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Mesenchymal hamartoma of the liver (MHL) is an uncommon benign tumor found primarily in children younger than 2 years of age. We report a rare case of MHL with a daughter nodule and atypical histological findings in a 14-month-old girl. On admission, computed tomography, magnetic resonance imaging, and angiography showed a solid hypovascular mass with a central cystic area in the liver. Laparotomy revealed a tumor, 8 cm in size, occupying segment 5 and parts of segments 4 and 6 of the liver, and a small nodule, 10 mm in size, in segment 7. Thus, we performed a partial hepatic resection (S4-6) and tumor extirpation (S7). The histological findings of both tumors were the same, but atypical of MHL. Recent studies on the pathogenesis of this tumor have found neoplastic features such as genetic anomalies and malignant transformation. These findings suggest that the conventional approach of completely resecting the tumor whenever possible is the best treatment.

But: II n'y a aucune etude nationale pour les patients avec un rhabdomyosarcome au Japon. Le but de cette etude est d'analyser l'etat actuel du traitement et du devenir de ces rhabdomyosarcomes. Patients et Methodes: De 1982 a 1996, 79 rhabdomyosarcomes etaient enregistres par ce groupe d'etude pour les tumeurs solides malignes dans la zone de Kyushu. Les facteurs pronostiques et les traitements etaient analyses, bases sur la survie a 5 ans. Le staging etait fait en accord avec la classification du groupe rhabdomyosarcome (IRS). Resultats: La survie a 5 ans pour tous les patients etait de 39,1 %. Le taux de survie pour chaque facteur etait le suivant: 1) rapport avec le groupe: 77,8% pour le groupe I, 51,9% pour le groupe II, 33,7% pour le groupe III et 20,2% pour le groupe IV; 2) en fonction de la localisation initiale: 56,3% pour tete et cou, 43,8% pour le tronc, 12,5% pour les extremites, 58,3% pour le pelvis et la localisation genito-urinaire et 30,5% pour les autres; 3) histologie: 35,8% etaient de type embryonnaire, 36,8% etaient de type alveolaire. Conclusion: Quoique le devenir de cette etude soit mediocre, pour ameliorer le devenir, une nouvelle etude nationale pour le rhabdomyosarcome doit etre realisee au Japon.

The survival outcome for patients with hepatoblastoma normally depends on the resectability of the tumor. In Japan, the pre and/or postoperative chemotherapy protocol using a combination of cisplatin (CDDP) and tetrahydropyranyl-Adriamycin (THP-ADR) has been the standard treatment since 1991. This study aims to assess exactly what influence the establishment of this chemotherapy protocol has had on both the tumor resectability and the outcome of patients with hepatoblastoma.From 1982 to 1997, 60 patients with hepatoblatoma were treated in the Kyushu area, Japan. Based on the pretreatment extent of disease (PRETEXT), the outcome and tumor resectability were compared between group A (1982 to 1990, n = 27, PRETEXT I:5, II:8, III:6, IV:8) and group B (1991 to 1997, n = 33, PRETEXT I:9, II:9, III:5, IV:10).The 5-year survival rates (group A and group B) were 33% and 73% for all cases (P.01), 100% and 89% for PRETEXT I, 38% and 89% for II (P.05), 17% and 80% for III (P.01), and 0% and 40% for IV (P.01), respectively. The 5-year survival rates for patients with metastases were 0% for group A (n = 5) and 57% for group B (n = 7; P.01). The rates of a complete resection of primary tumor were 48% for group A and 67% for group B. In particular, a significant difference was found regarding the complete resection rate between groups A and B in the patients with PRETEXT III (17% for group A and 80% for group B; P.01). In the patients with an incomplete tumor resection (14 for group A, 11 for group B), the 5-year survival rates were 0% for group A and 45% for group B (P.01).The optimal chemotherapeutic regimen of CDDP and THP-ADR was thus found to greatly contribute to the improved survival rate of hepatoblastoma patients. Preoperative chemotherapy resulted in an increased resectability of the tumor, whereas postoperative chemotherapy played an important role in the increased cure rate of cases with either an incomplete tumor resection or metastasis. However, refractory cases with PRETEXT IV or metastasis may still require the development of an even more effective treatment modality, including the use of blood stem cell transplantation.

小児消化器患者の経腸栄養では, n-6系またはn-3系多価不飽和脂肪酸の含有量が高い栄養剤(n-6PUFA-EN, n-3PUFA-EN)のいずれが,蛋白・脂質代謝,腸粘膜のintegrityに関して有用かは未だ明らかでない.今回,小児消化器術後患者11例を対象に, n-6PUFA-ENからn-3PUFA-ENに変更した栄養管理を行い,血清脂肪酸分画, albumin, rapid turnover protein,血中diamine oxidase (DAO)活性を, n-3PUFA-EN投与前後に測定し検討した.その結果,栄養剤の変更によりn-6/n-3比は15.1から6.4に低下した. EPA/AA比, prealbumin, retinol binding protein濃度, DAO活性はいずれも有意に上昇し正常化した. n-3PUFA-ENの投与は,蛋白・脂質代謝のみならず,小腸粘膜のintegrityを維持するのに有用であると考えられた.

