An intraosseous epidermal cyst is a rare benign cystic lesion. It is thought to result from congenital factors or trauma and can lead to bone destruction because the cyst develops at the soft tissue around the bone. Radiological findings of intraosseous epidermal cysts are a well-defined radiolucent lesion, with cortical expansion. It is important to differentiate an intraosseous epidermal cyst with other disease developed at distal phalanx because its clinical and radiological findings are similar. We report two rare cases of intraosseous epidermal cysts that developed at the distal phalanx.
Epidermal Cyst*

Transient neonatal diabetes mellitus (TNDM) is a rare form of diabetes mellitus that presents within the first 6 months of life with remission in infancy or early childhood. TNDM is mainly caused by anomalies in the imprinted region on chromosome 6q24; however, recently, mutations in the ABCC8 gene, which encodes sulfonylurea receptor 1 (SUR1), have also been implicated in TNDM. Herein, we present the case of a male child with TNDM whose mutational analysis revealed a heterozygous c.3547C>T substitution in the ABCC8 gene, leading to an Arg1183Trp mutation in the SUR1 protein. The parents were clinically unaffected and did not show a mutation in the ABCC8 gene. This is the first case of a de novo ABCC8 gene mutation in a Korean patient with TNDM. The patient was initially treated with insulin and successfully switched to sulfonylurea therapy at 14 months of age. Remission of diabetes had occurred at the age of 16 months. Currently, the patient is 21 months old and is euglycemic without any insulin or oral hypoglycemic agents. His growth and physical development are normal, and there are no delays in achieving neurological and developmental milestones.
ATP-Binding Cassette Transporters ; Child ; Diabetes Mellitus ; Humans ; Hypoglycemic Agents ; Infant ; Insulin ; Male ; Parents ; Potassium Channels, Inwardly Rectifying ; Receptors, Drug

BACKGROUND: Surgery may have a role when medical treatment alone is not successful in patients with multi-drug resistant (MDR) pulmonary tuberculosis (PTB). To document the role of resection in MDR PTB, we analyzed 4 years of our experience. METHODS: A retrospective review was performed on thirteen patients that underwent pulmonary resection for MDR PTB between May 1996 and February 2000. All patients had organisms resistant to many of the first-line drugs including isoniazid (INH) and rifampicin (RFP). RESULTS: The thirteen patients were 37.5±12.4 years old (mean±S.D.)(M:F=5:8), and their sputum was culture positive even with adequate medication for prolonged periods (109.7±132.0 months), resistant to 2-8 drugs including isoniazid and rifampin. All patients had localized lesion(s) and most (92.3%) had cavities. At least 3 sensitive anti-TB medications were started before surgery in all patients according to the drug sensitivity test. The preoperative FE1 was 2.37±0.83 L. Lobectomy was performed in 11 patients and pleuropneumonectomy in two. Postoperative mortality did not occur, but pneumonia occurred as a complication in one (7.7%). After 41.5±58.9 days (range 1~150 days) follow up, negative conversion of sputum culture was achieved in all patients within 5 months. Only one patient (7.7%) recurred 32 months after lung resection. CONCLUSION: When medical treatment alone is not successful, surgical resection can be a good treatment option in patients with localized MDR PTB.
Follow-Up Studies ; Humans ; Isoniazid ; Lung ; Mortality ; Pneumonia ; Retreatment* ; Retrospective Studies ; Rifampin ; Risk Factors* ; Sputum ; Tuberculosis, Pulmonary*

In order to evaluate the possible roles of secretory proteases in the pathogenesis of amoebic keratitis, we purified and characterized a serine protease secreted by Acanthamoeba lugdunensis KA/E2, isolated from a Korean keratitis patient. The ammonium sulfate-precipitated culture supernatant of the isolate was purified by sequential chromatography on CM-Sepharose, Sephacryl S-200, and mono Q-anion exchange column. The purified 33 kDa protease had a pH optimum of 8.5 and a temperature optimum of 55 degrees C. Phenylmethylsulfonylfluoride and 4- (2- Aminoethyl) -benzenesulfonyl-fluoride, both serine protease specific inhibitors, inhibited almost completely the activity of the 33 kDa protease whereas other classes of inhibitors did not affect its activity. The 33 kDa enzyme degraded various extracellular matrix proteins and serum proteins. Our results strongly suggest that the 33 kDa serine protease secreted from this keratopathogenic Acanthamoeba play important roles in the pathogenesis of amoebic keratitis, such as in corneal tissue invasion, immune evasion and nutrient uptake.
Acanthamoeba/*enzymology/isolation & purification/pathogenicity ; Acanthamoeba Keratitis/*parasitology ; Animals ; Cornea/parasitology ; Humans ; Hydrogen-Ion Concentration ; Korea ; Serine Endopeptidases/chemistry/*isolation & purification/*metabolism ; Substrate Specificity ; Temperature ; Virulence Factors

Recent studies suggest that alterations of the immune-inflammatory system contribute to the pathogenesis of Alzheimer's disease (AD), Parkinson's disease (PD) and Amyotrophic Lateral Sclerosis (ALS). Neuroinflammatory response initiated by innate immune mechanism that self-attack on neurons, known as "autotoxicity" could be an initial key mechanism of chronic neurodegenerative diseases. Numerous experimental and pathological evidences showing upregulated inflammatory cytokines and chemokines, and the activation of complement cascade and accumulation of activated microglia in damaged regions support the important role of immune-inflammatory mechanism in the pathogenesis of neurodegenerative diseases. Epidemiological studies on the non-steroidal anti-inflammatory drugs (NSAIDs), coupled with results from animal model of AD, PD and ALS, have prompted the studies to determine if immune-inflammatory modifying agents or molecules could be a new therapeutic paradigm of neurodegenerative diseases. Molecules inhibiting proinflammatory cytokines and chemokines released from microglia, agents that inhibit activation of microglia, COX2 and complement system are now considered as a good candidate of immune-inflammatory modulating treatment. By better understanding inflammatory and immunoregulatory processes, it should be possible to develop anti-inflammatory approach that may not completely cure AD, PD, and ALS but will likely help slow the progression or delay the onset of these devastating diseases.
Alzheimer Disease* ; Amyotrophic Lateral Sclerosis ; Chemokines ; Complement System Proteins ; Cytokines ; Microglia ; Models, Animal ; Neurodegenerative Diseases ; Neurons ; Parkinson Disease

