Objectives: . Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by recurrent epistaxis, telangiectasia, and visceral arteriovenous malformations (AVMs). Activin A receptor-like type 1 (ACVRL1/ALK1) and endoglin (ENG) are the principal genes whose mutations cause HHT. No multicenter study has yet investigated correlations between genetic variations and clinical outcomes in Korean HHT patients. Methods: . Seventy-two members from 40 families suspected to have HHT based on symptoms were genetically screened for pathogenic variants of ACVRL1 and ENG. Patients with genetically diagnosed HHT were also evaluated. Results: . In the HHT genetic screening, 42 patients from 24 of the 40 families had genetic variants that met the pathogenic criteria (pathogenic very strong, pathogenic strong, pathogenic moderate, or pathogenic supporting) based on the American College of Medical Genetics and Genomics Standards and Guidelines for either ENG or ACVRL1: 26 from 12 families (50%) for ENG, and 16 from 12 families (50%) for ACVRL1. Diagnostic screening of 42 genetically positive HHT patients based on the Curaçao criteria revealed that 24 patients (57%) were classified as having definite HHT, 17 (41%) as having probable HHT, and 1 (2%) as unlikely to have HHT. Epistaxis was the most common clinical presentation (38/42, 90%), followed by visceral AVMs (24/42, 57%) and telangiectasia (21/42, 50%). Five patients (12%) did not have a family history of HHT clinical symptoms. Conclusion . Only approximately half of patients with ACVRL1 or ENG genetic variants could be clinically diagnosed as having definite HHT, suggesting that genetic screening is important to confirm the diagnosis.

BACKGROUND: Malnutrition is associated with many adverse clinical outcomes. The present study aimed to identify the prevalence of malnutrition in hospitalized patients in Korea, evaluate the association between malnutrition and clinical outcomes, and ascertain the risk factors of malnutrition. METHODS: A multicenter cross-sectional study was performed with 300 patients recruited from among the patients admitted in 25 hospitals on January 6, 2014. Nutritional status was assessed by using the Subjective Global Assessment (SGA). Demographic characteristics and underlying diseases were compared according to nutritional status. Logistic regression analysis was performed to identify the risk factors of malnutrition. Clinical outcomes such as rate of admission in intensive care units, length of hospital stay, and survival rate were evaluated. RESULTS: The prevalence of malnutrition in the hospitalized patients was 22.0%. Old age (≥ 70 years), admission for medical treatment or diagnostic work-up, and underlying pulmonary or oncological disease were associated with malnutrition. Old age and admission for medical treatment or diagnostic work-up were identified to be risk factors of malnutrition in the multivariate analysis. Patients with malnutrition had longer hospital stay (SGA A = 7.63 ± 6.03 days, B = 9.02 ± 9.96 days, and C = 12.18 ± 7.24 days, P = 0.018) and lower 90-day survival rate (SGA A = 97.9%, B = 90.7%, and C = 58.3%, P < 0.001). CONCLUSION: Malnutrition was common in hospitalized patients, and resulted in longer hospitalization and associated lower survival rate. The rate of malnutrition tended to be higher when the patient was older than 70 years old or hospitalized for medical treatment or diagnostic work-up compared to elective surgery.
Cross-Sectional Studies ; Hospitalization ; Humans ; Intensive Care Units ; Korea ; Length of Stay ; Logistic Models ; Malnutrition ; Multivariate Analysis ; Nutrition Assessment ; Nutritional Status ; Prevalence ; Risk Factors ; Survival Rate

