Biliary atresia (BA) is an uncommon neonatal surgical disease that has a fatal outcome if not properly treated. The survival rates of the patients with native liver after Kasai's operation in countries outside Japan are not so good. We reviewed the results of 22 cases of biliary atresia treated in Kosin University Hospital between October 1987 and March 2001. There were 13 males and 9 females aged from 21 to 106 days (mean 52 days). There were 3 cases of Type I (13.6%), and 3 of Type II (13.6%), and 16 Type III (72.7%). The operative methods were resection of the common bile duct remnant and cyst followed by Roux-en-Y hepaticojejunostomy in 3 cases for Type I BA; Kasai I in 15 cases, Kasai II in 1 case, and Ueda's operation in 3 cases for Types II and III BA. There was no death within the first 30 days after operation. We were able to follow 21 of the 22 patients (95.4%) for more than 5 years. The actual 5 year survival rate (YSR) was 40.9%. One Type I case received a living-related liver transplantation at 6 years of age because of the multiple intrahepatic stones and liver cirrhosis. Five YSR after biliostomy group (Kasai II and Ueda op.) was 75% (3/4) while that of Kasai I was 20% (3/15). One case had no bile duct in the resected fibrotic plaque on microscopic review and died 8 months after Kasai I operation, would have been a strong candidate for early liver transplantation. From the above result, our conclusions are as follows; (1) early liver transplantation should be considered for cases of no bile duct after pathologic examination of the resected specimen, (2) measures to prevent postoperative cholangitis and prevention of postoperative liver cirrhosis are needed, (3) liver transplantation program should be available for failed cases.
Bile Ducts ; Biliary Atresia ; Cholangitis ; Common Bile Duct ; Fatal Outcome ; Female ; Humans ; Japan ; Liver ; Liver Cirrhosis ; Liver Transplantation ; Male ; Survival Rate

BACKGROUND: Lung cancer is the most common cause of cancer related death. Alterations in gene sequence, structure, and expression have an important role in the pathogenesis of lung cancer. Fusion genes and alternative splicing of cancer-related genes have the potential to be oncogenic. In the current study, we performed RNA-sequencing (RNA-seq) to investigate potential fusion genes and alternative splicing in non-small cell lung cancer. METHODS: RNA was isolated from lung tissues obtained from 86 subjects with lung cancer. The RNA samples from lung cancer and normal tissues were processed with RNA-seq using the HiSeq 2000 system. Fusion genes were evaluated using Defuse and ChimeraScan. Candidate fusion transcripts were validated by Sanger sequencing. Alternative splicing was analyzed using multivariate analysis of transcript sequencing and validated using quantitative real time polymerase chain reaction. RESULTS: RNA-seq data identified oncogenic fusion genes EML4-ALK and SLC34A2-ROS1 in three of 86 normal-cancer paired samples. Nine distinct fusion transcripts were selected using DeFuse and ChimeraScan; of which, four fusion transcripts were validated by Sanger sequencing. In 33 squamous cell carcinoma, 29 tumor specific skipped exon events and six mutually exclusive exon events were identified. ITGB4 and PYCR1 were top genes that showed significant tumor specific splice variants. CONCLUSION: In conclusion, RNA-seq data identified novel potential fusion transcripts and splice variants. Further evaluation of their functional significance in the pathogenesis of lung cancer is required.
Alternative Splicing* ; Carcinoma, Non-Small-Cell Lung* ; Carcinoma, Squamous Cell ; Exons ; Gene Fusion ; Lung ; Lung Neoplasms ; Multivariate Analysis ; Real-Time Polymerase Chain Reaction ; RNA* ; Sequence Analysis ; Sequence Analysis, RNA*

Neurological manifestations of internal carotid aretry (ICA) dissection include amaurosis fugax, cerebral ischemia, oculosympathetic paresis, and various cranial nerve palsies. Isolated hypoglossal nerve palsy is a rare manifestation of ICA dissection. A 55-year-old man developed dysarthria following sudden pain in the left retroauricular area. His tongue was paralysed on the left side. Magnetic resonance image and carotid angiogram showed characteristic features of left ICA dissection, which may be the most plausible cause of hypoglossal nerve palsy in this patient. Expanding hematoma of dissecting aneurysm of ICA seems to have compressed the nutrient artery of the hypoglossal nerve, although the possibility of direct compression of the hypoglossal nerve itself is not completely ruled out.
Amaurosis Fugax ; Aneurysm, Dissecting ; Arteries ; Brain Ischemia ; Carotid Artery, Internal* ; Carotid Artery, Internal, Dissection* ; Cranial Nerve Diseases ; Dysarthria ; Hematoma ; Humans ; Hypoglossal Nerve Diseases* ; Hypoglossal Nerve* ; Middle Aged ; Neurologic Manifestations ; Paresis ; Tongue

