Neurocristopathy is characterized as having a common origin in aberrant neural crest development. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by marked depression of respiratory drive during sleep and normal ventilation while awake because of no response to both hypercapnea and hypoxia. The girl was full-term, weighing 3020 grams. The girl had poor respiratory effort at birth, but improved with oxygen supply and stimulation. abdominal distention and calcification were noted. During laparotomy transitional zone was found at distal jejunum; a jejunostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an apnea work-up, including brain sonography, echocardiogram, were normal. The girl died of sepsis at 37 days of age. para-aortic ganglioneuroblastoma was found on autopsy. We experienced a newborn with congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma representative of neurocristopathy.
Anoxia ; Apnea ; Autopsy ; Brain ; Depression ; Female ; Ganglioneuroblastoma* ; Hirschsprung Disease* ; Humans ; Hypoventilation* ; Infant, Newborn* ; Jejunostomy ; Jejunum ; Laparotomy ; Neural Crest ; Oxygen ; Parturition ; Sepsis ; Ventilation

PURPOSE: Obesity is related to many diseases, including urological conditions. We investigated the prevalence, risk factors, and treatment of male obesity. MATERIALS AND METHODS: This study included 17,485 men older than 20 years of age who participated in the fourth, fifth, and sixth administrations of the Korean National Health and Nutrition Examination Survey. Two main cutoff points for obesity were defined: a body mass index (BMI) ≥25 kg/m2 and a BMI≥30 kg/m2. Additionally, we defined obesity requiring pharmacotherapy as the presence of a BMI≥30 kg/m2 or a BMI≥27 kg/m2 co-occurring with at least one associated comorbid medical condition, such as hypertension, dyslipidemia, or diabetes. RESULTS: The prevalence rates of a BMI≥25 kg/m2, a BMI≥30 kg/m2, and obesity requiring pharmacotherapy were 35.7%, 3.4%, and 10.5%, respectively. The prevalence of obesity increased over time for all definitions of obesity. The prevalence of obesity requiring pharmacotherapy was highest in Jeju (12.5%) and lowest in Gangwon-do (7.7%). Having a higher income, being a non-manual worker, and having completed a high level of education were significantly related to obesity requiring pharmacotherapy. More than 70% of patients with obesity requiring pharmacotherapy reported taking diet pills, eating functional foods, or consuming a one-food diet for weight reduction, but only 13.9% reported exercising for this purpose. CONCLUSIONS: Male obesity is a common condition, the prevalence of which is expected to continue to increase over time. A better strategy is required to manage male obesity in Korea.
Body Mass Index ; Diet ; Drug Therapy ; Dyslipidemias ; Eating ; Education ; Functional Food ; Gangwon-do ; Humans ; Hypertension ; Korea ; Male ; Nutrition Surveys ; Obesity* ; Prevalence ; Risk Factors ; Weight Loss

PURPOSE: The purpose was to evaluate outcomes according to different operative strategies of type A esophageal atresia (EA). METHODS: All patients who underwent surgery for type A EA between 1980 and 2011 were included. Patients were divided into 2 groups: E-E group included patients who received esophageal end-to-end anastomosis, whereas E-G group included patients who received esophago-gastric tube anastomosis. RESULTS: Twenty-two patients were included. The median gestational age was 37.5 weeks. The median birth weight was 2.5 kg. Twenty-one patients underwent gastrostomy as initial procedures, and one patient underwent primary esophageal end-to-end anastomosis. The median gap between both esophageal ends was six vertebral distance (VD). Seven patients underwent primary anastomosis of the esophagus, and 14 patients underwent gastric replacement. Three patients (13.6%) had anastomotic leakage and 10 patients (45.5%) had anastomotic stenosis. Most of the patients (90.9%) had gastroesophageal reflux, but only two patients required antireflux surgery. The median VD was significantly shorter in E-E group than in E-G group (3 VD vs. 6 VD). Stenosis was significantly more often in E-E group, but there was no significant difference in leakage and reflux symptoms. CONCLUSION: The treatment for type A EA can include E-E anastomosis or E-G anastomosis, depending on the length of the end-to-end interval after performing gastrostomy. Appropriate tension and blood flow in the anastomosis site are essential for preventing postoperative stenosis and leakage, and esophageal replacement with gastric tube is believed to be feasible and safe in cases where excessive tension is present.
Anastomotic Leak ; Birth Weight ; Constriction, Pathologic ; Esophageal Atresia* ; Esophagus ; Gastroesophageal Reflux ; Gastrostomy ; Gestational Age ; Humans

