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Purpose: The Soave procedure (SO) is performed most commonly for Hirschsprung disease. SO reduces the risk of injury to the pelvic structures; however, a residual aganglionic muscle cuff could interfere with bowel movement and lead to obstructive enterocolitis. The Swenson procedure is considered ideal in terms of peristalsis. Currently, laparoscopic surgery provides better visualization and facilitates precise dissection, possibly leading to feasible performance of the laparoscopic modified Swenson procedure (SW). We present our operative technique and the efficacy of the SW compared with that of SO. Methods: We retrospectively reviewed the records of 16 and 27 patients who underwent SW and SO, respectively, between 2012 and 2017. Results: Operative time, blood loss, length of stay, and frequency of bowel movements showed no significant difference between the two groups. In the SW group, temporary dysuria occurred in one patient, postoperative enterocolitis in two, wound infection in one, and severe perianal excoriation in four, whereas in the SO group, obstructive symptoms occurred in three patients, small-bowel obstruction in one, and severe perianal excoriation in three. The complications and outcomes were comparable between both groups. Conclusion: Laparoscopic SW was safe and feasible for the short-term follow-up outcomes., ファイル公開：2019/10/01

Background Transplanted organs from female donors are associated with less favorable prognoses and outcomes. This study aimed to determine whether donor gender affects levels of serum terminal complement component C5a and oxidative stress in pediatric living related liver transplantation (LRLTx) recipients. Material/Methods The subjects were 43 patients (20 males and 23 females) who underwent LRLTx during childhood (age range 1.2 years to 14.4 years; mean age 5 years). Serum samples were taken during the patients’ regular outpatient visits after LRLTx. Serum C5a was measured using the specific human C5a ELISA kit. Serum total hydroperoxide (TH) and biological antioxidative potential (BAP) were measured using the free radical analytic system, and the oxidative stress index (OSI) was calculated as the ratio of TH to BAP. Serum glutamic pyruvic transaminase (GPT), glutamic oxaloacetic transaminase (GOT), gamma-glutamyl transpeptidase (γGTP), and lactate dehydrogenase (LDH) were also measured as part of a typical outpatient examination for such patients. Results C5a serum levels were higher in the 29 recipients who received their grafted livers from female donors than in the 14 recipients who received their grafted livers from male donors. Recipients who received their grafted livers from female donors had higher incidence of post-LTx (liver transplantation) complications. Female recipients from female donors showed the highest serum GPT and GOT levels, but this difference was only significant when compared to the female recipients from male donors (41.4±9.8 IU/L vs. 17.3±1.8 IU/L for GPT and 42.2±7.5 IU/L vs. 23.4±2.2 IU/L for GOT; P Conclusions Pediatric LRLTx patients who receive their grafts from female donors exhibit higher levels of serum C5a that probably plays a role in the immunological response against grafted livers from female donors in LTx.

Purpose: Biliary atresia (BA) is characterized by progressive inflammation of the biliary system. This inflammation persists and causes liver fibrosis, although jaundice disappears after Kasai portoenterostomy (KP). We aimed to confirm whether the oral administration of eicosapentaenoic acid (EPA) suppresses liver fibrosis in postoperative patients with BA. Methods: We reviewed patients who underwent laparoscopic KP (lapKP) between January 2014 and September 2017. From December 2016, 30 mg/kg/day of EPA was orally administered to patients who opted to take medicine (EPA group). Patients who did not receive EPA were assigned to the non-EPA group. Mac-2 binding protein sugar chain modified isomer (M2BPGi) and hyaluronic acid (HA) levels were compared between the two groups in patients showing disappearance of jaundice at 6 months after the first lapKP. Results: Seventeen patients in the non-EPA group and 11 in the EPA group were enrolled. At 6 months after the first lapKP, 10 patients in the non-EPA group and six in the EPA group were without jaundice. M2BPGi and HA levels were significantly lower in the EPA group. Conclusions: Liver fibrosis was suppressed in patients without jaundice 6 months after lapKP, who were administered EPA. We believe that periductular inflammation was alleviated by EPA supplementation., ファイル公開：2019/10/01

ABO-incompatible liver transplantation (LTx) is becoming more common in response to the paucity of liver allografts. Several studies have expressed concern about the effect of ABO compatibility on graft survival.To evaluate the differences in serum cytokine levels between ABO-incompatible (ABO-i) and ABO-compatible (ABO-c; includes ABO-compatible and identical) pediatric LTx recipients during regular outpatient follow-up. Note that, in the field of organ transplantation, transplants are categorized as incompatible, compatible or identical; accordingly, these are the terms we use in the paper.A clinical outpatient study measuring serum transforming growth factor (TGF)-β1, interferon (IFN)-γ, interleukin (IL)-2 and IL-10 in 43 living related liver transplantation (LRLT) recipients, of whom 36 received ABO-c LRLT (34 were ABO-identical and 2 were non-identical) and 7 ABO-i LRLT. Serum glutamic pyruvic transaminase, glutamic oxaloacetic transaminase, gamma-glutamyl transpeptidase, alkaline phosphatase, lactate dehydrogenase and bilirubin were measured as part of the patients' regular follow-up visits.There were no differences between the ABO-c and ABO-i groups in terms of recipient's age [mean 12.6 vs. 11.1 years (y)], post-LTx duration (mean 7.3 vs. 7.3 y), donor's age (mean 35.5 vs. 34.6 y), body weight (28.9 ± 2.9 vs. 27.9 ± 6.9 kg), or gender (19 female and 17 male vs. 4 female and 3 male). Serum TGF-β1, IFN-γ and IL-2 were significantly higher in the ABO-i group than in the ABO-c group. IL-10, however, did not differ between the two groups. There was a tendency toward higher γGTP levels in the ABO-i group, but this difference did not reach significance.ABO-incompatible LRLTx patients have higher serum TGF-β1, IFN-γ and IL-2 levels as measured at regular outpatient visits. As a result, they face a higher risk of T-helper 1 cell polarization, which could make graft rejection more likely.

