The hereditary sensory neuropathy is a very rare disease characterized by prominent sensory loss without corresponding motor involvement, but may be associated with autonomic features. Currently, the disease is divided into five main types and most frequent are Type I and Type II. The type II hereditary sensory neuropathy is characterized by autosomal recessive inheritance, onset in utero or in infancy, loss of touch-pressure sense more than paintemperature sense, and almost total absence of myelinated nerve fibers. In this case, we describe a 23 years old female patient with acroosteolysis and heel ulcer who was diagnosed as hereditary sensory neuropathy type II.
Acro-Osteolysis* ; Female ; Heel ; Hereditary Sensory and Autonomic Neuropathies* ; Humans ; Nerve Fibers, Myelinated ; Rare Diseases ; Ulcer ; Wills ; Young Adult

OBJECTIVE: To understand the clinical manifestations and disease course of adult onset Still' s disease (AOSD). METHODS: 15 patients of AOSD diagnosed at Severance hospital, Yonsei University College of Medicine were retrospectively analysed in the period of September 1988 to September 1995. RESULTS: There were 3 men and 12 women (male to female ratio of 1:4). Age of disease onset ranged from 17-55 years, and over 86% of the patients were younger than age 40 at disease onset. The prevalence of clinical features were as follows fever (100%), arthritis (93%), skin rash (93%), sore throat (60%), abnormal liver function (73%), lymphadenopathy (47%), splenomegaly (47%), hepatomegaly (20%), serositis (13%). Fever was the most common initial symptom. Common labaratory features were leukocytosis with neutrophilia (87%), anemia' Hgb <10 g/dL (67%), increased serum ferritin (83%), ESR (87%) and CRP (93%). Serum ferritin was markedly raised at disease onset and correlated with disease activity. In 2 patients, the disease was controlled with NSAID alone, but most of the patients required steroid to control the disease activity. In 6 patients, MTX was added for steroid sparing effect and for steroid resistant arthritis. Most of AOSD patients had intermittent and chronic disease course. Root joint arthritis and polyarthritis were factors associated with chronicity. CONCLUSION: The clinical features of AOSD in our study generally resemble previous reports. Serum ferritin was a useful marker of disease activity. Most patients of AOSD had intermittent and chronic disease course. Root joint athritis and polyarticular pattern at disease onset were factors associated with chronicity.
Adult* ; Arthritis ; Chronic Disease ; Exanthema ; Female ; Ferritins ; Fever ; Hepatomegaly ; Humans ; Joints ; Leukocytosis ; Liver ; Lymphatic Diseases ; Male ; Pharyngitis ; Prevalence ; Retrospective Studies ; Serositis ; Splenomegaly ; Still's Disease, Adult-Onset*

BACKGROUND: Soman, a potent irreversible acetylcholinesterase (AChE) inhibitor, induces delayed neuronal injury by reactive oxygen species (ROS). Midazolam is used in patients with pathologic effects of oxidative stresses such as infection, hemodynamic instability and hypoxia. We investigated whether midazolam protects the Central Nervous System (CNS) from soman intoxication. The present study was performed to determine whether midazolam protects B35 cells from ROS stress for the purpose of exploring an application of midazolam to soman intoxication. METHODS: Glucose oxidase (GOX) induced ROS stress was used in a B35 neuroblastoma cell model of ROS induced neuronal injury. To investigate the effect of midazolam on cell viability, LDH assays and fluorescence activated cell sorting (FACS) analysis was performed. Western blotting was used for evaluating whether Akt-phosphorylation is involved in cell-protective effects of midazolam. RESULTS: GOX derived ROS injury decreased cell viability about 1.6-2 times compared to control; midazolam treatment (5 and 10 microg/ml) dose-dependently increased cell viability during ROS injury. On western blots, Akt-phosphorylation was induced during pretreatment with midazolam; it was diminished during co-treatment with LY-294002, an inhibitor of Akt-phosphorylation. FACS analysis confirmed that the cell protective effect of midazolam is mediated by an anti-apoptotic effect. GOX-induced apoptosis was inhibited by midazolam and the finding was diminished by LY-294002. CONCLUSIONS: Midazolam protects neuronal cells from GOX-induced ROS injury; this effect is mediated by an anti-apoptotic effect through Akt-phosphorylation. This shows that midazolam may be useful in soman intoxication.
Acetylcholinesterase ; Anoxia ; Apoptosis ; Blotting, Western ; Cell Survival ; Central Nervous System ; Chromones ; Flow Cytometry ; Glucose Oxidase ; Hemodynamics ; Humans ; Midazolam ; Morpholines ; Neuroblastoma ; Neurons ; Oxidative Stress ; Reactive Oxygen Species ; Soman

