Pachydermoperiostosis (PDP) is a primary hypertrophic osteoarthropathy characterized by digital clubbing, pachydermia, and periostosis, which is inherited as an autosomal dominant or recessive trait. We report on a patient suffering from bilateral knee arthritis for 6 years who was newly diagnosed as PDP. PDP was confirmed by bilateral digital clubbing, hyperhidrosis, and cutis verticis gyrata, findings of pachydermatosis on the forehead and scalp, X-ray findings of proliferative periostitis. This case indicates that PDP is one of several possible rare diseases that should be considered in patients with undifferentiated arthritis.
Arthritis* ; Forehead ; Humans ; Hyperhidrosis ; Knee ; Osteoarthropathy, Primary Hypertrophic* ; Periostitis ; Rare Diseases ; Scalp

Exercise-induced left bundle branch block is a rare condition that has been reported along with and without demonstrable cardiac abnormalities. We describe the case of a 73-year-old female with chest pain on execration. Coronary angiography revealed normal findings. She underwent a treadmill stress test. During the exercise left bundle branch block with concomitant chest pain was demonstrated. Chest pain was relieved with cessation of exercise.
Aged ; Bundle-Branch Block* ; Chest Pain* ; Coronary Angiography ; Exercise Test ; Female ; Humans

Pneumatosis cystoides intestinalis (PCI) is a rare disease with intramural gas formation in the gastrointestinal tract. The causes of PCI are various, and are commonly associated with collagen vascular disease. We present a case of a 48-year-old female with dermatomyositis (DM) who also developed PCI. Her risk of PCI may have been increased by multiple factors such as gender, DM itself, and medications including corticosteroids, methotrexate, and azathioprine. While the cause of, and risk factors for PCI in DM patients are not well known on a global scale, outcomes range from benign to life threatening. Therefore, we present a case study and review the literature to identify candidate risk factors for PCI.
Adrenal Cortex Hormones ; Azathioprine ; Collagen ; Dermatomyositis* ; Female ; Gastrointestinal Tract ; Humans ; Methotrexate ; Middle Aged ; Pneumatosis Cystoides Intestinalis* ; Rare Diseases ; Risk Factors ; Vascular Diseases

BACKGROUND/AIMS: The serum cytokeratin-18 (CK-18) has been suggested to be a surrogate marker of non-alcoholic fatty liver disease (NAFLD). The aim of this study was to investigate the correlation between CK-18 and metabolic parameter in NAFLD patients. Correlation between CK-18 and macronutrient composition was also assessed. METHODS: A total of 212 subjects were recruited. Blood chemistry including fasting glucose, cholesterol level, AST, ALT, and CK-18 were compared. Data on calorie intake and carbohydrate consumption were acquired by five-day-diet diary using 24 hour recall method. RESULTS: Plasma CK-18 were markedly increased in patient with NAFLD compared with control group (420.4+/-282.3 vs. 313.6+/-179, p<0.001). Plasma CK-18 were positively correlated with systolic blood pressure (r=0.130), ALT (r=0.503) and negatively correlated with HDL cholesterol (r=-0.246). NAFLD patients with metabolic syndrome had higher CK-18 level than those without metabolic syndrome (484.0 vs. 372.1 U/L, p=0.021). When NAFLD patients were subdivided into two groups with CK-18 cut-off value of 400 U/L, patients with CK-18 level over 400 U/L showed higher body mass index (28.0+/-4.5 vs. 25.5+/-4.3), subcutaneous abdominal fat (283.5+/-172.2 vs. 195.7+/-147.8), AST (52.7+/-26.3 vs. 40.7+/-23.5) and ALT (102.0+/-52.6 vs. 61.2+/-32.2). Calorie intake (r=0.301) and carbohydrate intake (r=0.305) also showed positive correlation with CK-18. CONCLUSIONS: Plasma CK-18 showed positive correlation with metabolic parameters as well as calorie and carbohydrate intake when its cut-off value of greater than 400 U/L was used.
Abdominal Fat ; Biological Markers ; Blood Pressure ; Body Mass Index ; Carbohydrates ; Chemistry ; Cholesterol ; Cholesterol, HDL ; Fasting ; Fatty Liver* ; Glucose ; Humans ; Keratin-18* ; Plasma ; Ultrasonography*

This correction is being published to correct the third author's Korean name.

