Serum and erythrocyte cholinesterase activity was measured in 16 normal controls, 9 pre-operative and 17 postoperative patients of Hirschsprung's disease. In concern with erythrocyte acetylcholinesterase activity, no significant difference was found among these three groups. However, serum cholinesterase activity in preoperative patients of Hirschsprung's disease was significantly higher than in normal controls. In patients undergone the Duhamel-Okamoto procedure, in whom the agangljonie segment remains in the anterior wall of the rectum, serum cholinesterase activity was high. On the other hand, serum ・cholinesterase activity in patients undergone Swenson's procedure was apparently lower than in preoperative patients. These results suggest that the elevated activity of serum cholinescterase observed in patients before operation and in those undergone Duhamel-Okamoto procedure for Hirschsprung's disease is due to the presence of the aganglionic segment.

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The experiment was designed for the purpose of evaluating the effect of exchange transfusion, especially fresh blood exchange transfusion, using arterio-venous line. Young rabbits, age around 3 weeks and weight of 258±33g (mean±SD), were used. Twelve rabbits (group I) were treated with sham operation. Twenty-eight rabbits (group II) were intravenously given 40mg/kg of lipopolysaccharide (LPS Difco, E. Coli 0111: B4). Sixty minutes after injection of LPS, 12 rabbits (group III) were given 30ml of exchange transfusion of fresh blood using a connecting circuit between the artery of adult rabbit and the vein of young rabbit. Sixty minutes after injection of LPS, 12 rabbits (group IV) were given 30ml of exchange transfusion of the arterial blood, which had been kept in the room air for 30 to 130 minutes after drawing. As the result, the survival rate after 24 hours was 100% in group I and III, 29% in group II and 58% in group IV. Significant difference (P<0.05) was observed between group II and III, group II and IV, and group III and IV. After injection of LPS, increase of the heart rate and decrease of the blood pressure were observed in group II, III and IV. Decrease of the blood pressure was smaller in group III than in group II and IV. After injection of LPS, respiratory alkalosis was observed in group III and IV. Hematologically, decrease of the white blood cell and platelet was observed after injection of LPS. But the recovery of platelet count in group III was remarkable after exchange transfusion via arterio-venous line. Histopathologically, in the groups with injection of LPS, noticeable changes and degenerations were observed in the kidneies, liver and thymus,but only little change was observed in the lung. These pathologically abnormal findings were marked in mortal cases.

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Five cases of Alagille's syndrome were experienced at Nagoya City University Hospital during from 1972 to 1979. Four cases were male and one female infant. Hypoplasia of the intrahepatic bile duct in Alagille's syndrome is usually recognized by pathological examination of the biopsied specimen, However, the size of the sample is not always large enough to detect changes of the bile ducts in the portal area. In our experience, we found the importance of cholangiographic studies for the diagnosis of various forms of obstructive jaundice. A new clasification of obstructive jaundice acording to cholangiographic findings in infants is proposed. 1) Biliary Alresia: Anywhere of the biliary system from the intrahepatic to common duct, one or more obstructions are found. 2) Bile duct Hypoplasia: There is no obstructive lesion in the total bile duct, but the diameter of the bile duct is very narrow or unclear on cholangiogram. 3) Infantile Hepatitis: There is no obstructive lesion in the total bile duct, and the cholangiogram can clearly demonstrates the second branch of intrahepatic bile duct. It is postulated that a successful out come of surgical treatment of biliary atresia will be expected if the diagnosis of intrahepatic bile duct hypoplasia is made in the early post natal period.