Intrahepatic cholangiocarcinoma (ICC) is a relatively rare malignancy arising from the biliary epithelium. Prognosis is typically poor. Currently, aggressive surgical resection is the only treatment modality that offers patients any chance of long-term survival. Here, we present the case of a 57-year-old woman in whom we diagnosed ICC, with the tumor occupying the entire left and caudate hepatic lobes and daughter nodules in the right lobe. She underwent hepatectomy of segments I to VI, combined with intraoperative microwave coagulation therapy for nodules in the residual liver. Three months after the surgical resection, she had recurrence of the disease. The patient subsequently received weekly intraarterial chemotherapy with irinotecan (CPT-11), and a partial response was observed which persisted for 18 months. Subsequent computed tomography revealed the regrowth of three tumors, and she therefore underwent a repeat resection 24 months after the first surgical operation. In postoperative-month (POM) 32, she received systemic chemotherapy with tegatur/gimeracil/oteracil potassium (S-1)/cisplatin for multiple small nodules in her lung. Following three cycles of chemotherapy with a stable disease response, partial resections of the lung were performed. Third and fourth hepatectomies were performed in POMs 46 and 59, respectively. Five years and 5 months after the first hepatectomy, she is alive with small lesions in her lung. This multimodal approach may be effective for ICC.

Background Main indications for liver transplantation in the pediatric population include biliary atresia and inherited metabolic diseases. The present study evaluated whether there are differences between pediatric patients undergoing living-related liver transplantation due to the two diseases in terms of their oxidative and immunological status during their regular outpatient follow-up visits. Material and methods A clinical outpatient study measuring serum oxidative stress index (calculated as serum oxidant/antioxidant ratio, in the form of serum total hydroperoxide/serum biological antioxidative potential), serum terminal complement component 5a, as an indicator of complement activity and immunological status, and transforming growth factor-s1, as a marker of liver fibrosis, in 16 patients (6 males and 10 females, 2.5-15 years old) who received living-related liver transplantation due to inherited metabolic diseases (n=6; in the form of propionic acidemia [n=1], methylmalonic acidemia [n=1], arginase deficiency [n=1], tyrosinemia [n=2], and glycogen storage disease type 1b [n=1], with an age range of 2.4-14.6 years old) and due to biliary atresia ([n=10], with an age range of 2.9-14.5 years old). Results Serum oxidative stress index, complement component-5a, and transforming growth factor-s1 were significantly higher in the inherited metabolic diseases group than in the biliary atresia group. In all patients, serum oxidative stress index correlated positively with complement component-5a and transforming growth factor-s1. Conclusions Patients who receive living-related liver transplantation due to inherited metabolic diseases are prone to higher oxidative stress, complement activity, and serum transforming growth factor-s1.

Oxidative stress has been suspected to influence graft survival and prognosis in pediatric recipients of living related liver transplantation (LRLT).We determined the oxidative status of pediatric LRLT recipients during their regular outpatient follow-up visits, and looked for a relationship between oxidative status and post-liver transplantation (post-LTx) duration.The study included 43 patients (20 males and 23 females) between the ages of 1.6 and 25.1 years (median 10.7 years) who had undergone LRLT from 5 months to 17.5 years (median 7 years) prior to the study, between the ages of 1.2 and 14.4 years (median 3.5 years).Serum glutamic pyruvic transaminase (GPT), glutamic oxaloacetic transaminase (GOT), gamma-glutamyl transpeptidase (γ-GTP), alkaline phosphatase (ALP), lactate dehydrogenase (LDH), direct bilirubin and choline-esterase were measured as part of the patients' regular follow-up visits. Serum total hydroperoxide (TH) and biological antioxidative potential (BAP) were measured using the free radical analytic system which requires 20 μl of serum and 10 min of processing time for each sample. Oxidative stress index (OSI) was calculated as the ratio of TH to BAP.Serum OSI correlated positively with serum levels of GOT, GPT, LDH, ALP, γ-GTP and direct bilirubin. Serum OSI, TH, LDH, ALP and GOT correlated negatively with post-LTx duration. Serum BAP correlated positively with post-LTx duration. Serum TH correlated positively with serum GOT and γ-GTP, but negatively with serum BAP.(1) The OSI, which can be calculated based on data acquired through a simple outpatient procedure, can serve as an index of our patients' laboratory results and oxidative status. (2) The LRLT recipients in our study were at risk for oxidative stress early in the post-operative period, but this risk subsided with time.

