Objective:To explore the application value of scenario simulation teaching combined with two-way evaluation in standardized training of pediatric nursing.Methods:A total of 34 trainees who received standardized training of pediatric nursing in The First Affiliated Hospital of Air Force Medical University from March 2018 to March 2019 were selected as the control group, and another 42 trainees from April 2019 to July 2020 were selected as the study group. The control group used traditional teaching, and the study group used scenario simulation teaching combined with two-way evaluation. Theoretical examination and scenario simulation exercise examination were used to assess the theoretical knowledge and clinical practice ability of the trainees, and questionnaire survey was used to evaluate the satisfaction of the trainees with the teaching effect. SPSS 22.0 was used for t-test. Results:The scores of theoretical examination[(95.12±6.24) vs. (91.05±5.12)] and scenario simulation exercise examination (nursing practice skill operation ability, ability to combine theory and practice, clinical thinking and judgment ability, emergency handling ability, communication ability, humanistic care and professional learning ability, and work attitude) of the trainees in the study group were higher than those in the control group, and the differences were statistically significant ( P<0.05). The satisfaction evaluation of the trainees in the study group with the teaching effect was higher than that in the control group, and the difference was statistically significant ( P<0.001). Conclusion:Scenario simulation teaching combined with two-way evaluation can improve the theoretical knowledge, clinical practice ability, and teaching satisfaction of pediatric nursing trainees.

OBJECTIVETo investigate the feature of phenylalanine hydroxylase (PAH) gene mutations and provide guidance for genetic and prenatal diagnosis of patients with phenylketonuria from Shaanxi.

METHODSFor 55 patients whose blood Phe concentration was over 2.0 mg/dL, potential mutations in 13 exons and flanking sequences of the PAH gene were detected by PCR and DNA sequencing.

RESULTSA total of 98 mutations were detected in 110 PAH alleles, with the detection rate being 89.10%. Nine mutations have been identified in exon 7, which accounted for 33.67% of all. Exon 12 (14.29%) and exon 3 (12.24%) have followed. Thirty eight mutations, locating in exon2-exon12 and the flanking sequence, were detected in the 55 PKU patients. p.R243Q (24.49%) was the commonest mutation, whilstp.A47E, p.I65S and p.A259T were first discovered in China. After querying international databases including PAHdb and HGMD, the p.C334X was verified as the novel PAH gene mutation.

CONCLUSIONThe mutation spectrum of the PAH gene in Shaanxi has been identified. And a novel mutation has been identified. This may facilitate the diagnosis of PKU in the future.

Alleles ; Base Sequence ; Child ; Child, Preschool ; China ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Mutation ; Phenylalanine Hydroxylase ; blood ; genetics ; Phenylketonurias ; enzymology ; genetics