Publications by authors named "Yuichi Takama"

38 Publications

Safety and Feasibility of Laparoscopic Resection of Neuroblastoma Without Image-Defined Risk Factors Performed by Pediatric Surgical Trainees: A Multicenter Comparison Study.

J Laparoendosc Adv Surg Tech A 2021 May 24. Epub 2021 May 24.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Japan.

Laparoscopic resection has gradually been adopted for neuroblastoma patients; however, some authors reported that, due to its technically demanding procedures, this operation should be performed only by highly experienced surgeons. The aim of this study was to evaluate the safety and feasibility of laparoscopic resection of abdominal neuroblastoma by pediatric surgical trainees. In this multicenter, retrospective study, including 18 children with abdominal neuroblastoma indicated for 19 laparoscopic resections (1 with bilateral neuroblastomas) from 1999 to 2018, the clinical data were retrospectively reviewed and compared between trainee and attending surgeons. None of the cases had image-defined risk factors (IDRFs) at surgery. All patients successfully underwent complete laparoscopic resection without blood transfusion, open conversion, or intraoperative or postoperative complications. Of the 19 cases, 6 were performed by pediatric surgical trainees under the guidance of attending surgeons, and 13 were performed by attending surgeons. With comparable tumor, largest diameter, operative time, and bleeding amount were not significantly different between the two groups. In the trainee surgeon group, one local recurrence occurred at a secondary resection site in the bilateral neuroblastoma patient with Stage L2. Laparoscopic resection of neuroblastoma could be safe and feasible when limited to absent IDRFs at surgery by pediatric surgical trainees under the guidance of experienced attending surgeons, as well as by attending surgeons.
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http://dx.doi.org/10.1089/lap.2020.0957DOI Listing
May 2021

Effect of microscopy-assisted portoenterostomy (MAPE) for biliary atresia.

Pediatr Surg Int 2021 Feb 2;37(2):223-228. Epub 2021 Jan 2.

Department of Pediatric Surgery, Osaka University of Graduation School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: Portoenterostomy (PE) is the standard treatment for biliary atresia (BA). However, micro-bile ducts are difficult to identify with surgical loupes and dissect systematically. We report the effects of our attempts to dissect hilar tissue using a surgical microscope.

Methods: Microscopy-assisted portoenterostomy (MAPE) was initiated in 2014. Patients born between 2000 and 2013 who underwent PE until day 70 without a surgical microscope for BA were gathered as historical control. MAPE in re-do PE cases (Re-MAPE) was evaluated in the same manner.

Results: Ten patients underwent MAPE for BA during the study period. 17 patients in the conventional PE group were gathered. In the MAPE group, the jaundice clearance rate was 80%, compared with 53% in the conventional PE group. Re-MAPE was performed in four patients, who had a jaundice clearance rate of 75%, essentially identical to the rate with initial MAPE. At age 4 years, the native liver survival rate was 58% in the MAPE group and 38% in the conventional PE group. The native liver survival rate in the Re-MAPE group was 75%.

Conclusion: MAPE is useful for sharing the surgical field during open PE in patients with BA. It may improve the rate of jaundice clearance.
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http://dx.doi.org/10.1007/s00383-020-04794-xDOI Listing
February 2021

Laparoscopic retroperitoneal splenopexy for wandering spleen: A novel technique using a three-incision retroperitoneal pouch.

Asian J Endosc Surg 2020 Nov 18. Epub 2020 Nov 18.

Department of Pediatric Surgery, Kindai University Nara Hospital, Ikoma, Japan.

Introduction: Wandering spleen is a rare condition for which splenopexy is indicated to prevent splenic torsion. We present a novel laparoscopic splenopexy technique for wandering spleen based on creation of a three-incision retroperitoneal pouch.

Materials And Surgical Technique: A 12-year-old male patient with abdominal distention and vomiting was transferred to our institution. Contrast-enhanced CT revealed a swollen wandering spleen with associated gastric volvulus, and the patient underwent laparoscopic surgery. A 5-mm camera port was inserted through an umbilical incision with two additional ports, one in the right upper abdomen and one in the left flank. Normal saline was injected into the retroperitoneal space from the left flank with a 23-G needle to create a retroperitoneal pouch. Three ventrodorsal peritoneal incisions were created at the same site in the peritoneum. The swollen spleen was inserted into the retroperitoneal pouch from the central incision, and the upper and lower poles of the spleen were exposed to the abdominal cavity from the cranial and caudal incisions to prevent splenic torsion. Finally, anterior gastropexy was performed. The postoperative period was uneventful. The patient was discharged on postoperative day 11 without complaints. As of 10 months after surgery, the patient had no recurrences of splenic torsion or gastric volvulus.

