Shwachman-Diamond syndrome (SDS) is an autosomal recessive genetic disorder, consisting of exocrine pancreatic insufficiency, chronic neutropenia, neutrophil chemotaxis defects, metaphyseal dysostosis, short stature, dental caries, and multiple organ involvements. Although SDS is the second most common hereditary abnormality of exocrine pancreas following cystic fibrosis in the Western countries, it has rarely been reported in Asia. We diagnosed a case of SDS in a 42-month-old girl, and genetic analysis including the relatives of the patient confirmed the diagnosis for the first time in Korea. She had short stature, steatorrhea, dental caries, and recurrent prulent otitis media and pneumonias. Laboratory studies revealed cyclic neutropenia, and serum levels of trypsin, amylase, and lipase were decreased. Simple radiography revealed metaphyseal sclerotic changes at the distal femur. A CT scan demonstrated a fatty infiltration and atrophy of the pancreas. On direct sequencing analysis of Shwachman-Bodian-Diamond Syndrome gene exon 2 region, the patient was homozygous for the c.258+2T>C mutation and heterozygous for the c.183_184TA>CT mutation and c.201A>G single nucleotide polymorphism. Treatment with pancreatic enzyme replacement, multivitamin supplementation, and regular to high fat diet improved her weight gain and steatorrhea.
Child, Preschool ; Dental Caries/*genetics ; Dysostoses/*genetics ; Exocrine Pancreatic Insufficiency/*genetics ; Female ; Humans ; *Mutation ; Neutropenia/*genetics ; Pedigree ; Syndrome

OBJECTIVES: he purpose of this study was to examine psychosocial factors related to burnout of social welfare officers working in Jeonnam Province.METHODS: A total of 395 social welfare officers (male 99, female 296) working in 22 areas of Jeollanam-do province, were subjects of this study. We examined socio-demographic factors, using a self-reporting questionnaire. Subjects were asked to complete the Maslach Burnout Inventory-General Survey (MBI-GS), Center for Epidemiological Studies Depression Scale (CES-D), Perceived Stress Scale (PSS), and the Generalized Self-Efficacy Scale (GSS), to assess psychosocial factors affecting to burnout of social welfare officers.RESULTS: Among 395 subjects, 221 (55.9%) reported recent experiences of burnout. There was no significant difference in age between two groups, divided by burnout. Sex (p < 0.001), rank (p=0.003), working period (p=0.034), depression (p < 0.001) revealed differences between the burnout group and control group. Scores of PSS (p < 0.001) were higher, while the scores of GSS (p < 0.001) were lower in the burnout group, than control group. Multivariate logistic regression analysis revealed that female (OR 2.840, 95%CI 1.466–5.504, p=0.002), depressive high-risk group (OR 6.824, 95%CI 2.893–16.096, p < 0.001) PSS (OR 1.247, 95%CI 1.153–1.349, p < 0.001) and GSS (OR 0.950, 95%CI 0.930–0.971, p < 0.001), were significantly associated with burnout.CONCLUSION: We found that some factors, were associated with experienced burnout in social welfare officers. Depressive symptoms were the strongest associative factor, for burnout in public servants in charge of social welfare. Sex, stress and self-efficacy also correlated with burnout, and especially self-efficacy was a protecting factor.
Depression ; Epidemiologic Studies ; Female ; Humans ; Jeollanam-do ; Logistic Models ; Psychology ; Social Welfare

Objectives: ：This study was aimed to investigate the changes in metabolic syndrome (MetS) status and long-term impact of its components over a 10-year period in severe mental illness (SMI) patients in a national mental hospital. Methods: ：A total of 93 patients (schizophrenia=88, bipolar disorder=5) who met the diagnosis of Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revision (DSM-IV-TR) and participated in the MetS study in 2011 were included. MetS was defined by revised National Cholesterol Education Program-Adult Treatment Panel III (revised NCEP-ATP-III) guidelines. Results: ：The prevalence of MetS was significantly increased from 40.9% in 2011 to 60.2% in 2020. There were significant differences in admission status and hospitalization months, compared to the groups with and without MetS. Upon reviewing the changes over a decade, systolic blood pressure (SBP) was a significant factor in the group without MetS. In the group with MetS, SBP, waist circumference, and BMI (body mass index) were significant factors. Multiple logistic regression analysis revealed that hospitalization during follow-up periods [odds ratio (OR)=0.969, 95% confidence interval (CI): 0.948-0.991] and BMI (OR=1.426, 95% CI: 1.196-1.701) were significantly associated with MetS in subjects. Conclusion ：The prevalence of MetS in patients with SMI significantly increased over time. The admission status and hospitalization were also confirmed to be the significant values of MetS.