BACKGROUND: The mechanical hyperalgesia that follows peripheral tissue injury results from peripheral and central sensitization. Central sensitization is initiated and maintained by windup that can be prevented by N-methyl-D-aspartate (NMDA) antagonists. NMDA antagonists, therefore, have the potential to prevent and treat pain, although clinical uses are limited because of their side effects. This study was designed to evaluate the analgesic action of intrathecal (IT) magnesium sulphate in a rat model of postoperative pain and investigate the analgesic mechanism of magnesium. METHODS: Forty-two Sprague-Dawley rats (300 +/- 20 g) were prepared with a chronic IT catheter. Under brief enflurane anesthesia, a 1-cm incision including skin, muscle and fascia was made in the plantar aspect of the hind paw and closed. Normal saline, magnesium (30, 100, 300, 600 microgram), NMDA 50 ng or NMDA 50 ng with magnesium 300 microgram was administered via the IT catheter after recovery. Response frequency, using Von Frey filaments, cumulative pain scores and motor deficits were assessed. RESULTS: The mechanical hyperalgesia and nonevoked pain behaviors decreased significantly at 1 h or 1 h and 3 h after IT injection of magnesium 100 microgram or 300 microgram compared to the saline group without profound motor deficits in a rat model of postoperative pain. However, the rats administered with magnesium 600 microgram were lethargic due to severe motor weakness. Effective duration of magnesium decreased significantly in the group of NMDA 50 ng with magnesium 300 microgram compared to that of magnesium 300 microgram administered alone, but the initial effects were similar between the two groups. CONCLUSIONS: We conclude that IT magnesium sulphate can modulate nociceptive processing after tissue injury and the analgesic mechanism of magnesium is involved in NMDA receptors. Magnesium,therefore, may offer a therapeutic agent for postoperative pain and may be an agent that prevents postoperative pain from changing to persistent pathological pain.
Anesthesia ; Animals ; Catheters ; Central Nervous System Sensitization ; Enflurane ; Fascia ; Hyperalgesia ; Magnesium* ; Models, Animal* ; N-Methylaspartate ; Pain, Postoperative* ; Rats* ; Rats, Sprague-Dawley ; Receptors, N-Methyl-D-Aspartate ; Skin

BACKGROUND: Preemptive analgesia is an antinociceptive treatment that prevents the development of central sensitization which contributes to the post-injury pain hypersensitivity. But controversies exist over the effectiveness and clinical value of preemptive analgesia. The aim of this study is to evaluate the preemptive effect of intrathecal bupivacaine on incisional pain in rats. METHODS: Thirty male rats were divided into 3 groups, saline-treated control group (n=10), post-treatment group (n=10), and pre-treatment group (n=10) according to the time which intrathecal administration of bupivacaine was done. To evaluate postoperative mechanical hyperalgesia in injured feet, withdrawal frequency and withdrawal thresholds were measured by von Frey filaments at 30 min, 1 hr, 2 hrs, 3 hrs, 1 day, 3 days and 7 days after incision. RESULTS: In control group, the withdrawal frequency increased from 0+/-0% before incision to 98.0+/-1.3% after the foot incision and the responses gradually declined during the postoperative 7 days to 52.0+/-4.7%. The median withdrawal threshold decreased from 148.43 mN before incision to 0.05 mN after foot incision and gradually increased during the postoperative 7 days to 6.79 mN. The post-treatment group showed no significant differences in the withdrawal frequency and withdrawal thresholds when compared with control group at post-operative 1 hour and thereafter (P<0.05). The pre-treatment group showed significantly lower withdrawal frequency and significantly higher withdrawal threshold compared with control group at postoperative 30 min and thereafter (P<0.05), and significantly lower withdrawal frequency and higher withdrawal threshold compared with post-treatment group at postoperative 2 hours and thereafter (P<0.05). CONCLUSION: We conclude that intrathecal bupivacaine administered before incision reduces postoperative delayed hyperalgesia in incisional pain model, and it may result from preventing the development of injury- induced central sensitization.
Analgesia ; Animals ; Bupivacaine* ; Central Nervous System Sensitization ; Foot ; Humans ; Hyperalgesia ; Hypersensitivity ; Male ; Pain, Postoperative* ; Rats*

