No abstract available.
Keratitis*

OBJECTIVE: To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis. METHODS: Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was assessed using Kaplan-Meier curves and multivariate analyses of the risk of death were performed using the Cox proportional hazards model. RESULTS: Among 212 suspicious subjects, definite ALS was diagnosed in 182 subjects. The survival rate at 3 and 5 years from onset was 61.5% and 40.1%, respectively, and the survival rate at 3 and 5 years post-diagnosis was 49.5% and 24.2%, respectively. Further, 134 patients (134/182, 73.6%) were initiated on NIV, and among them, 90 patients (90/182, 49.5%) underwent tracheostomy. Male gender and onset age of ≥65 years were independent predictors of adverse survival. CONCLUSION: The analysis of long term survival in ALS showed excellent outcomes considering the overall poor prognosis of this disease.
Age of Onset ; Amyotrophic Lateral Sclerosis* ; Diagnosis ; Humans ; Male ; Multivariate Analysis ; Noninvasive Ventilation ; Prognosis ; Proportional Hazards Models ; Survival Rate ; Tracheostomy

Envelope glycoprotein 1 (G1) and glycoprotein 2 (G2) of Hantaan (HTN) virus are believed to be major viral antigens that can induce neutralizing immunity against HTN virus infection. The purpose of this study is to clone and express G1 gene in an E. coli expression system. The truncated G1 gene (amino acid residues 35 to 123) of the HTN virus strain 76-118 was amplified by polymerase chain reaction (PCR). The 0.28 kb PCR product was cloned into pCR2.1 vector and named as pCGS1. The truncated G1 gene was excised from the pCGS1 and subcloned into the BamHI and SalI sites of pGEX-4T-2 and named pGGS1. The nucleotide sequence of the 0.28 kb truncated G1 gene was determined. It is revealed four non-silent nucleotide substitutions between the published sequence of strain HTN virus strain 76-118 and our stock of HTN virus strain 76-118 (passaged several times in our laboratory). The first G1 mutation was found to constitute an A to G nucleotide substitution, giving raise to an asparagine to serine mutation at residue 64. The second G1 mutation was found to constitute an A to C nucleotide substitution, giving raise to an lysine to threonine mutation at residue 112. The third G1 mutation was found to constitute an A to C nucleotide substitution, giving raise to an lysine to threonine mutation at residue 112. The fourth G1 mutation was found to constitute an G to A nucleotide substitution, giving raise to an glutamic acid to lysine mutation at residue 117. The truncated G1 gene was expressed as a 37 kDa protein fused to glutathione-S-transferase (GST). The GST fusion protein was purified by Glutathione Sepharose 4B affinity chromatography and reacted with the sera from patients of hemorrhage fever with renal syndrome (HFRS). One of 12 serum samples from HFRS patients was reactive with the 37 kDa fusion protein strongly. Three sera reacted moderately with the fusion protein. Six sera reacted only weakly with the protein, while remaing two were non-reactive. Control sera from patients with scrub typhus leptospirosis, or negative HFRS did not react with the recombinant fusion protein.
Antigens, Viral ; Asparagine ; Base Sequence ; Blotting, Western ; Chromatography, Affinity ; Clone Cells ; Fever ; Glutamic Acid ; Glutathione ; Glycoproteins* ; Hantaan virus* ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Leptospirosis ; Lysine ; Polymerase Chain Reaction ; Scrub Typhus ; Sepharose ; Serine ; Threonine

