Lectins can recognize the differences of sugar chains in glycoproteins. The sugar chain of a-fetoprotein (AFP) is known to have organ specificity and also tumor specificity. Subfractionation analysis of serum AFP by crossed immuno-affino-electrophoresis using Phytohemagglutinin-E_4 (PHA-E_4) lectin was carried out in 20 AFP-producing tumors in childhood and 10 adult carcinomas. AFPs in cord blood and infantile obstructive liver diseases were also analyzed with the same method. PHA-E_4 lectin can clearly separate serum AFP in 5 subfractions, i. e., tightly bound (TB) , loosely bound (LB 1 , LB 2 , LB 3) and unbound (UB) fractions. With this analysis, serum AFP could clearly be classified as the liver type and the yolk sac tumor type. The liver type could further be subdivided into fetal liver type and malignant liver tumor type. AFP in gastric cancer and pancreatic cancer as well as yolk sac tumor were the yolk sac tumor type. This analysis also disclosed that the recognition sites of sugars in AFP by PHA-E_4 and Lens culinaris agglutinin (LCA) were completely independent. This new method for glycosylation analysis of AFP is particularly useful in determining the origin of tumor and in early detection of hepatoma at risk in patients with liver cirrhosis.

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Immunohistochemical studies were carried out on tissue sections obtained from 5 patients with Hirschsprung's disease, aged 3 months to 7 months, using monoclonal anti-neurofilament antibodies. The following antibodies were used with Avidin-Biotin-peroxidase Complex method : monoclonal anti-neurofilament antibody "H" (molecular weight 200,000), "M"(160,000), "L" (68,000) [Bohringer Mannheim, West Germany]. Numerous fibers immunoreactive for anti-neurofilament "H" and "M" antibodies were present in both intermuscular and submucosal layers of the aganglionic segment in all of the 5 cases. These positively stained nerve bundles were hypertrophic and randomly distributed. The ganglionic segment showed, in the inter-muscular and submucosal layers, a reduced number of thin fibers that were positively stained by anti-neurofilament "H" and "M" antibodies. The immunoreactivity of the nerve fibers to anti-neurofilament "L" antibody was weak not only in the aganglionic segment but also in the ganglionic segment. These results indicate that the fundamental pathology in Hirschsprung's disease is not only the absence of ganglion cells but also the abundance of nerve fibers which are immunoreactive to monoclonal antineurofilament antibodies.

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Development of surgical technique and postoperative management provides substantial improvement for at least five years in one third of the patients with biliary atresia, although progressive cholestasis, hepatocellular decompression or severe portal hypertension occur with increasing age in some of them. One hundred and three patients have undergone hepatoportoenterostomy for biliary atresia during the period from January 1970 to December 1986 in our institute. Eighty-six patients (83%) had a bile dranage and two thirds of them showed jaundice free. There are thirty-six patients living without jaundice and twenty-eight of them living for more than five years. The survival rate is 36% at five years. Twnty-five patients have been expected curable. In this paper we did retrospectively examine in seventy-one patients of all with biliary atresia whether success of this procedure could be predicted for the individual patients by means of the data of operative age, serum bilirubin decreasing rate, clinical signs and various hepatic functional tests. We concluded as follows : (1) We suppose that serum bilirubin decreasing rate could be an important index for prediction of the success of hepatoportoenterostomy in biliary atresia, but the operative age should not be. (2) Transplantation of the liver should be early considered for biliary atresia patients with rapid increase of serum bilirubin or progressive hyperbilirubinemia exceeding 5 mg% at 3 months after hepatoportoenterostomy, who gradually decompensate hepatic functional reserve after this procedure. (3) In the postoperative patients with biliary atresia in hyperbilirubinemia exceeding or below 5 mg% of serum total bilirubin, who do not deteriorate hepatic functional reserve, liver transplantation should be delayed as long as possible to permit the children to achieve maxim growth. But, it should be performed for these patients within keeping in normal level of tha hepatic functional reserve.

