Background: The PLASMIC score is a convenient tool for predicting ADAMTS13 activity of ＜10%.Lactate dehydrogenase (LDH) is widely used as a marker of haemolysis in thrombotic thrombocytopenic purpura (TTP) monitoring, and could be used as a replacement marker for lysis. We aimed to validate the PLASMIC score in a multi-centre Asia Pacific region, and to explore whether LDH could be used as a replacement marker for lysis. Methods: Records of patients with thrombotic microangiopathy (TMA) were reviewed. Patients’ ADAMTS13 activity levels were obtained, along with clinical/laboratory findings relevant to the PLASMIC score. Both PLASMIC scores and PLASMIC-LDH scores, in which LDH replaced traditional lysis markers, were calculated. We generated a receiver operator characteristics (ROC) curve and compared the area under the curve values (AUC) to determine the predictive ability of each score. Results: 46 patients fulfilled the inclusion criteria, of which 34 had ADAMTS13 activity levels of ＜10%. When the patients were divided into intermediate-to-high risk (scores 5‒7) and low risk (scores 0‒4), the PLASMIC score showed a sensitivity of 97.1% and specificity of 58.3%, with a positive predictive value (PPV) of 86.8% and negative predictive value (NPV) of 87.5%. The PLASMIC-LDH score had a sensitivity of 97.1% and specificity of 33.3%, with a PPV of 80.5% and NPV of 80.0%. Conclusion Our study validated the utility of the PLASMIC score, and demonstrated PLASMIC-LDH as a reasonable alternative in the absence of traditional lysis markers, to help identify high-risk patients for treatment via plasma exchange.

Purpose: Factors associated with invasive recurrence (REC) of ductal carcinoma in situ (DCIS) are less known. This study was aimed at identifying better biomarkers to predict the prognosis of DCIS. Methods: RNA extracted from formalin-fixed paraffin-embedded blocks of twenty-four pure DCIS cases was subjected to differential gene expression analysis. The DCIS cases were selected by matching age and estrogen receptor status. Sixteen REC-free and 8 invasive-REC cases with disease-free interval of > 5 years were analyzed. Immunohistochemistry (IHC) staining was used to validate sixty-one independent pure DCIS cases, including invasive-REC (n = 16) and REC-free (n = 45) cases. Results: Eight differentially expressed genes (DEGs) were statistically significant (log 2-fold change [FC] < –1 or > 1 and p < 0.001). Less than ½ fold expression of CUL1, androgen receptor (AR), RPS27A, CTNNB1, MAP3K1, PRKACA, GNG12, MGMT genes was observed in the REC group compared to the no evidence of disease group. AR and histone deacetylase 1 (HDAC1) genes were selected for external validation (AR: log 2-FC − 1.35, p < 0.001, and HDAC1: log 2-FC − 0.774, p < 0.001). External validation showed that the absence of AR and high HDAC1 expression were independent risk factors for invasive REC (hazard ratio [HR], 5.04; 95% confidence interval [CI], 1.24–20.4; p = 0.023 and HR, 3.07; 95% CI, 1.04–9.04; p = 0.042). High nuclear grade 3 was also associated with long-term invasive REC. Conclusion Comparative gene expression analysis of pure DCIS revealed 8 DEGs among recurring cases. External validation with IHC suggested that the absence of AR and overexpression of HDAC1 are associated with a greater risk of long-term invasive REC of pure DCIS.

PURPOSE: Ductal carcinoma in situ (DCIS) is a high-risk disease for the development of invasive tumors. Although it is associated with excellent prognosis, many patients undergo extensive treatment with surgery, radiation, and endocrine therapy. This study evaluated the clinical and pathologic factors associated with invasive recurrence, particularly locoregional, distant disease after breast-conserving surgery (BCS). METHODS: This study included 431 patients diagnosed with pure DCIS after BCS between January 2000 and December 2008. The clinicopathological characteristics, margin status, adjuvant therapy, and duration of endocrine therapy were evaluated in hormone receptor-positive DCIS patients. RESULTS: The median duration of follow-up was 115 months. During this period, 37 cases (8.5%) of recurrence were observed (12 in situ and 23 invasive recurrence and 2 unknown cases). There was no distant metastasis as first event. Similarly, none of the initial in situ recurrence cases developed distant metastasis while eight (32%, 8/23) developed distant metastasis after invasive recurrence. Overall recurrence was associated with young age (≤40 years) (HR: 3.60, 95% confidence interval [CI]: 1.77–7.32) and hormone receptor negativity (HR: 3.33, 95% CI: 1.52–7.29). Invasive local recurrence was independently associated with young age (≤40 years) (HR: 3.86, 95% CI: 1.50–9.96), high nuclear grade (HR: 4.46, 95% CI: 1.62–12.27) and omission of adjuvant radiotherapy (HR: 6.45, 95% CI: 1.82–22.82). Notably, duration of endocrine treatment among the hormone receptor positive patient group, was numerically shorter for recurred patients, though not statistically significant. CONCLUSION Young age, high nuclear grade and absence of adjuvant radiotherapy were independently associated with an increased risk of invasive recurrence. Moreover, invasive locoregional recurrence as a first event was associated with worse outcomes, yet in situ recurrence didn't affect overall survival. Further studies with larger sample sizes are warranted to confirm the prognostic indicators of recurrence and the optimal strategy for adjuvant therapy in this setting.

