Pachyonychia congenita (PC) is a rare autosomal-dominant ectodermal dysplasia, which is characterized by hypertrophic nail dystrophy, diffuse or focal symmetrical hyperkeratosis of the palms and soles, oral leukokeratosis, follicular keratosis on the extensor surfaces of the extremities, and other ectodermal defects. However, the variable degree of gene mutation may result in nail abnormalities alone. The nail changes presented in our patient are the only clinical manifestation. Hypertrophic nail dystrophy and yellowish discoloration, without involving any other organs, were shown in both mother and baby since birth. To the best of our knowledge, the case of PC affecting only the nails has never been reported in Korea before. Herein, we report monosymptomatic form of PC, which is notable for its rareness and unique pattern, distinct from previously reported cases.
Ectoderm ; Ectodermal Dysplasia ; Extremities ; Humans ; Keratosis ; Korea ; Leukoplakia, Oral ; Mothers ; Nails ; Nails, Malformed ; Pachyonychia Congenita ; Parturition

Although the incidence of bleeding complications during extracorporeal membrane oxygenator (ECMO) support has decreased in various trials, bleeding is still the most fatal complication. We investigated the ideal dosage and efficacy of nafamostat mesilate for use with ECMO in patients with acute cardiac or respiratory failure. We assessed 73 consecutive patients who received ECMO due to acute cardiac or respiratory failure between January 2006 and December 2009. To evaluate the efficacy of nafamostat mesilate, we divided the patients into 2 groups according to the anticoagulants used during ECMO support. All patients of nafamostat mesilate group were male with a mean age of 49.2 yr. Six, 3, 5, and 3 patients were diagnosed with acute myocardial infarction, cardiac arrest, septic shock, and acute respiratory distress syndrome, respectively. The mean dosage of nafamostat mesilate was 0.64 mg/kg/hr, and the mean duration of ECMO was 270.7 hr. The daily volume of transfused packed red blood cells, fresh frozen plasma, and cryoprecipitate and the number of complications related to hemorrhage and thrombosis was lower in the nafamostat mesilate group than in the heparin group. Nafamostat mesilate should be considered as an alternative anticoagulant to heparin to reduce bleeding complications during ECMO.
Acute Disease ; Anticoagulants/*administration & dosage ; Dose-Response Relationship, Drug ; *Extracorporeal Membrane Oxygenation ; Female ; Guanidines/*administration & dosage ; Heart Failure/diagnosis/mortality/therapy ; Heparin/administration & dosage ; Humans ; Male ; Middle Aged ; Myocardial Infarction/diagnosis/mortality/therapy ; Respiratory Distress Syndrome, Adult/diagnosis/mortality/therapy ; Retrospective Studies ; Shock, Septic/diagnosis/mortality/therapy ; Survival Analysis

Primary cutaneous follicular B cell lymphomas are rare entities of an unknown cause, except for those induced by Epstein-Barr virus (EBV) or Borrelia burgdorferi. Follicular B cell lymphoma has been thought to rarely occur primarily in the skin. The lesions are generally solitary plaques or nodules that are localized on the head and neck. It shows considerable variation in the clinical presentation, the histological features, the immunophenotype and the prognosis. In this case, a 79-year-old man presented with a 3-month history of lesions on his head. Clinical examination revealed two deep-seated subcutaneous tumors with uneven surfaces located on the both sides of the frontoparietal area of the scalp. Biopsy of the skin lesion disclosed a massive dermal lymphocytic infiltrate with a follicular pattern. On immunohistochemical staining, the lymphocytes strongly expressed CD20 and Ki-67, but not Bcl-2. He was diagnosed with primary cutaneous follicular B cell lymphoma. This case illustrates a rarely reported example of primary cutaneous follicular B cell lymphoma that arose on the scalp.
Aged ; Biopsy ; Borrelia burgdorferi ; Head ; Herpesvirus 4, Human ; Humans ; Lymphocytes ; Lymphoma, B-Cell ; Neck ; Prognosis ; Scalp ; Skin