Chest wall osteoradionecrosis, one of the most serious complications of radiation therapy for breast cancer treatment, is usually treated by wide debridement followed by coverage with a well-vascularized flap. However, the extent of radiation-induced injury and limits in performing wide resection of the injured bones often present challenges in treatment. Herein, we present our experience treating chest wall osteoradionecrosis with a contralateral breast Y-V flap in an 81-year-old woman. She was diagnosed with chest wall osteoradionecrosis and had grade 3 ptotic breasts. Redundant contralateral breast tissue was used for reconstruction to cover the wound. The flap was elevated in the subfascial plane after an inverted-T incision was made in the lower pole and inframammary fold of the contralateral breast, while preserving the perforators of the left lateral thoracic artery. The flap was spread using the Y-V advancement fashion to cover the wound. The patient was discharged 2 weeks after surgery. At 19 months postoperation, there were no complications or recurrence. The patient was satisfied with the short recovery time and surgical results. The contralateral breast Y-V flap allows simple and quick reconstruction, potentially expanding the available treatment options and therefore increasing flexibility in choosing a treatment plan for patients.

The purpose of this study was to investigate relationships between physical activity and immanent fall risk factors in the elderly. The subjects were 138 (male: n=51; female: n=87) elderly who have no restriction in daily physical activity. Physical activity was measured over consecutive seven days by accelerometer. Immanent fall risk factors (Berg Balance Scale [BBS], Activities-specific Balance Confidence [ABC], and gait-related fitness) were measured. There are significant correlations between physical activity and BBS (male r=0.46, female r=0.36), gait-related fitness (male r=0.44, female r=0.43). And there are significant correlations between physical activity and ABC in female (r=0.38). Through this study, it is assumed that physical activity has correlation with immanent fall risk factors. Also, physical activity has correlation with gate-related fitness in the elderly.
Aged ; Female ; Humans ; Motor Activity ; Risk Factors

No abstract available.
Intracranial Hypotension*

Acanthamoeba keratitis (AK) is a rare ocular disease, but it is a painful and sight-threatening infectious disease. Early diagnosis and adequate treatment are necessary to prevent serious complications. While AK is frequently diagnosis via several PCR assays or Acanthamoeba-specific antibodies, a more specific and effective diagnostic method is required. This study described the production of a polyclonal peptide antibody against the periplasmic binding protein (PBP) of A. castellanii and investigated its diagnostic potential. Western blot analysis showed that the PBP antibody specifically reacted with the cell lysates of A. castellanii. However, the PBP antibody did not interact with human corneal epithelial (HCE) cells and the other 3 major causative agents of keratitis. Immunocytochemistry (ICC) results revealed the specific detection of A. castellanii trophozoites and cysts by PBP antibodies when A. castellanii were co-cultured with HCE cells. PBP antibody specificity was further confirmed by co-culture of A. castellanii trophozoites with F. solani, S. aureus, and P. aeruginosa via ICC. The PBP antibody specifically reacted with the trophozoites and cysts of A. polyphaga, A. hatchetti, A. culbertsoni, A. royreba, and A. healyi, thus demonstrated its genus-specific nature. These results showed that the PBP polyclonal peptide antibody of A. castellanii could specifically detect several species of Acanthamoeba, contributing to the development of an effective antibody-based AK diagnostics.

Previous studies have demonstrated a positive association between obesity and decreased lung function. However, the effect of muscle and fat has not been fully assessed, especially in a healthy elderly population. In this study, we evaluated the impact of low muscle mass (LMM) and LMM with obesity on pulmonary impairment in healthy elderly subjects. Our study used data from the Korea National Health and Nutrition Examination Survey from 2008 to 2011. Men and women aged 65 yr or older were included. Muscle mass was measured by dual-energy X-ray absorptiometry. LMM was defined as two standard deviations below the sex-specific mean for young healthy adults. Obesity was defined as body mass index > or = 25 kg/m2. The prevalence of LMM in individuals aged over 65 was 11.9%. LMM and pulmonary function (forced vital capacity and forced expiratory volume in 1 second) were independently associated after adjusting for age, sex, body mass index, smoking status, alcohol consumption, and frequency of exercise. LMM with obesity was also related to a decrease in pulmonary function. This study revealed that LMM is an independent risk factor of decreased pulmonary function in healthy Korean men and women over 65 yr of age.
Age Distribution ; Aged ; Aged, 80 and over ; Causality ; Comorbidity ; Female ; Humans ; Lung Diseases/*epidemiology/*pathology ; Male ; Nutrition Surveys ; Obesity/*epidemiology/pathology ; Organ Size ; Prevalence ; Reference Values ; Reproducibility of Results ; Republic of Korea/epidemiology ; Respiratory Function Tests ; Respiratory Muscles/*pathology ; Risk Factors ; Sarcopenia/*epidemiology/*pathology ; Sensitivity and Specificity ; Sex Distribution