安井 稔博

An 11-year-old girl presented with recurrent right lower quadrant (RLQ) pain. There was no evidence of inflammation and appendiceal swelling except at the initial onset. The repeated presence of a small amount of ascites at the time of abdominal pain triggered the performance of exploratory laparoscopy. Intraoperative examination revealed a non-inflamed, unswollen appendix with a cord-like atretic segment at the middle part and an appendectomy was performed. At 46 months follow-up, she remained asymptomatic. In patients with recurrent RLQ pain of unknown cause, it is necessary to consider diagnostic laparoscopy while keeping appendiceal atresia in mind as a differential diagnosis.

PURPOSE: We previously reported that polyphyllin D, a main component of the traditional Chinese medicinal herb Paris polyphylla, exhibited anticancer effects in vitro against human neuroblastoma cells. The aims of this investigation was to examine the presence or absence of in vivo anti-metastasis effects of polyphyllin D were to establish a liver metastasis model of neuroblastoma and to evaluate the anti-metastasis effects of polyphyllin D. METHODS: Subcutaneous and intraperitoneal tumors, and metastasis models were established in immune-deficient BALB/c nude and BALB/c Rag-2/Jak3 double-deficient (BRJ) mice using the human neuroblastoma cell lines IMR-32, LA-N-2, or NB-69. For evaluating polyphyllin D activity, we used a mouse model of liver metastasis with the IMR-32 cells line injected through the tail vein. We analyzed the livers number and area of liver tumors in of the phosphate buffer solution- and polyphyllin D-treated groups. RESULTS: Liver metastasis and intraperitoneal dissemination models were successfully established in immune-deficient BRJ mice using the three human neuroblastoma cell lines. In the liver metastasis, the model of IMR-32 cells, we found that polyphyllin D suppressed both the number and total area of metastatic foci the average number of metastatic foci, average focus areas, and number of cleaved caspase-3-positive cells were significantly lower in the polyphyllin D group (p = 0.016, 0.020, 0.043, respectively). CONCLUSIONS: We developed a mouse models of neuroblastoma metastasis and demonstrated for the first time that polyphyllin D has an antitumor effect on neuroblastoma liver metastases.

<title>Abstract</title>We report a case of a 13-year<bold>-</bold>old boy with arginase 1 deficiency carrying a new variant in <italic>ARG1</italic>. Sanger sequencing identified the compound heterozygous variants: NM_000045.4: c.365G&gt;A (p.Trp122*)/c.820G&gt;A (p.Asp274Asn). Although not previously reported, the p.Asp274Asn variant is predicted to have strong pathogenicity because it is located in a highly conserved domain in the protein core and arginase activity in the patient was below measurement sensitivity.

<title>Abstract</title><sec> <title>Background</title> Acute obstruction of the hepatic vein (HV) or the portal vein (PV), particularly when it occurs during liver surgery, is potentially fatal unless repaired swiftly. As surgical interventions for this problem are technically demanding and potentially unsuccessful, other treatment options are needed. </sec><sec> <title>Case presentation</title> We report two cases of acute, surgically uncorrectable HV or PV obstruction during liver resection or living donor liver transplantation (LDLT), which was successfully treated with urgent intraoperative placement of endovascular stents using interventional radiology (IVR). In Case 1, a patient with colonic liver metastases underwent a non-anatomic partial hepatectomy of the segments 4 and 8 with middle hepatic vein (MHV) resection. Additionally, the patient underwent an extended right posterior sectionectomy with right hepatic vein (RHV) resection for tumors involving RHV. Reconstruction of the MHV was needed to avoid HV congestion of the anterior section of the liver. The MHV was firstly reconstructed by an end-to-end anastomosis between the MHV and RHV resected stumps. However, the reconstruction failed to retain the HV outflow and the anterior section became congested. Serial trials of surgical revisions including re-anastomosis, vein graft interposition and vein graft patch-plasty on the anastomotic wall failed to recover the HV outflow. In Case 2, a pediatric patient with biliary atresia underwent an LDLT and developed an intractable PV obstruction during surgery. Re-anastomosis with vein graft interposition failed to restore the PV flow and elongated warm ischemic time became critical. In both cases, the misalignment in HV or PV reconstruction was likely to have caused flow obstruction, and various types of surgical interventions failed to recover the venous flow. In both cases, an urgent IVR-directed placement of self-expandable metallic stents (SEMS) restored the HV or PV perfusion quickly and effectively, and saved the patients from developing critical conditions. Furthermore, in Cases 1 and 2, the SEMS placed were patent for a sufficient period of time (32 and 44 months, respectively). </sec><sec> <title>Conclusions</title> The IVR-directed, urgent, intraoperative endovascular stenting is a safe and efficient treatment tool that serves to resolve the potentially fatal acute HV or PV obstruction that occurs in the middle of liver surgery. </sec>

<p>Objectives: Proximal stoma creation in neonates results in growth failure and distal intestinal atrophy. "Recycling stool" consists of stool injection from the proximal limb to the distal limb of a stoma. Because this method may prevent distal bowel atrophy and increase body weight, we investigated the effects of recycling stool upon distal intestinal mucosa by generating an ileostomy model in rats.</p><p>Methods: An ileostomy was created 5 cm proximal to the cecum in male Wistar/ST rats. Discharged stool or saline was injected into the distal limb, twice per day for 7 days. The intestinal adaptation was assessed by measuring the villus height and counting goblet cell number. Proliferation and apoptosis were analyzed by Ki67 and TUNEL immunostaining.</p><p>Results: The ratios of the height of the distal villi (D) to the that of proximal villi (P) were 0.97 (median [range] of D and P length: 421 [240–729] μm and 436 [294–638] μm, P<0.05) in the stool-injected group and 0.81 in the saline-injected group (442 [315–641] μm and 548 [236–776] μm, P<0.05). Compared with the saline-injected group, the stool-injected group showed elevated numbers of goblet cells (3.6 [2.0–7.6] vs. 4.9 [2.4–7.5] cells/100-μm villus length) and Ki67-positive cells (26.8% [13.8%–35.4%] vs. 40.1% [31.2%–45.7%]), along with a reduced number of apoptotic cells (5.0 [2.0–14.0] vs. 4.0 [1.0–9.0] cells/100-μm villus length).</p><p>Conclusions: Recycling stool prevented distal intestinal atrophy; this experimental design may facilitate further studies concerning alternative methods to prevent intestinal atrophy and growth failure.</p>