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This study aimed to evaluate the perioperative outcomes of laparoscopic fundoplication (LF) for gastroesophageal reflux disease (GERD) in children with scoliosis. Data of consecutive patients with GERD who underwent LF from January 2015 to December 2020 at a single pediatric institution were retrospectively analyzed. Eighty-two patients underwent laparoscopic Nissen fundoplication. The median [interquartile range (IQR)] body weight was 9.3 [7; 14] kg. Seventy-five patients were neurologically impaired (91%), and other comorbidities included scoliosis (n = 33), lung disease (n = 39), and cardiac disease (n = 14). The median (IQR) operative time including the creation of the gastrostomy and volume of bleeding were 160 [143; 190] min and 2 [1; 5] mL, respectively. There were no significant differences between patients with and those without scoliosis (p = 0.17 and p = 0.90, respectively). Patients with cardiac disease had a longer operative time (167 [161; 193] vs. 157 [141; 190] min, p = 0.01). There were three post-operative complications in children with neurological impairment; however, there was no clear relationship between the severity of scoliosis and complications. Severity of scoliosis did not correlate with perioperative results and post-operative complications. This suggests that the same LF technique can be used regardless of the presence or absence of scoliosis in children.

We present a 4-yr-old boy with adrenocortical carcinoma (ACC), diagnosed due to the appearance of gynecomastia as the presenting symptom. Six months prior to admission, an acute growth spurt along with the development of bilateral breast swelling was observed. He did not present any features of virilization, including enlargement of the testes, increase in testis volume, and penis size. Laboratory investigations showed gonadotropin-independent hypergonadism, with low LH/ FSH levels and elevated estradiol/testosterone levels. Abdominal computed tomography revealed a large heterogeneous mass adjacent to the right kidney and below the liver. Pathological investigations of the biopsy specimen demonstrated that the tumor was an ACC. Pre- and post-operative combination chemotherapy with mitotane was administered and surgical resection was carried out. Post-surgery, the elevated estradiol/testosterone concentrations reverted to within the reference range. Urinary steroid profile and tissue concentration analysis of estradiol and testosterone indicated the presence of estrogen in the ACC tissue. An investigation for TP53 gene aberrations revealed the presence of a germline point mutation in exon 4 (c.215C>G (p.Pro72Arg)). In ACC, the most common symptom is virilization, and feminization, characterized by gynecomastia, is very rare. However, a diagnostic possibility of ACC should be considered when we encounter patients who have developed gynecomastia without the influence of causative factors such as obesity or puberty, and do not present with the typical signs of virilization.

The prognosis of stage IV malignant rhabdoid tumor of the kidney (MRTK) has been extremely poor. However, a combination of ICE (ifosfamide, carboplatin, and etoposide) and VDCy (vincristine, doxorubicin, and cyclophosphamide) was recently reported to be effective for metastatic MRTK. We describe a 21-month-old girl with stage IV MRTK who was successfully treated with ICE, VDCy, and radiotherapy. She remained well, without recurrence, 24 months after diagnosis. Alternating therapy with ICE and VDCy might become a standard regimen for stage IV MRTK, although further study is required to confirm its effectiveness.

Background We have reported already that we succeeded in developing a normothermic liver perfusion system consisting of purely artificial products such as artificial blood. The aim of this study was to ascertain the metabolic functional integrity of the liver perfused in this system. Materials and methods A liver graft from a female pig weighing 20 kg was harvested in the usual manner. The perfusion solution consisted of artificial blood, L-15 medium, distilled water, bovine serum albumin, NaHCO3, NaOH, KCl, human regular insulin, 50% glucose solution, and dexamethasone. The isolated liver was perfused with this oxygenated perfusate through the portal vein at a rate of 300 ml/min for 9 h. We analyzed the changes of amino acids, ammonia, and urea concentrations in five livers, which showed high oxygen consumption (over 8 ml-O2/min during perfusion). Results Although urea did not exist in the perfusate at first, an extremely high level of the urea concentration appeared during the perfusion. On the other hand, the ammonia concentration was only slightly elevated during the perfusion. Arginine vanished from the perfusate with increases of citrulline and ornithine. Taurine was elevated with the disappearance of methionine. Alanine, glutamine, serine, histidine, and threonine concentrations decreased with an increase of the glucose concentration. Numerous liver cells exhibited PAS-positive cytoplasmic glycogen deposits not exhibited before perfusion. The ratios of branched-chain amino acids/aromatic amino acids were elevated during the perfusion. Conclusion Normothermic liver perfusion using artificial blood could sufficiently maintain the functional integrity of the liver.

