Epispadias is an extremely rare malformation that urethral opening is located on the dorsum of the penis. Depending upon its location epispadias is classified as balanic, penile, or penopubic, the latter being by far the most common condition. Most patients of epispadias will have severe urinary incontinence and vesicoureteral reflux. The objects of management in epispadias are to achieve continence, to facilitate normal sexual activity, and to preserve renal function. Recently we have experienced two cases of epispadias without incontinence.
Epispadias* ; Humans ; Male ; Penis ; Sexual Behavior ; Urinary Incontinence ; Vesico-Ureteral Reflux

No abstract available.
Female ; Pregnancy ; Pregnancy Outcome* ; Pregnancy*

It has been suggested that green juice supplementation may have some health promoting benefits. We evaluated the effects of green juice (Angelica keiskei) consumption on parameters of lipid profiles and plasma antioxidant status in healthy male smokers. Fifty-four smokers were supplemented with 300 ml of green juice for 6 weeks while maintaining their normal diet. Blood samples were collected on week 0 and week 6 in order to evaluate plasma lipid profiles (total cholesterol, triglyceride, LDL-cholesterol, HDL-cholesterol) , plasma antioxidant vitamin levels (ascorbic acid, alpha-tocopherol, gamma-tocopherol, alpha-carotene, beta-carotene, cryptoxanthin and lycopene) , the degree of LDL oxidation and GOT, GPT levels for liver function. Plasma ascorbic acid level remained at the same level. However, alpha-tocopherol and gamma-tocopherol normalized by total cholesterol (p<0.05) and beta-carotene (p<0.001) level were all significantly increased after green juice supplementation. Plasma cholesterol was reduced for 12%, LDL-cholesterol was reduced for 9.3% after green juice consumption, while plasma triglyceride and HDL-cholesterol was not changed. Oxidized LDL assessed by conjugated diene (CD) , was decreased (p<0.0001) after green juice consumption. These results further support a role for green juice supplementation in the improvement of lipid status, prevention of lipid peroxidation, and thereby reducing risk factors of numerous diseases associated with elevated oxidative stress in smokers.
alpha-Tocopherol ; Ascorbic Acid ; beta Carotene ; Cholesterol ; Diet ; gamma-Tocopherol ; Humans ; Lipid Peroxidation ; Liver ; Male ; Oxidative Stress ; Plasma* ; Risk Factors ; Triglycerides ; Vegetables* ; Vitamins

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by presence of α-synuclein-positive inclusions in the cytoplasm of oligodendrocytes. These glial cytoplasmic inclusions (GCIs) are considered an integral part of the pathogenesis of MSA, leading to demyelination and neuronal demise. What is most puzzling in the research fields of GCIs is the origin of α-synuclein aggregates in GCIs, since adult oligodendrocytes do not express high levels of α-synuclein. The most recent leading hypothesis is that GCIs form via transfer and accumulation of α-synuclein from neurons to oligodendrocytes. However, studies regarding this subject are limited due to the absence of proper human cell models, to demonstrate the entry and accumulation of neuronal α-synuclein in human oligodendrocytes. Here, we generated mature human oligodendrocytes that can take up neuronderived α-synuclein and form GCI-like inclusions. Mature human oligodendrocytes are derived from neural stem cells via “oligosphere” formation and then into oligodendrocytes, treating the cells with the proper differentiation factors at each step. In the final cell preparations, oligodendrocytes consist of the majority population, while some astrocytes and unidentified stem cell-like cells were present as well. When these cells were exposed to α-synuclein proteins secreted from neuron-like human neuroblastoma cells, oligodendrocytes developed perinuclear inclusion bodies with α-synuclein immunoreactivity, resembling GCIs, while the stem cell-like cells showed α-synuclein-positive, scattered puncta in the cytoplasm. In conclusion, we have established a human oligodendrocyte model for the study of GCI formation, and the characterization and use of this model might pave the way for understanding the pathogenesis of MSA.

In one-stage transanal endorectal pull-through operation (TERPT) for Hirschsprung disease, preoperative evaluation by contrast enema (CE) is important tool in aspect of planning of surgical procedure as well as diagnosis. This study was to evaluate the significance of CE for identifying the extent of aganglionic bowel. A retrospective analysis was performed in 40 patients who underwent TERPT between 2003 and 2011. The authors reviewed the CE studies and their correlation with pathologic extent of aganglionosis. Total 66 contrast enemas were performed in 40 patients. Twenty patients underwent single CE, but 20 patients required multiple CEs. In single CE group, 17 had clear radiographic transition zone, but 3 had less definite transition zone. In multiple CE group, 17 patients who had equivocal finding in first or second CE had definite radiographic transition zone, but 3 patients of this group had less definite radiographic transition zones. Overall, 34 patients (85%) had clear radiographic transition zone by single or repeated CE. One (2.9%) out of 34 patients with clear radiographic transition zone had discordance between radiographic and pathologic transition zone. In contrast 4 (66.7%) out of 6 patients with equivocal radiographic transition zone had discordance between radiographic and pathologic transition zone. Observation of clear radiographic transition zone is important in preparation of TERPT, and repeated CE is helpful to reduce the discordance between radiographic and pathologic transition zone. Awareness of the possibility of discordance is also important if radiographic transitional zone is not clear.
Enema ; Hirschsprung Disease ; Humans ; Retrospective Studies

