It has been reported that abnormal composition of serum lipids in patients with liver disease is associated with changes in erythrocyte membrane lipid composition. We speculate that similar changes may occur in other blood cells and impair their functions. The purpose of this study is to investigate the lipid composition of serum and erythrocyte membrane in patients with congenital biliary atresia (CBA). The serum was enriched in free cholesterol (FC) and phospholipids (PL) . Serum fatty acid compostion was abnormal, but the pattern of essential fatty acid deficiency (EFAD) was not observed. The erythrocyte membrane was also rich in FC. Phospholipid composition was also abnormal. The proportion of phosphatidylethanolamine was decreased and that of phosphatidylcholine was increased. The fatty acid composition of erythrocyte membrane was not indicative of EFAD, but was abnormal. There was a significant correlation between serum FC and that of erythrocyte membrane. In conclusion, it is suggested that the membrane fluidity may be decreased by FC enrichment and changes of PL composition and that similar changes may be found in polymorphonuclear leukocyte and other cells in CBA patients.

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Eleven cases of post-operative anorectal malformation with fecal incontinence were admitted to our institute. (9 cases: the intermediate or high type of anomaly, 2 cases: the low type of anomaly) The clinical assessment of anorectal function (full mark: 8 points) was performed on all patients. Ten cases showed 0-2 points and one case showed 4 points. The treatment protocol of fecal incontinence consists of the es tablis hment of habitual evacuatuin, the control of fecal condition to decrease frequency of incontinence or soiling, training of voluntary sphincteric contraction by bouginage, mental support by the family and revision of the operable anatomical abnormalities. The detected causes of the poor anorectal function were as follows. The abnormal distribution of sphincteric complex was found in 5 cases, abnormal distribution of puborectal muscle and sphincter complex in 3, cystic dilatation of the remained part of urethral fistula in 1, rectourethral fistula in 2, severe rectoanal stenosis after posterior sagittal anorectoplasty (PSARP) in 1, vesico-vaginal fistula in 1 and severe sacral anomaly in 2 cases. The surgical corrections were completed in 10 cases except for one low type anomaly with severe sacral deformity. An anal transplant was done in 3 cases, PSARP in 5, an division of the recto-urethral fistula through a sacroperineal approach in 1, and colostomy and bouginage of anorectum in 1 case. Seven cases showed marked improvement in the clinical assessment over 5 points. Four cases did not make any improvement and two cases with severe sacral anomaly learned rectal lavage at home.

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Clinical usefulness and limitation of DNA ploidy as a prognostic indicator was assessed in neuroblastoma by retrospective analysis in the patients with this disease experienced during the period from 1971 to 1990 at the Kobe Children's Hospital. Fifty-seven patients subjected to this study were divided into two groups by their DNA ploidy; DNA aneuploidy (AP) (n=33) vs DNA diploidy/tetraploidy (DTP) (n=24). Kaplan-Meier survival analysis (5 years) disclosed that there was a statistically significant difference between each combination of the two groups; AP vs. DTP (82% vs. 29% , p<0.001), AP vs. DTP in age below 12 months (100% vs. 43%, p<0.001), However, there was no significant difference in AP vs. DTP in age over 13 months (50% vs. 23%, p>0.05). In conclusion, DNA ploidy is useful in predicting prognosis in the patients with neuroblastoma of age below 12 months. However, that is not the case in those with age over 13 months.

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We experienced in patients undergoing CAPD a high incidence of peritoneal problems that included hydrocele, indirect inguinal hernia and umbilical hernia. Of 74 patients on CAPD, 5 had indirect inguinal hernia, one of whom had concomitant hydrocele, and 1 had 1 had umbilical hernia complicated with indirect inguinal hernia (overall incidence: 8/74 = 11%). For all of the patients, peritoneograms using 99mT-HSA demonstrated the presence of the patent processus vaginalis. Indirect inguinal hernia recurred in one patient. Umbilical hernia recurred twice. As the recurrences seemed to be related to increased intraabdominal pressure during CAPD, total exposure and tight high ligation of the hernia sac (processus vaginalis) seemed mandatory for the treatment of indirect inguinal hernia. Mattress sutures with pledgets in approximating the peritoneum-fascial defect were by far advantageous in preventing the recurrence of umbilical hernia.