The beneficial effects of high-monounsaturated fat (high-MUFA) diets on diabetic patients have been reported, whereas studies concerning the effects on animals have been few. Although experiments on animals should be useful in elucidating underlying mechanisms, it is not clear even whether there are benefits of a high-MUFA diet in animals. This study examined the short-term effects of a high-MUFA diet on normal and genetically diabetic mice. The high-MUFA diet supplied 38% of the total calories as fat (26% from MUFA), while a regular diet was 13% fat (3% from MUFA). Normal C5 7BL/6J and diabetic C57BL/KsJ-db/db mice were fed either the regular or the high-MUFA diet for 1 wk. Serum glucose and lipid levels were then measured. In normal mice, hepatic triglyceride production was also compared between the two dietary groups using the Triton WR1339 method. An oral glucose tolerance test was conducted on the diabetic mice. After 1 wk of feeding to normal mice, the high-MUFA diet was seen to lower serum triglyceride levels and reduce hepatic triglyceride production in comparison with the regular diet; it is suggested that the lowering of triglyceride consists of mechanisms including reduced hepatic triglyceride production. When diabetic mice were fed the high-MUFA diet with a controlled caloric intake, the serum glucose levels lowered without an accompanying deterioration in lipid metabolism and the impaired glucose tolerance was ameliorated. This study demonstrates that a high-MUFA diet can lower serum triglyceride levels in normal mice and improve disorders of glucose metabolism in diabetic mice.

慢性肝機能障害に対するcoenzymeQ10(以下CoQ10)経口投与の治療効果を検討する目的で,胆道閉鎖症術後12例を対象にCoQ10を1.6mg/kg/day, 100日間投与した. CoQ10の投与前後に,血中superoxide dismutase (SOD)活性, manganese (Mn)-SOD濃度,尿中8-hydroxydeoxyguanosine, 8-isoprostane濃度,肝機能を測定した.また,健常小児6例,喫煙成人6例の計12例を対照に投与前値を比較した. CoQ10の投与前,全例でSOD活性は高値で, insulin-like growth factor (IGF)-1, Type IV collagen値の異常5例はSOD活性, Mn-SOD濃度が異常高値を呈した. Mn-SODが200ng/mL以上の7例は, 200以下の症例に対し尿中酸化ストレスマーカーが有意に高値を呈した. Mn-SODはALT, AST, IGF-1値と強く相関した. CoQ10投与前の酸化ストレスマーカーはすべて,対照群より有意に高値を呈した.投与後は酸化ストレスマーカー,肝機能が改善傾向を呈し, CoQ10投与は酸化ストレスによる細胞傷害の防御に有用と考えられた.

The purpose of this study is whether the fungal deoxyribonucleic acid (DNA) examination is useful as a sensitive parameter for pediatric surgical patients with mycosis. The eleven episodes of five cases (4 cases; progressive liver disease after biliary atresia operation, 1 case; short bowel syndrome and long term total parenteral nutrition with megacystis microcolon intestinal hypoperistalsis syndrome) with mycosis were divided into two groups according to the difference of therapeutic protocols. The sensitivity of fungal DNA examination, serum Candida antigen level, plasma beta-D glucan level, and blood culture were evaluated at the onset of infection and at the quit of antifungal medication under the protocols respectively. The duration of medication and the medication free interval in two groups were compared. The 6 episodes (3 cases) were diagnosed and treated under the protocol not including fungal DNA examination, while the 5 episodes (2 cases) under the protocol including fungal DNA examination. The occurrence rate was not significant. The sensitivity of fungal DNA examination was complete, but others were not. Using the fungal DNA examination, the duration of medication became significantly short. We conclude that the fungal DNA examination could be a sensitive parameter not only to start but to quit antifungal medication in pediatric patients with mycosis.

Nesidioblastosis is a rare disorder in pediatric surgery. It is caused by hypertrophy and hyperplasia of the islands of Langerhans, and can lead to persistent hyperinsulinemic hypoglycemia. If appropriate treatment is delayed there is a high risk of the development of cerebral palsy, impaired mental development, epilepsy or other forms of irreversible brain damage, so early detection and early treatment to stabilize serum glucose levels is essential. Initial treatment consists of nutritional management with administration of an adequate amount of calories along with drug therapy using diazoxide. In cases that are resistant to drug therapy a subtotal pancreatectomy is performed. Conventional recommended practice has been to perform a 95% pancreatectomy, however this can lead to the onset of diabetes due to abnormal pancreatic endocrine function. We report here a case of nesidioblastosis in which we performed an 85% pancreatectomy. Postoperative clinical course has been excellent, with no recurrence or impaired glucose tolerance during the four years since the operation.

