• Clinical and Experimental Pediatrics
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• v.58 no.10
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.146-151
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• 1999

Neurocristopathy originates from aberrant development of the neural crest by genetic abnormality. Dysgenetic or neoplastic neurocristopathy mayor may not combine at one or more organs. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by the respiratory depression during sleep, although showing normal ventilation while awake, because the baby does not responde to hypercapnea or hypoxia. One newborn girl, full-term, 3,020 g of birth weight with neurocristopathy is reported. It showed poor respiration at birth, and temporary Improvement with oxygen and respiratory stimulations. Abdomen was distended. Abdominal x-ray revealed small bowel obstruction and calcified opacity at the right lower quadrant. Because transitional zone was noticed at the distal jejunum during laparotomy, jejunostomy was performed. Several times trial of extubation have failed becaused of the repeated apneas. Brain sonography and echocardiogram were normal. The patient died of sepsis at 37 days of age. Para-aortic ganglioneuroblastoma was found at autopsy. In this case, congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma are combined as a neurocristopathy.

• Advances in pediatric surgery
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• v.7 no.2
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• pp.118-125
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• 2001

The effectiveness of operative and non-operative management for postoperative adhesive ileus in children has been discussed. This study reviews the clinical characteristics and the treatment consequences of adhesive ileus in our institution. Department of Surgery of Chunbuk National University Hospital, retrospectively. A total of 62 cases of post-operative small bowel obstruction treated between January 1975 and December 1998 under the 15 years of age are included in this study. The patients were divided into two groups, operative(n=26) and non-operative(n=36) groups. The prevalent age was between 11 and 15 years(28 cases; 45.2 %), and the most common previous operation was appendectomy(28 cases; 45.2 %). The most common operative procedures were adhesiolysis(17 cases; 65.4 %). The interval between admission and operation was 1 day in 11 cases(42.3 %). The most common site of adhesion was the ileum in 13 cases(50.0 %) and band constriction was the most frequent pattern(8 cases; 30.8 %). Intestinal resection was significantly high in delayed operations of more than four days, in the patients with three or more classical signs of strangulation(fever, tachycardia, leukocytosis, abdominal pain, rebound tenderness), and in the cases of complete obstruction on plain abdomen film(p < 0.05). In conclusion, operation should be considered in cases with three or more signs of strangulation, no clinical improvement for over four days of conservative treatment, and signs of complete obstruction on plain abdomen film during the observation periods.

• Advances in pediatric surgery
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• v.10 no.2
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• pp.92-98
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• 2004

The history of esophageal replacement in infants or children is the history of development of various kinds of alternative conduits such as stomach, colon, and small bowel. The gastric tube has been the most widely used conduit. From January 1988 to May 2003, 23 esophageal replacements with gastric tube were performed at the Department of Pediatric Surgery, Seoul National University Childrens Hospital. Statistical analysis was performed using Windows SPSS11.0 Pearson exact test. There were Gross type A(n=10), type B(n=1), type C(n=11), type D(n=1). Ten patients who had long gap esophageal atresia (type A-8, type B-1, type C-1) and 13 patients (type A 2, type C-10, type D-1) who had stenosis, leakage, recurred tracheoesophageal fistula, and esophagocutaneous fistula after previous corrective operations, had esophageal replacement with gastric tube. Mean follow-up periods were 4 year 2 months (7 months-15 year 1 month). There were postoperative complications including GERD in 16 (69.6 %), leakages in 7 (30.4 %), diverticulum at anastomosis in 2 (8.7 %), anastomosis site stenosis in 4 (17.3 %), and distal stenosis of the gastric tube in 1 (4.3 %). There was no statistical significance between operation types and postoperative leakage and gastroesophageal reflux. In conclusion, esophageal replacement with gastric tube may be a useful surgical option in esophageal atresia with long gap and esophageal atresia complicated by previous corrective operation.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.89-92
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• 2010

Perianastomotic ulceration (PAU) rarely occurs after small bowel resection in infancy. Since the understanding of its pathogenesis is incomplete, an effective method of treatment has not yet been discovered. We report the first case in Korea of a 10-year-old girl with chronic iron deficiency anemia (IDA) and growth failure who was diagnosed with PAU at colonoscopy. Seven years were required to identify the cause of IDA. After surgical resection and revision of anastomosis, a close follow-up is being conducted due to the risk of recurrence. Here, we also review reports on 25 pediatric patients with PAU derived from a search of the English-language literature and describe the clinical features of PAU along with the results of treatment.

