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1.
International Journal of Pediatrics ; (6): 579-583, 2021.
Article in Chinese | WPRIM | ID: wpr-907282

ABSTRACT

Infantile liver failure syndrome-2(ILFS2)is a rare autosomal recessive disorder caused by neuroblastoma amplified sequence(NBAS)gene mutation, manifested as recurrent acute liver failure(ALF)with fever/infection-related pathogenesis.First-onset ALF is common in infants or early childhood(8 months to 3 years of age). The main characteristic of this disease is that the liver function can be recovered completely in the interval, and the definitive diagnosis is based on the identification of NBAS gene mutation in gene analysis.Until now, the pathogenesis of ILFS2 is not yet fully understood.Patients can be treated by supportive treatment clinically, while liver transplantation is the only treatment option currently available for patients with end-stage ALF.This review will focus on the recent progress in the pathogenesis and treatment of ILFS2.

2.
Chinese Journal of Practical Nursing ; (36): 1790-1792, 2019.
Article in Chinese | WPRIM | ID: wpr-803349

ABSTRACT

Objective@#To explore the application effect of improved drainage bag carrying method in the nursing of biliary drainage tube.@*Methods@#Sixty-five patients with biliary drainage tube were divided into two groups by convenient sampling. There were 33 cases in the control group and 32 cases in the observation group. When going out of bed, the observation group used the improved drainage bag carrying method to carry the drainage bag, and the control group used the traditional hand-held method or the S-hook external drainage bag. The catheter drainage failure rate, decoupling rate, retrograde infection rate, and satisfaction of tube activity in the two groups were compared between the two groups.@*Results@#There were no significant differences in catheter drainage failure rate, decoupling rate and retrograde infection rate between the two groups (P>0.05). The satisfaction of the tube with the improved drainage bag carrying method was (10.22±0.94) points, while (7.36±1.03) points in the contrast group. There was statistically significance between the two groups (t=11.685, P< 0.05).@*Conclusion@#The improved drainage bag carrying method has improved the quality of care in the department of catheter care, and the patient′s satisfaction with the nursing staff has also been improved.

3.
Chinese Journal of Practical Nursing ; (36): 1790-1792, 2019.
Article in Chinese | WPRIM | ID: wpr-752730

ABSTRACT

Objective To explore the application effect of improved drainage bag carrying method in the nursing of biliary drainage tube. Methods Sixty-five patients with biliary drainage tube were divided into two groups by convenient sampling. There were 33 cases in the control group and 32 cases in the observation group. When going out of bed, the observation group used the improved drainage bag carrying method to carry the drainage bag, and the control group used the traditional hand-held method or the S-hook external drainage bag. The catheter drainage failure rate, decoupling rate, retrograde infection rate, and satisfaction of tube activity in the two groups were compared between the two groups. Results There were no significant differences in catheter drainage failure rate, decoupling rate and retrograde infection rate between the two groups (P>0.05). The satisfaction of the tube with the improved drainage bag carrying method was (10.22±0.94) points, while (7.36±1.03) points in the contrast group. There was statistically significance between the two groups (t=11.685, P< 0.05). Conclusion The improved drainage bag carrying method has improved the quality of care in the department of catheter care, and the patient′s satisfaction with the nursing staff has also been improved.

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