Congenital hyperinsulinism associated with Hirschsprung's disease-a report of an extremely rare case.

BACKGROUND: Congenital hyperinsulinism (CH) is a rare disease, characterized by severe hypoglycemia induced by inappropriate insulin secretion from pancreatic beta-cells in neonate and infant. Hirschsprung's disease (HD) is also a rare disease in which infants show severe bowel movement disorder. We herein report an extremely rare case of combined CH and HD. CASE PRESENTATION: The patient was a full-term male infant who showed poor feeding, vomiting, and hypotonia with lethargy on the day of birth. He was transferred to tertiary hospital after a laboratory analysis revealed hyperinsulinemic hypoglycemia. The patient showed remarkable abdominal distension without meconium defecation. An abdominal X-ray showed marked dilatation of the large bowel. He was diagnosed with CH (nesidioblastosis) associated with suspected HD. He was initially treated with an intravenous infusion of high-dose glucose with the intermittent injection of glucagon. This was successfully followed by treatment with diazoxide and octreotide (a somatostatin analog). At 8 months of age, HD was confirmed by the acetylcholinesterase staining of a rectal mucosal biopsy specimen, and a transanal pull-through operation was performed to treat HD. At 14 months of age, subtotal pancreatectomy was performed for the treatment of focal CH located in the pancreatic body. His postoperative course over the past 12 years has been uneventful without any neurologic or bowel movement disorders. CONCLUSIONS: Although it is extremely rare for CH to be associated with HD, associated HD should be considered when a patient with CH presents severe constipation.

Laparoscopic fundoplication for a case of esophageal hiatal hernia after gastroschisis repair.

BACKGROUND: Esophageal hiatal hernia and gastroesophageal reflux have been recognized as inevitable complications after the definitive gastroschisis operation. Patients with refractory gastroesophageal reflux require anti-reflux surgery; however, the surgical adhesions may complicate subsequent surgical therapy, especially in the cases treated by staged repair. CASE PRESENTATION: A male infant who showed a severe gastroesophageal reflux due to hiatal hernia after staged abdominal fascial closure of gastroschisis. In spite of continuous conservative management, frequent vomiting and hematemesis had become progressively worse at the age of 8 months. Laparoscopic Nissen fundoplication was attempted and completed with no adverse events. CONCLUSIONS: Laparoscopic fundoplication may be applied, as a first-line approach, for the treatment of gastroesophageal reflux in this difficult group of patients, after the repair of congenital abdominal wall defect.

Association of lymphocyte crossmatch and the outcome of intestinal transplantation in swine.

BACKGROUND: The effect of preformed antidonor antibodies have been demonstrated in various types of solid organ transplantation. However, the significance of anti-donor antibodies in intestinal transplantation remains unclear. The aim of this study is to evaluate the impact that the extent of T cell crossmatch has on the outcome of swine intestinal transplantation. MATERIALS AND METHODS: All studies were performed on outbred domestic male pigs weighing from 15 to 20 kg. Intestinal transplantation was performed orthotopically with an exchange of grafts between white and black pigs. FK506 was administered intravenously (0.1 mg/kg per day, POD 0-7) for immunosuppression. A lymphocyte crossmatch test was performed using the direct CDC crossmatch. The results were considered positive when more than 10% of the donor lymphocytes were killed by the recipient's serum. In addition, 0-10, 11-20, 21-30, 31-80 and 81-100% of the killed lymphocytes were classified as grade 1, 2, 4, 6 and 8, respectively. RESULT: A total of 34 intestinal transplantations were performed. All but one case had positive donor specific T cell crossmatches. The number of grade 2, 4, 6 and 8 cases was 11, 14, 6 and 2, respectively. Although there was a tendency towards a decreased survival according to the grade, the survival rate was not statistically different among each different grade. Moreover, the rates of acute cellular rejection and vascular complications were not significantly different among the four grades. CONCLUSION: These results suggest that the extent of positive T cell crossmatch is not associated with the outcome of swine intestinal transplantation.