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We've experienced 63 cases with gastric rupture at our hospital from 1966 to 1991. Twin cases were detected in 7 (11%) of the 63 cases. In Japan, the frequency of twin is about 0.6% out of the delivery. In this study, we've evaluated the reason of the high frequency in twin cases. Of the 7 twin cases, 6 were of prematurities (birth weight l,690 ± 690g), 2 were with asphyxias, and 5 had another associated diseases (RDS, volvulus, NEC, hyperviscosity syndrome, sepsis). The site of perforation were detected at lesser curvature in 3, and size was very small in 3 cases. These findings suggest that the reason of the high frequency in twins is due to the disturbance of blood flow and ulcer formation contributing to anoxia in the stomach during perinatal period. We concluded that the frequency of gastric rupture in twins would be reducible by the improvement of perinaal care.

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Portal vein hemodynamics in 26 patients with biliary atresia was investigated using pulsed doppler flowmetry combined with B-mode ultrasonography. From the control study in normal subjects (n=37), the normal range of each parameter of portal vein hemodynamics in children was estimated as follows; (1) Cross-sectional area of the portal trunk: 0.137+0.010×W±0.138×√<1.027+0.0004×(W-21.2)^2>(cm^2), (2) portal venous velocity: 14.2 ±4.4 (cm/sec), (3) Portal venous flow: 104.4+9.24XW±151.4×√<1.027+0.0004×(W-21.2)^2> (ml/min), where W is body weight in kg. Z-score against the control value was applied to estimate the changes in portal vein hemodynamics in patients with biliary atresia. In the subgroup without liver dysfunction or portal hypertension (n=7), the cross-sectional area of the portal trunk and the portal venous flow were significantly increased. In the subgroup with liver dysfunction and no portal hypertension (n=8), the portal venous velocity decreased. In the subgroup with portal hypertension (n=11), the portal venous flow was well maintained owing to an increase of cross-sectional area of portal trunk in 6 patients, but portal vein hemodynamics was deteriorated in the other five patients. These changes in portal vein hemodynamics were found to correspond well to the patients' clinical course. In conclusion, analysis of portal venous hemodynamics is essential to estimate the actual status especially when to determine the timing of liver transplantation in patients with biliary atresia.

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Many studies have provided an evidence suggesting that platelet aggregation plays a key role in tumor metastasis. A number of antiplatelet agent have been used to prevent tumor metastasis in animal models and humans. Antiplatelet agent, a new stable prostaglandin I_2 analogue beraprost sodium (BPS) was examined to investigate its effect on hepatic metastasis model from neuroblastoma (C-1300) in A/J mice. The tumor cells were injected intrasplenically, and the animals were divided into control, BPS(10μg/week), BPS(20μg/week), and BPS(30μg/week)groups. BPS were administered subcutaneously with micro-osmotic pump after the tumor cell injction and the animals were killed on the 13th day. As a result, histological findings clearly showed that hepatic metastasis was suppressed in BPS(30μg/week)group, and an increase of hepatic metastasis was recognized in BPS(10μg/week)group. Hepatic weight in each group was as follows: control; 2.42+0.16g, BPS(10μg/week)group; 3.34±0.85g, BPS(20μg/week)group; 2.02±0.49g, and BPS(30μg/week)group; 1.5±0.37g. There was a significant difference between control and BPS(30μg/week)group(p=0.009). These results suggest that BPS(30μg/week)could prove of value in the prevention of hepatic metastasis from neuroblastoma.