BACKGROUND AND PURPOSE: Oxidative stress is involved in the pathophysiological mechanisms of stroke (e.g., atherosclerosis) and brain injury after ischemic stroke. Statins, which inhibit 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, have both pleiotropic and low-density lipoprotein (LDL)-lowering properties. Recent trials have shown that high-dose statins reduce the risk of cerebrovascular events. However, there is a paucity of data regarding the changes in the oxidative stress markers in patients with atherosclerotic stroke after statin use. This study evaluated changes in oxidative stress markers after short-term use of a high-dose statin in patients with atherosclerotic stroke. METHODS: Rosuvastatin was administered at a dose of 20 mg/day to 99 patients who had suffered an atherosclerotic stroke and no prior statin use. Blood samples were collected before and 1 month after dosing, and the serum levels of four oxidative stress markers-malondialdehyde (MDA), oxidized LDL (oxLDL), protein carbonyl content (PCO), and 8-hydroxy-2'-deoxyguanosine (8-OHdG)-were evaluated to determine the oxidation of MDA and lipids, proteins, and DNA, respectively, at both of those time points. RESULTS: The baseline levels and the degrees of reduction after statin use differed among the oxidative stress markers measured. MDA and PCO levels were associated with infarct volumes on diffusion-weighted imaging (r=0.551, p<0.05, and r=0.444, p=0.05, respectively). Statin use decreased MDA and oxLDL levels (both p<0.05) but not the PCO or 8-OHdG level. While the reduction in MDA levels after statin use was not associated with changes in cholesterol, that in oxLDL levels was proportional to the reductions in cholesterol (r=0.479, p<0.01), LDL (r=0.459, p<0.01), and apolipoprotein B (r=0.444, p<0.05). CONCLUSIONS: The impact of individual oxidative stress markers differs with time after ischemic stroke, suggesting that different oxidative markers reflect different aspects of oxidative stress. In addition, short-term use of a statin exerts antioxidant effects against lipid peroxidation via lipid-lowering-dependent and -independent mechanisms, but not against protein or DNA oxidation in atherosclerotic stroke patients.
Antioxidants* ; Apolipoproteins ; Atherosclerosis ; Brain Injuries ; Cholesterol ; Coenzyme A ; DNA ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors* ; Lipid Peroxidation ; Lipoproteins ; Oxidative Stress ; Oxidoreductases ; Stroke ; Rosuvastatin Calcium

PURPOSE: To determine which optic disc topographic parameters, obtained by the Heidelberg Retina Tomograph (HRT), are most useful to distinguish normal eyes from those with early to moderate glaucomatous visual field defects. METHODS: From each of 92 normal subjects and 154 patients with primary open-angle glaucoma one randomly selected eye was studied. The Humphrey perimeter, program C 30-2 visual fields, and 13 HRT parameters (software 2.01, includes rim to disc area ratio) were utilized. Because some of the optic disc parameters depend on the optic disc area, these parameters were corrected for the effect of disc area. The total glaucoma group was divided into three subgroups according to the visual field defects: early (mean deviation [MD] >-6 dB, 65 eyes), moderate (MD -6 to -12 dB, 49 eyes), and severe (MD <-12 dB, 40 eyes) glaucoma groups. RESULTS: In eyes with early glaucoma (MD = -3.43 +/- 1.60 dB), the sensitivity, specificity, accuracy, and areas under the receiver operating characteristic curve were, respectively: rim area (69%, 71%, 70%, 0.802), rim to disc area ratio (69%, 72%, 71%, 0.799), cup area (72%, 70%, 71%, 0.789) and cup to disc area ratio (69%, 70%, 69%, 0.790). In eyes with moderate glaucoma (MD = -8.75 +/- 1.88 dB), they were, respectively: rim area (78%, 86%, 83%, 0.872), rim to disc area ratio (76%, 86%, 82%, 0.869), cup area (76%, 87%, 83%, 0.864) and cup to disc area ratio (78%, 84%, 82%, 0.862). There was considerable overlap in HRT parameters between the normal and early glaucoma groups. CONCLUSIONS: The diagnostic value of single optic disc parameter had its limitations in the diagnosis of early stage glaucoma.
Diagnosis* ; Glaucoma* ; Glaucoma, Open-Angle ; Humans ; Retina* ; ROC Curve ; Sensitivity and Specificity ; Visual Fields

