No abstract available.
Ichthyosiform Erythroderma, Congenital*

Marchiafava-Bignami disease(MBD) is a rare complication of chronic alcoholism characterized by symmetrical demyelination of the corpus callosum. We report a case of MBD in a Korean patient having chronic alcohol dependence. The patient exhibited mental depression, weakness of all four limbs and dysarthria. Laboratory data showed mild hyponatremia. Magnetic resonance imaging(MRI) revealed unusual symmetrical resolving lesions of the putamen in addition to the typically observed lesion of the corpus callosum. The neurologic disturbances were gradually improved with the normalization of low plasma sodium levels. Marked improvement of abnormal MRI findings was noted after thiamine supplement, though the patient still exhibited severe cognitive impairment.
Alcoholism ; Corpus Callosum ; Demyelinating Diseases ; Depression ; Dysarthria ; Extremities ; Humans ; Hyponatremia ; Magnetic Resonance Spectroscopy ; Marchiafava-Bignami Disease ; Plasma ; Putamen ; Sodium ; Thiamine

PURPOSE: The purpose of this study is to identify the effects of job demand and recovery experience from job stress on job embeddedness among workers in the service industry. METHODS: The participants were 223 workers from the service industry in P and Y Cities with the help of a structured self-report questionnaire, administered between July 10 and August 20, 2017. The data were analyzed using descriptive statistics, t-test, ANOVA, Scheffé test, Pearson correlation coefficients and stepwise multiple regression. RESULTS: There were significant differences in job embeddedness in terms of satisfaction with salary, continuous service, perceived stress level and the perceived health status of the subjects. There were significant positive correlations between role clarification in job demands (r=.55), recovery experience from job stress (r=.27) and job embeddedness. From the multiple regression analysis, the most significant factors affecting job embeddedness were found to be role clarification in job demands (β=.47), recovery experience from job stress (β=.23), and perceived stress level (β=.18). These variables explain 34.0 % of the total variance in job embeddedness. CONCLUSION: In order to increase job embeddedness among workers in the service industry, it is necessary to prepare measures to increase recovery experience from job stress and to decrease role clarificationin job demand, and perceived stress level.
Commerce ; Personnel Loyalty ; Recovery of Function ; Salaries and Fringe Benefits

Purpose: : This study aimed to understand the current state of patient-centered care for people with disabilities by reviewing the key definitions, elements, and aspects of previously published care models/programs that borrow a patient-centered approach using a scoping review methodology. Methods : Following Arksey and O’Malley’s five-step methodology, we conducted a keyword search in EMBASE, Web of Science, and Pubmed for articles published from 2000 to January 2022, and a total of 30 articles were selected from 1,305 initially extracted articles. Results : A total of 13 studies developed models of care for people with disabilities based on the concept of patient-centeredness, 11 studies developed and evaluated care programs with patient- centered components, and 6 studies prospectively evaluated the effectiveness of patient-centered programs in clinical trials. The studies were generally based on multidisciplinary teams and emphasized two-way communication, collaborative relationships, multidimensional assessment, and measurement of patient needs. However, elements of patient-centeredness varied across studies, and methodologies for measuring needs and evaluating effectiveness were inconsistent. Conclusion : Based on this study, there is a need for systematic research to identify integrated and desirable patient-centeredness elements in healthcare models/programs for people with disabilities. This will enable the development of more patient-centered and friendly healthcare services for people with disabilities.

Purpose: : This study aimed to understand the current state of patient-centered care for people with disabilities by reviewing the key definitions, elements, and aspects of previously published care models/programs that borrow a patient-centered approach using a scoping review methodology. Methods : Following Arksey and O’Malley’s five-step methodology, we conducted a keyword search in EMBASE, Web of Science, and Pubmed for articles published from 2000 to January 2022, and a total of 30 articles were selected from 1,305 initially extracted articles. Results : A total of 13 studies developed models of care for people with disabilities based on the concept of patient-centeredness, 11 studies developed and evaluated care programs with patient- centered components, and 6 studies prospectively evaluated the effectiveness of patient-centered programs in clinical trials. The studies were generally based on multidisciplinary teams and emphasized two-way communication, collaborative relationships, multidimensional assessment, and measurement of patient needs. However, elements of patient-centeredness varied across studies, and methodologies for measuring needs and evaluating effectiveness were inconsistent. Conclusion : Based on this study, there is a need for systematic research to identify integrated and desirable patient-centeredness elements in healthcare models/programs for people with disabilities. This will enable the development of more patient-centered and friendly healthcare services for people with disabilities.

Purpose: : This study aimed to understand the current state of patient-centered care for people with disabilities by reviewing the key definitions, elements, and aspects of previously published care models/programs that borrow a patient-centered approach using a scoping review methodology. Methods : Following Arksey and O’Malley’s five-step methodology, we conducted a keyword search in EMBASE, Web of Science, and Pubmed for articles published from 2000 to January 2022, and a total of 30 articles were selected from 1,305 initially extracted articles. Results : A total of 13 studies developed models of care for people with disabilities based on the concept of patient-centeredness, 11 studies developed and evaluated care programs with patient- centered components, and 6 studies prospectively evaluated the effectiveness of patient-centered programs in clinical trials. The studies were generally based on multidisciplinary teams and emphasized two-way communication, collaborative relationships, multidimensional assessment, and measurement of patient needs. However, elements of patient-centeredness varied across studies, and methodologies for measuring needs and evaluating effectiveness were inconsistent. Conclusion : Based on this study, there is a need for systematic research to identify integrated and desirable patient-centeredness elements in healthcare models/programs for people with disabilities. This will enable the development of more patient-centered and friendly healthcare services for people with disabilities.