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One hundred one patients with advanced neuroblastoma entered in this study between April 1984 to January 1993. Dose-intensive cyclophosphamide-based and cisplatin-based chemotherapy regimen (original protocol) was used until May 1991 and adriamycin was added at April 1991 (new protocol). The 4-year survival rate for the 25 patients (14 Stage III and 11 Stage IV) younger than 1 year of age was 83% and that for the 76 patients (15 Stage III and 61 Stage IV) older than 1 year was 27%. Prognosis of the patients of 1 and 2 year of age was significantly worse than that of patients older than 3 year of age. One of 6 patients who underwent bone marrow transplantation (BMT) and all 4 patients who underwent peripheral blood stem cell transplantation (PBSCT) are alive without disease. Toxicities including hearing impairment, renal dysfunction, pneumonitis, GVHD, and sepsis were seen in 37 patients. There were 9 treatment-related deaths. The survival rate of new protocol was not significantly better than that of original protocol. In conclusion, an early diagnosis of poor-prognosis group patients by the second mass screening at about 18 months of age and the more effective dose-intensive chemotherapy supported by BMT or PBSCT are needed.

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Attachment and migration of the cultured neuroblastoma cells were potently stimurated by the substance consisted in the culture supernatant of LM-cell, murin lung fibroblast cell line. Purification of this substance was performed and this activity was eluted as a single peak at the fractions corresponding to M.W. 1,000,000 on gel-filtration column. This substance also stimulated the invasion of neuroblastoma cells through reconstituted basement membrane, Matrigel, and required Ca^<2+> for its activity. Since this activity was neutralized by the anti-laminin antibody, we concluded that this substance was soluble laminin. The effect of anti-laminin receptor antibodies were examined and this activity was potently inhibited when neuroblastoma cells were preincubated with anti-integrin β_1 antibody and anti-integrin α_6 antibody. These data indicate that adequate numbers of laminin receptors, such as integrin α_6 β_1 (VLA-6) must be expressed on invasive neuroblastoma cells and these integrins must play important roles in invasion and metastasis of neuroblastoma cells.

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This is a case report of a 15-year-old boy with recurrent tracheoesophageal fistula (TEF) found 15 years after the primary repair of Gross-C type congenital esophageal atresia. The patient suffered from recurrent pneumonia and atelectasis when he was operated upon for branchial arch syndrome at the age of 14 years. A diagnosis of recurrent TEF was made by esophagograms and cine-esophagogram. A guide wire was cannulated into the TEF just before operation using an esophagofiberscope. The site of closure of TEF in the tracheal wall was covered with a Gore-tex patch after the division of recurrent TEF. The incidence, symptoms, diagnosis and treatment of recurrent TEF in congenital esophageal atresia were discussed.

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We report a rare case of extrarenal Wilms'tumor in Childhood. A 2 year and 6 month old boy was admitted with abdominal distention. A smooth hard mass of fist size was palpated in the left upper abdomen. On intravenous pyelography, the left kidney and ureter were displaced toward the upper outer direction. However, the left renal calyx was not deformed. On MRI examination, a well-defined, solid mass existed on the lower inner side of the left kidney. Tumor markers were within normal range. At operation, an encapsulated tumor was found attached medially to the left kidney, close to hilus. The extirpated tumor measured 8X10X7cm. Histological diagnosis was extrarenal Wilms'tumor. The patient is currently managed with chemotherapy and is active and well at 1 year after surgery.

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Omental-mesenteric myxoid hamartoma was first described by Gonzalez-Crussi et al in 1983. We experienced a case of omental-mesenteric myxoid hamartoma. This 7-month-old female infant presented with abdominal distension. On clinical examination, an abdominal tumor mass was discovered. At sugery, a solid, well-circumscribed tumor attached to the ileum was identified. The tumor was removed with about 10cm of ileum. Microscopic examination disclosed a well vascularized myxoid tumor with mesenchymal cells. Immunohistochemmical findings showed positive staining for vimentin and desumin. Electromicroscopic examination showed dilated rough endoplasmic reticulum. These examinations confirmed the diagnosis of omental-mesenteric myxoid hamartoma. Her postoperative course has been uneventful without any evidence of recurrence.