褥瘡患者2症例に対して,創傷治癒促進因子である亜鉛,銅, n-3系脂肪酸高含有の経腸栄養剤を用いた栄養管理を行った. 症例1は軟菜全粥食の摂取にもかかわらず褥瘡が改善しないため,蛋白合成,創傷治癒に必要な栄養素の投与目的で,半消化態経腸栄養剤に変更した.エネルギー,蛋白,アルギニン投与量に変化はなく, n-3系脂肪酸,亜鉛,銅の投与量が増加して第6次改定の栄養所要量の摂取基準を満たし,蛋白合成能および脂質代謝の改善に伴って,治療後8週目には褥瘡は治癒した.症例2は高度の低栄養が認められ,経腸栄養剤の投与量を増加させて,摂取基準に見合うn-3系脂肪酸,亜鉛,銅の投与が行え,蛋白合成能および脂質代謝の改善に伴って治療後10週目には褥瘡は治癒した. 難治性の褥瘡患者の治療においては,エネルギーや蛋白の投与量のみでなく,蛋白合成能の改善や創傷治癒に必須となる栄養素の投与組成を考慮した栄養管理が重要といえる.

The prognosis of mediastinal neuroblastoma has been reported to be better than for other neuroblastomas. The reason for this is however not clear. Furthermore, a comparison between mediastinal neuroblastoma and the other neuroblastomas has been rarely reported so far. In this study, the characteristics of mediastinal neuroblastoma (84 cases) are investigated and compared with those of other neuroblastomas (440 cases). Regarding clinical factors, the age distribution and the rate of cases detected at mass screening were similar in both groups. According to Evan's staging system, the rates of early stage (I, II) were 62% in the mediastinal neuroblastoma and 38% in the other neuroblastomas (p

Since 1985, a nationwide program of mass screening for neuroblastoma has been available for 6-month-old infants throughout Japan. From 1985 to 1993, the authors studied 285 patients with neuroblastoma among their regional population of 15 million. There was an increase in the total number of patients per year in comparison to the previous 6-year period (1979 to 1984). However, no significant difference was noted in the number of patients older than 1 year or in the incidence of advanced-stage (stages III and IV) unscreened cases. The majority of neuroblastomas in the screened group showed favorable biological factors, even in the advanced stages. However, there was a small group with histologically and/ or biologically unfavorable factors; five of 115 had amplified N- myc oncogene, four of 74 showed unfavorable Shimada histological findings, and three of 33 had an unfavorable DNA ploidy pattern. One case from this group with unfavorable factors died of the tumor. 3) Thirty-eight cases were negative at the time of mass screening, but later presented with neuroblastoma. Most of them were diagnosed between 1 and 3 years of age, and 30 of the 38 cases (78.9%) were advanced stage with unfavorable prognostic factors. Thus, the authors conclude that mass screening at 6 months can detect a selected population of infants with neuroblastoma; some of the tumors may represent subclinical masses destined for spontaneous regression. However, some tumors with unfavorable factors have been detected by mass screening before progression and/ or dissemination. Infants in this group are considered to benefit most from early diagnosis and treatment.

An annual survey of the current national status of home parenteral nutrition (HPN) in Japan was begun in 1991, with a total of 231 registered patients from 142 institutions providing adequate data for evaluation and analysis. HPN was given for an average of 683±764 days to 93 patients with malignant diseases and 138 with benign disease, including 53 with inflammatory bowel disease and 79 with short bowel syndrome, 107 (46.3%) of whom were successfully rehabilitated. By the end of 1990, 61% of the patients investigated were still on HPN, 7% had already completed HPN, and 26% had died, the deceased accounting for 54% of the patients with malignant disease and 7% of those with benign diseases. A total of 321 catheters had been used by all 231 patients, the most common being the subcutaneously implanted type, accounting for 33% of all catheters. By the end of 1990, 32% of these 321 catheters were still in place, 18% had been removed on the termination of HPN and 44% had been removed due to complications of total parenteral nutrition, including 20% for catheter fever. Rehospitalization was required in 62% of the patients, the cause being HPN-related in 21% of the total patients. Metabolic complications were experienced by 60% of the patients, but none of these were severe although 6% required hospitalization. Thus, the total population of HPN patients and the success rate of rehabilitation in Japan were close to those reported in Europe, while the indications for HPN and its outcome were similar to those documented in the USA OASIS report, except that the incidence of rehospitalization from HPN-related causes in the Japanese survey was lower.

