Purpose: This study is descriptive research to examine the effects of the clinical nurse’s character and nursing informatics competencies of nurses working at veterans hospitals on their nursing performance. Methods: Data were collected from 192 nurses with over one year of clinical experience working at three veterans' hospitals in B, D, and G Metropolitan from September 6 to September 30, 2021. The collected data were analyzed with the SPSS 25.0 program in descriptive statistics, an independent t-test, ANOVA, Pearson’s correlation coefficient, and multiple regression. The Scheffé test was used as a post hoc test. Results: Nursing performance had a statistically significant positive correlation with the clinical nurse’s character (r=.69, p<.001) and nursing informatics competency (r=.73, p<.001). The study results showed that nursing informatics competency (β=.49, p<.001) and the clinical nurse’s character (β=.41, p<.001) significantly affected nursing performance. The total explanatory power of these variables was 62.4% (F=32.26, p<.001). Conclusion The above results of this study show that the most significant factors in nursing performance are nursing information competency and the clinical nurse’s character exhibit better nursing performance. Programs for improving nursing informatics competency and implementation methods need to be developed to improve nursing performance. It is also necessary to apply an effective clinical nurse’s character program to enhance the level of the clinical nurse’s character.

Purpose: This study is descriptive research to examine the effects of the clinical nurse’s character and nursing informatics competencies of nurses working at veterans hospitals on their nursing performance. Methods: Data were collected from 192 nurses with over one year of clinical experience working at three veterans' hospitals in B, D, and G Metropolitan from September 6 to September 30, 2021. The collected data were analyzed with the SPSS 25.0 program in descriptive statistics, an independent t-test, ANOVA, Pearson’s correlation coefficient, and multiple regression. The Scheffé test was used as a post hoc test. Results: Nursing performance had a statistically significant positive correlation with the clinical nurse’s character (r=.69, p<.001) and nursing informatics competency (r=.73, p<.001). The study results showed that nursing informatics competency (β=.49, p<.001) and the clinical nurse’s character (β=.41, p<.001) significantly affected nursing performance. The total explanatory power of these variables was 62.4% (F=32.26, p<.001). Conclusion The above results of this study show that the most significant factors in nursing performance are nursing information competency and the clinical nurse’s character exhibit better nursing performance. Programs for improving nursing informatics competency and implementation methods need to be developed to improve nursing performance. It is also necessary to apply an effective clinical nurse’s character program to enhance the level of the clinical nurse’s character.

Purpose: This study is descriptive research to examine the effects of the clinical nurse’s character and nursing informatics competencies of nurses working at veterans hospitals on their nursing performance. Methods: Data were collected from 192 nurses with over one year of clinical experience working at three veterans' hospitals in B, D, and G Metropolitan from September 6 to September 30, 2021. The collected data were analyzed with the SPSS 25.0 program in descriptive statistics, an independent t-test, ANOVA, Pearson’s correlation coefficient, and multiple regression. The Scheffé test was used as a post hoc test. Results: Nursing performance had a statistically significant positive correlation with the clinical nurse’s character (r=.69, p<.001) and nursing informatics competency (r=.73, p<.001). The study results showed that nursing informatics competency (β=.49, p<.001) and the clinical nurse’s character (β=.41, p<.001) significantly affected nursing performance. The total explanatory power of these variables was 62.4% (F=32.26, p<.001). Conclusion The above results of this study show that the most significant factors in nursing performance are nursing information competency and the clinical nurse’s character exhibit better nursing performance. Programs for improving nursing informatics competency and implementation methods need to be developed to improve nursing performance. It is also necessary to apply an effective clinical nurse’s character program to enhance the level of the clinical nurse’s character.

Purpose: This study is descriptive research to examine the effects of the clinical nurse’s character and nursing informatics competencies of nurses working at veterans hospitals on their nursing performance. Methods: Data were collected from 192 nurses with over one year of clinical experience working at three veterans' hospitals in B, D, and G Metropolitan from September 6 to September 30, 2021. The collected data were analyzed with the SPSS 25.0 program in descriptive statistics, an independent t-test, ANOVA, Pearson’s correlation coefficient, and multiple regression. The Scheffé test was used as a post hoc test. Results: Nursing performance had a statistically significant positive correlation with the clinical nurse’s character (r=.69, p<.001) and nursing informatics competency (r=.73, p<.001). The study results showed that nursing informatics competency (β=.49, p<.001) and the clinical nurse’s character (β=.41, p<.001) significantly affected nursing performance. The total explanatory power of these variables was 62.4% (F=32.26, p<.001). Conclusion The above results of this study show that the most significant factors in nursing performance are nursing information competency and the clinical nurse’s character exhibit better nursing performance. Programs for improving nursing informatics competency and implementation methods need to be developed to improve nursing performance. It is also necessary to apply an effective clinical nurse’s character program to enhance the level of the clinical nurse’s character.

