Objective To improve the nursing skills of low age and service seniority nurses and meet the needs of clinical nurses,in order to serve patients better and improve the quality of cave.Methods According to knowledge tests 50 points for nurse job-related skill issued by Chinese Ministry of Health,our hospital selected 12 commonly used basic operation skills and designed comprehensive cases through integration and reformation.We trained and examined nurses with undergraduate degree came to our hospistal in 2008.Results After training,the score of clinical nursing skill reached 91.81,86 nurses reached the standard and the effective compliance rate was 95%.Conclusions It proved to be an effective method to improve the nursing skills of low-age and service seniority nurses by carrying out the comprehensive casestyle training methods.

PTPN11 is the most common mutation gene of RAS disease, which is located in the upstream of RAS/MAPK pathway and participates in signal transduction. Because the molecular mechanism of RAS's disease involves the same pathway, it may present a certain commonality in clinic, but the different genotypes with PTPN11 mutation may also express different phenotypes. Therefore, it is not easy to identify and diagnose this disease early in clinic. The present article aims to analyze the correlation between the clinical phenotype and genotype of 4 patients with RAS disease.

Objective: To investigate the long-term effects of GnRHa treatment on final height gain, gonadal function, and body mass index(BMI) in children with central precocious puberty(CPP) or early and fast puberty(EFP), and to explore the influencing factors of height gain and early predictors. Methods: Fifty patients with CPP and 44 patients with EFP who were treated with GnRHa for more than 2 years were enrolled(80 females and 14 males). Body height, bone age, BMI, gonads hormone, uterus and ovarian volumes(female), testicular volume(male), and other parameters before and after treatment were measured. Results: (1)For girls: GnRHa plus GH treatment gained more final height compared with GnRHa treatment [(10.69±5.73) cm vs (7.42±5.76) cm, P<0.05]. Height lost >5cm at the initial treatment benefited much more for the final height compared with height lost<5cm [(10.65±3.32) cm vs (6.51±3.40) cm, P<0.01]. The proportion of overweight/obesity decreased when reaching the final height compared with the initial treatment and stopping the treatment. Serum LH level, uterine and ovarian volume were significantly decreased after stopping treatment compared with before treatment, and increased half a year to 1 year after stopping treatment.100% of girls had menarche and 95% reached the regular cycle 3 years after stopping treatment.(2)For boys: GnRHa plus GH treatment and GnRHa treatment gained height by(8.78±5.2) and(7.99±4.82) cm, respectively. Serum LH level and testicular volume were significantly decreased after stopping treatment as compared with those before treatment, and increased for half a year to 1 year after stopping treatment. Conclusion GnRHa treatment can significantly improve the final height for girls with CPP and EFP. The patients with more height lost could gain more height, which can be used as a predictor of height gain.

The clinical features and laboratory data of two patients with congenital lipoid adrenal hyperplasia (CLAH)were collected. The genomic DNA was extracted from the peripheral blood white cells in the two patients and their family members and the STAR gene was screened for mutations by PCR and Sanger sequencing. Patient 1 was a girl aged 2 years and 8 months,and she visited us because of continual cyanosis for more than two years. Physical examination showed no obvious pigmentation or clitoral hypertrophy,and Tanner stage was B1P1. Clinical examination revealed serum ACTH 1 284.1 pg/ml and 17α-hydoxyprogesterone(17-OHP)0.54 ng/ml, with Karyotype 46, XX. Genetic analysis showed compound heterozygous mutations of c.201_202delCT and c.229C>T in the STAR gene. Her father carried heterozygous c.201_202delCT mutation, and her mother showed heterozygous c.229C>T mutation. Patient 2 was a girl aged 22 years and referred to us because of dark skin for more than 21 years. Physical examination revealed generalized hyperpigmentation,with Tanner stage B5P2. Hormone examination showed ACTH>2 000 pg/ml and serum cortisol 0.77μg/dl. Karyotype analysis revealed 46,XX. Genetic analysis found compound heterozygous mutations of c.64+1G>C and c.707_708delinsCTT in the STAR gene,which descended from her father and mother respectively. Of note,c.64+1G>C is a novel splicing mutation of STAR gene.

Objective:To explore the real experience of parents caring for children with Kawasaki disease complicated with giant coronary artery aneurysm at home,in order to provide reference for targeted interventions.Methods:With phenomenological approaches in qualitative study,semi-structured interviews were conducted among 10 parents whose children with Kawasaki disease complicated with giant coronary artery aneurysm, and Colaizzi 's 7-step analysis method was used to analyze the data and extract the theme.Results:The care experience of parents were classified into four themes:continued negative emotions;lacked of family care knowledge;bore a huge burden of care; eager for support strength.Conclusions:Parents of children with Kawasaki disease complicated with giant coronary artery aneurysm have many difficulties in disease management,family care and other aspects in the process of caring for children.They also bear a greater burden of psychological pressure.Medical workers should pay attention to parent′s psychological status and related problems,and provide effective intervention measures to promote the rehabilitation of children and improve the quality of life of the family.