Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder usually showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. However, in some reported cases, Birbeck granules could not be demonstrated and only cytoplasmic dense bodies were seen. If the lesion is more advanced, Birbeck granules are transformed to lysosomes, i.e., 'unique phagosomes', in which they are degraded. A 2-month-old Korean girl presented with congenital, numerous red-brown pigmented papules on the left side of trunk and upper extremity without systemic symptoms. A biopsy specimen demonstrated papillary dermis containing epidermotropic infiltrates of histiocytes with abundant eosinophilic cytoplasm. Some had kidney-shaped nuclei and PAS-positive cytoplasmic inclusions. Immunohistochemically, infiltrating cells expressed S-100 protein and ultrastructurally, no Birbeck granules but many dense laminated bodies and unique phagosomes were found. It was ten months since the skin lesions developed that they have started resolving.
Biopsy ; Cytoplasm ; Dermis ; Diagnosis ; Eosinophils ; Female ; Histiocytes ; Humans ; Inclusion Bodies ; Infant ; Lysosomes ; Microscopy, Electron ; Phagosomes ; S100 Proteins ; Skin ; Upper Extremity

There are various reports referring to the clinical siignificances and removal of postgastrectomy bezoar. In this case, suture thread were thought to he the important factor of the bezoar formation, This bezoar was removed intaetly uaing fiber-optic endoscope in patinet who had postgastrectomy bezoar, by cutting the thread by biopsy forceps.
Bezoars* ; Biopsy ; Endoscopes ; Surgical Instruments ; Sutures*

No abstract available.
Emphysema* ; Tuberculosis*

Eosinophilic polymyositis (EPM) is a heterogeneous disorder characterized by eosinophilic infiltration into skeletal muscles. EPM is occasionally associated with a systemic disease, such as hypereosinophilic syndrome, connective tissue disease, or parasitic infection. However, EPM is rarely reported in drug rash with eosinophilia and systemic symptoms (DRESS) syndrome. We experienced a 17-year-old female who presented with EPM with DRESS syndrome associated with taking antitubercular agents.
Adolescent ; Antitubercular Agents* ; Connective Tissue Diseases ; Drug Hypersensitivity Syndrome ; Eosinophilia* ; Eosinophils* ; Exanthema* ; Female ; Humans ; Hypereosinophilic Syndrome ; Muscle, Skeletal ; Polymyositis*

BACKGROUND: We have found abnormal expression of ICAM-1 in thyroid follicular cells from patients with Graves disease and Hashimoto disease. In this report, we present the hormonal regulation of ICAM-1 mRNA expression and the primary structure of 5-regulatory region which is important for transcriptional regulation of ICAM-1 gene. A I.S kb fragment of the 5-regulatory sequences are identified and linked to luciferase as a reporter. METHOD: Those reporter constructs were used to evaluate the expression in response to cytokines and hormones. Deletion analysis of 1.8 kb fragment of ICAM-1 promoter in FRTL-5 cells provide the evidence for the existence of several regulatory elements of enhancer and silencer in ICAM-1 gene transcription in thyroid cells. RESULTS: ICAM-1 mRNA is easily detected by Northern analysis using total RNA from FRTL-5 cells regardless of culture conditions. The transcripts of rat ICAM-1 showed single band of 2.6 kb in length. The FRT cells which was come from early FRTL cell culture did not show ICAM-1 mRNA with usual Northern analysis, We found differential regulation of ICAM-1 RNA level in different culture condition in FRTL-5 cells, The cells maintained at 3H (no hydrocortisone, no insulin, no TSH) condition showed the highest expression level compared to 4H, 5H, or 6H medium. Hydrocortisone markedly decreased the ICAM-1 RNA and insulin partially recovered the hydrocortisone induced repression. TSH which is important in growth and function of FRTL-5 cells could independently downregulate the ICAM-1 RNA levels. Forskolin (10 mM) could mimic the action of TSH on ICAM-1 mRNA. TNF-a and interferon-y increase ICAM-1 expression in FRTL-5 thyroid cells. TSH/forskolin inhibited maximal expression of ICAM-1 by TNF-a and interferon-r. Promoter activity of the ICAM-1 gene was positively regulated by cytokines, TNF-a and IFN-r and negatively regulated by thyroid stimulating hormone. The addition of TSH and FSK caused a 50% decrease in ICAM-1 promoter activity within 24 hour. The TSH and FSK action was mapped at 175 bp and 97 bp of the start of translation. The mutant construct pCAM-175 delGAS which has no GAS sequence showed no TSH mediated suppression of promoter activity. CONCLUSION: These findings suggested that hormones and cytokines differentially regulated the ICAM-1 gene expression and TSH downregulated ICAM-1 gene transcription by inhibiting the activation of IFN-r induced transcription factors which can bind the GAS of ICAM-1 promoter.
Animals ; Cell Culture Techniques ; Clone Cells* ; Cloning, Organism* ; Colforsin ; Cytokines ; Gene Expression ; Graves Disease ; Hashimoto Disease ; Humans ; Hydrocortisone ; Insulin ; Intercellular Adhesion Molecule-1* ; Luciferases ; Rats* ; Repression, Psychology ; RNA ; RNA, Messenger ; Thyroid Gland* ; Thyrotropin ; Transcription Factors

