BACKGROUND: Trisomy 16 in mice is considered to be an animal model for Down's syndrome in human. We studied the morphologic characteristics of the heart and the edema, and their significance to the teratogenesis in this animal model. METHODS: A total of 30 dams were sacrificed to bear 125 (61.9%) normal fetuses 35 (17.3%) abnormal fetuses and 42 (20.8%) resorptions. Cytogenetic study and morphological examination were performed using microdissection agar-mount sectioning histologic examination and scanning electron microscope. RESULTS: The crown-rump length was significantly shorter in abnormal (trisomic fetuses) than the normal (eusomic) fetuses. Trisomic fetuses showed massive edema at the back from the vertex to the lumbar area. Four-chamber view section of the agar-mount and histologic section showed a common atrioventricular valve bridging the left and the right atrioventricular junction. Scanning electron microscopic examination on atrioventricular valves showed three types of atrioventricular valves:five cases with common atrioventricular orifice, three cases with partitioned atrioventricular orifice and two cases with atresia of the right atrioventricular orifice. CONCLUSION: This study confirmed the presence of massive edema and cardiac anomalies in the mouse with trisomy 16. But there was morphologic diversity of cardiac anomaly in this model.
Animals ; Crown-Rump Length ; Cytogenetics ; Down Syndrome ; Edema* ; Endocardial Cushion Defects ; Fetus* ; Heart ; Humans ; Hydrops Fetalis ; Mice ; Microdissection ; Models, Animal ; Pathology* ; Teratogenesis ; Trisomy*

Intestinal tuberculosis has been known since antiquity. After about 1950's, effective antituberculous chemotherapy and an improved standard of living resulted in a steady decline in all forms of tuberculosis. However, intestinal tuberculosis has been reported with impressive frequency in developing countries including Korea. Disease affecting the duodenum is reported to be rare and isolated duodenal tuberculosis sparing the rest of the intestine is uncommon. We experienced a case of tuberculosis of duodenal bulb associated pulmonary tuberculosis. The diagnosis was made on the basis of the histological study of the endoscopic biopsy specimen. We have confirmed the healing of the duodenal lesion by the follow-up endoscopy after 9 months treatment of antituberculous medication.
Biopsy ; Developing Countries ; Diagnosis ; Drug Therapy ; Duodenum* ; Endoscopy ; Follow-Up Studies ; Intestines ; Korea ; Socioeconomic Factors ; Tuberculosis* ; Tuberculosis, Pulmonary*

Double primary cancer means that more than two cancers with different origin exist independently in an individual. The diagnosis of double primary cancer was determined by following criteria. Each of the tumors must present a definite picture of malignancy, and each must be distinct, and the probability of one being a metastasis of the other must be excluded. Small cell carcinoma has a distinct biological behavior such as, early invasion and metastasis, a rapid clinical course, and significant sensitivity to chemotherapy. Small cell carcinoma in the esophagus is relatively rare, and rarer when it is combined with other malignant disease. We have experienced a case of double primary cancer, a 70-year-old man with esophageal small cell carcinoma and gastric adenocarcinoma, which were diagnosed by gastrointestinal endoscopic biopsy. A review of the Korean medical literature failed to reveal any previously described case of esophageal small cell carcinoma with gastric adenocareinoma. We report this case with review of literatures.
Adenocarcinoma* ; Aged ; Biopsy ; Carcinoma, Small Cell* ; Diagnosis ; Drug Therapy ; Esophagus ; Humans ; Neoplasm Metastasis

Double primary cancer is defined as the case of primary malignant tumors, which must be arise in different sites and have a different histologic appearences. The number of reported cases of double primary malignant tumors has increased in recent and the occurrence of multiple primary cancers in the same individuals is one of the interesting subjects for investigation and research in relation to the carcinogenic mechanism and/or genetic factors. We have experienced a case with double primary malignant tumors of different site origins such as stomach adenocarcinoma and rectal adenocarcinoma, which were diagnosed by biopsies with gastro and colono fiberscopy. For its great rarity, we report this case with review of literatures.
Adenocarcinoma ; Biopsy ; Rectum* ; Stomach*

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Roux- en-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.
Bile Ducts ; Birth Weight ; Cholangitis ; Choledochal Cyst* ; Dilatation ; Esophageal and Gastric Varices ; Female ; Follow-Up Studies ; Gestational Age ; Humans ; Infant, Newborn ; Male ; Parturition ; Postoperative Complications ; Prenatal Diagnosis ; Radionuclide Imaging ; Ultrasonography ; Ultrasonography, Prenatal