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The activity of ornithine decarboxylase (ODC) which catalyses the first step of polyamine Synthesis is expected to increase in the remaining bowel after massive intestinal resection in rat as well as observed in the partially resected liver and in the healing skin wound of rat. The ODC activity was highest in the upper jejunum and lower in the ileum in unoperated rat. After resection of 80% of the small intestine, the ODC activity of the proximal (duodenum and upper jejunum) and distal segment (lower ileum) increased very rapidly and reached maximum after 12 hours. The peak activity in the proximal segment was 3 times and that of distal segment was 43 times higher than the level of unoperated rats. These findings suggest that adaptation of the distal segment was greater than that proximal segment after massive intestinal resection. The increase of ODC activity in the remaining bowel was related to the amount of resected bowel. But the concentration of polyamine did not increase in the remaining bowel after resection. Although ODC activity of the lower ileum in operated rats increased only slightly by feeding, a marked increase of the enzyme activity was observed in the hyperplastic distal segment by feeding, 3 weeks after massive intestinal resection.

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The organo-axial and mesentero-axial classification of gastric volvulus has been accepted since proposed by Kocher in 1914. However, by analysing two cases of idiopathic gaslric volvulus which were successfully treated (one opcratively anather nonoperatively), we faced a difficulty to explain the sequence of gastric volvulus by the conventional organo-axial and mesentero-axial theory. The conventional classiffication which divides gastric volvulus into two patterns (organo-axial and mesenlero-axial volvulus) does not fit in many cases. The pure organo-axial type and mesentcro-axial type are rather of exception. And it should be emphasised that the axis itself is changing in the sequence of the volvulus. We speculate that the axis at the gastric volvulus moves from the more-organoaxial direction to more-mesenteroaxial direction as volvulus progress. Since the stomach is loosely or not fixed at the greater curvature, fundus and pyloric region, the greater curvature is lifted up or pulled up ventrally and cranially and sometimes toward lesser curvature. The fundus moves dorsally and caudally, and the stomach will finally become up side down. In some situation, the fundus may move doraslly and caudally prior to lifting of the greater curvature.

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Mitral regurgitation in chilldren is not common and congenital mitral regurgitation is especially rare. Four children with mitral valve replacement are reported. The age of patients ranged from 3 to 14 years. One was rheumatic valvular disease and the others considered to be congenital. Two patients were associated with PDA. Mitral regurgitation was found by cardiac catheterization and UCG. By the reason that the patients were troubled by congestive heart failure or growth returdation, open heart surgery was performed. Since the valve plasty procedure seemed unsuitable in all cases, mitral valve replacement with Bjork-Shiley valve was performed in three and with Carpentier-Edward valve in one patient. After surgery, anticoagulant therapy was employed in three patients with Bjork-Shiley valve except one with porcine Xenograft. These patients have been followed up for from 9 months to 6 years, and the result of surgery was satisfactory.

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To authors' knowlege, only two cases developing chylothorax following herniorrhaphy have been reported in the literature. This report discribes the third case of this unusual complication with Bochdalek hernia repair, and discusses the cause and method of treatment. A male infant weighing 3.55 kilograms, was born on July 17 1978, following normal pregnancy, labor and delivery. The patient's development was normal until two months of age. Vomiting first noted on October 3 1978 became to be seen after every oral feeding. The patient was transferred to our hospital. A chest x-ray film showed typical findings of left Bochdalek hernia, but no respiratory distress was noted. Through an abdominal approach, the left posterolateral diaphragmatic hernia with a thin sac was closed by mattress sutures with the sac removed. The immediate postoperative period was complicated with left pneumothorax treated by a chest tube insertion and continuous suction. On the second postoperative day, after oral feeding was started, drainage from the chest tube increased. Next day, the fluid became milky. Chylous fluid volume was small, but it did not decrease. Premilk started on the 7th day was changed to a medium-chain triglyceride milk, on the twelfth postoperative day, which seemed effective to stop drainage of chyle. The chest tube was removed 22 days after operation. The patient was discharged on the 36th hostital day in good condition. At present, he is one year old with normal growth and development.