PURPOSE: Nursing is regarded as a female-dominated profession. However, the number of Korean males entering the field of nursing is increasing. This study examines which of the following factors influence professionalism in male and female student nurses: area of nursing, gender identity, gender stereotypes, satisfaction with the field, emotional intelligence, and interpersonal relationships. METHODS: In total, 133 male and 293 female student nurses were selected from four Korean provinces between July 2013 and February 2014 to participate in this study. The data were analyzed with descriptive statistics, t-tests, chi2-tests of independence, and multiple regression analyses using SPSS/Win 20 software. RESULTS: In males, 47.4% of variance in nursing professionalism was accounted for by grades, interpersonal relationships, gender stereotypes, and satisfaction with the field. In females, 41.9% of variance in nursing professionalism was accounted for by satisfaction with the field, interpersonal relationships, area of nursing, age, emotional intelligence and grades. Common factors that influenced both genders included satisfaction with the field, interpersonal relationships and grades. CONCLUSION: Although males and females revealed no significant differences in overall mean scores of nursing professionalism, several factors influencing nursing professionalism did differ by gender. Therefore, gender-specific nursing educational and training programs may be necessary to promote professionalism among student nurses.
Education ; Emotional Intelligence ; Female ; Gender Identity ; Humans ; Male ; Nursing

OBJECTIVES: Shiftwork is known to be one of the common causes of sleep and health problems and finally causes the decreased quality of life. The purpose of this study was to investigate the sleep patterns of shiftworking and daytime psychiatric nurses using actigraphy and compare it with subjective assessment for sleep. METHODS: Twenty-three shift-working and 25 daytime nurses were enrolled. They rated their sleep quality using Pittsburgh Sleep Quality Index(PSQI) and other self-rating scales were measured for psychosocial aspects. Actigraphy was applied to the subjects for a total of 7 days to measure the sleep parameters. They also wrote sleep diaries during the period of wearing actigraphy. Sleep-related parameters of actigraphy, global score and components of PSQI, and the results of other self-rating scales were compared between shift-working and daytime nurses. RESULTS: Although the global score of PSQI did not show significant difference, the PSQI components showed significant differences between two groups: the shift-working nurses showed lower sleep quality, more sleep disturbance and hypnotic medication use, and worsened daytime dysfunction than daytime nurses. The shift-working nurses showed significantly shorter total time in bed and total sleep time, lower sleep efficiency, and longer average awakening time than those of daytime nurses in actigraphy. CONCLUSIONS: The results showed that shift-working nurses experienced more sleep disturbances in both subjective and objective aspects of sleep than daytime nurses. This study also suggests that actigraphy may be useful to measure the objective aspects of sleep that are difficult to assess with subjective questionnaires alone.
Actigraphy ; Quality of Life ; Weights and Measures

BACKGROUND: The aim of this study was to screen Korean college students for correlates, and comorbidities associated with attention-deficit hyperactivity disorder (ADHD).METHODS: A total of 2,593 college students participated in the study. Socio-demographic and clinical data were collected and self-report scales, such as the Adult ADHD Self-Report Scale-Version 1.1, the Center for Epidemiologic Studies Depression Scale, the Korean version of the Mood Disorder, a modified Korean version of the 16-item Prodromal Questionnaire, and the Alcohol Use Disorders Identification Test were included. Students with and without ADHD were compared using univariable analyses, and the association of ADHD with other psychiatric comorbidities was predicted using multivariable analyses.RESULTS: Of the total participants, 4.7% were diagnosed with ADHD. Multivariable analysis revealed that ADHD in college students was significantly associated with depression, psychotic-like experience, alcohol abuse, and female sex after adjustment. We found that ADHD in young college students was associated with several psychiatric comorbidities.CONCLUSION: These results suggest the need for early detection of ADHD in young adults and highlight the importance of implementing early psychiatric intervention for problems such as depression, psychotic-like experience, and alcohol abuse in adults with ADHD.
Adult ; Alcoholism ; Attention Deficit Disorder with Hyperactivity ; Comorbidity ; Depression ; Epidemiologic Studies ; Female ; Humans ; Mass Screening ; Mood Disorders ; Prevalence ; Weights and Measures ; Young Adult