BACKGROUND: The aim of this study was to evaluate the safety and analgesic efficacy of polmacoxib 2 mg versus placebo in a superiority comparison or versus celecoxib 200 mg in a noninferiority comparison in patients with osteoarthritis (OA). METHODS: This study was a 6-week, phase III, randomized, double-blind, and parallel-group trial followed by an 18-week, single arm, open-label extension. Of the 441 patients with knee or hip OA screened, 362 were randomized; 324 completed 6 weeks of treatment and 220 completed the extension. Patients were randomized to receive oral polmacoxib 2 mg (n = 146), celecoxib 200 mg (n = 145), or placebo (n = 71) once daily for 6 weeks. During the extension, all participants received open-label polmacoxib 2 mg. The primary endpoint was the change in Western Ontario and McMaster Universities (WOMAC)-pain subscale score from baseline to week 6. Secondary endpoints included WOMAC-OA Index, OA subscales (pain, stiffness, and physical function) and Physician's and Subject's Global Assessments at weeks 3 and 6. Other outcome measures included adverse events (AEs), laboratory tests, vital signs, electrocardiograms, and physical examinations. RESULTS: After 6 weeks, the polmacoxib-placebo treatment difference was −2.5 (95% confidence interval [CI], −4.4 to −0.6; p = 0.011) and the polmacoxib-celecoxib treatment difference was 0.6 (CI, −0.9 to 2.2; p = 0.425). According to Physician's Global Assessments, more subjects were “much improved” at week 3 with polmacoxib than with celecoxib or placebo. Gastrointestinal and general disorder AEs occurred with a greater frequency with polmacoxib or celecoxib than with placebo. CONCLUSIONS: Polmacoxib 2 mg was relatively well tolerated and demonstrated efficacy superior to placebo and noninferior to celecoxib after 6 weeks of treatment in patients with OA. The results obtained during the 18-week trial extension with polmacoxib 2 mg were consistent with those observed during the 6-week treatment period, indicating that polmacoxib can be considered safe for long-term use based on this relatively small scale of study in a Korean population. More importantly, the results of this study showed that polmacoxib has the potential to be used as a pain relief drug with reduced gastrointestinal side effects compared to traditional nonsteroidal anti-inflammatory drugs for OA.
Arm ; Celecoxib* ; Electrocardiography ; Hip ; Humans ; Knee ; Ontario ; Osteoarthritis* ; Outcome Assessment (Health Care) ; Physical Examination ; Vital Signs

PURPOSE: To evaluate the analgesic effect, efficacy and safety of aceclofenac controlled release (CR) in patients with chronic knee osteoarthritis (OA). MATERIALS AND METHODS: A total of 125 subjects with chronic knee OA were randomly divided into two groups: one group (n=62) was administered aceclofenac CR once daily and the other (n=63), aceclofenac immediate release (IR) twice a day for 4 weeks. A 100-mm visual analogue scale (VAS), Knee injury and Osteoarthritis Outcome Score (KOOS) and range of motoin (ROM) were evaluated as the outcome measures. To evaluate the safety of the drug, adverse events, vital signs, physical examination findings, clinical laboratory values and electrocardiographic findings were evaluated. RESULTS: The VAS, KOOS and ROM were improved after 4 weeks of administration in both groups, but the differences between the two groups were not statistically significant. Significant differences between the two groups were not shown in the evaluation of the adverse events, vital sign, physical examination results, clinical laboratory values, and electrocardiography. CONCLUSIONS: The aceclofenac CR and aceclofenac IR were equally effective in patients with chronic knee OA and the clinical trial results didn't show any significant difference in safety. The new aceclofenac CR formulation was found to be effective and safe with the practical advantage of once daily administration.
Delayed-Action Preparations ; Electrocardiography ; Humans ; Knee Injuries ; Knee ; Osteoarthritis ; Osteoarthritis, Knee ; Outcome Assessment (Health Care) ; Physical Examination ; Vital Signs

BACKGROUND: Sedation in spinal anesthesia can reduce patient's anxiety and discomfort. Dexmedetomidine has a sedative, hypnotic, analgesic, and minimal respiratory depression effect. However, use of the dexmedetomidine is associated with prolonged recovery. This study was designed to investigate the optimal dose of intravenous dexmedetomidine for proper sedation with minimal recovery time in spinal anesthesia. METHODS: One hundred twenty eight patients, aged 20-70 years (58.8 +/- 0.7), were recruited. After performing the spinal anesthesia with hyperbaric bupivacaine (13 mg), a loading dose of dexmedetomidine (1 microg/kg) was administered for 10 min, followed by the maintenance infusion of the following: Group A (n = 33; normal saline), Group B (n = 35; dexmedetomidine 0.2 microg/kg/hr), and Group C (n = 39; dexmedetomidine 0.4 microg/kg/hr). Heart rate, blood pressure, and the bispectral index score (BIS) were recorded during the operation. In the recovery room, modified aldrete score (MAS) was measured. RESULTS: There were no significant differences in mean blood pressure and heart rate among the three groups. BIS was not significantly different among the three groups from baseline to 60 min after the infusion of dexmedetomidine. BIS were significantly increased in Group A after 70 and 80 min, and Group A and B after 90, 100, 110 min of dexmedetomidine infusion (P < 0.05). MAS was higher in Group A as compared to Group B and C, within 30 min after admission in the recovery room (P < 0.05). CONCLUSIONS: The loading dose (1 microg/kg/10 min) of dexmedetomidine was sufficient for surgery of less than 60 min. Dexmedetomidine infusion followed by maintenance dose (0.2 microg/kg/hr) was sufficient for surgery within 90 min.
Aged ; Anesthesia, Spinal ; Anxiety ; Blood Pressure ; Bupivacaine ; Dexmedetomidine ; Heart Rate ; Humans ; Recovery Room ; Respiratory Insufficiency