BACKGROUND: Benzodiazepines acting on the GABA receptor complex in the spinal cord are known to induce antinociception. In the present study we aimed to evaluate the effect of intrathecal midazolam with bupivacaine following hemorroidectomy. METHODS: Forty five patients were involved in this study. Patients were randomly allocated to one of 3 groups: the control group received 0.5% heavy bupivacaine 5 mg and 0.9% NaCl 0.2 ml intrathecally, group BM1 0.5% bupivacaine and midazolam 1 mg (0.2 ml), group BM2 0.5% bupivacaine 5 mg and midazolam 2 mg (0.4 ml), respectively. The analgesic effects and side effects were compared among the 3 groups. RESULTS: There was a significant difference among the 3 groups in time-to-first-pain medication and total consumption of analgesics during the 24 h after surgery. Furthermore, the analgesic effect of midazolam was dose-dependent: 2 mg of midazolam showed better results than 1 mg. All the patients showed no considerable side effects. CONCLUSIONS: We conclude that intrathecal midazolam caused a significant delay in time-to-first-pain medication and reduced utilization of analgesics within 24 h. Therefore, intrathecal midazolam can be used as an effective adjunct for spinal anesthesia for postoperative pain control.
Analgesics ; Anesthesia, Spinal* ; Benzodiazepines ; Bupivacaine* ; Hemorrhoidectomy* ; Humans ; Midazolam* ; Pain, Postoperative ; Receptors, GABA ; Spinal Cord

Arteriovenous malformations (AVM) are an unusual cause of gastrointestinal (GI) bleeding. But most GI AVM can occur with GI bleeding. When conventional diagnostic studies have failed to reveal the source of GI bleeding, AVM have been one of the most common causes. But the incidence of AVM presented as bleeding of a gastric submucosal tumor is very low. A case of gastric submucosal tumor was experienced and reported as an AVM. This case is herein discussed with review of corresponding literature.
Arteriovenous Malformations* ; Hemorrhage ; Incidence ; Stomach

PURPOSE: To gain insight on transcriptional repression of Topo II a in HL-60 cells arrested to G2/M and M phase, the levels of Topo IIa mRNA and the binding activity of ATF have been investigated with Northern blot hybridization and DNA mobility shift assay, respectively. MATERIALS AND METHODS: HL-60 cells were grown in RPMI 1640 medium supplemented with 10% heat-mactivated fetal bovine serum and antibiotics in a humidified 5% CO2 at 37C degree. Total RNA was prepared by a modification of the method of Karlinsey et al. Northern blot hybridization was performed by the method of Virca et al. A Xho I-Mlu I fragment of phTOP2 was used as probe for Northern blot analysis of Topo II a mRNA. DNA mobility shift assay was performed by the method of Lim et al. End labeled DNA oligomer (upper strand, 5-TCTCCGCTATGACGCCGAGTGGTG-3) for ATF binding activity was mixed with nuclear extracts in a 20 pl reaction volume containing 60 mM KC1, 12 mM HEPES, pH 7.9, 5 mM MgCl2, 0.2 mM EDTA, 0.2 mM DTT, 12% glycerol, and 2 ug of poly [dI-dC]. RESULTS: HL-60 cells were arrested at G2/M phase and M phase after taxol or nocodazole treatment. The levels of Topo II a mRNA were reduced at 24 hours after exposure with nocodazole or taxol but the unknotting activities were not changed. DNA mobility shift assay using oligonucleotide containing the ATF binding site showed that ATF binding activity was reduced after pretreatment of nododazole or taxol. CONCLUSIONS: These results suggest that the reduction of ATF binding activity may be important to transcriptional repression of Topo II a gene by nocodazole and taxol in HL- 60 cells.
Anti-Bacterial Agents ; Binding Sites ; Blotting, Northern ; Cell Division* ; DNA Topoisomerases, Type I* ; DNA Topoisomerases, Type II* ; DNA* ; Edetic Acid ; Electrophoretic Mobility Shift Assay ; Genes, vif ; Glycerol ; HEPES ; HL-60 Cells ; Humans ; Hydrogen-Ion Concentration ; Magnesium Chloride ; Nocodazole ; Paclitaxel ; Repression, Psychology ; RNA ; RNA, Messenger

Campomelic syndrome is a very rare skeletal dysplasia with a characteristic pattern of deformity involving the proximal and distal extremities, pelvic and shoulder girdles, thoracic cage and palate. Respiratory compromise often leads to death in early infancy. Etiology has not been determined although evidence suggests genetic heterogeneity. Cytogenetic study revealed high incidence of a 46,XY karyotype in phenotypic females. Recently, we had experienced a case of campomelic dysplasia at amenorrhea 30weeks and termination was done, so we report with a brief review of literature.
Amenorrhea ; Campomelic Dysplasia* ; Congenital Abnormalities ; Cytogenetics ; Extremities ; Female ; Genetic Heterogeneity ; Humans ; Incidence ; Karyotype ; Palate ; Shoulder