Background: and Purpose The influence of imaging features of brain frailty on outcomes were investigated in acute ischemic stroke patients with minor symptoms and large-vessel occlusion (LVO). Methods: This was a retrospective analysis of a prospective, multicenter, nationwide registry of consecutive patients with acute (within 24 h) minor (National Institutes of Health Stroke Scale score=0–5) ischemic stroke with anterior circulation LVO (acute minor LVO). Brain frailty was stratified according to the presence of an advanced white-matter hyperintensity (WMH) (Fazekas grade 2 or 3), silent/old brain infarct, or cerebral microbleeds. The primary outcome was a composite of stroke, myocardial infarction, and all-cause mortality within 1 year. Results: In total, 1,067 patients (age=67.2±13.1 years [mean±SD], 61.3% males) were analyzed. The proportions of patients according to the numbers of brain frailty burdens were as follows: no burden in 49.2%, one burden in 30.0%, two burdens in 17.3%, and three burdens in 3.5%. In the Cox proportional-hazards analysis, the presence of more brain frailty burdens was associated with a higher risk of 1-year primary outcomes, but after adjusting for clinically relevant variables there were no significant associations between burdens of brain frailty and 1-year vascular outcomes. For individual components of brain frailty, an advanced WMH was independently associated with an increased risk of 1-year primary outcomes (adjusted hazard ratio [aHR]=1.33, 95% confidence interval [CI]=1.03–1.71) and stroke (aHR=1.32, 95% CI=1.00–1.75). Conclusions The baseline imaging markers of brain frailty were common in acute minor ischemic stroke patients with LVO. An advanced WMH was the only frailty marker associated with an increased risk of vascular events. Further research is needed into the association between brain frailty and prognosis in patients with acute minor LVO.

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.
Enzyme Assays ; Fibroblasts ; Humans ; Iduronate Sulfatase ; Leukocytes ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Phenotype ; Plasma ; Sensitivity and Specificity ; Skin

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.
Enzyme Assays ; Fibroblasts ; Humans ; Iduronate Sulfatase ; Leukocytes ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Phenotype ; Plasma ; Sensitivity and Specificity ; Skin

PURPOSE: A lateral neck node metastasis is common in patients with papillary thyroid carcinoma. If a preoperative diagnosis is not made or is uncertain, an intraoperative biopsy for a frozen examination should be considered. The aims of this study were to evaluate the factors predicting a lateral neck node metastasis and to suggest guidelines for an intraoperative neck node biopsy. METHODS: From March 2003 to January 2006, 79 patients (7 males, 72 females) with 89 intraoperatively biopsied lateral neck nodes were enrolled in this study. The median age was 45 years. Among these patients, two or more lateral neck nodes were biopsied intraoperatively in 9 patients and one node was biopsied in the others. The clinicopathological features and radiological findings were reviewed. RESULTS: Among the 79 patients with 89 lateral neck nodes, 25 patients with 26 lateral neck nodes (29%) showed a metastasis. Univariate analysis revealed the computed tomography (CT) findings of lateral neck nodes such as a longitudinal size >1 cm (P=0.001), postcontrast Hounsefield Unit (HU) >110 (P<0.001), presence of necrosis (P<0.001), absence of hilum (P<0.001), and irregular margin (P<0.001) were found to be significant predicting factors. The pathologic findings of tumors such as multifocality (P= 0.006), bilaterality (P=0.001), tumor size >2 cm (P=0.008), extracapsular invasion (P=0.005) had significant impact on a lateral neck node metastasis. Multivariate analysis revealed a longitudinal size >1 cm (P=0.039), postcontrast HU >110 (P<0.001), and bilaterality of the tumor (P= 0.001) with a suspiciously enlarged lateral neck node in CT to have a significant impact on node metastasis. However, bilateral tumor with lymph nodes >110 HU were the most important factors. CONCLUSION: The most significant factors influencing lateral neck node metastasis were multifocal, bilateral, large size (2 cm < or = ), extracapsular invasion of the primary tumor, and the CT findings of lymph nodes such as a large longitudinal size (1 cm < or =), necrosis, the absence of hilum, irregular margin and increased postcontrast HU (110 < or =).
Biopsy ; Carcinoma, Papillary ; Diagnosis ; Humans ; Lymph Nodes ; Male ; Multivariate Analysis ; Neck* ; Necrosis ; Neoplasm Metastasis* ; Thyroid Gland* ; Thyroid Neoplasms*