Foreign body in the airway could be a life-threatening risk, especially for young pediatric patients. A 6-day old male patient with foreign body, which was located deep in the right main bronchus was being admitted. Although we tried three times to remove it with rigid bronchoscopic forceps under the general anesthesia, we failed. Before switching to surgical treatment, we changed the Trendelenburg position and tapped his back several times in order to alter the foreign body toward the forcep. Finally we were able to catch and extract the foreign body successfully. We suggest that back percussion with the Trendelenburg position is a useful solution to remove a foreign body within a deep airway.
Anesthesia, General ; Bronchi ; Foreign Bodies ; Head-Down Tilt ; Humans ; Infant, Newborn ; Male ; Percussion ; Posture ; Surgical Instruments

Spinal dural arteriovenous fistula (SDAVF) is rare but still the most commonly encountered vascular malformation of the spinal cord. A 31-year-old male developed gait disturbance due to weakness of his lower extremities, voiding difficulty and sexual dysfunction with a progressive course since 3 months. He showed areflexia in both knees and ankles. Electromyographic findings were suggestive of multiple root lesions involving bilateral L2 to S4 roots of moderate degree. Magnetic resonance images showed high signal intensity with an ill-defined margin in T2-weighted images and intensely enhanced by a contrast agent through the lumbosacral spinal cord. Selective spinal angiography confirmed a dural arteriovenous fistula with a nidus at the L2 vertebral level. After selective endovascular embolization, his symptoms drastically improved except sexual dysfunction. We report a rare case of cauda equina syndrome due to spinal arteriovenous fistula with drastic improvement after endovascular embolization.
Adult ; Angiography ; Animals ; Ankle ; Arteriovenous Fistula ; Cauda Equina ; Central Nervous System Vascular Malformations ; Gait ; Humans ; Knee ; Lower Extremity ; Magnetic Resonance Spectroscopy ; Male ; Polyradiculopathy ; Spinal Cord ; Vascular Malformations

BACKGROUND: Etomidate frequently induces myoclonus when administered intravenously with bolus injection during anesthetic induction. This can be bothersome for the anesthesiologist. The dose of remifentanil appropriate for preventing myoclonus without side effects was investigated. METHODS: All patients with American Society of Anesthesiologists (ASA) physical status I-III were divided into three groups (n = 33 per group) according to the pretreatment effect site concentration of remifentanil (Ultiva, Glaxo-Wellcome, Munchen, Germany) of 0, 2 or 4 ng/ml (Group N: 0 ng/ml, Group R: 2 ng/ml, Group Q: 4 ng/ml) by a target controlled infusion (TCI) system. After a 0.3 mg/kg dose of etomidate was injected intravenously for over 1 minute for anesthetic induction, myoclonus was observed. Before the etomidate injection, the patients were pretreated with remifentanil and their side effects were monitored. RESULTS: The number of patients showing myoclonus was significantly different among the groups. The incidence of myoclonus was 81%, 12% and 0% (groups N, R, and Q, respectively, P < 0.01). Side effects including bradycardia and hypotension did not occur in either Group R or Q. Chest wall rigidity occured in 45% of patients in Group Q. CONCLUSIONS: Administration with a 2 ng/ml effect site concentration of remifentanil could reduce the incidence of myoclonus caused by etomidate bolus injection without chest wall rigidity.
Bradycardia ; Etomidate ; Humans ; Hypotension ; Incidence ; Myoclonus ; Piperidines ; Thoracic Wall

