The pathogenesis of bladder and cloacal exstrophy is an abnormal development of the cloacal membrane. The incidence of each is one in l0,000-50,000 and 200,000-400,000 live births. Male is more commonly affected than female. We experienced two cases of bladder exstrophy and one cloacal. First case was one-day-old girl who was referred to our department because of urine leakage through defected abdominal wall. We performed primary bladder closure with paraexstrophy skin flap urethroplasty, approximation and traction of pubis without osteotomy and application of hip-spica cast. The second was seven-day-old boy visited our department because of urine and stool leakage. We performed primary bladder closure, bilateral orchiectomy and ileostomy. He recurred bladder exstrophy due to infection of operation site. The third was twenty-day-old boy visited our department because of urine leakage. We recommended admission and treatment, but his parents refused further evaluation.
Abdominal Wall ; Bladder Exstrophy* ; Cloaca ; Female ; Humans ; Ileostomy ; Incidence ; Live Birth ; Male ; Membranes ; Orchiectomy ; Osteotomy ; Parents ; Skin ; Traction ; Urinary Bladder*

An intussusception in adulthood is an unusual cause of bowel obstruction. It accounts for up to 5% of all intussusception. Approximately 90% of cases are secondary to a definite lesion such as malignancy or lipoma. Most patients are asymptomatic and the lesion is often detected incidentally at colonoscopy, operation and autopsy. Strangulated intussuscetion is a rare case and also requires emergency operation. A 32-year-old woman visited our emergency room because of severe epigastric pain. Abdominal CT revealed a low density mass in bowel loop and distended small bowel loops filled with fluid. Colonoscopic finding showed huge purple-colored coil-spring lesion in the ascending colon. From this findings, we diagnosed a strangulated intussusception. Surgically removed specimen revealed a small intestinal lipoma.
Adult ; Male ; Female ; Humans

Most of treatments have been performed laparotomy, but the rapid advances in laparoscopic operative and laboratory techniques and instrument allowed to apply laparoscopic surgery to this disease. We have reviewed 342 cases of ectopic pregnancy from march 1994 to march 2001.The cornual pregnancies were 21 case (6.1%), and the laparoscopic management in 11 cases. The mean gestational age at the time of laparoscopic surgery was 9.1 1.2 (n=11) weeks, and laparotomy was 9.4 1.3 (n=10) weeks. There were no significant difference. The mean operation time of laparoscopic surgery was 72.8 25.5minutes, and laparotomy was 68.3 24.3minutes. The mean hospital days of laparoscopic surgery were 4.9 2.4days, and laparotomy were 6.6 2.3days. In conclusion, laparoscopic surgery can be used as effective and safe as laparotomy for cornual pregnancy.
Female ; Gestational Age ; Laparoscopy* ; Laparotomy* ; Pregnancy* ; Pregnancy, Ectopic

Transvenous implantation of an implantable cardioverter defibrillator in patients with a prosthetic valve in the tricuspid position is difficult because a defibrillator lead cannot be implanted into the right ventricle through the prosthetic valve. Hence, there are three options: epicardial implantation, subcutaneous implantable cardioverter defibrillator implantation, and cardiac vein implantation. Here, we report the transvenous implantation of an implantable dual-chamber cardioverter defibrillator in a patient who had undergone tricuspid valve replacement with a prosthetic valve. The patient was a 70-year-old female with a prosthetic valve in the tricuspid position who had experienced two events of sudden cardiac arrest. We successfully performed the procedure by implanting the defibrillator lead into the middle cardiac vein.
Aged ; Death, Sudden, Cardiac ; Defibrillators* ; Defibrillators, Implantable ; Female ; Heart Ventricles ; Humans ; Tricuspid Valve* ; Veins