PURPOSE: To compare short-term clinical results of femtosecond LASIK with those of epi-LASIK. METHODS: Twenty subjects (40 eyes) underwent femtosecond LASIK and 20 subjects (40 eyes) underwent epi-LASIK for myopia with astigmatism. The results of each surgery were compared with regard to visual acuity, spherical equivalent, safety, efficacy, stability, predictability and high order aberration. RESULTS: Postoperative uncorrected visual acuities were 0.51 +/- 0.11, 0.95 +/- 0.08, and 0.97 +/- 0.08 for epi-LASIK and 0.76 +/- 0.19, 0.97 +/- 0.07, and 0.98 +/- 0.06 for femtosecond LASIK at one week, one month, and two months after surgery, respectively. Femtosecond LASIK showed faster improvement in visual acuity. Postoperative spherical equivalents were -0.83 +/- 0.24, -0.31 +/- 0.19, and -0.27 +/- 0.09 for epi-LASIK and -0.47 +/- 0.21, -0.28 +/- 0.15, and -0.25 +/- 0.12 for femtosecond LASIK. Safety, efficacy, stability, and predictability showed no differences between the two groups. High order aberrations were increased significantly; however, no significant difference between the two groups was found. CONCLUSIONS: Both epi-LASIK and femtosecond LASIK are effective for surgical correction of myopia with fast visual rehabilitation. In addition, epi-LASIK and femtosecond LASIK showed good safeties, efficacies, predictabilities, and stabilities.
Astigmatism ; Keratomileusis, Laser In Situ ; Myopia ; Safety ; Visual Acuity

The purpose of this study was to evaluate the reliability of 6-axis model surgery simulator (6AMSS) for orthognathic surgery. A rectangular parallelepiped plastic block was assembled to model-mounting plate of 6AMSS. Left-right (X), anterior-posterior (Y), up-down (Z) translation and pitching (empty set X), rolling (empty set Y) and yawing (empty set Z) rotation was planned and performed using 6AMSS. The actual translation and rotation were measured with dial gauge and precisional protractor, respectively. Comparison between the planned and actual movements of plastic block for each variable were made using paired t- test. Statistical analysis for X, Y, Z, empty set X, empty set Y and empty set Z movement have shown no significant differences between planned and actual movement (P > 0.05). This indicate that model surgery performed with the aid of the 6AMSS is accurate in 3D translation and rotation. The 6AMSS is practically useful for accurate fabrication of surgical splint for orthognathic surgery.
Orthognathic Surgery ; Plastics ; Splints

PURPOSE: Smoking elicits airway inflammation and airflow obstruction in patients with asthma, even after smoking cessation. The aim of this study was to examine the effects of smoking cessation on lung function and quality of life (QOL) in asthmatic patients. METHODS: Thirty-two patients with asthma who were active smokers were recruited. After education on the effects of smoking on asthma, 22 patients continued to smoke, and 10 quit smoking. All patients were treated with inhaled fluticasone propionate (1 mg/day) for 3 months. We compared forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity (FVC), forced expiratory flow between 25 and 75% FVC (FEF(25-75%)), and scores on a QOL questionnaire at baseline, 1, 2, and 3 months. RESULTS: Quitters showed a greater percent change in FEV1 (19.1+/-6.3 vs. 7.9+/-2.4%, P=0.024) and FEV1/FVC (6.5+/-4.14 vs. 3.5+/-1.5%, P=0.05) than smokers. Both quitters and smokers showed improved QOL scores after 1, 2, and 3 months of fluticasone treatment. CONCLUSIONS: Patients with asthma who quit smoking showed less airway obstruction, suggesting that smoking cessation is crucial in the management of asthma.
Airway Obstruction ; Androstadienes ; Asthma ; Diethylpropion ; Forced Expiratory Volume ; Humans ; Inflammation ; Lung ; Quality of Life ; Smoke ; Smoking ; Smoking Cessation ; Vital Capacity ; Fluticasone ; Surveys and Questionnaires