BACKGROUND: Fiberoptic bronchoscope guided intubation is an important method of difficult airway management. The use of specific airways has been devised to assist the fiberoptic intubation. The authours compared effectiveness of separated airway with fiberoptic bronchoscope guided intubation and the hemodynamic responses. METHODS: 104 adult patients of American Society of Anesthesiologists grading (ASA) I-II who scheduled for surgery under general anesthesia were randomly divided into the Laryngoscope group (L group, n = 30) or the Fiberoptic bronchoscope group (F group, n = 36) or the Fiberoptic bronchoscope with separated airway (MF group, n = 38). A Fiberoptic bronchoscope guided intubation and a fiberoptic bronchoscope with separated airway and a direct laryngoscope was performed after inducing anesthesia. Intubation time, Jaw thrust incidence, mean blood pressure and heart rate after anaesthesia induction, at intubation and every two minute for a further 7 min were recorded. RESULTS: The intubation time was significantly shorter in the MF group (58.3 +/- 13.7 sec) than F group (71.9 +/- 22.1 sec). Jaw thrust incidence was lower in the MF group (60.5%) than F group (100%). The changes of MAPs and HRs during the observation were not significantly different in three group. CONCLUSIONS: Fiberoptic intubation using separated airway reduced intubation time and the incidence of jaw thrust.
Adult ; Airway Management ; Anesthesia ; Anesthesia, General ; Blood Pressure ; Bronchoscopes ; Heart Rate ; Hemodynamics ; Humans ; Incidence ; Intubation ; Jaw ; Laryngoscopes

The Fas (TNF receptor superfamily, member 6) (FAS)/FAS ligand (FASLG) interaction plays a central role in the regulation of programmed cell death. FAS and FASLG polymorphisms in promoter regions affect transcriptional activities. To investigate whether FAS and FASLG polymorphisms are associated with the development and clinical phenotypes of stroke, 2 promoter single nucleotide polymorphisms (SNPs) in FAS (rs1800682, -670C/T) and FASLG (rs763110, -844C/T) were selected and genotyped by direct sequencing in 220 stroke patients [107 ischemic stroke (IS), 77 intracerebral hemorrhage (ICH), and 36 subarachnoid hemorrhage (SAH)] and 369 control subjects. For the analysis of clinical symptoms, all stroke patients were divided into 3 clinical phenotypes according to the respective results of the National Institutes of Health Stroke Survey (NIHSS) and the Modified Barthel Index (MBI) and the presence or absence of complex regional pain syndrome (CRPS). The SNPStats, SNPAnalyzer, and Helixtree programs were used to analyze the genetic data. Multiple logistic regression models (codominant, dominant, and recessive) were used to estimate odds ratios (ORs), 95% confidence intervals (CIs), and p-values. The promoter SNP rs1800682 was associated with stroke in the codominant (OR=0.48, 95% CI=0.25-0.94, p=0.04) and dominant models (OR=0.51, 95% CI=0.30-0.87, p=0.011). However, a FASLG SNP (rs763110) was not in Hardy-Weinberg equilibrium (p<0.05). In the analysis of stroke types, rs1800682 was associated with IS in the codominant (OR=0.30, 95% CI=0.12-0.74, p=0.025), dominant (OR=0.44, 95% CI=0.23-0.88, p=0.018), and recessive models (OR=0.45, 95% CI=0.21-0.99, p=0.042). The genotype frequencies of rs1800682 were different between ICH and controls in the dominant model (OR=0.49, 95% CI=0.26-0.94, p=0.031) but not between SAH and controls. In the analysis of clinical symptoms, however, rs1800682 was not related to the 3 clinical phenotypes (NIHSS, MBI, and CRPS). These results suggest that a promoter SNP (rs1800682, -670C/T) in FAS may be associated with the development of stroke in the Korean population.
Cell Death ; Cerebral Hemorrhage ; Genotype ; Humans ; Logistic Models ; National Institutes of Health (U.S.) ; Odds Ratio ; Phenotype ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Stroke ; Subarachnoid Hemorrhage