BACKGROUND: Preemptive analgesia is an antinociceptive treatment that prevents the development of central sensitization which contributes to the post-injury pain hypersensitivity. But controversies exist over the effectiveness and clinical value of preemptive analgesia. The aim of this study is to evaluate the preemptive effect of intrathecal bupivacaine on incisional pain in rats. METHODS: Thirty male rats were divided into 3 groups, saline-treated control group (n=10), post-treatment group (n=10), and pre-treatment group (n=10) according to the time which intrathecal administration of bupivacaine was done. To evaluate postoperative mechanical hyperalgesia in injured feet, withdrawal frequency and withdrawal thresholds were measured by von Frey filaments at 30 min, 1 hr, 2 hrs, 3 hrs, 1 day, 3 days and 7 days after incision. RESULTS: In control group, the withdrawal frequency increased from 0+/-0% before incision to 98.0+/-1.3% after the foot incision and the responses gradually declined during the postoperative 7 days to 52.0+/-4.7%. The median withdrawal threshold decreased from 148.43 mN before incision to 0.05 mN after foot incision and gradually increased during the postoperative 7 days to 6.79 mN. The post-treatment group showed no significant differences in the withdrawal frequency and withdrawal thresholds when compared with control group at post-operative 1 hour and thereafter (P<0.05). The pre-treatment group showed significantly lower withdrawal frequency and significantly higher withdrawal threshold compared with control group at postoperative 30 min and thereafter (P<0.05), and significantly lower withdrawal frequency and higher withdrawal threshold compared with post-treatment group at postoperative 2 hours and thereafter (P<0.05). CONCLUSION: We conclude that intrathecal bupivacaine administered before incision reduces postoperative delayed hyperalgesia in incisional pain model, and it may result from preventing the development of injury- induced central sensitization.
Analgesia ; Animals ; Bupivacaine* ; Central Nervous System Sensitization ; Foot ; Humans ; Hyperalgesia ; Hypersensitivity ; Male ; Pain, Postoperative* ; Rats*

BACKGROUND: Recent findings in molecular pathology suggest that genetic translocation and/or overexpression of oncoproteins is important in salivary gland tumorigenesis and diagnosis. We investigated PLAG1, SOX10, and Myb protein expression in various salivary gland neoplasm tissues. METHODS: A total of 113 cases of surgically resected salivary gland neoplasms at the National Cancer Center from January 2007 to March 2017 were identified. Immunohistochemical staining of PLAG1, SOX10, and Myb in tissue samples was performed using tissue microarrays. RESULTS: Among the 113 cases, 82 (72.6%) were benign and 31 (27.4%) were malignant. PLAG1 showed nuclear staining and normal parotid gland was not stained. Among 48 cases of pleomorphic adenoma, 29 (60.4%) were positive for PLAG1. All other benign and malignant salivary gland neoplasms were PLAG1-negative. SOX10 showed nuclear staining. In normal salivary gland tissues SOX10 was expressed in cells of acinus and intercalated ducts. In benign tumors, SOX10 expression was observed in all pleomorphic adenoma (48/48), and basal cell adenoma (3/3), but not in other benign tumors. SOX10 positivity was observed in nine of 31 (29.0%) malignant tumors. Myb showed nuclear staining but was not detected in normal parotid glands. Four of 31 (12.9%) malignant tumors showed Myb positivity: three adenoid cystic carcinomas (AdCC) and one myoepithelial carcinoma with focal AdCC-like histology. CONCLUSIONS: PLAG1 expression is specific to pleomorphic adenoma. SOX10 expression is helpful to rule out excretory duct origin tumor, but its diagnostic value is relatively low. Myb is useful for diagnosing AdCC when histology is unclear in the surgical specimen.
Adenoma ; Adenoma, Pleomorphic ; Antibody-Dependent Cell Cytotoxicity ; Carcinogenesis ; Carcinoma, Adenoid Cystic ; Diagnosis ; Immunohistochemistry ; Oncogene Proteins ; Oncogene Proteins v-myb ; Parotid Gland ; Pathology, Molecular ; Salivary Gland Neoplasms ; Salivary Glands ; SOX Transcription Factors ; Translocation, Genetic