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A 2-month-old girl admitted to hospital because of a tumor shadow in the left upper lung field on the chest X-rays. The chest CT scan showed the tumor was in the left upper mediastinum. Left thoracotomy was performed to resect this tumor, which was dignosed as extralobar pulmonary sequestration. The arterial supply of this sequestrated lung tissue derived from a branch of the left pulmonary artery and venous drainage was into the left superior intercostal vein. We reviewed Japanese literatures and could not find such a case until now.

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An 8-year-old boy with intestinal malrotation is reprted on herein. He was admitted to our hospital because of abdominal pain with bilious vomiting. In an upper GI series, a complete obstruction at the duodenal 3rd portion and the dilatation of the proximal duodenal loop, which looked like a C-loop, were recognized. A barium enema showed no abnormal signs. In a second upper GI series, he was diagnosed as having intestinal malrotation due to the finding of an abnormal position of the duodeno-jejunal loop running towards the right side. He was treated successfully with Ladd's operation with an incidental appendectomy. The postoperative course was satisfactory and no sympotoms have recurred. We reviewed the diagnostic procedures of intestinal malrotation in older children. Although the upper GI series is favored by most pediatric surgeons and radiologists, we must recognize and pay attention to false negative findings such as those seen in our case.

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A 3-year and 2-month old boy, who had received chemotherapy and surgical treatment for his dumbbell type ganglioneuroblastoma discovered by the urinary mass screening program performed at 6-month old of age, developed renal cell carcinoma two years after the last course of the chemotherapy which included the alkylating agent, cyclophosphamide. The regional lymph nodes were involved, then right nephrectomy and lymph node dissection were performed. Pathological findings revealed renal cell carcinoma with clear cell type. Interferon-α was administered as post operative therapy, and the patient has been well with no evidence of recurrence nor metastasis during 8 months after nephrectomy.

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A 2-year-old girl was admitted with mobile abdominal mass. Abdominal X-ray showed a large lucent mass displacing the bowel loop laterally. CT and MRI showed non-homogeneous and fat-density mass which contained water-density area. Tumor was arising from mesentery of the terminal ileum and covered with thin capusule. The tumor was totally removed. The cut surface demonstrated yellowish, lobulated appearance and contained some myxoid tissue. Histologically it was formed by lobules composed of lipoblasts and diagnosed as lipoblastoma. Follow-up after three years showed no evidence of recurrence.

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Non-operative approach for various degrees of bile duct stricture was reported. Case 1: A 3y-old boy had a history of a partial liver transplantation after failed Kasai operation. He developed biliary stenosis 2 years after the transplantation. Case 2: A 9y-old girl, who had undergone hepatic portoenterostomy 14 days after T-tube drainage for perforated choledochal cyst, was diagnosed as intrahepatic cholelithiasis and biliary stenosis at the anastomosis 5 years after the operation. Balloon dilatation was performed in these two cases through the percutaneous and transhepatic access to the bile duct. Case 3: A 4y-old girl with 4 previous revisions for hepatic portoenterostomy was diagnosed as bile cyst due to obstruction at the porta hepatis. She underwent exteriolization of the Roux-en-Y jejunum. Through this exteriolization, re-communication was achieved using flexible puncture needle for TIPS and balloon dilatation was performed. All of these patients have been free from the biliary obstruction and repeated episodes of cholangitis. These procedures are proper alternatives to surgical interventions in children, avoiding potentially problematic re-laparotomy.

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Advanced neuroblastoma in childhood, especially with amplification of the N-myc gene, is associated with poor prognosis. Therefore, multimodal therapy is felt to be necessary for curing this tumor. We have experienced two cases with advanced neuroblastoma treated with high dose myeloablative chemoradiotherapy (VP-16, L-PAM, THP-ADR, plus 10-12 Gy fractionated total lymphoid irradiation), followed by peripheral blood stem cell transplantation (PBSCT) In those PBSC, we could not detect neuroblastoma cells by indirect immunofluorecence using anti-neuroblastoma monoclonal antibodies. One case died of DIG, however, the other has been alive in complete remission for 37 month.s posttransplant. As shown in these two cases, PBSCT may be a useful supportive method for patients ineligible for autologous bone marrow transplantation due to bone marrow metastases.

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