Extrarenal Wilms tumor in children with unfavorable histology: a case report.

Extrarenal Wilms tumor is extremely rare, and only 25 cases in children have been reported to date in Japan. A 2-year-old girl presented with a large left lower quadrant abdominal mass. Abdominal computed tomography revealed a retroperitoneal tumor located below the left kidney. At laparotomy, the tumor was encapsulated without evidence of metastasis to other abdominal organs. Pathologic diagnosis of the tumor was extrarenal Wilms tumor with diffuse anaplasia. After complete tumor resection, chemotherapy was administered according to the treatment protocol (Regimen I) of the Japan Wilms Tumor Study Group. Cyclophosphamide and etoposide were administered in combination with vincristine and doxorubicin. Two years after treatment, the patient has had no evidence of recurrence.

Recurrent acute pancreatitis caused by a gastric duplication cyst communicating with an aberrant pancreatic duct.

A 38-year-old woman was hospitalized in August 2007. This visit was her fifth episode of acute pancreatitis. Computed tomography revealed a cystic structure located near the antrum. Communication between this structure and the pancreatic duct was revealed by endoscopic retrograde cholangiopancreatography. Ultrasonography revealed that the cyst wall had a layered structure. Thus, we regarded it as a gastric duplication cyst. We thought that the gastric duplication cyst communicating with an aberrant pancreatic duct was responsible for the recurrent acute pancreatitis. In August 2008, a cyst gastrostomy was performed between the gastric duplication cyst and the stomach. No recurrence of acute pancreatitis has since occurred.

The influence of rejection on graft motility after intestinal transplantation in swine: the possibility of using this method for the real-time monitoring of acute cellular rejection.

BACKGROUND: We have previously reported that rejected allografts show dysmotility, which can be detected by real-time monitoring in swine. We examined the correlation between the motility and the mucosal histology to detect rejection at an early stage by real-time monitoring. METHODS: Intestinal transplantation was performed orthotopically using FK506. The distal segment of the allograft measuring about 20 cm was isolated and exteriorized as "Thiry-Vella" stoma for biopsies. Strain-gage force transducers were attached on a graft for the real-time monitoring of graft motility. The pigs without intestinal transplantation were used as controls (C). The rejection was classified into 4 groups based on the histologic findings: nonrejection, mild rejection, moderate rejection, and severe rejection. Migrating motor complex (MMC) phase 3 was estimated by the following parameters: duration, amplitude, interval, motility index, velocity, and frequency of the propagation. RESULTS: In the nonrejection group, all parameters were almost the same as in C group. In contrast, in the moderate rejection and severe rejection groups, most of the parameters were significantly lower than those in the C group. In the mild rejection group, the contractility of the MMC was not significantly altered, but the frequency of the propagation decreased significantly. CONCLUSIONS: The graft motility detected by the real-time strain-gage method correlated closely to the grade of mucosal histology. This method is therefore considered to be useful for detecting rejection at an early stage by examining the frequency of MMC propagation.

Safe techniques for inserting the hickman catheter in pediatric patients.

