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사립체 근병증 환자에서 발생한 자가항체 양성의 당뇨병성 케톤산증 1례
남순영,허림,권영희,이지은,조성윤,진동규,Nam, Soon Young,Huh, Rimm,Kwun, Younghee,Lee, Jieun,Cho, Sung Yoon,Jin, Dong-Kyu 대한유전성대사질환학회 2014 대한유전성대사질환학회지 Vol.14 No.2
사립체 근병증은 사립체 호흡 사슬의 장애로 인한 것으로 내분비계 관련 증상이 흔히 동반되고 그 중 당뇨병이 상대적으로 높은 빈도를 보인다고 알려져 있다. 사립체 근병증에서의 당뇨병은 사립체 기능 장애로 인한 인슐린 분비의 결함으로 발생하고, 대개 인슐린 의존성이나 당뇨병성 케톤산증으로 발현하거나 자가 항체가 검출되는 경우는 드물다. 저자들은 사립체 근병증 환자에서 당뇨병성 케톤산증으로 발현하고 Anti-GAD antibody와 Anti-insulin auto-antibody가 모두 양성으로 확인된 인슐린 의존성 당뇨병을 진단하였기에 이를 기존의 문헌과 비교하여 보고하는 바이다. Mitochondrial myopathy results from a primary dysfunction of the respiratory chain and is frequently accompanied with endocrine manifestations. Among the endocrine manifestations of mitochondrial disease, diabetes mellitus is relatively common. Diabetes mellitus in the mitochondrial myopathy is usually insulin dependent due to the defect in insulin secretion resulted from mitochondrial dysfunction. But it is seldom manifested as diabetes ketoacidosis and doesn't usually have an auto-antibody. We report a patient with mitochondrial myopathy who was diagnosed as having diabetes mellitus by presenting as diabetes ketoacidosis and had both of the auto-glutamic acid decarboxylase (GAD) antibody and anti-insulin auto-antibody.
Survival of Patients with Trisomy 18 Based on the Treatment Policy at a Single Center in Korea
남순영,안소윤,전지영,윤신애,박가영,최수영,성세인,유혜수,장윤실,박원순 대한신생아학회 2014 Neonatal medicine Vol.21 No.4
Purpose: Although discussion about active treatment of trisomy 18 is increasing,there are no previous articles regarding this subject in Korea. In order to provideobjective data about the clinical characteristics and survival of patients with trisomy18, based on the treatment policy, to medical teams and parents with trisomy 18, wereviewed the medical records of such patients at a single center in Korea. Methods: This is a retrospective study of 22 patients diagnosed with trisomy 18 at theSamsung Medical Center between 1995 and 2013. We collected data about the clinicalcharacteristics, including demographics, birth history, diagnosis method, and associatedanomalies. We analyzed the survival in days, according to three broad categoriesof treatment policy: give-up, conservative management and active treatment. Results: Of the 22 patients with confirmed trisomy 18, the majority were female (19,86%). The median gestational age was 39 weeks (range, 31–41 weeks) and the medianbirth weight is 2,029 g (range, 1,130–2,990 g). Among the anomalies associated withtrisomy 18, ventricular septal defect (86%) and patent ductus arteriosus (81%) werethe most common cardiac anomalies; giant cisterna magna (59%) was the mostcommon central nervous system anomaly; and clenched hands (73%) and low setears (59%) were the most common structural anomalies. The survival based on thetreatment policy was the highest in the active treatment group, followed by theconservative management group. The give-up group had the lowest survival. Conclusion: It is possible to achieve an improvement in both survival and symptomrelief for patients with trisomy 18, despite poor neurological outcome and highmortality. Medical personnel need to provide objective data on trisomy 18 to theparents, and determine the treatment policy through careful discussion.
가정 간호를 통한 수액 치료로 성공적으로 관리된 새로운 변이를 가진 프로피오닌산혈증 1례
양아람,남순영,김진섭,김현영,박형두,진동규,조성윤,Yang, Aram,Nam, Soon Young,Kim, Jinsup,Kim, Hyun-young,Park, Hyung-Doo,Jin, Dong-Kyu,Cho, Sung Yoon 대한유전성대사질환학회 2016 대한유전성대사질환학회지 Vol.16 No.1
프로피오닌산혈증은 propionyl-CoA carboxylase(PCC)의 결핍으로 인한 드문 상염색체 열성 유전 대사 질환이다. 이는 분지 아미노산 대사 장애로 혈장과 소변에서 프로피오닌산과 다른 대사 산물들의 축적되게 된다. 감염, 질병 혹은 스트레스 등 이화작용을 유발하는 스트레스적인 상황은 빈번하게 응급 혹은 입원 치료가 필요하며 심각한 경우에는 환자가 혼수나 사망에 이를 수도 있다. 하지만 이런 잦은 입원과 응급실 방문은 환자와 가족들에게 일상생활을 힘들게 하는 등 많은 스트레스가 된다. 이에 저자들은 프로피오닌산혈증 환자에게 중심정맥관을 유치하고 가정 간호를 통해 계획적으로 예방적인 수액 치료를 유지하여 급성 대사 위기로 인한 잦은 입원을 예방하였고 환자와 가족들의 부담을 줄이는데 기여할 수 있었다. 또한 이 환자에게서 확인된 돌연변이가 이전에 보고된 바 없는 새로운 돌연변이로 확인되어 이를 같이 보고하는 바다. Propionic acidemia (PA) is a rare autosomal recessive metabolic disease caused by the deficiency of propionyl-CoA carboxylase (PCC). PA affects the catabolism of branched chain amino acid and oddchain fatty acid then results in accumulation of propionic acid and other metabolites in plasma and urine. Catabolic stress such as infection, illness or any stress can precipitate cause acute metabolic decompensation, especially in the first years of life. Acute metabolic decompensation commonly calls for emergency treatment or admission and if the patient is in a serious condition, it can lead to coma or death. But frequent admissions or visiting the emergency room are much burden to the patients and their kins. And we experienced the propionic academia with a confirmed novel mutation and the patient suffered from frequent admission and visiting the emergency room. So, we tried the regular home carebased fluid therapy after securing a central venous line. Finally, we succeeded in preventing frequent admissions resulted from acute metabolic decompensation and could contribute to relieving the burden to the patient and their family.
신규간호사의 그릿과 회복탄력성이 재직의도에 미치는 영향
김지은,유준옥,김근희,남순영,박경옥,김문향 한국정신간호학회 2023 정신간호학회지 Vol.32 No.3
Purpose: This study was conducted to investigate the influence of grit and resilience on the retention intention of new nurses. Methods: The participants were 148 new nurses who had worked at two tertiary hospitals for less than 12 months. The data were collected on self-reported questionnaires from October 25, 2022, to November 28, 2022. T-tests, one-way analysis of variance, Mann-Whitney U test, Kruskal-Wallis test, Spearman’s correlation analysis, and multiple regression were used to analyze the data. Results: Retention intention was significantly different according to placement in the desired department and job satisfaction. Retention intention showed a significant positive correlation with grit (p<.001) and resilience (p<.001). Significant factors influencing the retention intention of new nurses were resilience (β=.30, p<.001), grit (β=.27, p=.001), and job dissatisfaction (β=-.16, p=.029). These variables accounted for 29% (F=21.41, p<.001) of new nurses’ retention intention. Conclusion: The results of this study showed that considering these factors influencing the retention intention of new nurses might be helpful for strategies addressing nurse staffing challenges.