Objectives: . To investigate the correlation of objective audiometry with user satisfaction as measured with the questionnaire scores. Methods: . Twenty patients with hearing loss, who agreed to wear a hearing aid and were referred for hearing aid fitting, were included in this prospective clinical study. All patients used the in-the-canal type of Wide7 hearing aid provided by BSL Co., Ltd. We performed the Korean version of the Hearing Handicap Inventory for the Elderly (K-HHIE) and the International Outcome Inventory for Hearing Aids (K-IOI-HA) before and 1, 3, and 6 months after wearing the hearing aid. We also performed pure tone audiometry (PTA), speech audiometry (SA), functional gain (FG), hearing in noise test (HINT), and central auditory processing disorder tests, such as frequency pattern test (CA-f), duration pattern test (CA-d), and dichotic test (CA-Di). Patients were divided into two groups (group A-HHIE, improved; group B-HHIE, same or worse) by comparing the score of K-HHIE before and 6 months after wearing the hearing aid. In the 6-month K-IOI-HA questionnaire, 21 points were considered as the average score. Based on this, we further divided patients into two groups (group A-IOI, >21 points; group B-IOI, ≤21 points). Results: . Group A-HHIE included six patients and group B-HHIE included 14 patients. In PTA, SA, HINT, CA-d, and CA-Di, group A-HHIE showed higher improvements than group B-HHIE, which were not statistically significant. Group A-IOI included 12 patients and group B-IOI included eight patients. No statistically significant difference was noted in the improvement of audiometric results over a period of 6 months after wearing the hearing aid between groups A-IOI and B-IOI. Conclusion . There were no significant and consistent audiometric results to reflect patient’s satisfaction with the hearing aid. Therefore, when analyzing the hearing aid-fitting outcome, both the objective audiometric tests and subjective questionnaire should be performed together for validating hearing aid performance.

BACKGROUND: The assessment of the severity of psoriasis is often subjective because of the lack of quantitative laboratory diagnostic tools. Histopathological examination is the most commonly performed procedure for psoriasis diagnosis; however, it is usually descriptive. Thus, there is currently no quantitative method of determining psoriasis severity. The clinical types of psoriasis are correlated with the severity of the disease, and a lesional severity index, such as the psoriasis severity index (PSI), could be used as a quantitative tool for assessing gross severity. OBJECTIVE: To correlate the histopathological findings of psoriasis with the PSI. METHODS: Psoriatic lesions in 98 patients were evaluated. The lesions were classified into the guttate, papular, small plaque, and large plaque types according to morphology, and were scored according to the PSI. Ten common histopathological features of psoriasis were evaluated for correlation with gross severity. RESULTS: The clinical types of psoriasis showed significant correlations with the histopathological severity. However, the PSI score showed no correlation with histopathological severity. CONCLUSION: In the future, subjective gross assessment should be modified by using objective measuring devices with detailed scales, in order to correlate the findings with the histological severity.
Adult ; Classification ; Diagnosis ; Evidence-Based Practice ; Humans ; Pathology ; Psoriasis* ; Weights and Measures

Mature cystic teratomas of the ovary (MCT) are usually observed in women of reproductive age with the most dreadful complication being malignant transformation which occurs in approximately 1% to 3% of MCTs. In this case report, we present a patient with squamous cell carcinoma which developed from a MCT during pregnancy. The patient was treated conservatively without adjuvant chemotherapy and was followed without evidence of disease for more than 60 months using conventional tools as well as positron emission tomography-computed tomography following the initial surgery. We report this case along with the review of literature.
Carcinoma, Squamous Cell ; Chemotherapy, Adjuvant ; Dermoid Cyst ; Electrons ; Female ; Humans ; Ovary ; Pregnancy ; Teratoma

Because nail psoriasis is difficult to treat, therapy with many biological drugs has been attempted. Ustekinumab is approved for chronic plaque psoriasis and psoriatic arthritis (PsA), with some trials reporting nail improvement using this agent. A 51-year-old man with severe chronic plaque psoriasis had severe involvement of all fingernails and toenails, with accompanying nail fold psoriasis. He also had PsA of the small joints of the fingers. Despite multiple conventional therapies, the nail lesions did not improve, and his nail psoriasis severity index score was 97. After a fourth ustekinumab injection, most of the fingernail psoriasis was resolved, and only hyperkeratosis remained on both large toenails. Because the nail plate, nail fold, and small joints of the fingers are closely apposed structures within a small area, cytokines produced from the nail units overflow to the nail fold and small joints and can induce nail fold psoriasis and PsA.
Arthritis ; Arthritis, Psoriatic ; Cytokines ; Fingers ; Humans ; Joints ; Middle Aged ; Nail Diseases ; Nails ; Psoriasis* ; Severity of Illness Index ; Ustekinumab