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During the period from 1982 to 1988, twenty-five patients with intestinal atresia underwent surgery. Total parenteral nutrition (TPN) administered in 13 patients and enteral nutrition by a trans-anastomotic feeding tube (TAFT) was given in 8. Efficacy of supportive nutritional therapy (SNT) was evaluated by weight gain, status of oral feeding and complication of SNT. SNT was evaluated as efficient in 61.5% of TPN and 50.0% of TAFT, but weight gain with SNT was not good. Complication rate of SNT was 19.2% in TPN group and 18.8% in TAFT group. In low birth weight infants, complication rate was higher and efficacy rate was lower than normal infants. Indications of SNT were concluded as follows : (1) SNT was not necessary in membranous type atresia of any location and in any type of ileal atresia. (2) SNT was absolutely indicated in high jejunal atresia. (3) Indication of SNT could not be predicted in annular pancreas or type III (disconnecting type) atresia of the duodenum.

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Eleven infants with biliary atresia during a two and half year period from May, 1985 through Octover, 1987 were treated with the following strategies; (1) hepatic mobilization, (2)anti-reflux valve in the Roux-Y loop , (3) aggressive antibiotics & choleretics, and (4)prompt reoperation. Hepatic mobilization was associated with no complications, and anti-reflux valve (Nakajo's method) was established in the Roux-Y loop, usually at the time of closure of Sawaguchi's fistula. All patients became free of jaundice, and are doing very well from 56 months to 28 months (average 43 months) with only one death due to septicemia. Details of our techniques are presented and discussed.

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The aim of preoperative treatment of infantile hypertrophic pyloric stenosis (IHPS) is to restore dehydration and metabolic alkaosis. One hundred and eighty-three cases of IHPS treated at Tokyo Metropolitan Kiyose Childre's Hospital were reviewed, and the relationship between serum electrolites, urinary electrolites and blood pH, BE and HCO_3~ were studied. Even if serum chloride concentration were 95mEq/ l or more (52 patients) , urinary chloride concentration were below 25mEq/ l in 46 (88.5%). Urinary sodium excretion was also low, and those results suggested the depletion of electrolites and persistent dehydration. When urinary chloride concentration was 25mEq/l or more, blood pH, BE, and HCO_3~ became normal. It is recommended that pyloromyoromy is planned when urinary chloride concentration is 25mEq/l or more.

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Polyethylene Glycol electrolyets solution (PEG) is well known as non-absorbable bowel lavage solution and established in preoperative bowel cleaning solution in USA. Although there were very few papers to date in Japan, all these paper described as a bowel cleaning for colonic endoscopy. We have been using this lavage solution from Feburary 1988 and analyse 21 Japanese children to determine proper amount of PEG and it's effect for bowel cleaning for pediatric surgical patients. The results were as follows; (1) Bowel cleaning using PEG solution could carry out with safety and get satisfactory results.(2) Infusion speed of PEG from N/G tube should not exceed 30 ml/kg/hr and get a excellent results for 4 hour infusion. (3) To reduce a number of bacterial colony, antibiotics should be prescribed.(4) We were able to relieve children from painful bowel cleaning using PEG solution.

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The investigation on the current state of nutritional care and parenteral nutrition in 1987 was performed by the questionnaire method to 170 pediatric surgical institutes in Japan. Replies submitted to the questuonnaire were obtained from 88 hospitals (52%) , though the rate of reply being 73% in 67 registered pediatric surgical institutes. The mean duration of parenteral nutrition was 50.5 days per patient, which was twice longer than 27.8 days in 1979. The frequency of the catheter-related sepsis was 4.3 of 1000 patient-days and decreased comparing to 7.9 of 1000 patient-days in 1979, while the catheter-related sepsis was occured in 16.4% of patients. Liver dysfunciton was pointed out as the most common metabolic complication for nutritional care.

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A case of 2-year-old boy with left giant hydronephrosis with congenital multiple stenosis and a contrateral moderate hydronephrosis with persistent fetal ureteral fold is reported. An ultrasonically guided percutaneous nephrostomy, a pyelo-ureterogram thruogh the nephrostomy and a manometric study are both useful for the diagnosis and evaluation for the treatments of this rare case.