The association of congenital colonic atresia with imperforate anus is extremely rare. The authors were able to find only three case on our investigation of the English literature. We have recently managed a female infant with imperforate anus associated with atresia of the transverse colon.
Anus, Imperforate* ; Colon* ; Colon, Transverse ; Female ; Humans ; Infant

Lymphangioma is a congenital malformation of the lymphatic system that easily expands into surrounding tissue similar to malignancies. Surgical excision has been the treatment of choice, but the results of surgical resection have been unsatisfactory due to incomplete resection, fluid collection and recurrence after surgery. Intralesional injection of OK-432(lyophilized incubation mixture of group A. streptococcus pyogenes of human origin) was employed in treating 24 patients with lymphangiomas between 1992 and 1997. The therapy was used as a primary therapy in 21 patients. Also it used in 3 patients with unresectable or recurrent lymphangioma. The results of intralesional injection of OK-432 were excellent in 8 patients (33.3%), good in 7 patients (21.8%) and poor in 7 patients (21.8%). The side effects were fever under 39.0degrees C and a local inflammation, however, were subsided in a few days without serious complications. These data suggest that intralesional injection of OK-432 is safe and effective for lymphangioma as a primary therapy and unresectable or recurrent lymphangioma as a secondary therapy.
Child* ; Fever ; Humans ; Inflammation ; Injections, Intralesional ; Lymphangioma* ; Lymphatic System ; Picibanil* ; Recurrence ; Streptococcus pyogenes

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1years (range, 7 - 13years). Six patients presented with an abdominal mass and/or pain, and one with weight loss. Tumor size ranged from 8.0x8.0cm to 15.0x15.0cm. Four tumors were located in right lobe, two in left lobe and one in both. One patient died during preparation for chemotherapy. Initial complete resection was performed in three patients. Two patients received the complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferentiated (embryonal) sarcoma of the liver is highly malignant, we think that the combination therapy of surgery, chemotherapy and radiotherapy can get good results.
Chemotherapy, Adjuvant ; Child* ; Diagnosis ; Drug Therapy ; Female ; Humans ; Liver* ; Radiotherapy ; Retrospective Studies ; Sarcoma* ; Seoul ; Sepsis ; Weight Loss ; Young Adult

Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood. Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4 - 32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from 10x8.5cm to 34x29cm. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion, it may be confused, and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore, complete excision of the tumor with surrounding normal liver tissue is recommended.
Child ; Female ; Hamartoma* ; Humans ; Liver* ; Recurrence ; Retrospective Studies ; Sarcoma ; Seoul

