ABSTRACT
Purpose@#: This study aims to explore nursing activities after the decision to discontinue life-sustaining treatment, awareness of a good death, and perception of life-sustaining treatment decisions among nurses in intensive care units (ICUs) at tertiary general hospitals. @*Methods@#: Participants were 173 nurses working in two tertiary general hospitals. The data were collected using structured questionnaires and analyzed using an independent t-test, paired t-test, one-way ANOVA, Scheffé’s test, and Pearson’s correlation coefficient. @*Results@#: Participants were 173 nurses working in two tertiary hospitals. The nursing activity increase was the greatest in the spiritual domain, and the physical domain was where the activities decreased the most. There were significant associations between Awareness of good death (Clinical) and Perception of life-sustaining treatment decision(r=.26, p＜.001), Awareness of good death (Closure) and Perception of life-sustaining treatment decision(r=.36, p＜.001), and Awareness of good death (Personal control) and Perception of life-sustaining treatment decision(r=.49, p＜.001). @*Conclusion@#: Based on the results, systematic education programs and job training are required to improve the awareness regarding good death and perception of life-sustaining treatment decision for nurses in ICUs where discontinuing lifesustaining treatment decisions are made.
ABSTRACT
The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12).This is a rare case report of CS with DEE-SWAS in a Korean patient.