小児の腸間膜嚢腫は比較的稀な疾患である.今回私共は,間歇的腹痛を主訴とした小児の腸間膜嚢腫(リンパ管腫)の1例を経験した.症例は5歳,男児.各種画像検査にて骨盤内に腫瘍を認め,摘出術を施行した.腫瘍はTreitz靱帯より肛門120cm,回盲部より口側340cmの部位の腸間膜に存在し,大きさは13×10×8cmであった.術後病理組織学的にlymphangiomaと診断された.

We experienced a case of malignant lymphoma of the duodenum presented as obstraction of the same organ. A 23-year-old female complained of nausea and vomiting. On admission, laboratory data revealed microcytic and hypochromic anemia and a decreased number of white blood cell, but its analysis and bone marrow were normal. Occult blood was admitted. Hypotonic duodenography and gastrointestinal fiberscopy showed that the third portion of the duodenum was completely obstructed by a tumor with irregular surface. The tumor was diagnosed as Non-Hodgkins' lymphoma of medium sized cell type by histological examination of the biopsy specimen. Laparotomy was performed, and an elevated tumor with irregular surface which ran up the whole-surcumference was seen at the third portion of the duodenum. Furthermore, metastases were seen in the mesocolic lymphonodes, but the liver and spleen were intact. On the following histologic examination, the preoperative diagnosis was confirmed. No monoclonality was observed in the tumor cell immunoglobulin. Surface phenotype studies of the tumor cells were all negative. ACOP theraphy was performed ten times from the second week after the operation. She was alive and well 1 year after the operation.

A 25-year-old male who had no significant medical history presented abrupt onset of high-grade fever and chills without noticeable trigger. The patient sought for medical attention for subsequently developed dyspnea and chest pain. Radiological examinations revealed bilateral lung peripheral multiple opacities, some of which were cavitating, suggesting of septic pulmonary emboli (SPE). Isolation of Staphylococcus aureus in blood and sputum culture confirmed the diagnosis. Extensive examinations disclosed neither underlying immunocompromising conditions nor infectious foci, which are usually notable in patients with SPE. The present patient illustrates that there are patients with SPE in whom underlying conditions or infectious foci are difficult to determine, and that suspicion of the disease based on characteristic radiological findings is critical for appropriate management in those patients.

Background/Purpose Since 1996, the standard treatment of Wilms tumors in Japan has been based on the regimen of the Japanese Wilms Tumor Study. However, in Japan, there have been no reports about Wilms tumors that analyzed the clinical features and patient outcome in a large series until now. This study aims to assess the clinical characteristics of patients with Wilms tumor with a favorable histology from a retrospective standpoint in the Kyushu area in Japan and, furthermore, to analyze the historical changes of clinical features and outcome from the 1980s to the 1990s. Methods Between 1982 and 1996, 90 cases of Wilms tumors with a favorable histology were registered in the Kyushu area. Regarding the clinical feature and outcome, they were divided into 2 groups (group A, 1982-1989, n=50; group B, 1990-1996, n=40). The outcome was analyzed based on the 5-year overall survival rate. Results The clinical features (age, sex, initial symptom, location, stage) demonstrated no definite differences between group A and group B. Regarding the operation, the rate of an initial complete resection in the early stages was significantly higher in group B than in group A. All stage V cases in group B undewent a bilateral tumor biopsy instead of a radical nephrectomy as the initial operation. The 5-year overall survival rate throughout the whole period was 87.8%, whereas the rates were 84.0% for group A and 90.0% for group B ( P = NS), respectively. Of particular note, the outcome of patients with stage I and stage V in group B substantially improved in comparison to that in group A. However, in advanced cases, no significant improvement in the outcome was noted. Conclusions This is the first report about the clinical features and outcome for Wilms tumors with a favorable histology in Japan from the 1980s to the 1990s. The present study suggested that in the early-stage cases, an initially complete resection followed by standard postoperative chemotherapy substantially improved the outcome of the patients in group B. In the stage V cases, the performance of renal salvage surgery may have positively contributed to the improvement in the outcome in group B. However, in the advanced stage cases, no definite improvement was noted. In the future, an improved efficacy of the treatments for Wilms tumors based on the standard protocol established by the Japanese Wilms Tumor Study in 1996 is expected in Japan.

We experienced a very rare case of a gastrointestinal perforation due to multiple ileal polyps. The patient was a 2-day-old boy who weighed 1,430 g at birth. At neonatal day 2 subdiaphragmatic free air was noted and an emergency operation was performed. During surgery a distended ileum approximately 5 cm in size was found approximately 40 cm distal from the ileocecum, and a perforation was discovered in the distended portion of the intestine. Approximately 8 cm of the ileum was resected, including the distended portion of the intestine, and an ileostomy was performed. Examination of the resected specimen revealed three pedunculated polyps with a perforation proximal to the polyps.