PURPOSE: "Chest compression-only" CPR encourages CPR by untrained lay people. Guidelines do not include victim's chest exposure, which might result in inaccurate compression point; therefore, this study compares accuracy of lay rescuer's chest compression point in dressed and undressed situations. METHODS: In this prospective observational cross-over study conducted in a simulation center of a tertiary academic hospital in the Republic of Korea, 96 non-medical college student volunteers were asked to point in random order to the location of four reference chest compression points [inter-nipple line (INL), center of the chest (CoC), lower quarter of the sternum (LQS), and lower end of the sternum (LES)]. In dressed and undressed situations, length from sternal notch to each of the subject-recognized compression and reference points and degree of deviation from sternum midline was compared. RESULTS: In both dressed and undressed situations, INL was closest while LQS, CoC, and LES were further away, in said order, from the sternal notch. The undressed situation showed association with lower reference and subjectrecognized chest compression point differences; subjectrecognized chest compression point deviation; and out-ofchest compression point frequency. CONCLUSION: Addition of "exposing victim's chest" to the CPR education program for untrained lay people might facilitate accurate and safe localization of chest compression points in cardiac arrest patients.
Cardiopulmonary Resuscitation* ; Cross-Over Studies ; Education ; Heart Arrest ; Humans ; Prospective Studies ; Republic of Korea ; Sternum ; Thorax* ; Volunteers

PURPOSE: Lynch syndrome, the commonest hereditary colorectal cancer syndrome, is caused by germline mutations in mismatch repair (MMR) genes. Three recently developed prediction models for MMR gene mutations based on family history and clinical features (MMRPredict, PREMM1,2,6, and MMRPro) have been validated only in Western countries. In this study, we propose validating these prediction models in the Korean population. MATERIALS AND METHODS: We collected MMR gene analysis data from 188 individuals in the Korean Hereditary Tumor Registry. The probability of gene mutation was calculated using three prediction models, and the overall diagnostic value of each model compared using receiver operator characteristic (ROC) curves and area under the ROC curve (AUC). Quantitative test characteristics were calculated at sensitivities of 90%, 95%, and 98%. RESULTS: Of the individuals analyzed, 101 satisfied Amsterdam criteria II, and 87 were suspected hereditary nonpolyposis colorectal cancer. MMR mutations were identified in 62 of the 188 subjects (33.0%). All three prediction models showed a poor predictive value of AUC (MMRPredict, 0.683; PREMM1,2,6, 0.709; MMRPro, 0.590). Within the range of acceptable sensitivity (> 90%), PREMM1,2,6 demonstrated higher specificity than the other models. CONCLUSION: In the Korean population, overall predictive values of the three models (MMRPredict, PREMM1,2,6, MMRPro) for MMR gene mutations are poor, compared with their performance in Western populations. A new prediction model is therefore required for the Korean population to detect MMR mutation carriers, reflecting ethnic differences in genotype-phenotype associations.
Area Under Curve ; Colorectal Neoplasms ; Colorectal Neoplasms, Hereditary Nonpolyposis ; DNA Mismatch Repair* ; Genetic Association Studies ; Genetic Testing ; Germ-Line Mutation ; Humans ; ROC Curve ; Sensitivity and Specificity

PURPOSE: Seizure and syncope are main causes of patient visits to an emergency department (ED). However, differentiating two diseases through history taking and several ancillary tests is difficult in an ED. Some reports have claimed that ammonia or lactate could differentiate between seizure and other medical diseases. Therefore, this study evaluated the ability of ammonia and lactate to differentiate seizure and syncope. METHODS: We reviewed the medical records of 388 patients who visited an ED with seizure or syncope. There were 271 patients in the seizure group (69.8%) and 117 patients in the syncope group (30.2%). Ammonia and lactate levels were compared between the two groups. The optimal level of ammonia and lactate, and their sensitivity and specificity, were evaluated to determine their ability to differentiate seizure and syncope. RESULTS: There were more male in seizure group (66%), but the average age was higher in the syncope group (51+/-21 yrs) (p<0.05). The time interval from symptom to ED was similar between the two groups (seizure group: 213+/-803 min and syncope group: 184+/-512 min, p=0.724). The mean ammonia level in the seizure group was higher than the syncope group (85+/-86 microg/dl and 49+/-29 microg/dl, respectively, p<0.001). The mean lactate level was also higher in the seizure group than the syncope group (3.5+/-2.9 mmol/L and 1.6+/-1.5 mmol/L, respectively, p<0.05). When the cutoff value for ammonia was 50 microg/dL [Area under curve (AUC): 0.617, 95% CI: 0.556-0.678] the sensitivity was 61% and the specificity was 52%. When the cut-off value for lactate was 2 mmol/L [AUC: 0.745, 95% CI: 0.692-0.798] the sensitivity was 60% and the specificity was 76%. CONCLUSION: Serum ammonia or lactate may serve as an ancillary method for differentiating seizure and syncope, but their sole application is unacceptable due to their low sensitivity and specificity.
Ammonia* ; Emergencies ; Humans ; Lactates ; Lactic Acid* ; Male ; Medical Records ; Methods ; Seizures* ; Sensitivity and Specificity ; Syncope*