PURPOSES: To investigate the viral etiology of community-acquired pneumonia in Korean adults, we have detected respiratory viruses (Respiatory syncytial virus, adenovirus, influenza virus and parainfluenza virus) in the way of prospective, multi-center study. METHODS: From July 1997 to April 2000, nasal aspirates or sputum were obtained from adults patients with community pneumonia admitted to the participating hospitals and transferred immediately to the central laboratory in the Seoul National University Children's Hospital. The specimens were divided into three parts. One part was used for indirect immunofluorescent test for respiratory viruses, the other part for the culture of RSV and adenovirus in HEp-2 cell monolayer. Another part was used for the culture of influenza virus and parainfluenza virus in MDCK or LLC- MK2 cell monolayers. RESULTS: Of 317 samples, 32 (10.1%) specimens were positive for viral isolation by indirect IF staining or culture, including one dual-infected specimen (adenovirus and parainfluenza virus). Influenza virus was most commonly detected (16 specimens). Parainfluenza virus, adenovirus and RSV were detected in 10, 4 and 3 patients, respectively. All isolated influenza viruses were type A (H3N2 in 9 patients, H1N1 in 2 and unspecified in 5), and 8 out of 10 parainfluenza virus isolates were type 3. CONCLUSION: Similar to previous foreign reports, a significant portion of community-acquired pneumonia in Korean adult is caused by respiratory viruses. Our data empathized the need of referral system for viral diagnosis and of nationwide investigation on respiratory virus infections.
Adenoviridae ; Adult* ; Diagnosis ; Humans ; Orthomyxoviridae ; Paramyxoviridae Infections ; Pneumonia* ; Prospective Studies ; Referral and Consultation ; Seoul ; Sputum

PURPOSE: Most of grafts used as interposition conduits for middle hepatic vein (MHV) in living donor liver transplantation (LDLT) have been allografts and autografts. Recently, polytetrafluoroethylene (PTFE) and bovine pericardium patch have also been used. Thus, we performed large-animal lab tests to assess the feasibility of interposition vessel graft substitutes for MHV. METHODS: The inferior vena cava was replaced in 9 dogs with allograft (3), PTFE (3), and bovine pericardium patch (3). After 28 days, patency rate, outer and inner diameter, intimal thickness, histology, and immunohistochemistry were evaluated according to interposition grafts. RESULTS: The allograft and PTFE groups were all patent at post-operative week 4, but the bovine group was not patent in all dogs. Outer diameter of anastomotic site at 4 weeks was 8.41+/-0.37, 10.83+/-0.51, and 7.41+/-0.86 mm in allograft, PTFE, and bovine group, respectively. Inner diameter of interposition graft at 4 weeks was 7.90+/-0.23, 6.33+/-0.68, and 0 mm in allograft, PTFE, and bovine groups, respectively. Intimal thickness was 48.0+/-8.6, 113.8+/-45.3, and 218.3+/-59.9microm in allograft, PTFE, and bovine groups, respectively. In histologic findings, inflammation was most severe in the bovine group. Intima of anastomotic site in the bovine group was thickest in all groups. Proliferation of smooth muscle cells was most severe in anti-alpha-actin antibody test in bovine group. CONCLUSION: Our data implicate that the use of allografts and PTFE grafts is more feasible than bovine pericardium for MHV reconstruction in LDLT.
Animals ; Dogs ; Glycosaminoglycans ; Hepatic Veins ; Humans ; Immunohistochemistry ; Inflammation ; Liver ; Liver Transplantation ; Living Donors ; Myocytes, Smooth Muscle ; Pericardium ; Polytetrafluoroethylene ; Pyridines ; Thiazoles ; Transplantation, Homologous ; Transplants ; Vena Cava, Inferior