PURPOSE: The purpose was to compare the marginal fidelity and the fracture resistance of the zirconia crowns according to the various coping designs with different thicknesses and cement types. Material and METHODS: Zirconia copings were designed and fabricated with various thicknesses using the CAD/CAM system (Everest, KaVo Dental GmbH, Biberach., Germany). Eighty zirconia copings were divided into 4 groups (Group I: even 0.3 mm thickness, Group II: 0.3 mm thickness on the buccal surface and the buccal half of occlusal surface and the 0.6 mm thickness on the lingual surface and the lingual half of occlusal surface, Group III: even 0.6 mm thickness, Group IV: 0.6 mm thickness on the buccal surface and the buccal half of occlusal surface and the 1.0 mm thickness on the lingual surface and the lingual half of occlusal surface) of 20. By using a putty index, zirconia crowns with the same size and contour were fabricated. Each group was divided into two subgroups by type of cement: Cavitec(R) (Kerr Co, USA) and Panavia-F(R) (Kuraray Medical Inc, Japan). After the cementation of the crowns with a static load compressor, the marginal fidelity of the zirconia crowns were measured at margins on the buccal, lingual, mesial and distal surfaces, using a microscope of microhardness tester (Matsuzawa, MXT-70, Japan, x100). The fracture resistance of each crown was measured using a universal testing machine (Z020, Zwick, Germany) at a crosshead speed of 1 mm/min. The results were analyzed statistically by the two-way ANOVA and oneway ANOVA and Duncan's multiple range test at alpha= .05. RESULTS: Group I and III showed the smallest marginal fidelity, while group II demonstrated the largest value in Cavitec(R) subgroup (P < .05). For fracture resistance, group III and IV were significantly higher than group I and II in Cavitec(R) subgroup (P < .05). The fracture resistances of Panavia-F(R) subgroup were not significantly different among the groups (P > .05). Panavia-F(R) subgroup showed significantly higher fracture resistance than Cavitec(R) subgroup in group I and II (P < .05). CONCLUSION: Within the limitation of this study, considering fracture resistance or marginal fidelity and esthetics, a functional ceramic substructure design of the coping with slim visible surface can be used for esthetic purposes, or a thick invisible surface to support the veneering ceramic can be used depending on the priority.
Cementation ; Ceramics ; Collodion ; Crowns ; Esthetics ; Japan ; Zirconium

PURPOSE: This study aimed to examine the factors influencing the appearance of headache and backache following diagnostic lumbar puncture in children, focusing on the need for strict bed rest after lumbar puncture. METHODS: We studied 70 two-fifteen-year-old pediatric patients who underwent diagnostic lumbar puncture from July 2005 to July 2007 at Konkuk University Hospital. We divided them into two groups. Patients in the first group (n=24) were allowed free mobility and patients in the second group (n=46) were to have strict bed rest for four hours after puncture. Data were analyzed by age, sex, number of puncture attempts, cell counts and pressure in the cerebrospinal fluid (CSF), duration of bed rest, and occurrence of headache and backache. RESULTS: The rate of complications was not significantly related to sex, age, presence of enterovirus, CSF pressure, or postural headache. The occurrence of headache was significantly correlated with white blood cell (WBC) count in CSF (P=0.043). Symptom frequency did not differ significantly between the groups. Backache was significantly related to the frequency of puncture attempts (P=0.046). CONCLUSION: Strict bed rest following diagnostic lumbar puncture in children does not influence headaches and backaches. These are respectively related to the WBC count on the CSF profile and the frequency of attempts. Therefore, after lumbar puncture, absolute bed rest is not necessary and patients are more comfortable with free mobility.
Back Pain ; Bed Rest ; Cell Count ; Child ; Enterovirus ; Headache ; Humans ; Leukocytes ; Punctures ; Spinal Puncture

PURPOSE: Kawasaki disease(KD) is an important acute febrile systemic vasculitis disease. Various cytokines have been studied to evaluate the pathogenesis of vascular injury. Some cytokines have been implicated for vascular injury, but there has been no direct evidence. We compared cytokine profiles of KD with Henoch-Schonlein purpura(HSP), and febrile infections. METHODS: We investigated the serum concentrations of six cytokines and intracellular TNF-alpha and IL-6 levels in T cells and monocytes with acute KD, HSP and infections. We determined serum cytokine concentrations using the cytometric bead array method and detected intracellular TNF-alpha and IL-6 levels using QuantiBRITE and dual color flow cytometry. RESULTS: Serum IFN-gamma concentrations were increased more in KD and infections than in HSP and normal controls. Serum TNF-alpha concentrations were higher in KD and infections than in HSP and normal controls. And TNF-alpha concentrations in KD were lower than in infections. Serum IL-10 concentrations were higher in KD and in infections than in HSP and normal controls. And IL-10 concentrations in KD were higher than in infections. Serum IL-6 concentrations were higher in KD and in infections than in HSP and normal controls. Serum IL-4 and IL-2 concentrations were lower and similar to those of normal controls, respectively. However, intracellular IL-6 levels in T cells were higher in KD than in infections and intracellular IL-6 levels in monocytes were higher in KD than in infections. CONCLUSION: These results suggest that the alterations of various cytokines in KD are related to fever rather than to vasculitis. KD showed higher IL-10 levels in serum and intracellular IL-6 levels in T cell and monocyte than HSP. This reveals that the pathogenesis of KD is different from that of HSP.
Cytokines ; Fever ; Flow Cytometry ; Interferons ; Interleukin-10 ; Interleukin-2 ; Interleukin-4 ; Interleukin-6 ; Monocytes ; Mucocutaneous Lymph Node Syndrome* ; Purpura ; Systemic Vasculitis ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Vascular System Injuries ; Vasculitis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea.
Down Syndrome/complications ; Female ; Humans ; Korea ; Moyamoya Disease/*complications/*diagnosis ; Thyrotoxicosis/*complications ; Young Adult