Healthcare accreditation program was started in November 2010. The program was operated by newly born institute, Korea Institute for Healthcare Accreditation. Major characteristic of healthcare accreditation program in Korea is spontaneous program that hospitals voluntarily request for accreditation survey. Goals of healthcare accreditation program in Korea are to change the culture of healthcare supply side from supplier oriented to consumer oriented culture and to change regulation for quality improvement from government initiated regulation to self regulated quality improvement activities by hospitals. Objectives of accreditation are to assure the patient safety and to improve quality of healthcare services in hospitals. Korean Association of Hospital Management conducted a survey on the impact of accreditation program on hospital management, the survey was supported by Korea Institute for Healthcare Accreditation. Results of the survey showed that accredited hospital had changed to positive direction in patient safety, decision making process, organizational culture and hospital management. Assignments of accreditation program in Korea to be solved are to develop incentive for active participation of small size hospitals, to improve quality of surveyors and to increase the apprehension of accreditation of general population. Korea Institute for Healthcare Accreditation endeavor to improve quality of surveyors under in depth training program and to advertise accreditation program through radio and TV to the general population. However the incentive program must be developed by government policy.
Accreditation ; Decision Making ; Delivery of Health Care ; Korea ; Motivation ; Organizational Culture ; Patient Safety ; Quality Improvement ; Quality of Health Care

Purpose : The main genetic contribution to type 1 diabetes susceptibility is the human leukocyte antigen (HLA) class II gene. Several non-HLA chromosomal regions are also known to be involved. We studied the association of HLA class II and non-HLA candidate genes, which are cytotoxic T lymphocyte antigen-4 (CTLA4), tumor necrosis factor (TNF), lymphotoxin-alpha(LT-alpha, and vitamin D receptor (VDR) gene, polymorphisms with disease susceptibility in Korean children with type 1 diabetes.Methods : Fifty Korean children with type 1 diabetes (29 girls and 21 boys) and 166 healthy Koreans were investigated in this study. HLA class II alleles were determined by PCR-SSP (sequence-specific primer) and PCR-SSOP (sequence specific oliogonucleotide probe) method. CTLA4 exon 1 polymor phism was analyzed by PCR-SSCP (single strand conformation polymorphism), and TNF promotor and LT-alphagene polymorphism by PCR-RFLP (restriction fragment length polymorphism), respectively. VDR gene polymorphisms were analyzed by PCR-RFLP using restriction enzyme FokI, ApaI, TaqI, and BsmI. Results : The frequencies of HLA-DRB1*04, DRB1*09, and DQB1*04 were significantly increased and those of HLA-DRB1*14, DRB1*15, DQB1*05 and DQB1*06 were significantly decreased in the patients with type 1 diabetes compared with the control subjects. No significant differences in the distribution of CTLA4 exon 1, TNF promotor, LT-alpha and VDR gene polymorphisms were observed between the patients with type 1 diabetes and the control subjects. Conclusion : These data suggest that HLA-DRB1*04, DRB1*09, and DQB1*04 are susceptible genes for type 1 diabetes, whereas HLA-DRB1*14, DRB1*15, DQB1*05, and DQB1*06 are protective genes in Korean children. CTLA4 exon 1, TNF promotor, LT-alpha and VDR gene polymorphisms are not associated with susceptibility to type 1 diabetes in Korean children.
Alleles ; Child* ; Diabetes Mellitus, Type 1* ; Disease Susceptibility* ; Exons ; Female ; Genes, MHC Class II ; Humans ; Leukocytes ; Lymphocytes ; Receptors, Calcitriol ; Tumor Necrosis Factor-alpha

