BACKGROUND: Kawasaki disease (KD) is an acute inflammation of middle-sized arteries such as the coronary artery. KD is an important disease due to its high mortality rate, which approaches 1-2% without appropriate treatment. Since the various mucocutaneous lesions of KD are related to the dermatologic field, dermatologists should take an interest in this disorder and understand the lesions. OBJECTIVE: To evaluate the characteristics of cutaneous lesions in Korean patients with KD. METHODS: A total of 919 patients with KD were assessed at the department of pediatric cardiology and dermatology, Myongji hospital between January 2006 and December 2010. RESULTS: Of the 919 patients, 566 (61.6%) were diagnosed with typical KD and 353 (38.4%) with atypical KD. KD was most frequent in the 4 year-old group (22.7%), and the ratio of male to female was 1.5:1. Among the mucocutaneous manifestations, erythema of the oropharyngeal mucosa (75.7%) occurred most frequently, followed by polymorphous trunk rash (41.7%), and changes of palms and soles (25.6%). A total of 606 patients (65.9%) with KD had associated diseases as follows: pneumonia (211 patients; 34.8%), pharyngotonsilitis (168 patients; 27.7%), gastroenteritis (132 patients; 21.8%), asthma (15 patients, 2.5%), atopic dermatitis (11 patients, 1.8%). CONCLUSION: Our study shows that change in the lips or oral cavity is the most common mucocutaneous manifestation in both classic and atypical KD. Also, morbiliform rash is the most common pattern of polymorphous rashes. Interestingly, erythema multiforme-like lesions occupied accounted for a considerable portion of KD cases compared to previous studies.
Arteries ; Asthma ; Cardiology ; Coronary Vessels ; Dermatitis, Atopic ; Dermatology ; Erythema ; Exanthema ; Female ; Gastroenteritis ; Humans ; Inflammation ; Lip ; Male ; Mouth ; Mucocutaneous Lymph Node Syndrome ; Mucous Membrane ; Pneumonia

Macrophages play a role in innate immune responses to various foreign antigens. Many products from primary tumors influence the activation and transmigration of macrophages. Here, we investigated a migration of macrophages stimulated with cancer cell culture-conditioned medium (CM). Macrophage activation by treatment with CM of B16F10 cells were judged by the increase in protein levels of inducible nitric oxide synthase (iNOS) and cyclooxygenase 2 (COX2). The location where macrophages were at 4 h-incubation with control medium or CM was different from where they were at 5 h-incubation in culture dish. Percentage of superimposed macrophages at every 1 h interval was gradually increased by CM treatment as compared to control. Total coverage of migrated track expressed in coordinates was smaller and total distance of migration was shorter in CM-treated macrophages than that in control. Rac1 activity in CM-treated macrophages was also decreased as compared to that in control. When macrophages were treated with CM in the presence of dexamethasone (Dex), an increase in COX2 protein levels, and a decrease in Rac1 activity and total coverage of migration were reversed. In the meanwhile, biphasic changes were detected by Dex treatment in section distance of migration at each time interval, which was more decreased at early time and then increased at later time. Taken together, data demonstrate that macrophage motility could be reduced in accordance with activation in response to cancer cell products. It suggests that macrophage motility could be a novel marker to monitor cancer-associated inflammatory diseases and the efficacy of anti-inflammatory agents.
Anti-Inflammatory Agents ; Cell Movement* ; Cyclooxygenase 2 ; Dexamethasone ; Immunity, Innate ; Macrophage Activation ; Macrophages* ; Nitric Oxide Synthase Type II ; Track and Field

Basaloid follicular hamartoma (BFH), uncommon rare benign neoplasm connected to the adnexal structures, presents with multiple clinical manifestations that can develop into basal cell carcinoma. BFH may be congenital or acquired, and the congenital form can be further divided into the generalized and unilateral type, and the acquired form may present as localized and solitary lesions. Congenital, generalized BFH is associated with systemic diseases such as alopecia, cystic fibrosis, hypohidrosis, and myasthenia gravis. In contrast, sporadic cases are observed as unilateral or localized lesions. BFH shows thick cords and thin strands of anastomosing basaloid proliferations that arise from hair follicles and are enclosed by loose fibrous stroma. Here, we report a 70-year-old man with an acquired, solitary form of BFH.
Aged ; Alopecia ; Carcinoma, Basal Cell ; Cystic Fibrosis ; Hair Follicle ; Hamartoma ; Humans ; Hypohidrosis ; Myasthenia Gravis

