Congenital Microgastria: Treatment with a Hunt- Lawrence Pouch

Congenital microgastria results in growth retardation early in a patient’s life due to poor oral intake, malnutrition and failure to gain weight. An one-month-old female was admitted with a history of poor oral intake, vomiting and recurrent pneumonia after birth. She had an extremely small, tubular stomach in the midline of the abdomen, a megaesophagus and severe gastroesophageal reflux (Reflux Index: 41.1%, DeMeester score: 152.2). A Hunt- Lawrence Pouch (Roux-en-Y double barrel jejunal food pouch formation) procedure was performed for augmenting the small stomach after failure of conservative management such as small, frequent feedings and prokinetics. The postoperative UGI and esophageal pH study showed marked improvement of the GER (13.7%, 68.9 respectively). Although her preoperative symptoms have subsided, her weight is still below normal (third percentile). Creating a Hunt-Lawrence pouch is the treatment of choice for congenital microgastria that is refractory to conservative management.ope

Sudden Death Caused by Enterocolitis after Duhamel's Operation for Hirschsprung's Disease

Hirschsprung's disease is a common functional obstructive disease in infants, and a lot of knowledge and experience have been accumulated about this disease. But the exact genesis of Hirschsprung's disease is still unknown, as well as there is a lack of knowledge about the pre- or postoperative complications. Enterocolitis is a common and serious complication that can cause death. The authors experienced sudden death with three cases that were treated with Duhamel's operation under the confirmed diagnosis of the Hirschsprung's disease. To prevent such sudden and unexpected death, we report here on some of the medical procedure that we performed for these 3 patients.ope

The role of thioredoxin 1 in the mycophenolic acid-induced apoptosis of insulin-producing cells

Mycophenolic acid (MPA) is one of many effective immunosuppressive drugs. However, MPA can induce cellular toxicity and impair cellular function in β-cells. To explore the effects of MPA and the relation between MPA and Trx-1, we used various methods, including an Illumina microarray, to identify the genes regulated during pancreatic β-cell death following MPA treatment. INS-1E cells (a pancreatic β-cell line) and isolated rat islets were treated with MPA for 12, 24, or 36 h, and subsequent microarray analysis showed that (Trx1) gene expression was significantly reduced by MPA. Further, Trx1 overexpression increased the cell viability, decreased the activations of c-jun N-terminal kinase (JNK) and caspase-3 by MPA, and attenuated ROS upregulation by MPA. Furthermore, siRNA knockdown of Trx1 increased MPA-induced cell death and the activations of p-JNK and caspase-3, and MPA significantly provoked the apoptosis of insulin-secreting cells via Trx1 downregulation. Our findings suggest that the prevention of Trx1 downregulation in response to MPA is critical for successful islet transplantation.ope

One-Stage Operation for Cantrell's Pentalogy

Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operationope

한국인에서의 TNF-alpha 유전자 다형성과 HLATNF-alpha 일배체형의 분포

학위논문(석사)--서울대학교 대학원 :의학과 임상병리학전공,2002.Maste

Prenatal diagnostic clue for fetus in fetu

The purpose of this paper is to define a prenatal diagnostic clue for fetus in fetu (FIF) based on the characteristic findings of four FIF cases studied by the authors. A retrospective analyses of prenatal ultrasonography (US), postnatal US, computed tomography (CT) scans and operative findings were carried out on each of the four FIF cases collected from a multi-center. Prenatal US findings for each of the four cases showed a fluid-filled sac with a solid portion “floating” within it. In addition, bony structures were found in the solid portion. In each of the four cases, the postpartum imaging studies were consistent with the prenatal US findings. Operative findings revealed a solid mass within a fluid-filled sac surrounded by a transparent membrane. The solid mass was connected to the membrane by a stalk. Histopathologically, the cells making up the sac were of the same type as those of the amnion, while the solid mass had the general characteristics of FIF. In conclusion, FIF can be suspected when prenatal US shows a solid mass with bony structures within a fluid-filled sac in a newborn.ope