Discussion: In the present method, the retroperitoneal pouch was created without difficulty by injection of normal saline. Even in a patient with a swollen spleen, this novel method could prevent splenic torsion without using artificial materials or extensively dissecting the retroperitoneal space.
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http://dx.doi.org/10.1111/ases.12894DOI Listing
November 2020

Can plain chest X-ray replace computed tomography for the follow-up of children who have undergone the Nuss procedure?

Surg Today 2020 Oct 26;50(10):1249-1254. Epub 2020 May 26.

Department of Pediatric Surgery, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka Suita, Osaka, 565-0871, Japan.

Purpose: To establish whether new indices on plain chest X-ray (CXR) can replace those on computed tomography (CT) for the follow-up of children who have undergone the Nuss procedure.

Methods: The subjects of this retrospective study were 45 children who underwent the Nuss procedure between 2000 and 2016. The Haller index (HI) was measured by preoperative CT. Preoperative and postoperative chest deformities were evaluated by two CXR measurements: the concave rate on the lateral view (CR; the depth of the concavity divided by the anterior-posterior diameter of the rib cage) and the tracheal bifurcation angle (TBA) on the anterior view. Data are expressed as the median with range.

Results: The median age and HI of the children, when they underwent the Nuss procedure, was 9.3 (3.8-17.3) years and 4.5 (3.2-10.1), respectively. The preoperative CR was correlated significantly with the HI. The postoperative CR was significantly lower than the preoperative CR [pre: 0.17 (0.08-0.37), post: 0.09 (0.01-0.18), p < 0.05], and the low value was sustained after bar removal. The TBA decreased significantly after the Nuss procedure from 74.2° (55-104) preoperatively to 65.0° (45-92) postoperatively (p < 0.05).

Conclusions: These results suggest that CXR can replace CT for the follow-up of patients after the Nuss procedure, with lower radiation exposure.
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http://dx.doi.org/10.1007/s00595-020-02020-8DOI Listing
October 2020

Outcome and Late Complications of Hepatoblastomas Treated Using the Japanese Study Group for Pediatric Liver Tumor 2 Protocol.

J Clin Oncol 2020 08 18;38(22):2488-2498. Epub 2020 May 18.

Center for Integrated Medical Research, Hiroshima University Hospital, Hiroshima, Japan.

Purpose: We report here the outcomes and late effects of the Japanese Study Group for Pediatric Liver Tumors (JPLT)-2 protocol, on the basis of cisplatin-tetrahydropyranyl-adriamycin (CITA) with risk stratification according to the pretreatment extent of disease (PRETEXT) classification for hepatoblastoma (HB).

Patients And Methods: From 1999 to 2012, 361 patients with untreated HB were enrolled. PRETEXT I/II patients were treated with up-front resection, followed by low-dose CITA (stratum 1) or received low-dose CITA, followed by surgery and postoperative chemotherapy (stratum 2). In the remaining patients, after 2 cycles of CITA, responders received the CITA regimen before resection (stratum 3), and nonresponders were switched to ifosfamide, pirarubicin, etoposide, and carboplatin (ITEC; stratum 4). Intensified chemotherapeutic regimens with autologous hematopoietic stem-cell transplantation (SCT) after resection were an optional treatment for patients with refractory/metastatic disease.

Results: The 5-year event-free and overall survival rates of HB patients were 74.2% and 89.9%, respectively, for stratum 1, 84.8% and 90.8%%, respectively, for stratum 2, 71.6% and 85.9%%, respectively, for stratum 3, and 59.1% and 67.3%%, respectively, for stratum 4. The outcomes for CITA responders were significantly better than those for nonresponders, whose outcomes remained poor despite salvage therapy with a second-line ITEC regimen or SCT. The late effects, ototoxicity, cardiotoxicity, and delayed growth, occurred in 61, 18, and 47 patients, respectively. Thirteen secondary malignant neoplasms (SMNs), including 10 leukemia, occurred, correlating with higher exposure to pirarubicin and younger age at diagnosis.