The Mayer-Rokitanski-Kuster-Hauser Syndrome refers to the clinical entity consistingof primary amenorrhea associated with congenital absence of the vagina, 46XX Karyotype,a rudimentary uterus in the form of bilateral noncanaliculated muscular band or completeabsence, normal ovarian function and normal ovulation, normal breast development, bodyproportion and body hair, frequent associated of renal, skeletal and other congenitalanomalies. A case of Mayer-Rokitanski-Kuster-Hauser syndrome was presented and the literatureswere reviewed briefly.
Amenorrhea ; Breast ; Female ; Hair ; Ovulation ; Uterus ; Vagina*

Nelumbo nucifera Gaertn. (Nymphaeaceae) is commonly called lotus and its leaves are widely been used as functional ingredients due to its antioxidant activity. For maximum efficacy, optimized extraction condition was established using response surface methodology. The high F-values, low p-values and insignificant p-value for lack-of-fit supported the fitness of the model and yielded the second-order polynomial regression for the antioxidant activity. The optimized extract was obtained by the extraction of 1 g of lotus leaves with 40 mL of 50% MeOH at 10.0℃, which exerted 70.1% antioxidant activity. Close correlation between phenolic content and antioxidant activity suggested phenolic compounds as active constituents of lotus leaves. In addition, comparison of different parts of lotus demonstrated the most potent antioxidant activity of flowers, followed by leaves and roots. Taken together, these results provide useful information about lotus leaves for the development as antioxidant ingredients. In addition, flowers and roots as well as leaves are suggested as good sources for antioxidant activity.
Flowers ; Lotus ; Nelumbo ; Phenol

Objectives: This study aims to compare attitude toward suicide among the elderly with suicide attempt, suicide ideation, and without suicide ideation and attempt. Methods: We recruited study groups according to suicide risk. Suicide attempt/ideation group were recruited from Busan Regional Suicide Prevention Center. Control group was recruited from general population. Suicide attempt group was those who attempted suicide within 6 months prior to study participation. Suicide ideation group was those who had suicide ideation within 6 months prior to study participation but did not have lifetime history of suicide attempt. Control group was those who did not have either lifetime history of suicide ideation and attempt. Attitude toward suicide was evaluated by Korean version of Attitude Toward Suicide-20 (ATTS-20) Questionnaire. Results: Total of 141 elderly people aged over 60 were recruited. Four subscale scores of ATTS-20 (permissiveness and nonintervention; preventability and incomprehensibility; universality; unpredictability) were significantly lower in the suicide attempt group than suicide ideation and control group. Conclusion The results of present study suggest that the elderly who had history of suicide attempt are more permissive toward and have lack of comprehension of suicide. These results can be useful to develop effective suicide intervention and prevention strategy for the elderly.

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.
Adult ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Extracorporeal Membrane Oxygenation ; Follow-Up Studies ; Heart ; Heart Transplantation ; Hemodynamics ; Humans ; Infant* ; Oxygenators, Membrane* ; Tissue Donors

PURPOSE: This study performed a comparative evaluation of nutritional condition's improvement and clinical effects in accordance with the Nutrition Support Team (NST) consultation compliance of critically ill pediatric patients. METHODS: The medical records of 64 critically ill pediatric patients (2 to 18 years old), who were officially referred to a NST consultant in pediatric intensive care unit from January to August 2015, were reviewed. The patients were divided into 2 groups according to the compliance of NST consultation answers. The total delivered/required caloric and protein ratio, weight, serum total protein, serum albumin, hemoglobin, and hematocrit were compared. RESULTS: According to the NST consultation answer, 'nutrition support increase' occupied the largest proportion at 38.5%; 'maintenance' and 'decrease' accounted for 35.7% and 18.2% respectively. The NST compliance group and non-compliance group were 20 and 14 patients, respectively. Although total delivered/required caloric ratio was significantly increased in the NST compliance group (19.7%, P=0.036), there was no significant difference in the NST non-compliance group (5.1%, P=0.692). The total delivered/required protein ratio was increased (15.1%, P=0.163) in the NST compliance group and decreased (-4.7%, P=0.774) in the NST non-compliance group. The NST non-compliance group (-8.6%, P=0.219) was further reduced weight than the NST compliance group (-1.0%, P=0.820). The serum albumin was significantly increased in the NST compliance group (13.1%, P=0.003), but there was no difference in the NST non-compliance group (7.1%, P=0.433). CONCLUSION: Although 56.7% of NST consultations were needed for nutritional interventions, a lower NST compliance (53.8%) is the limit of nutritional support. The NST compliance group was supplied adequately with more calories and protein than before consultation and a more improved nutritional status. Therefore, aggressive NST consultation can help increase the therapeutic effect by improving the nutritional status. This study will form the basis to seek ways to further enhance NST compliance.
Compliance* ; Consultants ; Critical Illness* ; Hematocrit ; Humans ; Intensive Care Units ; Medical Records ; Nutritional Status ; Nutritional Support ; Pediatrics ; Referral and Consultation ; Serum Albumin