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. MATERIALS AND METHODS: Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. RESULTS: All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. CONCLUSION: Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.
Abnormalities, Multiple ; Child ; Chromosome Aberrations ; Cytogenetic Analysis ; Cytogenetics ; Face ; Fingers ; Genetic Counseling ; Heart Defects, Congenital ; Hematologic Diseases ; Hip Dislocation ; Humans ; Intellectual Disability ; Joint Instability ; Karyotyping ; Medical Records ; Muscle Hypotonia ; Natural History ; Retrospective Studies ; Strabismus ; Vestibular Diseases

A 9-year-old Korean boy with lissencephaly was found dead at home. He had previously been diagnosed with lissencephaly that presented with infantile spasm on the basis of magnetic resonance imaging and electroencephalogram results. Antemortem chromosomal banding revealed a normal karyotype. A legal autopsy was requested to eliminate the possibility of neglect or abuse by his parents. The autopsy findings revealed type I lissencephaly with the associated microcephaly. No external wounds or decubitus ulcers were noted. Postmortem fluorescence in situ hybridization for the LIS1 locus and nucleotide sequence analysis of the whole coding regions of the LIS1 gene did not reveal any deletions. The antemortem and postmortem findings revealed that lissencephaly syndrome was associated with isolated lissencephaly sequence. External causes of death were excluded by the full autopsy and toxicology test results. Because patients with mental retardation are frequently victimized and suffer neglect or abuse, thorough external and internal examinations should be conducted at the time of autopsy.
Autopsy ; Base Sequence ; Cause of Death ; Child ; Child Abuse ; Classical Lissencephalies and Subcortical Band Heterotopias ; Clinical Coding ; Electroencephalography ; Fluorescence ; Forensic Pathology ; Humans ; In Situ Hybridization ; Infant ; Infant, Newborn ; Intellectual Disability ; Karyotype ; Lissencephaly ; Magnetic Resonance Imaging ; Microcephaly ; Parents ; Pressure Ulcer ; Spasms, Infantile ; Toxicology

Townes-Brocks syndrome (TBS) is a rare genetic disorder characterized by the classic triad of congenital anomalies of the anus, thumbs, and ears, with variable expressivity. Additionally, renal malformations, cardiac anomalies, and endocrine and eye abnormalities can accompany TBS, although less frequently. TBS is inherited in an autosomal dominant fashion; however, about 50% of patients have a family history of TBS and the remaining 50% have de novo mutations. SALL1, located on chromosome 16q12.1, is the only causative gene of TBS. SALL1 acts as a transcription factor and may play an important role in inducing the anomalies during embryogenesis. Clinical features of TBS overlap with those of other multiple anomaly syndromes, such as VACTERL syndrome, Baller-Gerold syndrome, Goldenhar syndrome, cat eye syndrome, and Holt-Oram syndrome. Consequently, there are some difficulties in differential diagnosis based on clinical manifestations. Herein, we report a Korean family with two generations of TBS that was diagnosed based on physical examination findings and medical history. Although the same mutation in SALL1 was identified in both the mother and the son, they displayed different clinical manifestations, suggesting a phenotypic diversity of TBS.
Anal Canal ; Animals ; Anus, Imperforate ; Cats ; Diagnosis, Differential ; Ear ; Embryonic Development ; Eye Abnormalities ; Family Characteristics ; Female ; Goldenhar Syndrome ; Hearing Loss, Sensorineural ; Humans ; Mothers ; Physical Examination ; Polydactyly ; Pregnancy ; Thumb ; Transcription Factors

Long QT syndrome(LQTS) is characterized by prolongation of the QT interval, frequent episodes of syncope, and ventricular tachycardia leading to sudden cardiac death in adolescents and young adults. Early diagnosis and prompt treatment to reduce the risk of life-threatening cardiac events is crucial, however sudden onset convulsive syncope may be misdiagnosed as epilepsy and lead to antiepileptic drug therapy for many years. We experienced a case of six-year-old boy who were having seizure triggered by exercise and being treated with antiepileptic drugs. He had normal EEG and brain MRI but his ECG revealed prolonged QT interval(QTc=477 ms) and treadmill test induced polymorphic ventricular tachycardia. After applying beta-blocker, he had no more seizure attacks. We report a case of long QT syndrome misdiagnosed as epilepsy with a brief review of related literatures.
Adolescent ; Anticonvulsants ; Brain ; Death, Sudden, Cardiac ; Early Diagnosis ; Electrocardiography ; Electroencephalography ; Epilepsy ; Exercise Test ; Humans ; Long QT Syndrome ; Seizures ; Syncope ; Tachycardia, Ventricular ; Young Adult

No abstract available.
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