Conclusion: The JPLT-2 protocol achieved up-front resectability in PRETEXT I/II patients with no annotation factors, and satisfactory survival in patients who were CITA responders in the remaining patients. However, outcomes for CITA nonresponders were unsatisfactory, despite therapy intensification with ITEC regimens and SCT. JPLT-2 had a relatively low incidence of cardiotoxicity but high rates of SMNs.
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http://dx.doi.org/10.1200/JCO.19.01067DOI Listing
August 2020

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report.

Surg Case Rep 2020 Apr 17;6(1):73. Epub 2020 Apr 17.

Department of Pediatric Surgery, Osaka City General Hospital, Osaka, Japan.

Background: Congenital portosystemic shunt (CPSS) is a rare malformation that leads to hyperammonemia, hypermanganesemia, and various symptoms. CPSSs are divided into intrahepatic and extrahepatic shunts. In patients with persistent CPSS including an intrahepatic portosystemic shunt (IPSS), early intervention to occlude the shunt reverses the associated complications.

Case Presentation: The patient was a 1-year-and-7-month-old girl. She presented with hypergalactosemia and elevation of blood ammonia level (75 μg/dL) and total bile acid levels (68.2 μmol/L) during the neonatal period. Two IPSSs were detected using ultrasound and enhanced computerized tomography. Magnetic resonance imaging (MRI) at 1 year and 3 months of age showed abnormally high signal intensity in the pallidum of her brain. Spontaneous closure was not observed. We performed a right hepatectomy at 1 year and 7 months of age. The portal vein pressure was 16 mmHg after temporary occlusion of the right portal vein. Blood ammonia and serum manganese levels decreased immediately after the operation. The abnormal signal on brain MRI disappeared. She had a favorable course with no sign of recurrence of IPSS 5 years postoperatively.

Conclusion: Liver resection for an IPSS to control the symptoms of a portosystemic shunt is reasonable in a child for whom interventional radiological treatment is not indicated.
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http://dx.doi.org/10.1186/s40792-020-00838-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7165232PMC
April 2020

Thoracoscopic repair of esophageal atresia.

Surg Today 2020 Sep 14;50(9):966-973. Epub 2019 Oct 14.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, 565-0871, Osaka, Japan.

Thoracoscopic repair (TR) of esophageal atresia (EA) has been performed with increasing frequency over the last two decades, with the expectation of improved outcomes by avoiding thoracotomy. To understand the current practice and outcomes of TR of EA, we reviewed the relevant literature, including 15 case series, 7 comparative studies, and 3 meta-analysis comparing TR with conventional open repair (COR). Most of the studies had a retrospective design and small numbers of patients. Although the evidence level is low because of the lack of prospective studies, this review found that TR is as safe as COR, with comparative outcomes. Moreover, there were several advantages of TR over COR, such as less blood loss and a shorter hospital stay. The long-term outcomes of TR remain unclear because of limited data. Moreover, there is a significant learning curve over the first 10-20 TRs performed. We conclude that TR of EA, when conducted by experienced surgeons, is a safe and minimally invasive alternative to COR and may yield better results than COR in appropriately selected patients.
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http://dx.doi.org/10.1007/s00595-019-01884-9DOI Listing
September 2020

The effect of a novel immunosuppressive drug, a PAK-2 inhibitor, on macrophage differentiation/polarization in a rat small intestinal transplantation model.

Transpl Immunol 2019 12 14;57:101246. Epub 2019 Sep 14.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Japan.

Objective: PQA-18 (Prenylated quinolinecarboxylic acid-18) has been reported to be a novel immunosuppressant that attenuates the production of various cytokines, and the differentiation of macrophages by inhibiting PAK2. In this study, we investigated the function of this drug mainly on macrophages using a rat small intestinal transplant model.