OBJECTIVE: To investigate the relationship between serum creatine kinase (CK) level and pulmonary function in Duchenne muscular dystrophy (DMD). METHODS: A total of 202 patients with DMD admitted to the Department of Rehabilitation Medicine, Gangnam Severance Hospital were enrolled from January 1, 1999 to March 31, 2015. Seventeen patients were excluded. Data collected from the 185 patients included age, height, weight, body mass index, pulmonary function tests including forced vital capacity (FVC), peak cough flow, maximal expiratory pressure (MEP), and maximal inspiratory pressure (MIP), and laboratory measurements (serum level of CK, CK-MB, troponin-T, and B-type natriuretic peptide). FVC, MEP, and MIP were expressed as percentages of predicted normal values. RESULTS: Serum CK activities were elevated above normal levels, even in the oldest DMD group. Serum CK level was strongly correlated with pulmonary functions of sitting FVC (p<0.001), supine FVC (p<0.001), MIP (p=0.004), and MEP (p<0.001). CONCLUSION: Serum CK level is a reliable screening test even in patients with advanced DMD, and is a strong predictor of pulmonary functions.
Body Weight ; Cough ; Creatine Kinase* ; Creatine* ; Humans ; Mass Screening ; Muscular Dystrophy, Duchenne* ; Reference Values ; Rehabilitation ; Respiratory Function Tests ; Troponin T ; Vital Capacity

A quantitative assessment of the density of the protein gene product 9.5 (PGP9.5), the neural cell adhesion molecule (NCAM), and the low-affinity nerve growth factor receptor (NGFR) expressing nerve fibers in the circular muscle layer in the colon was carried out by morphometric analyses from 13 patients with Hirschsprung's disease (HD). The difference in the nerve fiber density between the ganglionic and aganglionic segments was compared by calculating the ratio of the sum of the areas occupied by positively stained nerve fibers per unit area of the muscle after immunohistochemical staining on paraffin embedded tissue sections using computer software. There was an obvious difference in the density of the PGP9.5 stained nerve fibers between the ganglionic (0.0380 +/- 0.0171) and aganglionic segments (0.0143 +/- 0.01661). The NCAM-positive nerve fibers were fewer in number than those of both the PGP9.5-positive fibers and NCAM-positive fibers, which were also markedly lower in number in the aganglionic segment (0.0066 +/- 0.0076) than in the ganglionic segment (0.0230 +/- 0.0195). Immunostaining for low-affinity NGFR revealed much fainter staining in the ganglionic and aganglionic segment without a statistically significant difference in their density. Considering the fact that PGP9.5 is a very sensitive marker for nerve fibers, the results of this study reaffirm the innervation failure of the proper muscle in HD. The decreased NCAM expression level in the aganglionic segment appears to be caused not by the selective down-regulation of NCAM expression among the nerve fibers but by a markedly reduced number of nerve fibers.
Colon/*innervation ; Hirschsprung Disease/*pathology ; Human ; Muscle, Smooth/*innervation ; Nerve Fibers/*chemistry/pathology ; Neural Cell Adhesion Molecules/*analysis ; Receptor, Nerve Growth Factor/analysis ; Thiolester Hydrolases/*analysis

Background and Objectives: The 10-item Standardized Cosmesis and Health Nasal Outcomes Survey (SCHNOS) is a recently developed questionnaire for assessing both nasal obstruction and nasal cosmetic satisfaction. It is widely used in rhinoplasty clinics based on its high level of reliability and efficacy in Western countries. In this study we evaluated the validity of the Korean version of the SCHNOS (K-SCHNOS).Subjects and Method The SCHNOS was translated into easily-understandable Korean following the guideline of the developers. Twenty-seven patients undergoing rhinoplasty and a control group of 29 non-rhinoplasty patients completed K-SCHNOS twice at intervals of 2 weeks regardless of pre- or postoperative state. The K-SCHNOS was evaluated for internal consistency, test-retest reliability, and validity. Results: Of the 56 study participants, 39 (69.6%) were male and 17 (30.4%) were female, with the mean (standard deviation) age of 33.5 (12.9) years. Both obstructive domain and cosmetic domains showed high internal consistency with their respective Cronbach’α values being 0.92 and 0.91. The correlations in the test-retest analysis for each item were acceptable, ranging from 0.6-0.82. The association between the obstructive domain and cosmetic domain was 0.48 (95% confidence interval, 0.32-0.61), indicating a very weak correlation. Conclusion The K-SCHNOS has proven to be a reliable and valid questionnaire to be used by Korean-speaking patients undergoing functional or cosmetic rhinoplasty.