No abstract available.
Wilms Tumor*

Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by widespread occurence of epithelioic cell. The authors experienced a case of sarcoidosis in 34-year-old female who has lacrimal gland enlargement, severe subcutaneous nodules and bilateral granulomatous uveitis. Histopathologic examination of the subcutaneous nodule of lid revealed sarcoidosis. In the course of corticosteroid therapy, subcutaneous nodules and enlarged lacrimal gland subsided gradually.
Adult ; Female ; Granulomatous Disease, Chronic ; Humans ; Lacrimal Apparatus ; Sarcoidosis* ; Uveitis*

No abstract available.
DNA* ; Lymphoproliferative Disorders*

No abstract available.
DNA* ; Lymphoproliferative Disorders*

BACKGROUND: The Korean Journal of Dermatology and the Annals of Dermatology are representative journals of dermatology in Korea. Evaluation of their content, authors and affiliations are valuable for our understanding of the situation, trends and interests of the Korean dermatologic society. OBJECTIVE: To analyze the original contributions in the major dermatology journals of Korea, and compare these with those of renowned international dermatology journals in terms of original contribution. METHODS: A total of 318 original articles from the last 3 volumes of the Korean Journal of Dermatology (2003, vol. 41 to 2005, vol. 43) were collected and analyzed for their characteristic features, such as categories, authors, and affiliations. We also compared these characteristic features with those of 18 original articles from the last 3 volumes of Annals of Dermatology (2003, vol. 15 to 2005, vol. 17), an English dermatologic journal in Korea. RESULTS: Thirty-two percent of original articles fell into the category of drug evaluation and clinical research, followed by laboratory and experimental research. Most articles had three to six co-authors (78%), and one (55.3%) or two (31.1%) affiliations. The number of articles by a specific corresponding author ranged from one to eight, and the majority of articles by a specific corresponding author were one (43.6%) during 3 years. These situations were similar with those of the Annals of Dermatology. The evaluation of the Korean Journal of Dermatology about charateristics of original articles showed a small number of original articles by a corresponding author and cooperative researches with other afilliations. The main subjects of articles were centralized to 3 major categories in the Korean Journal of Dermatology, whereas the other famous international journals showed a relatively uniform distribution. CONCLUSION: The participation of non-dermatologists was fewer in the Korean journals than the other famous international journals. The Korean dermatologic society should make an effort to involve the specialists of other fields in submitting excellent original articles to the Korean Journal of Dermatology. And for the academic position of the Korean dermatologic society, we should make an effort to register one of our journals to the SCI (science citation index). The Korean Journal of Dermatology should deal with more varied articles from new categories for a more uniform distribution of themes. It should also make more effort to improve the Korean Journal of Dermatology.
Dermatology* ; Drug Evaluation ; Korea* ; Specialization

No abstract available.
Heterotaxy Syndrome*

BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency of erythrocyte membrane protein defects in hereditary spherocytosis and correlation between some of the hereditary spherocytosis biochemical subsets and the selected clinical phenotype. METHODS: We evaluated the clinical and laboratory characteristics of 14 normal healthy persons and 23 hereditary spherocytosis patients and 8 their family members. The patients were divided into three groups based on clinical and hematological severity(mild, typical, severe). In addition to routine hematologic determlnatlons, osmotic fragility and autohemolysis, RBC membrane protein analysis were performed in all patients by densitometric tracing of SDS-PAGE(sodium dodecyl sulphate polyacrylamide gel electrophoresis) stained by Coomassle blue utilizing both the discontinuous buffer system of Laemmli with acrylamide linear gradient from 4% to 12% and the continuous buffer system of Fairbank with exponential gradient of acrylamide from 3.5% to 17%. RESULTS: 1) The patients could be seperated into three classes of different clinical severity as mild(3 cases), moderate(16 cases) and severe(4 cases) on the clinical feature. 2) Eighteen patients(82.6%) among 23 hereditary spherocytosis revealed abnormal erythrocyte membrane protein and we detected six patients(26.1%) with spectrin deficiency combined with ankyrin reduction, 4 patients(17.4%) with ankyrin deficiency, 4 patients(17.4%) with isolated spectrin deficiency and 3 patients(13.0%) with band 3 deficiency. Five HS patients(21.7%) showed normal RBC membrane protein. 3) Eight HS and their family members showed same RBC membrane protein deficiency. 4) The type and degree of RBC membrane protein reduction were variale with spectrin at 66~94%, with ankyrin at 48~82% of normal levels. These showed that each patient had different clinical severities according to different RBC membrane protein levels and type. CONCLUSION: RBC membrane protein abnormalities were observed in 82.6% of HS patients. The combined spectrin and ankyrin deficiency is the most common molecular defect in HS. The clinical severity and biochemical expression is heterogeneous. SDS-PAGE analysis of RBC membrane protein was provided the diagnosis of RBC membrane defects and basic molecular studies. We believed that the early identification of the biochemical defect responsible for HS is important because it is helpful starling point for the identification of the primary molecular defect, and it could help to anticipate the clinical outcome of the disease. For these reasons, we consider the SDS-PAGE of the red cell membrane to be of crucial importance for a complete evaluation of children with HS. Further studies with more cases would be to clarify the correlation between clinical and biochemical phenotypes.
Acrylamide ; Ankyrins ; Cell Membrane ; Child ; Diagnosis ; Electrophoresis, Polyacrylamide Gel* ; Erythrocyte Membrane* ; Erythrocytes* ; Erythrocytes, Abnormal ; Humans ; Membrane Proteins ; Membranes ; Osmotic Fragility ; Phenotype ; Population Characteristics* ; Spectrin ; Starlings