Methods: Male Dark Agouti (DA) and Lewis rats (LEW), 7-9 weeks of age, were used as donor and recipient, respectively. Approximately 15 cm intestinal grafts were heterotopically transplanted to the recipient rats. The recipient rat was treated with PQA-18 (4 mg/kg/day) by intraperitoneal injection (ip) from postoperative day 1 for 2 weeks. The in vivo effects of this drug were evaluated based on changes in body weight, and the population of each type of blood cell. Mixed lymphocyte reaction (MLR) was also assessed, using the T cells from intestinal mesenteric lymph nodes (MLN) of the grafts on POD6. Total cells from MLN and graft Payer's patch (PP) were next collected on POD6, and the number of infiltrated macrophages was determined.

Results: While the survival time was 7.0 ± 0.77 days for the control group (n = 9), that for the PQA-18 group was 10.7 ± 1.26 days (n = 10) (p < .001). Histological examinations showed a relatively clear difference in the grafts for both groups. In addition, the MLR response was significantly lower in recipients treated with PQA-18, suggesting PQA-18 well suppressed the T cells. Moreover, while a significant increase of both MHC class II and CD11b/c positive cells, estimated as differentiated/polarized macrophages, in MLN & PP was observed in the control group, PQA-18-administration significantly suppressed the differentiation of macrophages in the MLN & PP.

Conclusion: PQA-18 significantly prolonged the survival of the rats with intestinal grafts, and also suppressed the infiltration of lymphocytes, and macrophages to the grafts.
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http://dx.doi.org/10.1016/j.trim.2019.101246DOI Listing
December 2019

Serum Mac-2-binding protein (M2BPGi) as a marker of chronological liver fibrosis in biliary atresia patients with cirrhosis.

Pediatr Surg Int 2019 Oct 7;35(10):1065-1070. Epub 2019 Aug 7.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, 565-0871, Osaka, Japan.

Purpose: Biliary atresia (BA) is characterized by progressive liver fibrosis, but it is difficult to assess the progression after the patient develops cirrhosis. Mac-2-binding protein glycosylation isomer (M2BPGi) is a new marker for hepatic fibrosis. We examined the chronological changes in M2BPGi levels in BA patients with cirrhosis.

Methods: Patients with cirrhosis were selected from among pediatric BA patients who had their native livers. Serum M2BPGi levels and Child-Pugh classification were evaluated. A total of 11 pediatric BA patients with cirrhosis were recruited.

Results: Initial M2BPGi level after diagnosis of liver cirrhosis based on liver biopsy was on average 3.4, and the most recent M2BPGi level under observation was on average 4.3. The follow-up period from the initial M2BPGi measurement averaged 22.6 months. The ratio of the initial and most recent values (M2BPGi ratio) was on average 1.3 (0.5-2.4). Three cases with improved fibrosis (M2BPGi ratio < 1.0) remained in Child A, as did six cases (1.0 ≤ M2BPGi ratio < 2.0), but two cases with marked fibrosis progression (2.0 ≤ M2BPGi ratio) advanced to decompensated cirrhosis Child B.

Conclusion: M2BPGi is useful as a prognostic factor for BA patients with liver cirrhosis. In addition, fibrosis improved even after the development of cirrhosis.
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http://dx.doi.org/10.1007/s00383-019-04535-9DOI Listing
October 2019

Recombinant human soluble thrombomodulin reduces the severity and incidence of necrotizing enterocolitis in a newborn rat model.

Surg Today 2019 Nov 12;49(11):971-976. Epub 2019 Jun 12.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: Necrotizing enterocolitis (NEC) remains the leading cause of death in preterm infants. Recombinant human soluble thrombomodulin (rTM) has been reported to have anti-inflammatory effects as well as antithrombogenic effects. The aim of this study was to evaluate the effect of rTM in a rat NEC model.

Methods: NEC was induced by enteral feeding with hyperosmolar formula, gavage administration of lipopolysaccharide and asphyxia stress. Controls were fed by their mother ad libitum. In the treatment group, rTM was administered subcutaneously twice (once each on the first and second day). All animals surviving beyond 96 h or that developed signs of distress were euthanized. The ileum was harvested for a histological evaluation and the measurement of the mRNA and protein expression.

Results: The rate of NEC-like intestinal injury in the treatment group (9/25, 36%) was significantly lower than in the NEC group (25/34, 73.5%). Tissue levels of TNF-α, IL-6 and HMGB1 were significantly elevated in the NEC group, whereas those in the treatment group were decreased to similar values as in the control group.