Background. Previously, we clarified the role of inducible nitric oxide synthase (iNOS) and the protective effect of an iNOS inhibitor in warm ischemia and reperfusion model. In this study, we investigated whether the same effects would be obtained by iNOS inhibitor in liver transplantation model. Material and methods. Orthotopic liver transplantation was performed in pigs in the usual manner after about 6 h of cold preservation in University of Wisconsin solution. Aminoguanidine hemisulfate (AG) was used as the iNOS inhibitor and AG was administered intraportally at the dose of 10 mg/kg just after reperfusion. Two experimental groups were subjected, control group (n = 10), and AG group (n = 10). We investigated changes of serum nitrite/nitrate (NOx) and aspartate aminotransferase (AST). Expression of iNOS was examined by immunohistochemistry, including a double immunofluorescnce technique in combination with cofocal laser scanning microscopy. Results. Serum NOx and AST were significantly lower in the AG group. Histological hepatic damage and thrombocyte thrombi were attenuated in the AG group. Expression of iNOS was recognized strongly at Kupffer cells and neutrophils in the centrilobular region of liver after reperfusion by cofocal laser scanning microscopy. Moreover, iNOS staining was attenuated in AG group compared with control group. Conclusions. These results indicate that hepatic ischemia and reperfusion injury in liver transplantation might be triggered by iNOS expression of Kupffer cells and neutrophils, and attenuated by administration of an iNOS inhibitor. Moreover, AG showed down regulation of iNOS expression after reperfusion.

Background. It is still controversial whether a major mode of cell death during hepatic ischemia-reperfusion (I/R) injuries is apoptosis or necrosis. Moreover, the correlation between these cell deaths and the effects of a novel inducible nitric oxide synthase inhibitor (ONO-1714) has not been studied before. Methods. Pigs were subjected to 180 min of hepatic warm I/R under extracorporeal circulation. The control group was not administered ONO-1714. In the ONO-1714 group, ONO-1714 was administered 5 min before ischemia at a dose of 0.05 mg/kg through a portal vein catheter. The apoptotic and necrotic changes after reperfusion were examined by terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling and hematoxylin-eosin staining. Nitrotyrosine, active caspase-3, and cytochrome c were examined by immunohistochemistry. The plasma NO 2 - + NO 3 -, aspartate aminotransferase, and lactate dehydrogenase levels were also examined. Results. In the control group, the frequency of apoptotic cells was only 2.6%; nevertheless, that of necrotic cells was 37% at 24 hr after reperfusion. ONO1714 significantly attenuated apoptosis and necrosis, the expression of nitrotyrosine, and the increases of the plasma aspartate aminotransferase, lactate dehydrogenase, and NO 2 - + NO 3 -levels in the reperfusion phase. Conclusions. A major mode of cell death during hepatic warm I/R injury was necrosis, and apoptosis was not dominant. These necrotic changes were caused by the excess production of peroxynitrite, and ONO-1714 greatly attenuated I/R injuries as the result of inhibition of the peroxynitrite production.

Background. Non-heart-beating donors (NHBDs) are not yet acceptable in orthotopic liver transplantation (OLTX) because of the high frequency of primary graft nonfunction. In this study, we aimed to develop a new predictive method of graft viability in OLTX from NHBDs. Materials and methods. (1) Pigs were subjected to 15 min of hepatic ischemia and reperfusion (I/R). (2) Porcine OLTX was performed using grafts obtained from NHBDs subjected to in situ warm ischemia (0, 30, and 60 min). During both operations, hepatic hypoxanthine levels were measured by a microdialysis method. Results. In the I/R model, hypoxanthine accumulated during ischemia and decreased after reperfusion, whereas marked xanthine and uric acid production were observed after reperfusion. In the NHBDs model, all of the 0-min group, 6 of 13 pigs in the 30-min group, and 1 of 6 pigs in the 60-min group survived more than 7 days. Significant increases of hypoxanthine levels were seen dependent on warm ischemic time. In the 30-min group, hypoxanthine levels were significantly higher in the pigs that died than in those that survived, and correlated with aspartate aminotransferase, lactate dehydrogenase, and adenosine triphosphate levels of recipients. Histological examination revealed that graft injury was severe in the pigs with higher hypoxanthine levels. Conclusions. It is suggested that hepatic microdialysate hypoxanthine levels during warm ischemia in NHBDs were correlated with graft viability in the recipient. By using of this technique, prediction of the graft viability may be possible during donor operation.