Familial Parkinson's disease (PD) has been linked to point mutations and duplication of the α-synuclein (α-syn) gene. Mutant α-syn expression increases the vulnerability of neurons to exogenous insults. In this study, we developed a new PD model in the transgenic mice expressing mutant hemizygous (hemi) or homozygous (homo) A53T α-synuclein (α-syn Tg) and their wildtype (WT) littermates by treatment with sub-toxic (10 mg/kg, i.p., daily for 5 days) or toxic (30 mg/kg, i.p., daily for 5 days) dose of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). Tyrosine hydroxylase and Bcl-2 levels were reduced in the α-syn Tg but not WT mice by sub-toxic MPTP injection. In the adhesive removal test, time to remove paper was significantly increased only in the homo α-syn Tg mice. In the challenging beam test, the hemi and homo α-syn Tg mice spent significantly longer time to traverse as compared to that of WT group. In order to find out responsible proteins related with vulnerability of mutant α-syn expressed neurons, DJ-1 and ubiquitin enzyme expressions were examined. In the SN, DJ-1 and ubiquitin conjugating enzyme, UBE2N, levels were significantly decreased in the α-syn Tg mice. Moreover, A53T α-syn overexpression decreased DJ-1 expression in SH-SY5Y cells. These findings suggest that the vulnerability to oxidative injury such as MPTP of A53T α-syn mice can be explained by downregulation of DJ-1.
1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine ; Adhesives ; Animals ; Apoptosis ; Dopamine* ; Dopaminergic Neurons* ; Down-Regulation* ; Hominidae ; Humans ; Mice* ; Mice, Transgenic ; Neurons ; Parkinson Disease ; Point Mutation ; Synucleins ; Tyrosine 3-Monooxygenase ; Ubiquitin

OBJECTIVE: To determine the frequency of fetal anomaly and perinatal outcome in pregnant women with polyhydramnios. METHODS: Ultrasound examinations from January 1998 to August 2001 were reviewed to identify 170 patients with singleton pregnancy which diagnosed polyhydramnios. Frequency of fetal anomaly and perinatal outcomes were compared with amniotic fluid index (AFI) 20.0-25.0 cm as group 1 and AFI >25.0 cm as group 2. RESULTS: The prevalence of polyhydramnios was 0.54% (170/31,358). The types of structural anomalies were gastrointestinal system (10/34, 29.4%), genitourinary system (9/34, 26.5%), central nervous system (CNS, 6/34, 17.7%), cardiovascular system (CVS, 3/34, 8.8%), neuromuscular system (2/34, 5.9%), respiratory system (1/34, 2.9%) and others (3/34, 8.8%). The structural anomaies between two groups were significantly different. The rate of cesarean section, low 5-min Apgar score (<7), large for gestational age (LGL, 95 percentile), Nursery Intensive Care Unit (NICU) visitation and perinatal death were not significantly different between two groups. CONCLUSION: The prevalence of fetal anomaly was 20% (34/170) and significantly different between two groups (p=0.0143). However, the perinatal outcome was not significantly different between two groups. CNS, gastrointestinal system and CVS anomalies were more frequent in group 2 (AFI>25.0 cm) and genitourinary system anomaly was dominant in group 1 (AFI 20.0-25.0 cm). Polyhydramnios (AFI>25.0 cm) indicated an increased risk of severe fetal anomaly.
Amniotic Fluid ; Apgar Score ; Cardiovascular System ; Central Nervous System ; Cesarean Section ; Female ; Gestational Age ; Humans ; Intensive Care Units ; Nurseries ; Polyhydramnios* ; Pregnancy ; Pregnant Women ; Prevalence ; Respiratory System ; Ultrasonography ; Urogenital System

BACKGROUND/AIMS: CD11c is a dendritic cell marker in humans, which potentially induces a cytotoxic effect on lymphoma cells. Forkhead boxP3 (FOXP3) is a regulator of T lymphocyte in the microenvironment of the lymphoma. The principal objective of this study was to determine whether the tumors' microenvironment expressions of CD11c and FOXP3 are predictive of clinical outcomes in diffuse large B-cell lymphoma (DLBCL) patients receiving treatment with rituximab, cyclophosphamide, anthracycline, vincristine, and prednisone (R-CHOP) combination chemotherapy. METHODS: The study population consisted of 100 patients with DLBCL. The CD11c and FOXP3 expression in primary tumors' microenvironment were evaluated using an immunohistochemistry (IHC). RESULTS: CD11c and FOXP3 expression positivity in microenvironment were 25% and 35%, respectively. Each one counted for 1 point. In CD11c and FOXP3 stain, positive was counted as 0 and negative was 1. The points were separated into low risk (0 to 1) and high risk (2) groups. Only the extranodal DLBCL patient group analysis conveyed significant differences of progression-free survival (p = 0.019) and overall survival (p = 0.039) between the two groups. CONCLUSIONS: We can achieve possible clinical significance of lymphoma tumor microenvironments through CD11c and FOXP3 IHC stains in extranodal DLBCL patients receiving R-CHOP therapy.
B-Lymphocytes* ; Coloring Agents ; Cyclophosphamide* ; Dendritic Cells ; Disease-Free Survival ; Drug Therapy, Combination* ; Humans ; Immunohistochemistry ; Lymphocytes ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, Large B-Cell, Diffuse ; Prednisone* ; Rituximab* ; Tumor Microenvironment* ; Vincristine*