It is not unusual for patients with severe motor and intellectual disabilities to present with eating and swallowing disorders, and such patients often require long term enteral nutrition. These patients tend to receive all their nutrients in the form of a single nutrient solution that is administered over a long period, and there are concerns about the impact of the composition of these nutrient formulas on patient health. Therefore, it is very important that adequate care be taken when selecting a nutrient formula for patients of this type. In the present study we administered two types of enteral nutrient solutions and examined the effect of the differences in nutrient composition on the degree of oxidative stress experienced by human patients. Subjects were 5 patients (mean age: 27.2 years; male/female ratio: 4:1) with severe motor and intellectual disabilities who were incapable of oral feeding, and who were receiving long term enteral nutrition. The subjects were administered a standard elemental diet for 2 months, and this was followed by administration of an n-3 polyunsaturated fatty acid enriched enteral nutrient solution for three months. Results showed that the n-3 polyunsaturated fatty acid enriched diet improved serum concentrations of trace elements, protein synthesis, and the balance between n-6 polyunsaturated fatty acids and n-3 polyunsaturated fatty acids, which are essential fatty acids. At the same time oxidative stress was reduced, and serum IgE levels declined significantly. Patients with severe psychophysiological disorders often suffer from repeated bouts of pneumonia due to immune system suppression, and there are reports that such patients are subject to increased allergic diathesis. The present results indicated that the in vivo membrane damage induced by oxidative stress may be closely related to the onset of these disorders. The results also suggested that in addition to attaining an adequate understanding of the effect of each of the various nutrients in enteral nutrient formulas, it will also be important to consider not only trace elements but also fatty acid composition in the nutritional management of patients with such disorders.

Despite the numerous approaches described for the management of neonates with “long gap” esophageal atresia, controversy still exists as to the preferred method. Delayed primary anastomosis is probably the most frequently adopted practice but often the native esophagus is abandoned. We report a case of a 2.98 kg newborn with pure esophageal atresia . Although the elongation of the distal esophageal pouch by mechanical bougienage was initiated at 1 year and 8 months, a successful tension-free anastomosis with minimum dissection of the lower esophagus was performed at 2 years and 7 months. Her postoperative quality of life has been quite excellent. This report emphasizes that a tension-free anastomosis without operating on the lower esophagus and stomach is essential for the treatment of long-gap esophageal atresia.

The aims of this study were to determine the normal values of plasma diamine oxidase (pDAO) activity in children and to examine the influence of several factors (nutritional management, dietary fiber, and chemotherapy) on pDAO activity. The activity of pDAO was measured in 138 healthy children with minor surgical conditions such as inguinal hernia or undescended testis. In order to define normal values patients were subdivided into 5 groups according to age. Next, changes in pDAO activity under different nutritional conditions were studied in 14 patients with adhesive ileus. The influence of chemotherapeutic drugs on pDAO activity was also studied in 19 neuroblastoma patients. I. The normal values of pDAO activity at year1, 1or = years3, 3or = years6.6or = years12, 12or = years were 6.65 +/- 1.75, 7.70 +/- 2.29, 6.53 +/- 1.68, 5.85 +/- 1.87, 5.06 +/- 1.84 units/l, respectively. II. The pDAO activities in patients with ileus were 4.73 +/- 1.02 (total parenteral nutrition), 6.84 +/- 1.18 (enteral, nutrition), 7.62 +/- 0.67 (soluble dietary fiber added enteral nutrition) and 8.82 +/- 1.26 units/l (oral feeding). The difference in pDAO activity at enteral or oral feeding vs. total parenteral nutrition was significant (p.0001). III. The pDAO activity decreased significantly and remained low during the first 4 days after cyclophosphamide administration in neuroblastoma patients. The preadministration of dietary fiber inhibited the influence of cyclophosphamide. Plasma DAO activity was greatly influenced by nutritional management and administration of dietary fiber and/or cyclophosphamide. Plasma DAO activity may be a sensitive marker of intestinal function in children.

We report a case of pancreatic injury, caused by a stab wound, in which ductal injury and wound depth were clearly identified by intraoperative ultrasonography. A 65-year-old woman was emergently admitted to our hospital after stabbing herself in the abdomen in a suicide attempt. Preoperative computed tomography (CT) and laboratory examination revealed liver and pancreatic injury with massive abdominal bleeding and free air. Operative findings included injuries of the stomach, small bowel, colon, liver, and pancreas. The pancreatic lacerations were 1 cm in length, in the body. Intraoperative ultrasonography enabled the diagnosis of a lacerated main pancreatic duct with no damage to the major vessels posterior to the pancreas. Distal pancreatectomy; simple repairs of the liver, small bowel, and stomach; exteriorization of the injured colon; cholecystostomy; gastrostomy; and jejunostomy were performed. The patient recovered and was transferred to a psychiatric hospital 87 days after surgery. In this patient, intraoperative ultrasonography was successfully used to identify the degree of injury to the pancreatic duct, as well as the depth of the stab wound. In conclusion, intraoperative ultrasonography should be routinely performed to detect main pancreatic duct injury in penetrating pancreatic trauma.