Nicolau syndrome or embolia cutis medicamentosa is a rare complication of a still largely unidentified pathogenesis at the site of intramuscular injections of various drugs. It is characterized by development of an acute, severe pain around the injection site followed by a localized erythema, a reticular rash, a hemorrhagic patch and varying degrees of tissue damage. The disease outcomes vary from atrophic ulcers and pigmentation to lower limb paralysis and limb amputation. We describe a 34-year-old woman with the diagnosis of this syndrome after she received an intramuscular diclofenac-beta-dimethyl-aminoethanol injection to the lateral aspect of the right upper buttock. The patient was treated by surgical debridement with concomitant primary closure.
Adult ; Amputation ; Buttocks ; Debridement ; Diclofenac ; Erythema ; Exanthema ; Extremities ; Female ; Humans ; Injections, Intramuscular ; Lower Extremity ; Paralysis ; Pigmentation ; Ulcer

OBJECTIVE: Lethality of the chosen method during a suicide attempt is a strong risk factor for completion of suicide. We examined whether annual changes in the pattern of suicide methods is related to annual changes in suicide rates among older adults in South Korea and Japan. METHODS: We analyzed annual the World Health Organization data on rates and methods of suicide from 2000 to 2011 in South Korea and Japan. RESULTS: For Korean older adults, there was a significant positive correlation between suicide rate and the rate of hanging or the rate of jumping, and a significant negative correlation between suicide rate and the rate of poisoning. Among older adults in Japan, annual changes in the suicide rate and the pattern of suicide methods were less conspicuous, and no correlation was found between them. CONCLUSION: The results of the present study suggest that the increasing use of lethal suicide methods has contributed to the rise in suicide rates among older adults in South Korea. Targeted efforts to reduce the social acceptability and accessibility of lethal suicide methods might lead to lower suicide rate among older adults in South Korea.
Adult* ; Humans ; Japan* ; Korea* ; Methods* ; Poisoning ; Risk Factors ; Suicide* ; World Health Organization

Immunoglobulin G4 (IgG4)-related kidney disease is a chronic immune-mediated fibro-inflammatory disorder characterized by multiple organ infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis or tumefactive lesions. Previous studies have explored IgG4-related kidney disease, increasing our understanding of its clinical manifestations, and pathological and radiologic findings. However, IgG4-related kidney disease can be misdiagnosed since it mimics malignancies. We report a case of a 77-year-old Korean man diagnosed with IgG4-related kidney disease with membranous proliferative glomerulonephritis, presenting with a renal pelvic mass suspected of being malignant.

Anoctamin 2 (ANO2 or TMEM16B), a calcium-activated chloride channel (CaCC), performs diverse roles in neurons throughout the central nervous system. In hippocampal neurons, ANO2 narrows action potential width and reduces postsynaptic depolarization with high sensitivity to Ca2+ at relatively fast kinetics. In other brain regions, including the thalamus, ANO2 mediates activity-dependent spike frequency adaptations with low sensitivity to Ca2+ at relatively slow kinetics. How this same channel can respond to a wide range of Ca2+ levels remains unclear. We hypothesized that splice variants of ANO2 may contribute to its distinct Ca2+ sensitivity, and thus its diverse neuronal functions. We identified two ANO2 isoforms expressed in mouse brains and examined their electrophysiological properties: isoform 1 (encoded by splice variants with exons 1a, 2, 4, and 14) was expressed in the hippocampus, while isoform 2 (encoded by splice variants with exons 1a, 2, and 4) was broadly expressed throughout the brain, including in the cortex and thalamus, and had a slower calcium-dependent activation current than isoform 1. Computational modeling revealed that the secondary structure of the first intracellular loop of isoform 1 forms an entrance cavity to the calcium-binding site from the cytosol that is relatively larger than that in isoform 2. This difference provides structural evidence that isoform 2 is involved in accommodating spike frequency, while isoform 1 is involved in shaping the duration of an action potential and decreasing postsynaptic depolarization. Our study highlights the roles and molecular mechanisms of specific ANO2 splice variants in modulating neuronal functions.