OBJECTIVE: To investigate the predictive index of functional recovery after primary pontine hemorrhage (PPH) using the combined motor evoked potential (MEP) and somatosensory evoked potential (SEP) in comparison to the hematoma volume and transverse diameter measured with computerized tomography. METHODS: Patients (n=14) with PPH were divided into good- and poor-outcome groups according to the modified Rankin Score (mRS). We evaluated clinical manifestations, radiological characteristics, and the combined MEP and SEP responses. The summed MEP and SEP (EP sum) was compared to the hematoma volume and transverse diameter predictive index of global disability, gait ability, and trunk stability in sitting posture. RESULTS: All measures of functional status and radiological parameters of the good-outcome group were significantly better than those of the poor-outcome group. The EP sum showed the highest value for the mRS and functional ambulatory category, and transverse diameter showed the highest value for "sitting-unsupported" of Berg Balance Scale. CONCLUSION: The combined MEP and SEP is a reliable and useful tool for functional recovery after PPH.
Evoked Potentials, Motor ; Evoked Potentials, Somatosensory* ; Gait ; Hematoma ; Hemorrhage* ; Humans ; Posture

OBJECTIVES: This study aimed to determine the effects of smoking on alcohol cravings in alcoholdependent individuals. METHODS: Study participants were 123 male smokers, 18 to 65 years of age, diagnosed with alcohol dependence. The study questionnaire consisted of demographic characteristics, alcohol drinking habits, smoking habits, the Korean Obsessive-Compulsive Drinking Scale (OCDS), the 12-item Tobacco Craving Questionnaire (12-item TCQ), and the Fagerstrom Test for Nicotine Dependence (FTND). We divided the subjects into high-OCDS score and low-OCDS score groups. RESULTS: The high-OCDS group had a higher alcohol drinking frequency and greater average amount of alcohol consumed than did the low-OCDS group. Also, the high-OCDS group smoked a higher average number of cigarettes per day and had higher 12-item TCQ scores and FTND scores. Logistic regression analysis showed scores of the 12-item TCQ and the FTND were the most significant variables for predicting alcohol cravings. CONCLUSION: This study shows that alcohol cravings are significantly related to cigarette cravings and to severity of nicotine dependence.
Alcohol Drinking ; Alcoholism ; Drinking ; Humans ; Logistic Models ; Male ; Nicotine ; Surveys and Questionnaires ; Smoke ; Smoking ; Tobacco ; Tobacco Products ; Tobacco Use Disorder

INTRODUCTIONLong QT syndrome (LQTS), an inherited cardiac arrhythmia, is a disorder of ventricular repolarisation characterised by electrocardiographic abnormalities and the onset of torsades de pointes leading to syncope and sudden death. Genetic polymorphisms in 5 well-characterised cardiac ion channel genes have been identified to be responsible for the disorder. The aim of this study is to identify disease-causing mutations in these candidate genes in a LQTS family.

MATERIALS AND METHODSThe present study systematically screens the coding region of the LQTS-associated genes (KCNQ1, HERG, KCNE1, KCNE2 and SCN5A) for mutations using DNA sequencing analysis.

RESULTSThe mutational analysis revealed 7 synonymous and 2 non-synonymous polymorphisms in the 5 ion channel genes screened.

CONCLUSIONWe did not identify any clear identifiable genetic marker causative of LQTS, suggesting the existence of LQTS-associated genes awaiting discovery.