Purpose: This study aims to assess the prognostic factors and optimal treatments for malignant germ cell tumors (MGCT) in childhood. Methods: Among 117 MGCT, the clinical features were analyzed. Regarding the histology, there were 89 embryonal carcinomas, 13 dysgerminomas, 4 choriocarcinomas, and 11 others. The prognostic factors and treatments were assessed based on the 5-year survival rate. Results: (1) Stage: 100% for stage I (n = 54), 75.0% for stage II (n = 4), 67.3% for stage III (n = 14), and 54.8% for stage IV (n = 33); Unknown: n=12. (2) Primary site: 93.4% for the testis (n = 52), 86.7% for the ovary (n = 31), 56.9% for the sacrococcygeal (n = 21), and 60.6% for others (n = 12); unknown: n=1. (3) Surgical intervention for primary tumor: 100% for stage I with a complete resection (n = 53), 78.4% for stage III, IV with a complete resection (n = 26), and 33.3% for stage III, IV with an incomplete resection (n = 21). (4) Type of chemotherapy for the stage III and IV: 83.9% for the PVB (cisplatin, vinblastin, bleomycin; n=13), 66.7% for the VAC (vincristine, actinomycin D, cyclophosphamide; n=6), and 47.1% for other regimens (n = 25). Conclusions: An early stage, a diagnosis under 1 year of age and a primary site in the gonads were favorable prognosis factors, whereas histologic findings of choriocarcinoma and liver or lung metastasis were unfavorable. Radical complete resection alone is a sufficient treatment for localized MGCT. The PVB regimen is optimal chemotherapy for advanced MGCT; however, high-risk cases still may require more aggressive treatment. J Pediatr Surg 37:1703-1706. Copyright 2002, Elsevier Science (USA). All rights reserved.

BACKGROUND It is said that most cases detected by neuroblastoma mass screening at 6 months of age tend to have a favorable clinical course after a surgical resection either with or without mild chemotherapy. However, a few cases have an unfavorable outcome. In the current study, the authors analyzed the clinical and biologic characteristics for recurring neuroblastoma in mass screening cases. METHODS In 245 cases detected through mass screening in the Kyushu area in Japan, the clinical data and biologic features (N-myc status, DNA ploidy, Shimada histology, neuron-specific enolase (NSE), ferritin) were investigated, whereas, in particular, the data for recurring cases also were analyzed. RESULTS Of 245 cases, 28 tumors had one or more biologically unfavorable prognostic factors, and 6 patients experienced recurrence. Three of the six patients with recurring disease underwent a complete resection of the primary tumor, whereas three cases had undergone an incomplete resection of the tumor. Regarding the initial chemotherapy, three cases received mild chemotherapy, two cases received no chemotherapy, and one case had high-dose multidrug chemotherapy. Regarding biologic prognostic factors, four of six cases with recurring disease had one or more unfavorable factors, whereas two cases had no unfavorable factors. Regarding the outcome after recurrence, four cases are CR, one case has a stable residual tumor, and one case died of disease with N-myc amplification. CONCLUSIONS Most neuroblastomas detected by mass screening at 6 months of age have biologically favorable factors. However, approximately 10% of the cases had one or more unfavorable factors and thus might have a higher risk of recurrence than the patients with no unfavorable factors. Conversely, some cases with recurring disease had no unfavorable factors; however, the reason for this is still unclear. A long-term follow-up for mass screening cases is important, and it also might be necessary to research the established biologic factors and identify other new prognostic factors. Cancer 2001;92:349–53. © 2001 American Cancer Society.