Background. Recently, a novel inhibitor of inducible nitric oxide synthase, ONO-1714, was developed. We evaluated the effect of ONO-1714 on a critical warm I/R model of the pig liver. Methods. Pigs were subjected to 180 min of hepatic warm I/R under the extracorporeal circulation. We investigated the time course of changes in the serum NO2− + NO3− (NOx), the cellular distribution of endothelial and inducible nitric oxide synthase, thrombocyte-thrombi, and nitrotyrosine by immunohistochemistry. The hepatic tissue blood flow (HTBF) was measured continuously using a laser-Doppler blood flowmeter. Results. ONO-1714 at 0.05 mg/kg improved the survival rate from 54 (control group) to 100%. The serum NOx levels in the ONO-1714 group were significantly lower than those in the control group at 1, 1.5, 2, 3, and 6 hr after reperfusion. The serum aspartate aminotransferase (AST) and lactate dehydrogenase (LDH) levels of the ONO-1714 group were significantly lower than the control group, and the HTBF of the ONO-1714 group was significantly higher than the control group. The formation of thrombocyte-thrombi and nitrotyrosine after reperfusion was significantly lower in the ONO-1714 group. Conclusions. These results indicated that ONO-1714 improved the survival rates and attenuated I/R injury in a critical hepatic warm I/R model of the pig. ONO-1714 will be beneficial for hepatectomy or liver transplantation in the clinical field.

It has been reported that ingested magnets can cause intestinal fistula formation or perforation, leading to intestinal obstruction. However, there are no previous case reports that magnet ingestion additionally caused an intestinal volvulus. We report herein the case of a 1-year-old boy in whom the ingested magnets caused a volvulus of part of the small intestine leading to the resection of the necrotic portion. We think that if more than one magnet is found as a foreign body in the intestine, they should be removed immediately by laparotomy. Clinicians who care for children should be aware of this unexpected risk.

We tried to make an ex vivo functioning liver with an artificial perfusate that consisted of artificial blood in the pig liver. A liver graft from a female pig weighing 20 kg was harvested in the usual manner. The perfusion solution consisted of artificial blood, L-15 medium, distilled water, bovine serum albumin, NaHCO3, NaOH, KCl, human regular insulin, 50% glucose solution, and dexamethasone. The isolated liver was perfused with this oxygenated perfusate through the portal vein at a rate of 300 ml/min for 9 hours. Seven livers were perfused for 9 hours in this system. Five of the livers showed mean oxygen consumption of over 8 ml-02/min during perfusion. Histological findings showed that the hepatic architecture was almost completely preserved and numerous hepatocytes exhibited PAS-positive cytoplasmic glycogen deposits in these livers. These observations indicate that we have succeeded in developing an ex vivo functioning liver with an artificial perfusate employing artificial blood.

To clarify the changes that occur in hepatic venous oxygen saturation (ShVO2) during hepatic ischemia/reperfusion (I/R) injury, we examined the relationship between ShVO2, hepatic tissue blood flow (HTBF), and portal vein pressure (PVP) in a warm I/R model using pig livers. Female pigs weighing 18-23 kg were subjected to warm I/R under extracorporeal circulation between the superior mesentric vein and the left jugular vein to avoid portal congestion. The warm ischemic times were 120 min (n = 4), 180 min (n = 14), and 240 min (n = 4). ShVO2, HTBF, and PVP were measured after reperfusion. The survival rates of the pigs 3 days after reperfusion were 100% in the 120-min group, 57% in the 180-min group, and 25% in the 240-min group. In the 180-min group, the ShVO2 was lower in the pigs that died than in those that survived. There was a significant correlation between ShVO2 and both PVP and HTBF after reperfusion. Histological examination revealed findings of severe I/R injury in pigs with a low ShVO2, and mild I/R injury in pigs with a stable ShVO2. These observations suggest that the changes in ShVO2 could reflect the degree of hepatic I/R injury, especially that related to microcirculatory disturbances occurring at the sinusoid levels.