The placement of the Hickman catheter in the central veins is thought to be an effective method for providing venous access in various clinical situations in children. The catheter is usually inserted by the percutaneous approach, but in some cases various troublesome complications can occur, such as sheath introducer kinking or damage, in addition to other major ones. Therefore, some modified techniques, using vascular dilators, both to dilate the route and to avoid such complications, have been developed and investigated to obtain a smooth and safe percutaneous insertion of the Hickman catheter in children. A total of 41 Hickman catheters were inserted by the percutaneous method in 41 pediatric patients from 1996 to 2004 in our department. Sixteen catheters were inserted by means of a standard method, using the manufacturer's insertion kit, and 25 catheters were inserted by means of a modified method, namely, using various sized vascular dilators. The length of time for the procedure, the complication rate, and the changes in the serum C-reactive reaction (CRP) levels were then compared between the standard and the modified methods. Those parameters were also compared between a right-side and left-side approach using both methods, to clarify which side was better for the insertion of this catheter. The length of time for the catheter replacement procedure in the standard group was significantly longer than that in modified one. The occurrence rate for both the kinking and small damage to the sheath introducer in the standard group was higher than that in the modified one. The peak of serum CRP in the modified group was significantly lower than that in the standard one. When comparing a right-side and left-side approach, 7 catheters out of 16 were inserted by the right-side approach in the standard group, while 10 catheters out of 25 were done by the right-side approach in the modified group. The length of time for the procedure for the left-side approach was significantly shorter than that for the right-side one in both groups. No difference in technical complications was observed between the two different approaches in the modified group, while complications when using the right-side approach often occurred in the standard group. The peak of serum CRP in the left-side approach was lower than that in the right-side one in both groups. The use of the modified percutaneous method, using various sized vascular dilators and the left-side approach, was therefore found to be useful for the safe and smooth placement of the Hickman catheter in children.

Decrease in hepatic circulation induces hepatic fibrosis in a neonatal piglet model with short bowel syndrome.

BACKGROUND: Regarding the complications associated with short bowel syndrome (SBS), progressive liver failure is one of the most severe complications known to occur: Although several studies have suggested that many factors interactively influence this clinical condition we investigated the relationship between hepatic circulation and hepatic fibrosis using a neonatal piglet SBS model. MATERIALS AND METHODS: This study used the following 4 groups of neonatal piglets: a group with an 80% resection of the small bowel (SBS group), a group with a bypass operation of the small bowel (functional SBS group), a group with only a laparotomy as a sham operation (sham group), and a no operative treatment group (control group). We measured the hepatic circulation just before and after the reconstruction of the intestine, as well as on the 7th and 14th postoperative day. In addition, both blood and hepatic tissue samples were collected to investigate them both biochemically and morphologically. RESULTS: Regarding the biochemical liver function and the tissue blood flow of liver, there were no significant differences among all groups on any investigated days. However, on both the 7th and 14th postoperative days, the portal venous flow in the SBS group was significantly lower than that in other groups. According to a histological analysis, only hepatic samples on the 14th postoperative day showed mild hepatic fibrosis in the SBS group. Regarding the alpha-smooth muscle actin staining findings that expresses active stellate cells, numerous positive cells were found to be distributed in the perisinusoidal space on the 14th postoperative day in the SBS group. CONCLUSION: Based on our data, a decrease in the hepatic circulation, especially in the portal venous flow, after a massive resection of the intestine may cause progressive liver dysfunction because of the activation of hepatic stellate cells.

Efficacy of OK-432 local injection for the treatment of a neonatal branchial cleft cyst: a case report.

We herein present a case of a neonatal cervical cyst, which was diagnosed prenatally, and markedly decreased in size and disappeared after a local injection therapy of OK-432. A 0-day-old boy had an abnormal prenatal ultrasonography scan suggestive of rt. cervical cyst, measuring about 25 mm in diameter at 29 weeks' gestation. At birth, an elastic soft mass, measuring about 30 mm in diameter, was found on the right side of his neck. Computed tomography (CT) scans showed a giant cyst, which extended from the upper level of epipharynx to the upper mediastinum, and the contents were air and fluid. At 20 days of age, ultrasonography (US)-guided needle aspiration was performed. The aspirated fluid contained no epithelial cells, but many lymphocytes and neutrophils based on a cytological analysis. After the local injection of OK-432 had been performed four times, the right neck cyst had almost completely disappeared on US scans. During the local injection therapy, we analyzed the other sample of the second aspiration fluid of the neck cyst. Several clusters of epithelial cells, columnar epithelium, squamous cells, and ciliated epithelium were thus cytologically observed. Therefore, a final diagnosis of a branchial cleft cyst was made. The local injection of OK-432 was thus found to be an effective treatment for branchial cleft cysts.