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This is a report of a case of choledochal dilation in a 5-year-old boy associated with an aberrant hepatic bile duct draining the posterior segment of the right hepatic lobe. Episodes of repeated abdominal pain and vomiting brought him to our consultation. At laparotomy, 2 ductal structures, 3 mm in diameter each, were found at the uppermost aspect of the choledochal dilation, which was about the size of 4 × 3 × 3cm. Intraoperative cholangiograms revealed that the post-erior segment duct did not join its anterior counterpart, but terminated independently at the choledochal cyst. The anterior segment duct united witin the liver with the left hepatic duct and then entered the cyst just to the right of the posterior segment duct entry. Three hundred and nineteen cases of aberrant hepatic bile duct were collected from Japanese and English literatures and analysed. The anterior segment of the right hepatic lobe is the commonest site of origin for an aberrant hepatic bile duct, which comes from the right hepatic lobe in 96.9% of the cases. It is emphasized that intraoperative cholangiography should be so performed as attention be paid not only to the pancreaticobiliary ducts relationship, but to the anatomical communication between the intra-and the extrahepatic biliary systems. Identification of the anterior as well as the posterior segmental bile ducts of the right hepatic lobe is mandatory for an uncomplicated biliary reconstruction. The role of an aberrant hepatic bile duct in the pathogenesis of congenital choledochal dilation is doubtful as yet from embryological point of view and remains to he further studied.

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A 8 -month-old of Cornelia de Lange syndrome complicated by esophageal hiatus hernia is presented. This is the second case of Cornelia de Lange syndrome with esophageal hiatus hernia in Japan. The patient showed low-birth-weight, growth retardation, generalized hirsutism, synophrys, characteristic facial feature, hand abnormalities and vomiting. An upper gastrointestinal series examination revealed esophageal hiatus hernia, and pH study revealed marked gastroesophageal reflux (GER), for which we performed Nissen's cardiac fundoplication. Though the vomiting disappeared after surgery, she has been unable to take oral feeding.

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Laryngotracheoesophageal cleft is a rare but potentially curable anomaly. A five-month-old boy with the type I cleft was preseted. Though the difficulty in making diagnosis is stressed in literatures, esophagogram with barium and laryngofiber scope were diagnositc in our case. We succeeded in repairing the cleft 15mm long, through an anterior approach to obtain a permanent cure.

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Epignathus or congenital nasopharyngeal teratoma occurring from intracranial space, growing out through complete cleft palate, and occupying oral and retropharyngeal cavities is an extremely rare abnormality. This unusual tumor may cause severe respiratory obstraction which is often associated with fetal prognosis in the newborn patients. Therefore, the antenatal diagnosis is essential for early management in the cases with congenital epignathus. Endotracheal intubation or tracheostomy should be caried out immediately after birth. We reported a case of nasopharyngeal teratoma which was diagnosed antenatally by ultrasonic echogram and amniography. Abnormal band between the tongue and the retropharygeal wall was excised by pediatric surgeons, enabling endotracheal intubation soon after birth in this case. Subsequently tracheostomy and gastrostomy were performed. The tumor has been excised successfully by three staged operations. She has been doing well taking food orally and breathing normally. CT and MRI was useful in delineating the tumor.

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Xanthogranulomatous pyelonephritis (XPN) is usually seen in middle-aged women and is much rarer in children. We encountered a case of XPN in a 2 mouth-old-boy whose right kidney and its surrounding fat were affected, with widespread lesions in the retroperitoneum. Such a case with large retroperitoneal involvement is rare in infants. It was considered difficult to diagnose the difference between XPN and Xanthogranuloma. The removal of the kidney, perinephritic fat and peritoneal lesion are curative in this case.

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A successful surgical treatment of a newborn with IAA and VSD by staged operation is presented. The patient was diagnosed as IAA associated with VSD on the 15th day and aortic arch reconstruction by Blalock-Park procedure and pulmonary artery banding were carried out on emergency bases on the day of diagnosis. The postoperative recovery was uneventful and he had satisfactory physical development. Cardiac catheterization and angiography at 1 year and 9 months after the initial operation revealed stenosis at the site of aortic reconstruction with a pressure gradient of 65 mmHg. Aortic stenosis was releaved by patch aortoplasty through lateral thoracotomy. At the age of 2 years and 3 months, open heart definitive repair (patch closure of VSD, debanding and patch plasty of the pulmonary artery) was performed successfully. Postoperative angiography demonstrated satisfactory correction of both aorta and pulmonary artery. We conclude that : 1) Aortic arch reconstruction and pulmonary artery banding can be safely and reliably performed even in a critically ill neonate with IAA and VSD, 2 ) Staged definitive intracardiac repair can be done safely without any incremental risk factors.

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