Immunoglobulin G4 (IgG4)-related kidney disease is a chronic immune-mediated fibro-inflammatory disorder characterized by multiple organ infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis or tumefactive lesions. Previous studies have explored IgG4-related kidney disease, increasing our understanding of its clinical manifestations, and pathological and radiologic findings. However, IgG4-related kidney disease can be misdiagnosed since it mimics malignancies. We report a case of a 77-year-old Korean man diagnosed with IgG4-related kidney disease with membranous proliferative glomerulonephritis, presenting with a renal pelvic mass suspected of being malignant.

The purpose of this study was to investgate and evaluate whether the recording information contained in the work authorization was performed properly and master casts were designed accurately according to the rationale and principles of the removable partial denture when the work authorization and the master casts were sent to the dental framework. the accurate recording of the work authorization sent to dental laboratory in Hwasun for removable partial denture framework, the normal form of the occlusal rest guiding plan of the surveyed crown using the surveyor, the location analysis of the occlusal rest seat, the design patten of the maxillary and mandibulary major connectors, the use of the surveyor on the master cast, the status of the secondary impression taken in the distal extension edentulous areas were investigated for this study. The following results were obtained : 1. In the status of the occlusal rest seat prepared in the abutment, the occlusal rest seat with normal form was 81.25%, 95.45%, that without form was 6.25%, 0.00%, respectively in Class I and Class II on the maxillary cast and the occlusal rest seat with normal form was 76.92%, 95.45%, that without form was 7.69%, 0.00% respectively in Class I and Class II on the mandibular cast. 2. In the status of the guiding plan, the guiding plan with the normal form was 79.17%, 86.36% that with abnormal form was 20.84%, 13.64% respectively in Class I and Class II on the maxillary cast and the guiding plan with normal form was 79.49%, 77.27%, that with abnormal form was 20.51%, 22.73% respectively in Class I and Class II on the mandibular cast. 3. The abutment splinting in the distal extension removable partial denture was 20.78%, 27.27%, respectively in Class I and Class II on the maxillary cast and was 25.64%, 13.64% respectively in Class I and Class II on the mandibular cast. 4. The location and form of the major connector delineated on the maxillary and mandibular master cast was 95.83%, 98.41% respectively. 5. The delineation and recording on the work authorization was incomplete as group II(73.33%), group I(14.07%), group III(12.59%) and group IV (0.00%) in investigating the contents of the work authorization. 6. Aker's clasp of the types of the direct retainer was nots used 72.22%, 74.60%, in the maxillary and mandibular casts respectively. 7. The location of the indirect retainer delineated on the maxillary and mandibular master cast was 51.43%, 50.82% respectively. 8. The types of metal used were mostly Cr-Co non-precious metal alloy and the cases using color coding on the drawing paper of work authorization were not observed. 9. The cases taking secondary impression in the distal extension removable partial denture were 45.71%, 52.46% respectively in the maxillary and mandibular master cast. 10. The delineation of tripoding or reference line on the master cast was not observed in surveying procedure.
Alloys ; Clinical Coding ; Crowns ; Denture, Partial, Removable ; Laboratories, Dental ; Splints

PURPOSE: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. METHODS: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. RESULTS: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). CONCLUSION: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.
Alagille Syndrome* ; Bilirubin ; Cardiovascular Diseases ; Cholestasis ; Cyanosis ; Embryonic Development ; Female ; Heart Diseases ; Hemodynamics ; Humans ; Jaundice ; Labor Presentation ; Liver ; Liver Transplantation ; Mortality ; Pregnancy ; Prognosis ; Pulmonary Valve Stenosis ; Retrospective Studies