PURPOSE: Primary central nervous system lymphoma (PCNSL) is defined as lymphoma limited to the cranial-spinal axis without evidence of systemic disease and its incidence has risen threefold during the last fifteen years among apparantly healthy population. This study was intended to analyze the clinicopathologic features and treatment outcome of the patient with PCNSL. MATERIALS AND METHODS: Twenty one patients were diagnosed and treated for the PCNSL limited to brain parenchyme at Asan Medical Center between March 1989 and December 1996. We reviewed clinical records of these patients and analyzed clinicopathologic features, treatment response, survival time and prognostic factors. RESULTS: The ratio of male to female was 1.3: 1 and the most prevalent age group was the 4th decade. Most patients had diffuse large cell (19/21) and B-cell type (8/8). Seventeen (94.4%) among 18 evaluable patients achieved complete remission (CR) as initial response, but 53% of patients showed recurence of the disease. Median times of disease-free and overall survival were 40 and 50 months, respectively and 5 year overall survival rate was 35.3 %. Prognostic factors such as age and performance status, had a statistically significant influence on the overall survival but not on disease-free survival. CONCLUSION: CR rate of the patients with PCNSL was high, but relapses were frequent. There fore further studies are needed to define the pmgnostic factors and to decrease relapse rate.
Axis, Cervical Vertebra ; B-Lymphocytes ; Brain ; Central Nervous System* ; Chungcheongnam-do ; Disease-Free Survival ; Female ; Humans ; Incidence ; Lymphoma* ; Male ; Recurrence ; Survival Rate ; Treatment Outcome

BACKGROUND: Congenital adrenal hyperplasia (CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. It appears that the frequency and the type of the responsible mutations differ according to the ethnic background and the type of mutation can predict the clinical outcomes such as salt losing type (SL), simple virilizing type (SV) and non-classic type (NC). METHODS: We have analyzed CYP21 genes in 55 Korean cases (110 chromosomes) of CAH by Southern blotting, PCR-dot hybridization and PCR amplification-created restriction site method. The patients include 43 cases of SL and 12 of SV. None of the NC was found. RESULTS: We found the mutations in 94% (103/110) of the examined chromosomes. A total of 10 types of mutations were discovered. The mutations include aberrant splicing of intron 2 (i2, 35%), CYP21 gene deletion (32%) and I172N (11%) in order. When the relationship between the clinical types and genotypes were correlated, most of the SL patients have either i2 (42%) or CYP21 gene deletion (41%), while SV patients have I172N (33%) or P30L (21%). The parents' mutation was investigated in 20 cases. In 4 families, one of the parents was not the obligatory heterozygote carrier i.e. did not have a mutation. The results suggest the high incidence of de novo mutation. CONCLUSION: We have identified the frequency of mutations of the CYP21 in Korean AGS patients. Our results shows that the clinical type of AGS can be predicted from the genotypes of CYP21. Also the high incidence of de novo mutation of CYP21 confirmed the genetic instability of major histocompatibility III region where the CYP21 is located.
Adrenal Hyperplasia, Congenital ; Adrenogenital Syndrome* ; Blotting, Southern ; Cholesterol ; Gene Deletion ; Genotype* ; Heterozygote ; Histocompatibility ; Humans ; Hydrocortisone ; Incidence ; Introns ; Parents ; Phenotype* ; Polymerase Chain Reaction ; Steroid 21-Hydroxylase*

PURPOSE: The effect of intra-portal infusion of glucose-insulin-potassium (GIK) solution on the energy metabolism during cold preservation was investigated using a small-animal liver transplantation model. METHODS: Fifteen white rats were divided into 3 groups: the group A (feeding group) were fed normally before experiment. The group B (fasting group) and group C (GIK group) were fasted from 3 days before experiment, by which acute nutritional deficiency state was induced. In group A and B, the whole liver was procured after intra-portal perfusion of HTK solution and serial liver biopsies were performed during the cold preservation period with 4degrees C HTK solution. In group C, intra-portal GIK solution infusion for 1 hour preceded liver graft harvest. From the liver tissues, the relative intracellular glycogen contents and the ATP concentration were measured. RESULTS: Relative glycogen contents in group A were 100% at 0 h, 64.6% at 2 h, 54.9% at 4 h, and 16.2% at 8 h; 10.3%, 8.3%, 4.9% and 0%, respectively in group B; 109.2%, 96.9%, 54.2% and 9.7%, respectively in group C. There was a temporary supercharge of ATP level in group C only at 0 h. Apoptosis was less expressed in group C comparing with group A and B. CONCLUSION: Rapid intra- portal infusion of GIK solution could make intrahepatic glycogen content fully restored to the normal level. Considering that intracellular glycogen is the main energy source during immediate post-transplant period, its restoration may contribute to improvement of post-transplant graft function.
Adenosine Triphosphate ; Animals ; Apoptosis ; Biopsy ; Cold Temperature ; Energy Metabolism ; Glucose ; Glycogen ; Humans ; Insulin ; Liver ; Liver Transplantation ; Malnutrition ; Mannitol ; Perfusion ; Potassium ; Potassium Chloride ; Procaine ; Rats ; Transplants