To evaluate the optimal timing of closure of external biliary fistula after definitive surgery with Suruga II in biliary atresia, complications and liver function were studied retrospectively in 15 patients. The following results were obtained: 1) The most common complication at enterostomy closure was ascending cholangitia (AC). 2)Most AC before enterostomy closure occurred by one year of age. 3) The patinets who had a stoma closed after 3 years of age had a high incidence of AC. 4) A history of AC, especially at over one year of age. was correlated with the occurrence of AC at enterostomy closure. 5) The liver function of non-jaundiced patients after enterostomy closure was significantly improved and stable compared with that before closure. In conclusion, our results suggest that the optimal timing for enterostomy closure in non-jaundiced patients is about 1 year of age.

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Two children with short bowel syndrome, undergoing long-term total parenteral nutrition, had low plasma selenium concentrations and we had to treat them with intravenous selenium compounds (selenious acid) because one of the children was symptomatic. During daily administration of 5 icrograms/kg of selenious acid, the patients plasma selenium levels (P-Se) and glutathione peroxidase (GSH-Px) acitvity reached the normal range within 4 weeks. In contrast, the erythrocyte selenium concentration (E-Se) barely reached the normal range after 16 weeks of treatment, but there was marked improvement in the clinical symptoms of the symptomatic patient. The erythrocyte GSH-Px activity level reached the normal level after 30 weeks of the treatment. Urinary excretion of selenium in boty patients after administration of selenious acid in proportion to the dose, and decreased as P-Se concentration diminished. Serum zinc and copper levels were uninfluenced by selenious acid therapy. However, in the one patient who had low vitamin E levels, the level of vitamin E recovered after administration of a certain amount of selenious acidl. In conclusion, selenium supplementation is mandatory in children on long-term parenteral nutrition. We feel that selenium therapy can be performed safely on the patients undergoing long-term parenteral nutrition, if the several indices found below are serially monitored : 1) plasma-and erythrocyteselenium levels, 2) glutathione peroxidase activity in plasma and in erythrocyte, and 3) selenium excretion in urine.

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The authors present 4 cases with perforated choledochal cyst and review the reported cases in Japan. The age ranged from 13 days to 3 years, one boy and three girls. The type of dilatation was cystic in 3 and fusiform in one. Abdominal pain, fever and vomiting were observed in 3. and abdominal distension, jaundice and drowsiness in one. The duration from onset to operation ranged from 2 to 13 days. The diagnosis was made by the operative findings of bile stained ascites in two, biliary scintigraphic finding of leakage into the free peritoneal space in one, and sonographic findings of bile duct dilatation and subhepatic fluid collection in one. From the investigation of the 93 collected cases in the literature, we suggest that reflux of the pancreatic juice into the bile duct and increase in the bile duct pressure might be the main causes of the perforation in choledochal cyst. In addition to these factors, weakness of the bile duct wall may play an important role in perforation, because the perforation seldom occurs in children at the age of 5 or more.

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Intraoperative ultrasonography (IOUS) was performed in 13 children with a solid abdominal tumor: 7 hepatic; 2 adrenal; 2 renal; 1 ovarian and 1 retraperitoneal. Nine out of the 13 tumors were successfully resected with the help of IOUS. IOUS was thought to be useful in the flollowing aspects. For the hepatic tumors, tumors too somall to be palpated could be detected in one case, qualitative diagnosis could be given in two cases, resectability or range of resection was precisely indicated in five cases and therapeutic injection could safely be done in two cases. In the adrenal and retroperitoneal toumors. the anatomical relationships between the tumor, vessels and surrounding organs were clearly depicted, leading to a complete removal. IOUS for the ovarian or renal tumors proved to be with no vessel invasion, no tumor embolism or no tumor in the contralateral ofgan.

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The expression of cellular adhesion molecules, i.e. neural cell adhesion molecule (NCAM) anil the CD44 molecule in neuroblasloma were examined. Fresh nonfixed specimens were studied in 9 patients with neuroblastoma (NB) or ganglioneuroma (GN). Three cases of 8 neuroblaslomas showed amplification of N-myc oncogene. Frozen sections were stained with monoclonal antibodies that recognize the NCAM and CD44 common region (CD44c). The expression of CD44 mRNA (standard form and variant 6) were examined by the RT-PCR Southern blot hybridization method. All cases had positive antibody reactions to NCAM. Three cases with N-myc amplification did not react to the CD44c antibody. However, five neuroblastomas without N-myc amplification and one ganglioneuroma showed positive staining with the CD44c antibody. The studies by the RT-PCR Southern blot hybridization method showed that the cases with N-myc amplification did not expresss mRNA for standard form CD4-1 and the variant 6. The other cases did express CD44 mRNA for both. These data may indicate that the expression of NCAM correlates to neuronal phenotypic differentiation. It also suggests that CD44 expression has an inverse relationship to N-myc amplification. This suggests that CD44 expression is a good prognostic marker for neuroblastoma.

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