Efficacy of real-time monitoring to determine motility in porcine small intestinal transplantation.

Orthotropic small intestinal transplantation (SIT) was performed in outbred 20 pigs. The interdigestive motor patterns were evaluated using strain gage (SG) force transducers. Seven pigs without SIT were treated as control (C) group. Based on the obtained data, the group, which could be detected the migrating motor complex (MMC) in the graft and alive with adequate oral feeding, was regarded as functional graft (FG) group, whereas the group which had available data recorded within 10 days before the death due to rejection was regarded as rejection (R) group. The MMC was analyzed using following parameters: duration; amplitude; and interval. In group FG, all parameters were almost same as group C, thus suggesting that the allograft in group FG had a normal motor function. In contrast, all parameters in group R were significantly lower than those in group FG, suggesting that the motility in group R was impaired. The SG method could monitor the real-time motility and was efficient for detecting the rejection of SIT.

Peutz-Jeghers syndrome in children: high recurrence rate in short-term follow-up.

We have managed four cases of Peutz-Jeghers syndrome (PJS) in children. Fathers of three of these patients had PJS. There was also a family history of cancer in three cases, with pancreatic cancer in a father, colonic and laryngeal cancers in a grandfather, and hepatic and gastric cancers in a grandmother. It is presumed that in each of the cases, the largest polyp was responsible for initial symptoms. Preoperative examination revealed additional small polyps in the whole alimentary tract except for the oesophagus. Patients underwent laparotomy to remove the largest polyps and subsequent intraoperative endoscopic polypectomy for other small polyps, to minimize intestinal resection. Follow-up gastrointestinal examinations, including upper gastrointestinal series, small intestinal contrast study, and barium enema, were repeated about once a year. Three of four cases showed recurrent small intestinal polyps, and one required a second laparotomy because of recurrent abdominal pain. In conclusion, patients with PJS occurring in childhood have a strong hereditary family history of cancer and a high incidence of recurrence. Careful follow-up examination is mandatory for the gastrointestinal tract, as well as other solid organs that are susceptible to malignant change, throughout a patient's life.

The efficacy of autologous cord-blood transfusions in neonatal surgical patients.

PURPOSE: Allogenic blood transfusions have a risk of infection owing to unknown organisms, graft-versus-host reaction, and immunosupression; however, the use of autologous blood has been reported to be safe. Cord blood has been reported to be useful as a source of stem cell transplantation for the treatment of leukemia and genetic disease. Furthermore, autologous cord-blood transfusions (ACBT) have been reported to be effective for the treatment of anemia in premature infants. The authors examined the efficacy of ACBT in neonatal surgical patients. METHODS: Autologous cord-blood was stored from 12 infants at delivery, including 2 transvaginal and 10 cesarean section deliveries, from 1998 to 2001. All infants had surgically correctable malformations diagnosed antenatally. The mean gestational age was 37.2 +/- 1.6 weeks, and the birth weight was 2,597 +/- 1.6 g. The results of the blood count, serum electrolyte, and liver function tests of the patients who underwent ACBT only (group 1, n = 7) were compared with those of the 7 neonates who underwent an allogenic transfusion during the same period (group 2, n = 7). RESULTS: The mean volume of the stored blood was 64 +/- 35.6 g (range, 20 to 100). Eleven of the 12 patients underwent transfusions. Ten of 11 patients received autologous cord blood. A mean of 44.1 +/- 37.3 g of cord blood was used. Three of 10 cases also required an allotransfusion because of ECMO circuit preparation and a shortage of the stored blood. One patient underwent allotransfusion only. As a result, 7 of 11 babies (64%) who required transfusion were able to avoid an allotransfusion. The blood potassium levels were lower in group 1 than in group 2. No significant complications were recognized clinically. CONCLUSIONS: ACBT is considered beneficial because it enables neonatal surgical patients to avoid allotransfusions. Therefore, autologous cord-blood storage should be considered in the patients antenatally diagnosed to have surgical malformations. However, the storage volume varies for each case. Improved techniques to obtain an adequate amount of blood also should be developed.