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A term male infant weighing 3,050 g developed necrotizing enterocolitis 2 days after birth. A perforation of the intestine occurred on the 6th day. A laparotomy was carried out for creating an enterostomy at the perforation. On the 10th day after operation, the patient developed diabetes insipidus, which was successfully managed by transnasal Desmopressin administration. The cause of diabetes insipidus seems to be a transient disorder of the neurohypophyseal axis, which is secondery to septicemia orginated form NEC.

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In 1976, Berdon et al. described a new type of neonatal intestinal obstruction. They reported five newborn girls who presented with marked abdominal distension and bilious vomiting with specific clinical features including dilated small bowel and rnicrocolon without anatomic obstruction, short intestine and malrotation, defective intestinal peristalsis, megacystis without vesicoureteral reflux, and hydronephrosis or dilated ureter. Pathorogical studies show almost normal ganglion cells in both dilated and narrowed areas of the intestine. Recently we encountered a patient with this condition. A 4-day-old girl underwent a tube-ileostomy for intestinal obstruction described above. But the ileostomy drainage was minimal. The patient required continuous intravenous hyperalimentations. She could survive for 46 postoperative days.

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Hypoglycernia-producing extrapancreatic neoplasms in infants are extremely rare. To our knowledge, only two cases under 10 years of age (Wilms' tumor and neuroblastoma) have been reported. A five-year-old male was admitted with symptoms of hypoglycemia. Thereafter, in the course of studies, a hepatoblastoma was found. After resection of the tumor, hypoglyce mia disappeared. A high concentration of insulin-like activity (ILA) was found in the tumor extract. Hypoglycemia in this patient seemed to depend mainly on ILA and partly on liver enzyme activities related to carbohydrate metabolism.

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Two casee of choledochal cyst accompanying multiple cystic dilatation of the intrahepatic bile ducts are reported. In one case, operative cholangiogram showed multiple cystic dilatation of the left intrahepatic bile duct with a stenosis at the hepatic hilus and diffuse dilatation of the right intrahepatic duct. Liver fibrosis was noted. The other case demonstrated multiple cystic dilatation of the bilateral intrahepatic bile ducts and high level of amylase in the bile juice. As to the cause of these findings, it is difficult to consider that extrahepatk bile duct dilatation has extended to the intrahepatic biliary system. The findings were similar to Caroli's disease, of which the etiologic factors are discussed. Long term follow up is stressed for the late complications such as cholangitis, intrahepatic cholelithiasis, portal hypertension or neoplasm.

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A female infant was hospitalized at 49 days of age, with congestive heart failure and hepatomegaly. A diagnosis of congenital heart disease was made, and the patient was treated with digoxin and diuretics. At 61 days of age, cardiac catheterization and angiography were performed. These studies revealed a large multiple angiomatous lesins in the bilateral lobes of the liver with variant blood supply and massive intrahepatic shunt. The supplying arteries included the right internal thoracic, common hepatic, right inferior phrenic, and left internal thoracic arteries. The right internal thoracic artery was the main supplying artery. Ligation of the bilateral internal thoracic arteries and prednisonc therapy provided a a dramatic improvement in the patient's condition. Prednisonc therapy was discontinued two weeks after the initiation. At present, 9 months after cessation of therapy, the liver is in normal size. The abdominal aortography revealed normal hepatic artery and complete disappearance of the angiomatous lesions.

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A 17 year old female was admitted for occasional pain in the quadrant, initially noted at 9 years of age. At one month prior to admission, she had the same pain. By ERCP, a cystic dilatation of the extrahepatic bile duct was found extending up to the right and left hepatic duct with a stricture at the bifurcation. A large narrow segment was found in the distal bile duct with a junctional abnormality to the pancreatic duct. After removal of the cystic bile duct and gall bladder, a band like fibrous bridge was found in the distal part of the left main hepatic duel with stricture. After removal of this band, hepaticojejunostomy was performed. The postoperative course was uneventful. The specimen of the removed cystic bile duct was 8 x 3.8cm in size without any malignant change.

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