BACKGROUND: Accurate measurement of blood glucose concentrations is essential for defining diabetes, and the minimization of ex vivo glycolysis has been recommended. Recent guidelines advocate two kinds of methods for sample collection and processing: either the sodium fluoride (NaF) method or immediate refrigeration using a serum separation tube (SST). We investigated the difference between the two methods in measuring subsequent glucose concentrations using blood specimens from participants recruited for the fourth Korean National Health and Nutrition Examination Survey. METHODS: Paired venous blood samples were collected in an SST and a NaF tube from 1,103 men and women. SST serum was separated within 30 min, including standing for 15 min, and then refrigerated. The NaF samples were refrigerated, but not separated until immediately before analysis. We compared the blood glucose concentrations between the SST (SST glucose) and NaF (NaF glucose) methods. RESULTS: The mean SST glucose was significantly higher than NaF glucose (99.0 mg/dL vs 96.5 mg/dL, P<0.05). NaF glucose showed a negative mean bias of 2.6 mg/dL vs SST glucose but showed high correlation (R=0.9899). There was no significant correlation between the bias of blood glucose concentrations by two methods and the storage time of NaF glucose. CONCLUSIONS: The negative bias associated with the use of NaF tubes may significantly affect the prevalence of diabetes. Serum separation and refrigeration within 30 min after venous sampling is recommended over NaF method, not only to minimize the preanalytical impact on detecting diabetes but also to reduce sample volume and number of tubes.
Blood Glucose/*analysis ; Blood Specimen Collection/*methods ; Diabetes Mellitus/diagnosis ; Female ; Glycolysis/*drug effects ; Humans ; Male ; Nutrition Surveys ; Republic of Korea ; Sodium Fluoride/*pharmacology ; Specimen Handling

Condyloma acuminatum, commonly known as anogenital warts, is caused by human papillomavirus (HPV). The most common location of condyloma acuminatum in women is the vulva. Other locations are the vagina, anus and perianal area, perineum and cervix. Condyloma acuminatum most commonly occur due to receptive anal intercourse, and can enlarge to form exophytic masses on the perianal skin, but rarely involve the rectum. We experienced an occurrence of a 12 mm polypoid lesion in the rectum of a heterosexual woman detected during a colonoscopy. The polypoid lesion was excised and was diagnosed as condyloma acuminatum. The lesion was positive for HPV type 11 based on the use of an HPV DNA chip test.
Anal Canal ; Cervix Uteri ; Colonoscopy ; Female ; Heterosexuality ; Humans ; Oligonucleotide Array Sequence Analysis ; Perineum ; Polyps ; Rectum ; Skin ; Vagina ; Vulva ; Warts

Choledochoduodenal fistula (CDF) occurring simultaneously with pancreaticoduodenal fistula is extremely rare. CDF has known to be a chronic sequela of cholelithiasis, but it is unknown whether pancreaticoduodenal fistula results from chronic cholelithiasis as well. We report a case of cholelithiasis accompanied with choledochoduodenal and pancreaticoduodenal fistula opening into small suprapapillary diverticulum in a 80-year-old woman.
Aged, 80 and over ; Biliary Fistula/*complications ; Cholelithiasis/complications ; Common Bile Duct Diseases/*complications ; Diverticulum/*complications ; Duodenal Diseases/*complications ; Female ; Humans ; Intestinal Fistula/*complications ; Pancreatic Fistula/*complications

PURPOSE: To study the availability of the radiological classification for incongruent hallux valgus deformities, which has been used as the key to the algorithm for selecting surgical options. MATERIALS AND METHODS: To determine radiological severity, 257 cases of incongruent hallux valgus deformities were studied. The hallux valgus angle (HVA) and the 1/2 intermetatarsal angle (IMA) were measured in each case. Following Mann's radiological classification system, the HVA and IMA were compared to determine any similarity in severity. The distal metatarsal articular angle (DMAA) was also measured to observe the extent of its effect on the incongruent deformity. RESULTS: The HVA was 36.1degrees on average (range, 16-60degrees) and the 1/2 IMA was 15.8degrees on average (range, 5-30degrees). In 126 cases (49.1%), the severity between the 1/2 IMA and HVA coincided with the index classification, whereas. 131 cases (50.9%) did not correspond. Overall, the severity of approximately half the cases, could not be graded. The DMAA was 15.6degrees on average (range, 0-40degrees) and 133 cases (51.8%) demonstrated a value above normal value (>15degrees). CONCLUSION: The radiological classification system used as a guide for surgical decision-making needs to be revised, due to the overt limits of correspondence between the severity of the 1/2 IMA and HVA. Because the HVA would be influenced by both the lateral subluxation and bony deformity around the first metatarsophalangeal joint, it is reasonable to consider these factors on the HVA as elements to be corrected individually, instead of the HVA alone.
Classification* ; Congenital Abnormalities* ; Hallux Valgus* ; Hallux* ; Metatarsal Bones ; Metatarsophalangeal Joint ; Reference Values