Mesenchymal hamartoma of the liver accompanied by a daughter nodule: report of a case.

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.

Clinical characteristics and outcome of Wilms tumors with a favorable histology in Japan: a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan.

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.

A young adult patient with septic pulmonary emboli of undetermined origin.

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.

Multiple ileal polyps associated with gastrointestinal perforation in a 2-day-old neonate.

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.

The fungal DNA examination is useful as a sensitive parameter for the initiation and the quit of antifungal therapy in immunocompromised pediatric patients after surgery.

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.

Intraoperative ultrasonography is useful for diagnosing pancreatic duct injury and adjacent tissue damage in a patient with penetrating pancreas trauma.

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.

Effects of a high-monounsaturated fat diet on glucose and lipid metabolisms in normal and diabetic mice.

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.

Efficacy of n-3 polyunsaturated fatty acid enriched enteral nutrient solution in relieving oxidative stress in patients with severe psychophysiologic disorders.

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.

Nesidioblastosis treated successfully by 85% pancreatectomy.

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.

Clinical significance of plasma diamine oxidase activity in pediatric patients: influence of nutritional therapy and chemotherapy.

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 year < 1, 1 < or = years < 3, 3 < or = years < 6.6 < or = years < 12, 12 < or = 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.

The elongation of distal esophageal pouch by mechanical bougienage for a year resulted in a tension-free anastomosis in a patient with long gap esophageal atresia: a case report.

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.