In spite of many different kinds of chemotherapy for neuroblastoma, the prognosis for advanced neuroblastoma remains unsatisfactory. In particular, the outcome of advanced neuroblastoma with high copies of the N-myc gene tend to be poor. Therefore, the new high-dosage combined chemotherapy regimens for advanced neuroblastoma based in part on the N-myc amplification status has been utilized in the Kyushu area of Japan since 1991. This study aims to investigate whether these new regimens based in part on N-myc amplification have improved the survival rate of stage III and stage IV patients in comparison with the old regimens.Between 1983 and 1995, 77 patients over 1 year of age and with stage III or IV neuroblastoma were registered in the Kyushu Area. Between 1983 and 1990, 49 patients received 1 of 2 combined chemotherapy regimens consisting of cyclophosphamide, cisplatin plus VM-26, and Adriamycin plus DTIC. Since 1991, two new regimens (New A1 and A3) have been administered based on the N-myc amplification status in a total of 28 patients. The New A1 regimen, which consists of cyclophosphamide, cisplatin, Adriamycin, and VP-16 has been administered in cases of less than 10 copies of N-myc, whereas the A3 regimen, consisting of a higher dose of cyclophosphamide, cisplatin, Adriamycin, and VP-16, has been administered in cases of more than 10 copies of N-myc. The survival rate was then compared between the old regimens and the new regimens.The 3-year survival rate (61.5%) for patients treated by the new regimens was significantly higher than that (32.7%) for patients treated by the old regimens (P.01). Regarding the 24 cases of more than 10 copies of N-myc, the 3-year survival rate (35.9%) of the 13 patients treated by the A3 regimen was higher than that (0%) of the 11 patients treated by the old regimens (P.05). However, in the 19 stage IV patients treated by the new regimens, the 3-year survival rate (11.1%) of the 9 cases of more than 10 copies was significantly lower than that (77.8%) of the 10 cases of less than 10 copies of N-myc (P.01).These results suggest that high-dose combined chemotherapy based in part on the N-myc amplification status significantly improved the prognosis of patients with advanced neuroblastoma. However, stage IV patients with N-myc amplification still require a more effective treatment modality.

Hepatic hemangiomas are benign tumors, and therefore minimally invasive treatment such as irradiation or steroid therapy is often recommended. However, in patients who have a hemangioma complicated by Kasabach-Merritt syndrome, surgical intervention should also be considered because of its confirmative therapeutic effect. We present herein the case of a 32-day-old male infant in whom a huge hepatic hemangioma associated with Kasabach-Merritt syndrome was treated by surgical intervention together with strong antidisseminated intravascular coagulation therapy.

Duplication of the biliary tract is extremely rare. In fact, to the best of our knowledge, only four previous reports of pediatric patients with this disease have been documented in the Japanese literature. This anomaly was diagnosed by perioperative endoscopic retrograde cholangiopancreatography (ERCP) in three of these patients, and incidentally during surgery for congenital biliary dilatation in the other one. We report herein a case of biliary tract duplication which was diagnosed by T-tube cholangiography, only in the oblique view, after cholecystectomy and choledochotomy with T-tube drainage. The patient was a 13-year-old girl who was initially diagnosed as having choledocholithiasis based on the results of preoperative and intraoperative cholangiography; however, a postoperative T-tube cholangiography revealed residual stones. At this point, duplication of the biliary tract was diagnosed only in the first oblique view. Reoperation was carried out 26 days after the first operation. The biliary tract was dissected completely from the pancreaticobiliary junction to the left and right hepatic ducts in the porta hepatis and found to be duplicated from the level of porta hepatis to the suprapancreatic area. We report our experience of this case, followed by a discussion on the relevant literature.

Background/Purpose: A statistical analysis of the mass screening for neuroblastoma in Japan based on a population study rarely has been reported. This study aims to evaluate retrospectively the effectiveness of mass screening at 6 months of age using the available population data. Methods: The data on the neuroblastoma cases registered by the Committee for Pediatric Solid Malignant Tumors in the Kyushu area were analyzed based on both screened and unscreened populations in the Kyushu area. Results: From 1988 to 1992, the cumulative incidence of neuroblastoma in children less than 5 years of age was 82 in 484,599 for screened children, and 11 in 92,966 for unscreened children, respectively. Fourteen of the 82 screened patients had negative findings at 6 months of age (MS-negative cases). No significant difference was observed in the cumulative mortality rates from neuroblastoma in children younger than 5 years of age between the screened children and the unscreened children. Six of seven patients who died among the screened children were MS-negative cases with stage III or IV disease. In addition, no significant difference was found in the cumulative mortality rates from the neuroblastoma cases in patients less than 5 years of age between the children screened from 1988 to 1992 (7 of 484,599) and all children from 1980 to 1984 (14 of 668,084). Conclusions: These findings suggests that the majority of the patients detected by mass screening had a favorable prognosis, and, mass screening in Japan for children less than 6 months of age was not observed to reduced the incidence and mortality from neuroblastoma. Therefore, mass screening at 6 months of age was not found to improve substantially the prognosis of patients with unfavorable neuroblastoma identified over 1 year of age, which is the primary purpose of such mass screening for neuroblastoma.