PURPOSE: Cholestatic liver disease is a frequent complication of prolonged parenteral nutrition, especially in premature infants. The purpose of present study is to evaluate the clinical and biochemical efficacy of ursodeoxycholic acid (UDCA) in premature infants with total parenteral nutrition (TPN) cholestasis. METHODS: Retrospective chart review of 31 prematures with TPN cholestasis in Neonatal Intensive Care Unit of Chonnam University Hospital from January 1995 and December 2002 was done. Prematures were divided into two groups based on UDCA treatment: the study group (n=22, with UDCA treatment) and the control group (n=9, without UDCA treatment). Treatment efficacy of UDCA was evaluated by monitoring the changes of serum direct bilirubin level for more than one to two months. RESULTS: In study group, cholestasis appeared at a mean age of 23+/-18.8 days after a mean of 21+/-19.7 days of TPN. UDCA was initiated (ranged 15-30 mg/kg/day) at a mean age of 54+/-22.3 days for a mean of 32+/-19.0 days. There was no significant difference in decrease of direct bilirubin level between the study and control group. However, the day of initial enteral feeding and full enteral feeding were earlier in control group compared with study group. CONCLUSIONS: This study shows that UDCA treatment is not effective in the treatment of TPN cholestasis among premature infants unlike that of results reported in children and adults. To reduce the incidence of TPN cholestasis in premature infants, early introduction of enteral feeding as soon as possible is most important.
Adult ; Bilirubin ; Child ; Cholestasis* ; Enteral Nutrition ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Jeollanam-do ; Liver Diseases ; Parenteral Nutrition ; Parenteral Nutrition, Total* ; Retrospective Studies ; Treatment Outcome ; Ursodeoxycholic Acid*

Trisomy 14 mosaicism is a rare chromosomal abnormality with distinct and recognizable clinical features. We report a patient with presumed retinal dystrophy having diffuse retinal pigment epithelial abnormalities, which has not been previously reported in association with trisomy 14. This case expands the clinical spectrum of this rare entity.
Abnormalities, Multiple/genetics ; Chromosomes, Human, Pair 14 ; Female ; Humans ; Infant, Newborn ; Mosaicism ; Retinal Diseases/*congenital/genetics ; *Trisomy

Extramammary Pagets disease of the vulva is an uncommon vulvar neoplasm with a high rate of recurrence. We report a young woman in whom vulval Paget's disease occurred in association with local adenocarcinoma. The patient presented at the age of 28 years with slowly growing vulval mass which had some mucoid discharge. Biopsy of the mass showed Paget's disease with associated local adenocarcinoma. This patient received modified radical vulvectomy with unilateral inguinofemoral lymphadenectomy. Until now she is under follow up without evidence of recurrence for 24 months. According to our medline research from 1980 to 2000, this case is the youngest asian patient yet recorded. We recommend that abnormally growing vulvar mass need to be biopsied eventhough she is young.
Adenocarcinoma* ; Asian Continental Ancestry Group ; Biopsy ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Paget Disease, Extramammary ; Recurrence ; Vulva ; Vulvar Neoplasms

PURPOSE: To describe the ophthalmoscopic features and natural history in a case series of eyes that developed intraocular hemorrhages associated with perinatal distress and to evaluate their clinical courses. METHODS: A retrospective chart review of 289 neonates with a medical history of perinatal distress was conducted. Among these 289 patients (578 eyes), 29 eyes of 17 neonates were found to have had retinal hemorrhages or vitreous hemorrhages (VH). A comprehensive chart review, including details of fundoscopic findings and perinatal history, was conducted. RESULTS: Intraocular hemorrhage was present in 5.5% of the patients. Most hemorrhages (82.7%) were intraretinal. In our population, 17% (n = 5) of hemorrhages resolved within two weeks, but 31% (n = 9) did not resolve even after four weeks. Most hemorrhages spontaneously resolved without any specific sequelae; however, one infant's dense unilateral VH persisted up to three months after birth. When the patient was seen again at 3.5 years of age, she had developed axial myopia and severe amblyopia of the involved eye. CONCLUSIONS: In asphyxiated newborns, the possibility of intraocular hemorrhages should be considered. Long-standing, dense hemorrhages obscuring the macula may lead to severe vision deprivation amblyopia. Therefore, ophthalmic examination should be considered in neonates with perinatal distress, and close observation is necessary for hemorrhages that do not resolve in this amblyogenic age group.
Asphyxia Neonatorum/*complications ; Female ; Follow-Up Studies ; Humans ; Infant, Newborn ; Male ; Ophthalmoscopy ; Retinal Hemorrhage/diagnosis/*etiology ; Retrospective Studies ; Time Factors ; Visual Acuity ; Vitrectomy