Nephrotic syndrome is a rare manifestation of malignancy associated with paraneoplastic syndrome. Paraneoplastic nephrotic syndrome has been reported in various malignancies: malignant lymphoma, colon cancer, lung cancer and prostate cancer. However, an ovarian carcinoma associated with nephrotic syndrome has rarely been reported. Only six cases of ovarian carcinoma associated paraneoplastic nephrotic syndrome has been reported worldwide, but no cases have been reported in Korea. Here, we report a case of paraneoplastic nephrotic syndrome in a patient with an ovarian carcinoma. The patient presented with ascites, proteinuria and hypoalbuminemia. An initial computed tomography (CT) scan and ultrasonography evaluations showed no specific findings suggestive of an ovarian tumor. Despite treatment for nephrotic syndrome, the symptoms became more aggravated. There after, follow up evaluation at Yonsei University Medical Center, including serum CA 125, pelvis MRI and peritoneal fluid examination were performed. On the pelvis MRI, a left ovarian mass was detected with an ascitic fluid collection. The serum CA 125 level was elevated to 2211 U/ml. The peritoneal fluid cytological examination showed malignant cells suggestive of an ovarian carcinoma. Combination chemotherapies including paclitaxel plus carboplatin, topotecan plus gemcitabine and oxaliplatin plus capecitabine were administered to the patient, and complete remission was achieved on image and tumor marker studies. There was complete recovery from the nephrotic syndrome with no evidence of ascites and proteinuria. These findings suggest that nephrotic syndrome caused by paraneoplastic syndrome can be resolved only after the complete control of the underlying malignancy.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Carcinoma/*complications/diagnosis/drug therapy ; Female ; Human ; Magnetic Resonance Imaging ; Middle Aged ; Nephrotic Syndrome/*complications/drug therapy ; Ovarian Neoplasms/*complications/diagnosis/drug therapy ; Paraneoplastic Syndromes/*complications/drug therapy ; Remission Induction ; Support, Non-U.S. Gov't ; Tomography, Emission-Computed ; Tomography, X-Ray Computed

BACKGROUND/AIMS: The competency of the gastroesophageal junction (GEJ) holds the key in unlocking pathophysiologic mechanisms of gastroesophageal reflux disease (GERD). However, a relationship between GERD and the incompetent GEJ has not been established. The aim of our study was to assess the relationship between the shape of the GEJ and gastroesophageal acid reflux. METHODS: Forty six patients with reflux symptoms underwent an endoscopy, esophageal manometry and 24-hour esophageal pH monitoring. Patients were placed in 3 groups according to the shape of their GEJ, categorized by a retroflex view of the endoscopy; type I - gastroesophageal fold without a pouch, type II - no pouch and no fold, and type III - a pouch without a fold. RESULTS: In type II and III, LESP was reduced. However, % of time with the pH < 4.0 was increased in type III only. There was a significant correlation between the size of a hiatal hernia and the shape of the GEJ. There was a relationship between the grade of esophagitis and the shape of the GEJ. CONCLUSIONS: The retroflex endoscopic finding of the GEJ focusing on the presence or absence of a GE fold and hiatal pouch, could be an indicator of whether a patient has GERD.
Endoscopy ; Esophageal pH Monitoring ; Esophageal Sphincter, Lower ; Esophagitis ; Esophagogastric Junction* ; Gastroesophageal Reflux ; Hernia, Hiatal ; Humans ; Hydrogen-Ion Concentration ; Manometry

BACKGROUND/AIMS: Gastroesophageal reflux disease (GERD) is known to be one of the contributing factors to cause epigastric soreness, which we often experience after intake of flour-based meals. The aim of this study was to evaluate gastroesophgeal reflux (GER) and its mechanism according to intake of different ingredients of meals. METHODS: We investigated the effect of meals of different ingredients on the lower esophageal sphincter (LES) pressure, the frequency of transient LES relaxation (tLESR) and GER in seven healthy volunteers. The meals used in this study were either rice cakes or flour cakes, which were isocaloric and isovolumetric. A mylohyoid electromyogram, LES pressure and esophageal pH were simultaneously recorded for 3 hours after ingestion of each meal on two different occasions. RESULTS: There was no significant difference in incidence of GER between two meals. CONCLUSION: The intake of isocaloric and isovolumetric meals of either flour or rice cake did not result in significant discrepancies in frequency of GER. The increase in the frequency of swallow- and strain-associated GER observed in the case of a flour cake may be caused by reduced LES pressure after ingestion.
Eating ; Esophageal Sphincter, Lower ; Flour* ; Gastroesophageal Reflux ; Healthy Volunteers ; Hydrogen-Ion Concentration ; Incidence ; Meals* ; Relaxation

