AIM: An inherited deficiency of human lysosomal acid lipase (LAL)results in the rare conditions of Wolman disease and cholesteryl ester storage disease (CESD). We want to present the rare case of CESD in an adult.METH...AIM: An inherited deficiency of human lysosomal acid lipase (LAL)results in the rare conditions of Wolman disease and cholesteryl ester storage disease (CESD). We want to present the rare case of CESD in an adult.METHODS: We report about an adult female patient with severe chronic diarrhea and weight loss as a consequence of CESD. Clinical examination revealed signs of malabsorption and slightly elevated liver enzymes.RESULTS: Histopathologic changes in the liver tissue and DNA sequence analysis confirmed the diagnosis of CESD due to homozygosity for the most common CESD mutation,a G934A splice site defect encoded by exon 8 of the lysosomal acid lipase (LIPA) gene.CONCLUSION: It is the first case in the literature with diarrhea as a putative symptom of CESD in adult patients.展开更多
Objectives:This study was to longitudinally investigate the association between occupational change trajectories and mental health in the Korean population aged 45 years and older from the Korean Longitudinal Study of...Objectives:This study was to longitudinally investigate the association between occupational change trajectories and mental health in the Korean population aged 45 years and older from the Korean Longitudinal Study of Aging(KLo-SA).Methods:After excluding missing values,the data of 6,224 participants from thefirst to eighth waves of the KLo-SA were analyzed using t-test,Analysis of variance(ANOVA),Group-based Trajectory Model(GBTM)from 1–5th KLoSA and Time-Lagged Generalized estimating equation(GEE)model from 5–8th KLoSA to analyze the association between occupational change trajectories and mental health in the Korean population aged 45 years and older.Results:An analysis of the association between occupational change trajectories and mental health demonstrated that,among all age group,the“sustained in unemployment”group had a worse MMSE and CESD score compared to“sustained WC”group.unlike the under-54 age group,those aged≥55 years demonstrated reductions in the MMSE and CESD scores in the sustained unemployment and the changed working condition from Standard BC to Unemployment groups,whereas those who underwent a change from the Standard WC to unemployment group only demonstrated a reduction in MMSE scores.Conclusions:There was a close relationship between occupational change trajectories and mental health.Therefore,this study can serve as the foundation for policies and institutional measures to manage the deteriorate mental health in the late middle-aged and older population.展开更多
文摘AIM: An inherited deficiency of human lysosomal acid lipase (LAL)results in the rare conditions of Wolman disease and cholesteryl ester storage disease (CESD). We want to present the rare case of CESD in an adult.METHODS: We report about an adult female patient with severe chronic diarrhea and weight loss as a consequence of CESD. Clinical examination revealed signs of malabsorption and slightly elevated liver enzymes.RESULTS: Histopathologic changes in the liver tissue and DNA sequence analysis confirmed the diagnosis of CESD due to homozygosity for the most common CESD mutation,a G934A splice site defect encoded by exon 8 of the lysosomal acid lipase (LIPA) gene.CONCLUSION: It is the first case in the literature with diarrhea as a putative symptom of CESD in adult patients.
文摘Objectives:This study was to longitudinally investigate the association between occupational change trajectories and mental health in the Korean population aged 45 years and older from the Korean Longitudinal Study of Aging(KLo-SA).Methods:After excluding missing values,the data of 6,224 participants from thefirst to eighth waves of the KLo-SA were analyzed using t-test,Analysis of variance(ANOVA),Group-based Trajectory Model(GBTM)from 1–5th KLoSA and Time-Lagged Generalized estimating equation(GEE)model from 5–8th KLoSA to analyze the association between occupational change trajectories and mental health in the Korean population aged 45 years and older.Results:An analysis of the association between occupational change trajectories and mental health demonstrated that,among all age group,the“sustained in unemployment”group had a worse MMSE and CESD score compared to“sustained WC”group.unlike the under-54 age group,those aged≥55 years demonstrated reductions in the MMSE and CESD scores in the sustained unemployment and the changed working condition from Standard BC to Unemployment groups,whereas those who underwent a change from the Standard WC to unemployment group only demonstrated a reduction in MMSE scores.Conclusions:There was a close relationship between occupational change trajectories and mental health.Therefore,this study can serve as the foundation for policies and institutional measures to manage the deteriorate mental health in the late middle-aged and older population.