Conclusions: Our experimental study showed that rTM is able to reduce the severity and incidence of NEC. It may be an alternative option for the treatment of NEC.
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http://dx.doi.org/10.1007/s00595-019-01832-7DOI Listing
November 2019

The effect of maternal grafts in early acute cellular rejection after pediatric living-donor liver transplantation.

Pediatr Surg Int 2019 Jul 20;35(7):765-771. Epub 2019 May 20.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: Living-donor liver transplantations (LDLTs) with maternal grafts can be more successful than those with paternal grafts because of their tolerance to non-inherited maternal antigens. We reviewed LDLT patients to investigate the relationship between acute rejection and donor sex.

Methods: LDLT patients between January 2010 and November 2015 were enrolled. ACR was defined by a rejection activity index of > 3.

Results: Forty-six patients (22 males and 24 females), of whom 28 had biliary atresia, were enrolled. The median age of the patients was 2.8 years and the donor types were maternal (n = 25) and paternal (n = 21). Acute cellular rejection (ACR) was observed in 22 patients. Twelve (48%) of the 25 patients in the maternal group had at least one episode of rejection compared with 10 (48%) of the 21 in the paternal group. Among the patients with ACR, the first rejection in the maternal group occurred significantly earlier than that in the paternal group (p < 0.01). In the multivariable analysis, the only variable significantly related to the first rejection day after LDLT was donor sex (male) (p < 0.005).

Conclusion: Our results showed that maternal grafts had an effect on causing earlier ACR in LDLT.
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http://dx.doi.org/10.1007/s00383-019-04487-0DOI Listing
July 2019

Long-term outcomes of patch tracheoplasty using collagenous tissue membranes (biosheets) produced by in-body tissue architecture in a beagle model.

Surg Today 2019 Nov 16;49(11):958-964. Epub 2019 May 16.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka Suita, Osaka, 565-0871, Japan.

Purpose: Although various artificial tracheas have been developed, none have proven satisfactory for clinical use. In-body tissue architecture (IBTA) has enabled us to produce collagenous tissues with a wide range of shapes and sizes to meet the needs of individual recipients. In the present study, we investigated the long-term outcomes of patch tracheoplasty using an IBTA-induced collagenous tissue membrane ("biosheet") in a beagle model.

Methods: Nine adult female beagles were used. Biosheets were prepared by embedding cylindrical molds assembled with a silicone rod and a slitting pipe into dorsal subcutaneous pouches for 2 months. The sheets were then implanted by patch tracheoplasty. An endoscopic evaluation was performed after 1, 3, or 12 months. The implanted biosheets were harvested for a histological evaluation at the same time points.

Results: All animals survived the study. At 1 month after tracheoplasty, the anastomotic parts and internal surface of the biosheets were smooth with ciliated columnar epithelium, which regenerated into the internal surface of the biosheet. The chronological spread of chondrocytes into the biosheet was observed at 3 and 12 months.

Conclusions: Biosheets showed excellent performance as a scaffold for trachea regeneration with complete luminal epithelium and partial chondrocytes in a 1-year beagle implantation model of patch tracheoplasty.
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http://dx.doi.org/10.1007/s00595-019-01818-5DOI Listing
November 2019

Postoperative decrease in plasma acyl ghrelin levels after pediatric living donor liver transplantation in association with hepatic damage due to ischemia and reperfusion injury.

Pediatr Surg Int 2019 Jun 5;35(6):709-714. Epub 2019 Mar 5.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: Ghrelin was recently reported to promote recovery from hepatic injury. We hypothesized that it could also be associated with clinical recovery of the transplanted liver from ischemia and reperfusion injury. Our aims were to investigate perioperative ghrelin changes following pediatric living donor liver transplantation (LDLT) and to analyze the association of these changes with postoperative hepatic function.

Methods: We measured plasma acyl ghrelin (AG) concentrations before surgery, at the end of surgery and on postoperative days (PODs) 1, 3 and 7 in 12 children who underwent LDLTs, and, as controls, pre- and post-operatively and on POD1 in 7 children who underwent benign abdominal mass resection. The correlations between the participants' ghrelin profiles and hepatic function-related data were evaluated.