Cortisol and cytokine responses after surgery in different age groups of pediatric patients.

We investigated the cortisol and cytokine responses to surgical stress in the different age groups of pediatric patients. This study included 19 neonates (0-6 days old, group I), 19 infants (1-11 months old, group II), and 20 pre-school children (1-5 years old, group III), undergoing major thoracic and abdominal surgery. We obtained blood samples preoperatively and 0, 3, 6, 12, and 24 h postoperatively to measure the plasma levels of C-reactive protein (CRP), cortisol, interleukin (IL)-6, and IL-10. The plasma CRP level in each group reached a peak value on postoperative day 2; however, the peak value was significantly lower in group I than in groups II or III (I vs II, III; p=0.0134, p=0.0017, respectively). The plasma cortisol level in each group reached a peak value just after surgery; however, the peak value was also significantly lower in group I than in groups II or III (I vs II, III; p<0.001, p=0.0104, respectively). The plasma IL-6 level in each group reached a peak level hours postoperatively; however, the peak values in groups I and II were higher than in group III (I, II vs III; p=0.003, p=0.0458, respectively). The plasma IL-10 level in each group reached a peak value just after surgery and did not differ among the three groups. The endocrine and cytokine responses to the surgical stress vary among the different age groups of pediatric patients.

Changing profile of parenteral nutrition in pediatric surgery: a 30-year experience at one institute.

BACKGROUND: Due to technical refinements and steady advances in the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients, parenteral nutrition (PN) is now playing an important role in patient management. However, some PN-associated complications, such as catheter-related sepsis (CRS) and cholestasis, continue at high incidence, particularly in neonates. The objective of this study was to investigate the changing profiles of PN over the past 30 years in our department. METHODS: The medical records of 893 children (225 neonates, 245 infants, 261 preschool-age children, and 162 school-age children) who were placed on PN for >7 days in our department were reviewed, and the following data were extracted: birth weight, underlying disease, indications for PN, PN delivery route, type of catheter used, duration of PN, substrate and energy intake, type of amino acid solution used, and incidence of complications including CRS and liver dysfunction. The results were analyzed by dividing the patients into 3 groups according to their basic stages in management of PN and consisted of group 1 (1970 to 1979), group 2 (1980 to 1989), and group 3 (1990 to 1999). The parameters were compared in each group. RESULTS: The total number of patients in each group showed no significant difference; however, the percentage of low birth-weight neonates increased in group 3. In group 1, 85% of PN was administered through the peripheral vein; in group 2, 51.2%; and in group 3, 9.7%. The total calorie and nutrient intake decreased in groups 2 and 3 compared with group 1, particularly regarding fat intake. In groups 1 and 2, commercially available amino acid solution based on the Food and Agriculture Organization/World Health Organization formula was usually used as the nitrogen source, but in group 3, it was changed to an amino acid solution for children. CRS decreased significantly, particularly in neonates, and occurred at a rate of 45.4% in group 1, 10.7% in group 2, and 1.5% in group 3. The incidence of liver dysfunction also showed a decrease: 35.7% in group 1, 22.3% in group 2, and 18.0% in group 3. A multivariate analysis showed a strong relationship between PN-related liver dysfunction and the duration of PN, the presence of infection, and the type of amino acid solution used. CONCLUSIONS: PN via central venous catheters has been regarded as safe and effective treatment in pediatric surgical patients. Over the past 30 years, the incidence of CRS has decreased. However, PN-related liver dysfunction remains a problem, particularly in patients receiving long-term PN.