BACKGROUND/AIMS: The nonspecific esophageal motility disorder (NEMD) has been reported to be related to gastroesophageal reflux disease (GERD) in some cases. However, the pathophysiologic mechanism of NEMD has not been estabilished. The aim of this study was to assess the prevalence of esophagitis and gastric acid reflux following abnormal contractions in patients with NEMD. METHODS: 122 NEMD patients were enrolled (76 male and 46 female) and their endoscopic findings and 24 hour esophageal pH data were compared with 24 healthy subjects. The abnormal contractions were classified as 1) non-transmitted contraction, 2) low amplitude contraction, 3) non-transmitted and low amplitude contraction, and 4) others. RESULTS: Among the 122 patients with NEMD, 62 patients (50.8%) had GERD, 53 patients (43.4%) had endoscopic reflux esophagitis, and 41 patients (33.6%) had both. Acid exposed time studied by 24 hour pH monitoring was more increased in NEMD patients than in healthy controls (7.48 +/- 10.68 vs 1.42 +/- 1.17), but there were no differences among abnormal contraction patterns. Moderate to severe reflux esophagitis were frequently seen in patients with combined abnormal contractions as the results of endoscopic findings. Pre-existing factors for the gastric acid reflux in NEMD patients were male and esophageal hiatal hernia. CONCLUSIONS: Large numbers of NEMD patients were found to have concurrent GERD in our study. However, the esophageal peristaltic dysfunction was more closely related to the severity of esophagitis rather than to the amount of refluexed gastric acid itself.
Esophageal Motility Disorders* ; Esophagitis ; Esophagitis, Peptic ; Gastric Acid ; Gastroesophageal Reflux* ; Hernia, Hiatal ; Humans ; Hydrogen-Ion Concentration ; Male ; Prevalence*

This study is verifying the hypothesis that distraction followed by recompression may promote fracture healing of the osteoporotic bone on the assumption that distraction induces increased callus formation at the distracted gap and recompression results in fusiform callus, having biomechanically stronger fracture stiffness. Sixty-six Sprague-Dawley rats, in which osteoporosis were induced 6-8 weeks after oophorectomy, were separated into two groups. Group I was control group that had fracture healing by simple external fixation. Group II was experimental group that had fracture healing by monofocal distraction-compression. Radiomorphometric study revealed that there was significantly increased amount of callus and ensuing bone formation, which becehe obvious from 20 days after osteotomy in group II compared with group I. Biomechanical study also showed that tensile break strength was significantly higher in group II compared with group I with time. It is concluded that monofocal distraction-compression method may be one of the useful method to promote fracture healing of the estrogen deficient osteoporotic bone.
Animals ; Bony Callus ; Estrogens ; Female ; Fracture Healing* ; Osteogenesis ; Osteoporosis* ; Osteotomy ; Ovariectomy ; Rats* ; Rats, Sprague-Dawley

To evaluate the effect of intertrochanteric femoral derotational osteotomy(IFDO) on the sagittal plane kinematics and kinetics of the hip and pelvis in spastic cerebral palsy, we compared the preoperative and post-operative results of 3 dimensional gait analysis. Intertrochanteric femoral derotational osteotomy alone without psoas procedure was performed in 34 hips of cerebral palsy patients with increased femoral anterversion regardless of preoperative dynamic or static hip flexion contracture. Those who had other concomitant hip procedures were excluded. There were 24 diplegics and 10 hemiplegics. Mean age of the patients at the time of operation was 9.1 years (range, 4.9 to 22). They were divided into three subgroups according to the degree of dynamic hip flexion contracture; 13 patients with normal hip extension in terminal stance (group I), 13 patients with maximum hip extension in terminal stance between 0 and 15 degrees (group II), and 8 patients with maximum hip extension in terminal stance of more than 15 degrees (group III). The gait analysis included clinical assessment, video-taping, 3D-kinematics and kinetics, and dynamic EMG. Linear parameters of gait, kinematic parameters, sagittal plane hip moment parameters, and total hip energy parameters were compared. Postoperatively, cadence and double support time decreased, whereas walking velocity and stride length increased in all groups. Maximum and minimum pelvic tilt were improved in all groups. The range of pelvic tilt improved in all groups except group I. Hip flexion-extension curve shifted into extension in all groups. Marked improvement in maximum hip extension in stance and the range of hip motion were observed in all groups. The sum of extensor moment decreased, whereas that of flexor moment increased significantly in all groups. The conversion timing from extensor to flexor moment significantly improved in group I and II. The decrease of power generations of Hl and the increase of power absorptions of H2 were significant in all groups. However, there were no significant changes in power generation of H3 in all groups. The changes of peak power generation timing of H3 was not consistent among the groups. Femoral derotational osteotomy at the intertrochanteric level brings the lesser trochanter forward resulting in iliopsoas lengthening effect. We found significant improvement of the sagittal plane kinematics and kinetics of the hip and pelvis when IFDO alone was performed without psoas tenotomy. The psoas lengthening procedure may be considered secondarily at the time of hardware removal after full evaluation of the psoas lengthening effect.
Absorption ; Biomechanical Phenomena ; Cerebral Palsy* ; Contracture ; Family Characteristics ; Femur ; Gait ; Hip* ; Humans ; Kinetics ; Muscle Spasticity* ; Osteotomy* ; Pelvis* ; Tenotomy ; Walking