and Migrants ; psychology ; Young Adult

Objective Evaluation of left ventricular ( LV) function has great clinical significance in patients with early chro-nic kidney disease (CKD).The study was to evaluate whether two-dimensional speckle tracking imaging (2D-STI) technique can offer additional information of cardiac dysfunction in early CKD patients . Methods Conventional echocardiography and 2D-STI echocar-diography were performed in CKD group of 20 patients at stage Ⅱ-Ⅲ and control group of 20 healthy volunteers .LV structure and function were evaluated by conventional echocardiography .Multidirectional strain , strain rate and LV twist parameters were analyzed by 2D-STI technique. Results There was no statistical difference in conventional echocardiographic parameters such as left atrial diam -eter, end-diastolic LV diameter and ejection fraction between CKD group and control group .By applying 2D-STI technique, CKD group had significantly decreased value of global longitudinal strain (-19.8 ±2.4)%and strain rate(-1.1 ±0.3)S-1 in comparison to control group(-22.6 ±2.1)%、( -1.8 ±0.2)S-1, along with significant increase in the values of maximum LV rotation (11.6 ± 4.4)°and LV twist(19.6 ±3.8)°in comparison to control group(8.2 ±3.7)°、(13.5 ±3.6)°(P<0.05).There was no differences in both global circumferential and radial deformation parameters as well as maximum rotation value of LV assist device between two groups(P>0.05). Conclusion 2D-STI can detect subclinical LV dysfunction sensitively in early CKD patients .

Gene targeted mutagenesis and replacement can be used to modify gene sequence in genomic background without position effect or insertion inactivation in transgenic plants. Targeted mutagenesis organism has little biosafety concerns free of transgenes or marker genes. Gene targeted mutagenesis and replacement in high plants now appears to be a potential tool for gene functional analysis in situ, crops genetic improvement and molecular design. Zinc finger nuclease(ZFN)is most important and would be widely used in gene targeted mutagenesis and replacement through introducing double-strand breaks in genome. Strategies for gene targeted mutagenesis and replacement in plants is discussed. ZFN is described in detail from its structure, operation model and application in plants. Developmental prospect of ZFN in plant gene targeted mutagenesis and replacement is also discussed.

Adolescent ; Child ; Child, Preschool ; Core Binding Factor Alpha 3 Subunit ; genetics ; metabolism ; DNA Methylation ; Female ; Humans ; Infant ; Lymphoma ; genetics ; metabolism ; Male ; Promoter Regions, Genetic

Objective To design an appropriate preoperative questionnaire in Chinese bariatric surgery.Methods Bariatric surgery center carried out laparoscopic bariatric operation from January 2010,preoperative questionnaire before the operation were supplied.Till January 2014,a total of 401 patients replied to the survey.The questionnaires were designed through three stages:translated from the Cleveland Clinic (USA) and applied in 30 patients for the first survey; adjustments and changes to the contents of the questionnaire for 90 patients according to Chinese conditions in the second phase; in the third stage,the third edition questionnaire was used in 281 patients.The satisfaction degree of the doctors and patients about the content of the questionnaire was evaluated.Results For the three versions of the questionnaire,the doctor satisfaction rate was 73.3％,86.7％ and 96.8％.Patients' satisfaction rate of three versions were 30.0％,40.0％,76.9％,not satisfactory rate was 20.0％,11.1％,2.8％.Conclusions The current preoperative questionnaire includes 11 categories with 101 items of the third edition,doctors and patients satisfaction rate is higher,and it is suitable for Chinese bariatric surgery.

Hematologic Diseases ; Hemolysis ; Humans ; Infant, Newborn

Objective To explore the distribution and correlation of gross motor function and manual ability in 4～12 year old children with cerebral palsy of different types. Methods 143 children with cerebral palsy (CP) were recruited according to the inclusion and exclusion criterions. They were assessed with Chinese version of Gross Motor Function Classification System （GMFCS） and Manual Ability Classification System （MACS）. Results The classification of GMFCS and MACS was Ⅰ～Ⅱ in 33 (60.0%) children with spastic diplegia; Ⅲ～Ⅴ in 28 (75.7%) children with spastic quadriplegia; Ⅰ in 21 (55.3%) children with spastic hemiplegia, and GMFCS Ⅱ in 2 children, MACS Ⅱ in 14 children. There was moderate correlation between GMFCS and MACS （r=0.67，P<0.05）. Conclusion GMFCS and MACS can be used to determine the gross motor function and manual ability in CP children of different types.

Abstract: Objective　To investigate the distribution and drug resistance of pathogenic bacteria separated from ascites of patients in Children’s Hospital Affiliated to Zhengzhou University from 2015 to 2021, and to provide a basis for rational clinical antimicrobial agents. Methods　Bacterial culture, bacterial identification and drug sensitivity analysis were performed on 1 058 non-duplicate ascites culture specimens from January 2015 to December 2021. The clinica1 and microbiologica1 data were ana1yzed by WHONET 5.6 and SAS 9.4 Results　Of the 1 058 specimens, 586 (55.39%) were positive for pathogenic bacteria, with a total of 781 strains isolated. There was no significant trend of increase or decrease in the positivity rate over different years. Male children (63.99%) were more prevalent than female children. Appendicitis (59.22%) was the most common disease and Escherichia coli was the most common causative bacteria. Among neonates (≤28 d), the bacteria with the highest detection rate were Klebsiella pneumoniae (23.50%) and Enterococcus faecium (23.50%), while among children (>28 d), the highest detection rate was Escherichia coli (35.98%). Gram-negative bacteria accounted for 64.79% of the 781 strains, mainly Escherichia coli (38.28%), Klebsiella pneumoniae (8.58%), and Pseudomonas aeruginosa (5.89%); Gram-positive bacteria accounted for 29.45%, mainly Enterococcus faecium (8.58%), Streptococcus constellatus (2.69%), and Enterococcus avium (2.43%); fungi accounted for 1.66% and anaerobic bacteria accounted for 4.10%. The resistance rates of Escherichia coli to cefoperazone/sulbactam, piperacillin/tazobactam, imipenem and meropenem were 6.02%, 4.35%, 4.35%, and 3.68%, respectively. The resistance rates of Klebsiella pneumoniae to these drugs were 59.70%, 59.70%, 50.75% and 53.73% respectively. Linezolid-resistant strains of Enterococcus faecium were found. Conclusion　Appendicitis is the most common abdominal infection in children, and the distribution of ascites pathogens varies with ages and diseases. The pathogenic bacteria are mainly Gram-negative bacteria, and the drug resistance of Klebsiella pneumoniae was more serious. It is particularly important to use antibiotics correctly and rationally to reduce the emergence of drug resistant bacteria.