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage, but improved resectability and survival is being reported with the help of neoadjuvant chemotherapy. Twenty biopsy proven hepatoblastoma patients were diagnosed and managed during the period between January, 1987 and June, 1995. Median age at diagnosis was 13 months (2mo~7yr 10mo) with M : F = 13 : 7. The histologic profile included 13 epithelial (5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial types. Chemotherapy effectively reduced the tumor volume with statistical significance (p=0.008) and was able to convert 7 out of 9 initially unresectable cases (78%) to resectable ones. Fourteen operations were done, 12 radical and 2 palliative with or without adjuvant chemotherapy. The whole population was followed with a median duration of 33 months and the median survival in the whole group was 26 months. The curative resection group displayed a 5 year survival rate of 61.1% and none in the non-curative group survived for more than 13 months (p=0.0001). In univariate analysis for prognostic factors, large tumor size at diagnosis and absence of thrombocytopenia was associated with poor survival, but these differences were not statistically significant. Pure fetal histology was not associated with better prognosis and this may be due to a different histologic classification. In this new era of neoadjuvant chemotherapy, the optimal management strategy for hepatoblastoma is still debated with radical surgical resection at earliest possible time being the final goal. For now, individualized approach appears to be the choice.
Biopsy ; Chemotherapy, Adjuvant ; Classification ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Prognosis* ; Survival Rate ; Thrombocytopenia ; Tumor Burden

Balloon dilatation has been applied in treatint of various pathologic narrowing of the hollow viscus. It is now accepted as very effective modality especially in treating esophageal stenosis obviating surgical procedure. We performed 128 balloon dilatations in 29 patients with the number of dilatations in each patient ranging from once to 12 times. The age distribution of the patients was from 3 weeks to 6 years, with the median age of 3 months. Twenty nine patients consisted of 25 postoperative esophageal strictures (21 esophageal atresia with tracheoesophageal fistula, 1 congenital esophageal stenosis, 2 tracheobronchial remnant, and 1 congenital esophageal stenosis with esophageal atresia), 2 achalasia, 1 congenital esophageal stenosis, and 1 corrosive esophagitis. We had successful dilation in 22 patients, who showed subsequent relief of symptoms and improvement in the diameter of stenotic segment. In 4 patients, esophageal perforation occurred during the procedure, one requiring emergency thoracotomy and the other threeconservative management. Seven patients had no improvement in stenotic sites after several balloon dilatations. Failed cases were congenital stenosis, achalasia, corrosive esophagitis and four postoperative strictures. We believe that balloon dilatation is the procedure of choice in the treatment of postoperative esophageal stricture in infants and children and is a safe method as the perforation which can complicate the procedure could be managed conservatively.
Age Distribution ; Child* ; Constriction, Pathologic* ; Dilatation* ; Emergencies ; Esophageal Achalasia ; Esophageal Atresia ; Esophageal Perforation ; Esophageal Stenosis ; Esophagitis ; Humans ; Infant* ; Methods ; Thoracotomy ; Tracheoesophageal Fistula

PURPOSE: To review the type, incidence and radiologic findings and to evaluate the clinical significance of intrahepatic duct dilatation of choledochal cysts in children. MATERIALS AND METHODS: Ultrasonography and operative cholangiography of 49 consecutive children with surgically confirmed choledochal cyst were reviewed and classified according to the Todani's classification. Patients with intrahepatic duct dilatation in preoperative evaluation were routinely followed by ultrasonography after surgery of extrahepatic bile duct. The status of bileduct was evaluated by comparing with the preoperative ultrasonography. RESULTS: According to the preoperative evaluation, there were 19 cases(39%) of type Ia, three cases(6%) of type Ib, ten cases(20%) of type Ic, 15 cases(31%) of type IVa, two cases(4%) of type V, but no case of the type II, III, IVb in Todani's classification.In twelve of 13 patients (five cases of type I and eight cases of type IVa) who had been followed up after surgery of choledochal cyst, the previously noted intrahepatic ductal dilatation were no longer seen. CONCLUSION: The incidence of the types of choledochal cysts by Todani's classification shows no significant difference between our result and the other authors'. The intrahepatic ductal dilatation seems to be reversible and mainly secondary to extrahepatic obstruction by choledochal cyst. Thus, postoperative follow up examination is more important than preoperative classification of bile duct dilatation.
Bile Ducts ; Bile Ducts, Extrahepatic ; Child* ; Cholangiography ; Choledochal Cyst* ; Classification ; Dilatation ; Follow-Up Studies ; Humans ; Incidence ; Ultrasonography