Adolescent ; Adult ; Child ; DNA Mutational Analysis ; ERG1 Potassium Channel ; Ether-A-Go-Go Potassium Channels ; analysis ; genetics ; Female ; Frameshift Mutation ; Humans ; KCNQ1 Potassium Channel ; analysis ; genetics ; Long QT Syndrome ; genetics ; Male ; Middle Aged ; Muscle Proteins ; analysis ; genetics ; NAV1.5 Voltage-Gated Sodium Channel ; Polymorphism, Genetic ; genetics ; Potassium Channels, Voltage-Gated ; analysis ; genetics ; Sodium Channels ; analysis ; genetics ; Trans-Activators

BACKGROUND: Type 2 diabetes develops because of defects in both insulin secretion and action. The half-life of insulin in uremia is prolonged because the metabolic clearance rate of insulin in diabetic end stage renal disease (ESRD) patients is reduced with consequence that the dose of insulin and/or oral hypoglycemic agent (OHA) administered in normal renal function make them increase the risk of hypoglycemia. Therefore, we should usually reduce the dose of insulin and/or OHA, or stop administration of insulin and/or OHA if type 2 diabetic patients are progressed to ESRD. But in some patients, that is not true. The aim of this study was to test the hypothesis that insulin resistance plays an important role in (re)evaluation of optimal insulin and/or OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD. METHODS: Insulin resistance was examined in 23 type 2 diabetic ESRD patients with tight control of glycemia using the K index of the insulin tolerance test (Kitt). We divided 23 patients into three groups. Group 1 (n=10) was defined as patients who were administered neither insulin nor OHA after ESRD. Group 2 (n=9) was defined as patients who were changed from insulin to OHA as drug for glycemic control after ESRD. Group 3 (n=4) was defined as patients in whom insulin or OHA was continuously administered after ESRD without a change of them for glycemic control. We compared the degree of insulin resistance among these three groups. RESULTS: Insulin resistance determined by Kitt was significantly different between group 1 (Kitt, 2.1422/0.94-4.01%/min), group 2 (Kitt, 1.3811/0.79- 3.90%/min) and group 3 (Kitt, 0.8550/0.44-1.81%/min) by using Kruskal-Wallis test (p=0.048). Kitt in group 3 was significantly lower than in group 1 by using Mann-Whitney test (p=0.016). CONCLUSION: Although metabolic clearance of insulin is reduced by renal failure, demand of insulin/ OHA for optimal glycemic control is not reduced in higher insulin-resistant type 2 diabetic ESRD patients on hemodialysis. Insulin resistance plays an important role in determination of optimal insulin/ OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD.
Half-Life ; Humans ; Hypoglycemia ; Insulin Resistance* ; Insulin* ; Kidney Failure, Chronic* ; Metabolic Clearance Rate ; Renal Dialysis* ; Renal Insufficiency ; Uremia

Branchioma ; Carotid Body Tumor ; Diagnosis ; Diagnosis, Differential ; Head ; Hemangioma ; Hodgkin Disease ; Humans ; Lymph Nodes ; Lymphangioma, Cystic ; Lymphoma ; Lymphoma, Non-Hodgkin ; Neck ; Neoplasm Metastasis ; Oropharynx ; Parotid Gland ; Parotid Region ; Rare Diseases ; Retrospective Studies ; Tongue

Absorptiometry, Photon ; Adult ; Aged ; Bone Density ; Chungcheongbuk-do ; Female ; Humans ; Ilium ; Male ; Middle Aged ; Osteoporosis ; Tibia ; Tissue Donors ; Transplants ; Volunteers

BACKGROUND AND AIMS: Various gastric and duodenal lesions with gastrofiberscopy were observed in patients with obstructive biliary disease. METHODS: A clinical analysis of the endoscopic findings was carried out on 88 patients with obstructive biliary disease, from February 1994 to January 1998, in the department of Internal Medicine, Chungnam National University Hospital. RESULTS: 1) In the 88 patients, sex distribution showed predominance in the males (47/41) and most of the cases involved those in their 60's. 2) The obstructive biliary diseases were included, common bile duct stones (53.4%), common bile duct cancer (18.2%), pancreatic cancer (18.2%), Klatskin tumor (4.5%) and common hepatic duct cancer (3.4%). 3) The gastroduodenal lesions involved in the obstructive biliary diseases were, erosive gastritis (22.7%), duodenal ulcer (8.0%), gastric ulcer (5.7%), and acute duodenitis (3.4%) in orders. CONCLUSIONS: Significant upper gastrointestinal lesions were found in obstructive biliary disease.
Chungcheongnam-do ; Common Bile Duct ; Duodenal Ulcer ; Duodenitis ; Gastritis ; Hepatic Duct, Common ; Humans ; Internal Medicine ; Klatskin's Tumor ; Male ; Pancreatic Neoplasms ; Sex Distribution ; Stomach Ulcer