PURPOSE: This study was performed to identify perceptions on fixed night shift system and turnover intention of general hospital nurses and to explore a desirable application plan for a fixed night shift system. METHODS: This study was a cross-sectional survey. Participants were 300 nurses working at one general hospital in GyeongGi-Do, Korea. Data were collected from November 3 to 7, 2014, with a structured questionnaires and analyzed using SPSS/PC ver 20.0 programs. RESULTS: Of the participants 73.5% had thought about turnover because of night shifts and the preconditions for desirable fixed night shift system were a payment plan differentiated according to work conditions and an adequate number of nursing staff. Economic and administrative supports like 'raising extra-pay for night shift' and 'increasing the number of paid holidays' were very important for a desirable fixed night shift system. CONCLUSION: The results indicate that the most important factor for a desirable fixed night shift system to decrease nurses' turnover intention is economic and administrative support according to the needs of the nurses. So nursing managers need to find a desirable fixed night shift system considering nurses' demographic characteristics and organizational characteristics.
Cross-Sectional Studies ; Gyeonggi-do ; Hospitals, General* ; Humans ; Intention* ; Korea ; Nursing ; Nursing Staff

Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the lower airway, generally characterized by blood supply received from the systemic circulation. We present a rare case of a 19-year-old man with incidentally detected BPS supplied by a branch of a pulmonary artery, rather than a systemic artery. Computed tomography showed a sequestered segment supplied by a branch of the left pulmonary artery and containing an ectopic bronchus. As chest computed tomography revealed necrosis in the sequestered tissue, infection was presumed, and the tissue was surgically removed. This may represent a very unusual occurrence, as such cases have yet to be reported in the literature.

A 3 year and 3 months old boy with recurrent infections since his age of 5 months was presented with clinical data and autopsy findings. He was the 4th product of healthy parents. His elder brother died of recurrent perianal abscess and sepsis at his age of 3 years. His 2nd elder sister died on the 14th day of life probably from the complication of BCG vaccination. Beginnig with perianal abscesses at his age of 5 months, he has been continuously suffering from recurrent infections such as arthritis, ostomyelitis, pneumonia, epididymitis, subcutaneous abscesses and perianal abscesses. In spite of meticulous supportive and aggressive antibiotic therapy persistent positive cultures for staph. Aureus, klebsiella, E. Coli, Enterococcus and coliform bacilli from different sited were noted. Erythrocyte sedimentation rate of 25 to 40 were constant. White cell count varied frem 15500 to 33400 with polymorphonucleocytes predominance. NBT test showed persistent low scoring of 2% throught the course. He finally died of pneumonia and empyema. At postmortem examination, multiple abscesses and grnulomas of right lung and multipe granulomas in the liver, spleen, lymph node, bone, marrow, adrenal gland, kidney and intestinal wass were noted. At microscopic examination histiocytic granulomas with lipid containing histiocyte infiltrations were noted in every organs described including brain.
Abscess ; Adrenal Glands ; Arthritis ; Autopsy ; Blood Sedimentation ; Bone Marrow ; Brain ; Cell Count ; Empyema ; Enterobacteriaceae ; Enterococcus ; Epididymitis ; Granuloma ; Granulomatous Disease, Chronic* ; Histiocytes ; Humans ; Infant ; Kidney ; Klebsiella ; Liver ; Lung ; Lymph Nodes ; Male ; Mycobacterium bovis ; Parents ; Pneumonia ; Sepsis ; Siblings ; Spleen ; Vaccination