PURPOSE: To investigate the role of radiotherapy for squamous cell carcinomas of the external auditory canal and middle ear. MATERIALS AND METHODS: A series of 35 patients who were treated at a single institution from 1981 through 2007 were retrospectively analyzed. Thirteen patients were treated by radiotherapy alone; four by surgery only and 18 by a combination of surgery and radiotherapy. The total radiation dose ranged from 39~70 Gy (median, 66 Gy) in 13~35 fractions for radiotherapy alone and 44~70 Gy (median, 61.2 Gy) in 22~37 fractions for the combined therapy. Clinical end-points were the cause of specific survival (CSS) and local relapse-free survival (LRFS). The median follow-up time was 2.8 years (range, 0.2~14.6 years). RESULTS: The 3-year CSS and LRFS rate was 80% and 63%, respectively. Based on a univariate analysis, performance status and residual disease after treatment had a significant impact on CSS; performance status and histologic grade for LRFS. Patients treated by radiotherapy alone had more residual disease following the course of treatment compared to patients treated with the combined therapy; 69% vs. 28%, respectively. CONCLUSION: Our results suggest that radiation alone was not an inferior treatment modality for CSS compared to the combined therapy for squamous cell carcinoma of the external auditory canal and middle ear. However, local failure after radiotherapy is the main issue that will require further improvement to gain optimal local control.
Carcinoma, Squamous Cell ; Ear Canal ; Ear, Middle ; Follow-Up Studies ; Humans ; Retrospective Studies

Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.
Biopsy ; Cyclophosphamide ; Diagnosis ; Humans ; Kidney ; Korea ; Purpura ; Renal Insufficiency ; Respiratory System ; Rhinitis ; Sinusitis ; Skin ; Vasculitis ; Wegener Granulomatosis

A total of 90 Acinetobacter isolates from freshwater and seawater in Gangjin Bay of Korea was investigated for the distribution of genomic species, antimicrobial resistance patterns and clonal relatedness. By amplified ribosomal DNA restriction analysis, eighty-nine Acinetobacter isolates were classified into 11 Acinetobacter genomic species. A. johnsonii (n=23) was the most prevalent, followed by A. baumannii (n=13), A. calcoaceticus (n=13), Acinetobacter genomic species 11 (n=10), A. phenon 6/ct13TU (n=9), A. junii (n=5), A. venetianus (n=5), Acinetobacter genomic species 17 (n=4), 14BJ (n=3), A. phenon 10/1271 (n=2), Acinetobacter genomic species 3 (n=1), and ungrouped (n=1). The majority of Acinetobacter genomic species were isolated from the site A and B, and some known nosocomial pathogens in the clinical environment were observed among them. Of the 11 antimicrobial drugs tested, several A. johnsonii isolates exhibited high-frequency resistance to a wide variety of antimicrobial agents, including ampicillin-sulbactam, piperacillin, ceftazidime, cefotaxime, and sulfamethoxazole (p < 0.001). Some Acinetobacter genomic species were resistant to currently used antibiotics but all isolates were susceptible to imipenem, amikacin, and tetracycline. Based on the results of antimicrobial resistance pattern and phylogenetic analysis, 23 A. johnsonii isolates were classified into 19 pulsotypes. In conclusion, there was a significant difference in the distribution of Acinetobacter species between freshwater and seawater. Predominance of A. johnsonii strains was probably due to their ability to proliferate in the contaminated aquatic environment originated from local geographic features. Therefore, the waste effluent from animals and humans plays an important role in the distribution of Acinetobacter species in aquatic environment.
Acinetobacter ; Amikacin ; Ampicillin ; Animals ; Anti-Bacterial Agents ; Anti-Infective Agents ; Bays ; Cefotaxime ; Ceftazidime ; DNA, Ribosomal ; Fresh Water ; Humans ; Imipenem ; Korea ; Piperacillin ; Seawater ; Sulbactam ; Sulfamethoxazole ; Tetracycline