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.

Extracellular Ca2+ uptake by T cells might help to make a diagnosis of acute rejection.

To more non-invasively diagnose acute rejection, we focused on the uptake of extracellular Ca2+ by T cells as a result of the activation of Ca2+ release activated Ca2+ channels. A full thickness of the skin allograft model was established using BN rats as the donors and LEW rats as the recipients, and similar LEW rats as both donors and recipients in the control group. After transplantation, the grafts were staged histopathologically in both rats. The uptake of extaracellular 45Ca pre T cell was measured in the macrophage-treated and non-treated groups, and the ratios between the two groups were calculated and the results were compared according to the post-operative day. No histopathological findings of acute rejection were observed in the control group. The allograft model group showed acute rejection histopathologically beginning on day 2 and increased through day 5. The macrophage-treated model/non-treated model 45Ca uptake ratio (CAR) was significantly higher in the allograft rats on day 2. No significant difference was observed on day 4. Measuring the uptake of extracellular Ca2+ by recipient T cells using donor macrophages might be useful for making a diagnosis of acute rejection.

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.

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.