Objective To improve the data disaster response capacity of hospital information system and protect the safety of hospital data. Methods The data for disaster-related technical characteristics was comparatively analyzed. Results The variety of disaster recovery technologies has the advantages and disadvantages to choose the best program. Conclusion Only the establishment of disaster recovery applications can solve the data security and protect the hospital's business continuity.

Objective To discuss the relationship between clinical pathway management and semi-structured electronic medical record(EMR),and explore the effects of EMR on hospital information system and its management quality.Methods EMR was used in combination with quality management and clinical pathway management.The relationship between clinical pathway management and semi-structured electronic medical record(EMR) was researched.Results Semi-structured EMR can increase the clinical pathway managerment and improve the clinical quality of management,the competitiveness of hospital can be upgraded.Conclusion Hospital should widely develop semi-structured EMR and promote the clinical pathway managerment in the future.

Objective The optimal treatment for chronic type B dissection remains controversial. The purpose of this study was to report early and mid-term results of thoracic endovascular aortic repair (TEVAR) of chronic type B aortic dissection. Methods Methods From June 2001 to September 2007, 84 patients with chronic type B aortic dissection received TEVAR. The time between onset of dissection and TEVAR was (13.9 ± 22.0) months (ranged 1 - 120 months). All patients were followed for 6 - 86 months [mean (33.2 ± 19.2) months]. Results The entry tear was completely sealed in 77 cases ( 91.7% ) during TEVAR. The incidence of incomplete seal was 8.3%. One-month mortality was 1. 2%. One patient had retrograde type A dissection 1 month after operation. Four patients received a second TEVAR during follow-up :3 for endoleaking and 1 for newly formed intima tear. Seven patients (8.3%) died during follow-up: 3 thoracic aorta rupture due to endoleaking, 1 abdominal aorta rupture caused by continuous dilation of the abdominal aorta, unrelated to aortic dissection deaths in 2 and 1 died of unknown cause. The Kaplan Meier actuarial survival curve showed a 7-year survival rate of 84.4%. Conclusion Early and mid-term results showed that TEVAR was effective in treating chronic type B aortic dissection. Endoleak was the main cause of death during follow-up. With increasing of physician's experience and refinement of the stent-graft, results are likely to improve in the future.

OBJECTIVES: To investigate genotype-phenotype characteristics and long-term prognosis of neonatal carbamoyl phosphate synthetase 1 (CPS1) deficiency among children through newborn screening in Zhejiang province. METHODS: The clinical and follow-up data of children with CPS1 deficiency detected through neonatal screening and confirmed by tandem mass spectrometry and genetic testing in Zhejiang Province Newborn Disease Screening Center from September 2013 to August 2023 were retrospectively analyzed. RESULTS: A total of 4 056 755 newborns were screened and 6 cases of CPS1 deficiency were diagnosed through phenotypic and genetic testing. Ten different variations of CPS1 genewere identified in genetic testing, including 2 known pathogenic variations (c.2359C>T and c.1549+1G>T) and 8 unreported variations (c.3405-1G>T, c.2372C>T, c.1436C>T, c.2228T>C, c.2441G>A, c.3031G>A, c.3075T>C and c.390-403del). All patients had decreased citrulline levels (2.72-6.21 μmol/L), and varying degrees of elevated blood ammonia. The patients received restricted natural protein intake (special formula), arginine and supportive therapy after diagnosis, and were followed-up for a period ranging from 9 months to 10 years. Three patients experienced hyperammonemia, and one patient each had attention deficit hyperactivity disorder, transient facial twitching and increased muscle tone. One patient died, while the other five surviving patients had normal scores of the Ages & Stages Questionnaires (ASQ) and Griffiths Development Scales up to the present time; 4 cases had combined height or weight lag and one case was normal in height and weight. CONCLUSIONS Low citrulline levels and hyperammonemia are common in CPS1 deficiency patients in Zhejiang. Most gene variants identified were specific to individual families, and no hotspot mutations were found. Early diagnosis through newborn screening and following standardized treatment can significantly improve the prognosis of the patients.
Child ; Humans ; Infant, Newborn ; Carbamoyl-Phosphate Synthase I Deficiency Disease/therapy* ; Neonatal Screening ; Follow-Up Studies ; Hyperammonemia ; Citrulline/genetics* ; Retrospective Studies ; Mutation