Although the role of hepatectomy for patients with colorectal liver metastases is well established, few reports exist of hepatectomy for patients with metastases of gastric cancer origin. This study reviews cases of hepatectomy for metastatic gastric cancer at Fujita Health University Hospital.Between 1989 and 2004, 18 patients underwent hepatectomy for liver metastases from gastric cancer. The patients consisted of 16 men and 2 women and their ages ranged from 51-76 (median 64) years. Hepatic resection was indicated for patients with synchronous metastases who did not have peritoneal dissemination or any other distant metastases (11 patients), and patients with metachronous metastases who did not have any other recurrent lesions (7 patients).Overall survival rate for 1, 2, 3, and 5 years are 56.3, 36.5, 27.3, and 27.3%, respectively. Although the 5-year survival rate was considerable, an early and rapid decrease of survival rate occurred in the first 1-2 years (compared with the colorectal patients). Univariate analysis showed serosal invasion and lymphatic invasion of the primary tumor as significant prognostic factors for survival.Surgical resection for liver metastases of gastric cancer is thought to be beneficial for small part of the patients. For other patients, the procedure may only provide the limited beneficial effects on survival.

We had previously reported that using CT during angiography as a preoperative evaluation improves the detecting rate of minute (less than 10 mm) metastases and non-recurrent rate in the liver. We here evaluate the impact of CT during angiography on the survival after putative curative liver resection in our series.During the period between 1990 and 2000, single detector helical CT was used for preoperative examination. There were 85 patients who underwent the examination before hepatectomy for colorectal metastases. Among them, there were 37 patients who underwent preoperative evaluating CT during angiography, and 48 who did not. The survival curves of the patient with/without CT during angiography, after the first hepatectomy, were calculated.Although recurrences in the residual liver after hepatectomy were observed in 9 out of 37 patients with CT-during angiography and 19 out of 48 patients without, the actual 5-year survival rates of the patients with and without CT during angiography are 42.6 and 43.2%, respectively, after more than 5 years' follow-up period. There is no significant difference between them.The present data show that the benefits from the approach to improve the detection of minute liver metastases are limited for the survival of putative curative liver resection.

5-FU plus Cisplatin combination therapy had been employed against primary liver carcinomas for years. S-1 is a fourth-generation oral fluoropyrimidine and attracts considerable interest for the activity against gastric cancer. We herein examined the effect and adverse effects of S-1 plus Cisplatin combination therapy for primary liver carcinomas.4 patients with hepatocellular carcinoma (HCC) and 3 with cholangiocellular carcinoma (CCC) were employed for this study. They all had far-advanced diseases in and/or out of the liver at the time of the therapy initiation. They were 4 men and 3 women. Their ages were in the range of 42-73 (58 +/- 9.73, mean +/- SD) years old. The protocol of the therapy is a 3-week period of S-1 (70-80 mg/m2/day) oral administration combined with 2 intravenous administration of CDDP (20-35 mg/m2) during the period. With two weeks of intermission, the therapy was repeatedly performed 2-11 times for each patient.Three patients had PR and 2 had NC response with the therapy. Two patients with HCC and pre-treatment with 5-FU had PD response. Although the patients developed leukopenia and thrombocytopenia, the therapy was well tolerable also in the outpatient basis.S-1 plus Cisplatin combination therapy is a potential therapy for advanced primary liver carcinomas.

Humoral hypercalcemia of malignancy (HHM) is a rare complication of malignant pediatric tumors, specifically those that secrete humoral factor(s), such as parathyroid hormone-related peptide (PTHrP). The authors report a case of severe hypercalcemia associated with ovarian dysgerminoma in a 10-year-old girl. In this case, the humoral factor was considered to be 1,25-dihydroxyvitamin D. HHM is extremely resistant to medical therapy. Therefore, tumor resection or volume reduction is necessary to control serum calcium levels.

Hepatic resection for colorectal metastases was performed for 188 patients. Overall survival rates after the first hepatectomy are 41.4% and 32.7% for 5 and 10 years, respectively. The survival rate of 116 cases with unilobar hepatic metastases (H1) is significantly higher than those of 48 cases with two to four bilobar metastases (H2) and 24 cases with more than four (H3), respectively. However, the differences between the survival rates from H1 with multiple metastases, H2, and H3 are not significant, even though the H3 group has no 10-year survivors. The 5-year survival rates after the second hepatectomy (30 patients) and the resection of the lung (26 patients) are 30.3% and 35.2%, respectively, in this series. In those patients, the 5-year survival rates from the first metastasectomy are 43.4% and 50.3%, respectively. There are 14 5-year survivors with multiple metastases and 8 of those patients underwent multiple surgeries. There are 13 patients with three or more repeat resections of the liver and/or lung. The 5-year survival rates of the patients from the first and third metastasectomy are 53.9% and 22.5%, respectively. Repeat operations for the liver and the lung contribute to the improving prognosis.