• Archives of Pharmacal Research
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• v.26 no.4
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• pp.258-263
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• 2003

Colon-specific delivery of glucocorticoids is highly desirable for the efficient treatment of inflammatory bowel disease. We synthesized prednisolone 21-sulfate sodium (PDS) as a colon-specific prodrug of prednisolone (PD) and investigated its properties using rats as test animals. We expected that introduction of sulfate ester as a sodium salt might increase the hydrophilicity and restrict the absorption in the GI tract. If PDS is stable and nonabsorbable in the upper intestine, it will be delivered to the colon as an intact form, where it hydrolyze by the sulfatase to release PD. Compared with PD, the solubility of PDS increased and the apparent partition coefficient decreased greatly. PDS was stable on incubation with pH 1.2 and 6.8 buffer solutions and with the contents of the stomach and small intestine. On incubation with the cecal contents, PDS decreased to 9.6％ of the dose in 10 h producing PD. The amount of PD increased to give a maximum 54％ of the dose and decreased. As a control, when PD was incubated with the cecal contents, it decreased to 29％ of the dose in 8 h, which implied that reduction of PD proceeded under such conditions. These results suggested that hydrolysis of PDS took place to produce and accumulate PD, which decreased by reduction as the incubation period extended. Our results suggested that PDS can be a promising colon-specific prodrug of PD, and sulfate ester group might serve as a potential colon-specific promoiety, especially for the drugs which are resistant to reduction in the colon.

• Archives of Pharmacal Research
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• v.26 no.4
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• pp.264-269
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• 2003

5-Aminosalicylic acid (5-ASA) is an active ingredient of therapeutic agents used for Crohn s disease and ulcerative colitis. Because it is absorbed rapidly and extensively in the upper intestine, delivery of the agent specifically to the colon is necessary. We selected taurine as a colon-specific promoiety and designed 5-aminosalicyltaurine (5-ASA-Tau) as a new colon-specific prodrug of 5-aminosalicylic acid (5-ASA). It was expected that introduction of taurine would restrict the absorption of the prodrug and show additive effect to the anti-inflammatory action of 5-ASA after hydrolysis. 5-ASA-Tau was prepared in good yield by a simple synthetic route. The apparent partition coefficient of 5-ASA-Tau in 1-octanol/pH 6.8 phosphate buffer or $CHCl_3$/pH 6.8 phosphate buffer was 0.10 or 0.18, respectively, at $37^{\circ}C$. To determine the chemical and biochemical stability in the upper intestinal environment, 5-ASA-Tau was incubated in pH 1.2 and 6.8 buffer solutions, and with the homogenates of tissue and contents of stomach or small intestine of rats at $37^{\circ}C$. 5-ASA was not detected from any of the incubation medium with no change in the concentration of 5-ASA-Tau. On incubation of 5-ASA-Tau with the cecal and colonic contents of rats, the fraction of the dose released as 5-ASA was 45% and 20%, respectively, in 8 h. Considering low partition coefficient and stability in the upper intestine, 5-ASA-Tau might be nonabsorbable and stable in the upper intestine. After oral administration, it would be delivered to the colon in intact form and release 5-ASA and taurine. These results suggested 5-ASA-Tau as a promising colon-specific prodrug of 5-ASA.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.222-225
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• 2005

Meckel's diverticulum is generally acknowledged to be the most prevalent congenital anomaly of the gastrointestinal tract. The preoperative diagnosis of Meckel's diverticulum is difficult, especially in neonates, because of the lesions ability to masquerade as one of a variety of much more common abdominal pathologies. Recently we experienced a case of perforated Meckel's diverticulum with mild inflammatory reaction, intestinal adhesion, and small bowel obstruction in a previous healthy 9-day-old neonate. The spontaneous perforation of Meckel's diverticulum in neonate is very rare but serious entity. The course was rapid and progressed to sepsis. So despite its varied presentation, Meckel's diverticulum should be kept in mind as a cause of acute abdomen in neonates.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.208-214
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• 2003

Juvenile polyposis is an uncommon condition characterized by the development of multiple juvenile polyps predominantly in the colon but also in the rest of the gastrointestinal tract. Patients with juvenile polyposis commonly present with rectal bleeding, diarrhea, abdominal pain, anemia, prolapse of the polyp. We experienced a juvenile polyposis in a 7 year-old male patient with protein losing enteropathy who was diagnosed by $^{99M}Tc$-human serum albumin abdominal scintigraphy, colonoscopy, and small bowel series. Proctocolectomy with ileostomy was performed and then protein losing enteropathy was resolved.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.213-220
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• 2006

Laparoscopic cholecystectomy has been increasingly used because of several advantages, less pain, better expectation for cosmesis (requires small incisions), and more rapid recovery compared with open cholecystectomy. Oral intake is tolerated on the day of operation or on the next. In this study, we evaluated the effectiveness and safety of laparoscopic cholecystectomy in children. Nine cases of laparoscopic cholecystectomy for acute and chronic cholecystitis in children were performed at Asan Medical Center between April 2002 and April 2004. Laparoscopic cholecystectomy was performed on a total of 10 patients, but one of them was excluded because of the simultaneous splenectomy for sickle cell anemia. Clinical presentation, operative findings, operation time, length of hospital stay, and postoperative complications were analyzed. Mean age was 10.4 (4.15) years, and only 3 of patients were less than 10 years. One patient was female. In 8 the diagnosis was calculous cholecystitis. Mild adhesions were found in 3 cases and intraoperative bile leakage in 2. There was no conversion to open surgery and there were no vascular, bowel, or bile duct injuries. Mean operation time was 82.2 (20.160) minutes; mean length of hospital stay was 2.1 (1.3) day. There was no postoperative complication. Laparoscopic cholecystectomy in children was remarkably free of side effects and complications and had a short recovery time. Laparoscopic cholecystectomy for cholecystitis is considered to be a standard procedure in children.