Functional dyspepsia is defined as the presence of the symptoms that originate from the gastroduodenal area, without evidence of any organic, systemic, or metabolic disease that could explain these symptoms. The pathophysiology of functional dyspepsia includes chronic duodenal inflammation, Helicobacter pylori, excessive gastric acid secretion, dietary factors, and psychosocial, genetic, and environmental factors. However, gastric dysmotility and visceral hypersensitivity are thought to play a major role in the pathogenesis of functional dyspepsia, and other factors may affect gastric motility and visceral hypersensitivity. In the diagnosis of functional dyspepsia, upper gastrointestinal endoscopy is important to rule out organic diseases; an H. pylori test-and-treat strategy is currently not recommended for the initial diagnostic step in Korea where both H. pylori prevalence and gastric incidence are very high. Anti-secretory drugs (proton pump inhibitors and histamine 2 receptor antagonists) and prokinetics can be used as a first-line treatment depending on the patient's symptoms (postprandial distress syndrome vs. epigastric pain syndrome). If initial treatment is not effective, tricyclic antidepressants and/or anxiolytics (fundic relaxants) could be used in selected patients.
Anti-Anxiety Agents ; Antidepressive Agents, Tricyclic ; Diagnosis* ; Dyspepsia* ; Endoscopy, Gastrointestinal ; Gastric Acid ; Helicobacter pylori ; Histamine ; Humans ; Hypersensitivity ; Incidence ; Inflammation ; Korea ; Metabolic Diseases ; Prevalence

PURPOSE: We report clinical and radiologic outcomes after metacarpal extension osteotomy for mild osteoarthritis of the thumb carpometacarpal joint. MATERIALS AND METHODS: From 1999 to 2008, 11 patients were diagnosed with mild thumb carpometacarpal arthritis (Eaton stage I, II), and extension osteotomies were performed. Of these, seven patients with at least 6 years follow-up were analyzed retrospectively. Male to female ratio was 2:5, and mean age at time of surgery was 38.9 years old. Symptom onset period was a mean of 11.2 months. Two patients were I, and five patients were II in Eaton stage. Preoperative visual analogue scale (VAS) and disabilities of the arm, shoulder and hand scale (DASH) scores were 3.7 points (3-4 points), and 40.1 points (32-51 points). Radial abduction was 38.5degrees (30degrees-45degrees), and volar abduction was 42.1degrees (40degrees-45degrees). Grip strengths and pinch powers, compared with the normal contralateral side were 82% (64%-90%) and 72% (40%-100%), respectively. RESULTS: The mean follow-up period was 8.5 years, and all patients except one maintained their occupational activity during the follow-up period. Final VAS and DASH scores were 0.7 points (0-2 points) and 11.7 points (8-16 points), respectively, and were statistically significant. Volar abduction, grip strengths, and pinch power were improved to 45degrees (40degrees-50degrees), 92.3% (73%-117%), and 94.4% (75%-117%) with statistical significances. In five patients, Eaton stages did not change, and two patients advanced to the next stage (stage I to II in one patient, stage II to III in one patient). CONCLUSION: Among the various treatment options for mild thumb carpometacarpal arthritis, metacarpal extension osteotomy may be considered as an effective treatment.
Arm ; Arthritis* ; Carpometacarpal Joints ; Female ; Follow-Up Studies ; Hand ; Hand Strength ; Humans ; Male ; Osteoarthritis ; Osteotomy* ; Retrospective Studies* ; Shoulder ; Thumb*

Average anteversion of the femur is 15.3 degree. Ultrasound, computerized tomogram, and three dimensional reconstruction from CT or MRI have been used for more accurate measurement of the anteversion. There are two methods in measuing anteversion angle from CT scan:one is by drawing a mid line through long axis of the femoral neck (conventional method). Another is by drawing a line from the center of the head to that of the neck at the base of the trochanter using several cut slices (Murphy’s method). We compared these methods with fluoroscopic measurement of the Anteversion. We traced the change of the anteversion before and after closed femoral intramedullary (IMO) nailing to evaluate the origin of malrotation of the femur. 1. Normal anteversion angles were measured in 15 cases. Average anteversion angle was 6.9 degrees by conventional method, 12.3 degrees by Murphy’s method, and 12.2 degrees by fluoroscope. 2. Anteversion angles were measured after IM nailing in 18 cases. Average was 17 degrees by Murphy’s method and 15.2 degrees by fluoroscope. Mean of difference between these two methods sea 6.3 degrees. That was 1.7 degrees in normal side. 3. Change of the anteversion angle between before and after IM nailing was measured in 17 cases by fluoroscope. Average anteversion angle before the operation was 11.9 degrees and it was change to 15.8 degrees after operation. Mean of these change was 7.1 degrees. 4. Pereperative traction provides important information on change of anteversion. Reduction excessive flexion of proximal fragment was a origin of change of anteversion during nailing procedure. Conclusion : Malrotation of the femur after IM nailing must be keep in mind and it may be preventable by fluoroscopic control of the rotation in nailing procedure.
Femur ; Femur Neck ; Fracture Fixation, Intramedullary ; Head ; Magnetic Resonance Imaging ; Methods ; Neck ; Traction ; Ultrasonography