Infants with neuroblastoma are known to have a better prognosis than older children. In Japan in 1985, mass screening for neuroblastoma in infants aged 6 months was introduced. With this policy, there has been an increase in the number of patients seen with neuroblastoma between 6 and 11 months of age. In a previous report the authors described the management and prognosis of infants with disease detected by mass screening, but there is still little information regarding the strategies of management for infants with neuroblastoma aged less than 6 months. The authors analyzed the data regarding 27 patients aged less than 6 months registered in their region (population 15 million) from 1985 to 1992, and compared it with that of the previous 8-year period. In the younger age group, there was a significantly higher rate of advanced disease stages (III and IV). In spite of the variation in treatment related to the choice of individual institutions, infants with stages I, II, and III disease had a good outcome, suggesting that aggressive chemotherapy is not necessary unless poor prognostic factors are present. One patient with stage IV disease died of disseminated disease and one with stage IVs and 22 copies of N-myc oncogene also died of tumor relapse in spite of aggressive chemotherapy. It is therefore concluded that the prognosis in infants with stage IV and IVs neuroblastoma under the age of 6 months is not as good as had previously been believed, and that such patients, therefore, require special consideration.

Multiple Organ Failure that occurred in 35 cases out of a total of 1498 cases after intestinal surgery were studied by an analysis of the clinical and statistical data from the past 5 years (1984-1988). 1. The incidence of MOF after intestinal surgery was 2.3%. 2. Twenty-eight of MOF resulted from severe infections. 3. The incidence of infection was highest after esophageal operations. 4. Hepatic failure was the most common type of primary organ failure and renal failure was the second most common. 5. The prognosis for MOF was very poor with a survival rate of 8.7%.

Changes in acid-base balance, water-electrolyte metabolism and CVP were evaluated in 315 patients with postoperative ileus during the past 21 years. Infusion therapy for postoperative ileus is described.

A prospective, randomized, controlled trial of nutritional effects of branched-chain-enriched amino acid (BCAA) solution was undertaken in 173 surgical patients with gastric cancer. Eighty-six and 87 patients underwent subtotal and total gastrectomy, respectively. The effects were evaluated in total parenteral nutrition (TPN) in an isocaloric/isonitrogenous setting where the major difference between the group was the amount of BCAA received. Each 80 patients in the control and the BCAA groups completed the trial. The group receiving BCAA-enriched amino acid solution demonstrated a statistically significant improvement on days 2 and 3 in nitrogen balance in patients with total gastrectomy. Three-methyl-histidine excretion gradually decreased after day 1, and the values on day 7 were significantly lower than those on day 1 in the BCAA group in both those receiving subtotal and total gastrectomy. There were no significant differences of serum albumin and rapid turnover proteins between the control and BCAA groups in both those receiving subtotal and total gastrectomy. Plasma BCAA level and BCAA to aromatic amino acid (AAA) ratio were significantly higher, and AAA level was significantly lower in the BCAA group than in the control group. There were no serious complications encountered during the observation period in both groups. These results indicated that a BCAA-enriched amino acid solution can improve metabolism and maintains good nitrogen retention without increasing side effects as compared with a conventional amino acid solution for nutritional support of patients who have received subtotal or total gastrectomy.

Lipid and protein metabolism were examined in 36 patients who received parenteral hyperalimentation following total gastrectomy by dividing them into three groups receiving 1) glucose + amino acid (group G), 2) glucose + lipid emulsion + amino acid (group G + Lip) and 3) an infusion solution (GFX) consisting of glucose, fructose and xylitol combined in the ratio of 4:2:1 plus amino acid (group GFX). The results were as follows:Postoperative levels of triglyceride and phospholipid recovered favorably in group GFX. Preoperative levels of essential fatty acid were maintained in group G + Lip alone, and ω-3 fatty acid decreased in all three groups. C-peptide increased until the 3rd day in all patients, and decreased satisfactorily thereafter in groups G + Lip and GFX. No difference was found in N-balance among three groups, and rapid turnover of protein recovered best in group G + Lip, followed by gorup GFX and group G, in that order.GFX proved equally effective with glucose used alone as a carbohydrate source for parenteral hyperalimentation, also showing an insulin-saving effect. Protein-saving was further improved by the combined use of lipids.

An adult case of congenital segmental dilatation of the jejunum with malrotation was treated by resecting the dilated tract and performing an end-to-end anastomosis. The progress of this case was reported along with a review of the literature. 1) 9 cases of congenital segmental dilatation of the intestine with malrotation, including this case (Babut et al. 1977; Komi 1974; Kusaba et al. 1983; Rossi 1973; Swenson et al. 1959) have been reported . The relative morbidity for males and females was 2 : 7. The location in the jejunum was high in 6 cases. 2) the morbidity was the highest in infants . The etiology of this disease still remains unclear. 3) Differential diagnosis of this disease from Hiruschsprung's disease in infants and from mechanical ileus in adults is important. 4) With resection of the dilated tract and end-to-end anastomosis, g results can be obtained. This method is recognized as the best treatment. 5) The etiology of this disease is unknown. Physiological and biochemical research must be continued until the etiology is clarified.

This is a case report of two cases of mesenteric cysts in a seven-monthold female and a two-year-8-month-old male, we reviewed and analysed the reported cases in Japan and applied this knowledge to the evaluation and the statistical analysis of our cases.