Results: AG levels significantly declined to 15.6% of preoperative levels after LDLT and almost returned to baseline on POD3. Post-operative AG levels were significantly reduced to a greater extent following LDLT than benign abdominal mass resection. AG levels on POD1 inversely correlated with aspartate aminotransferase levels and cold/total ischemia time (P < 0.05).

Conclusion: These results suggest that reduced AG levels on POD1 may reflect the degree of damage to the transplanted liver due to ischemia and reperfusion injury.
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http://dx.doi.org/10.1007/s00383-019-04463-8DOI Listing
June 2019

Safety and efficacy of laparoscopic percutaneous extraperitoneal closure for inguinal hernia in infants younger than 6 months: A comparison with conventional open repair.

Asian J Endosc Surg 2019 Oct 18;12(4):439-445. Epub 2018 Dec 18.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Japan.

Introduction: This study aimed to compare the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) and conventional open repair (OR) for inguinal hernia in infants younger than 6 months.

Methods: The medical records of 202 patients who had undergone LPEC or OR at an age younger than 6 months between 2010 and 2016 were reviewed. Patient characteristics and surgical outcomes were compared between LPEC and OR.

Results: In total, there were 120 LPEC (76 males, 44 females) and 83 OR (76 males, 7 females) cases. There were no differences in median age, median bodyweight, and presence of incarceration between the two groups (LPEC group: 3 months (range, 0-5 months), 5.7 kg (range, 2.3-9.6 kg), and 13.3%; OR group: 3 months (range, 1-5 months), 5.5 kg (range, 2.1-9.0 kg), and 12.0%). LPEC was completed in all cases without any intraoperative complications or open conversions. In the LPEC group, 60.0% of unilateral hernia patients had a contralateral patent processus vaginalis and underwent prophylactic closure. The incidence of metachronous contralateral hernia was significantly lower in the LPEC group than in the OR group (0% vs 10.0%, P < 0.01). Operative time was significantly shorter during LPEC than during OR (P < 0.01) for male patients. The recurrence rate was lower in the LPEC group (0.83%) than in the OR group (2.4%), but the difference was not significant.

Conclusion: The present data indicate that LPEC is comparable to OR in terms of safety and efficacy in infants younger than 6 months. Moreover, among the LPEC group, there were fewer incidences of metachronous contralateral hernia, and the procedure had a shorter operative time.
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http://dx.doi.org/10.1111/ases.12676DOI Listing
October 2019

Laparoscopic ligation of a congenital extrahepatic portosystemic shunt for children with hyperammonemia: a single-institution experience.

Surg Today 2019 Apr 3;49(4):323-327. Epub 2018 Nov 3.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2, Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: A congenital extrahepatic portosystemic shunt (CEPS) associated with hyperammonemia requires occlusion of the shunt vessels. We evaluated the effectiveness and safety of laparoscopic ligation of a CEPS in children with hyperammonemia.

Methods: The subjects of this retrospective study were seven children with hyperammonemia who underwent laparoscopic ligation of a CEPS. Their median age was 5.2 years (range 1-16 years). Before the laparoscopic procedure, a catheter was inserted through the femoral vein and placed in the portal vein via the shunt vessel. The shunt vessel was dissected and taped laparoscopically. After measuring the portal vein pressure under temporal occlusion, the shunt vessels were ligated.

Results: The types of shunts according to location were patent ductus venosus (n = 2), splenorenal shunt (n = 2), gastrorenal shunt (n = 2), and superior mesenteric vein-inferior vena cava shunt (n = 1). Laparoscopic ligation of the shunt vessel was completed uneventfully in all patients. The median portal vein pressure was 19 mmHg after ligation. The median preoperative blood ammonia level was 94 µg/dL (range 71-259 µg/dL), which decreased after ligation in all patients. There was no incidence of postoperative liver failure.

Conclusion: Laparoscopic ligation of a CEPS is safe and effective for children with hyperammonemia.
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http://dx.doi.org/10.1007/s00595-018-1731-yDOI Listing
April 2019

Tracheal Replacement Using an In-Body Tissue-Engineered Collagenous Tube "BIOTUBE" with a Biodegradable Stent in a Beagle Model: A Preliminary Report on a New Technique.

Eur J Pediatr Surg 2019 Feb 2;29(1):90-96. Epub 2018 Nov 2.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Introduction:  Tracheal reconstruction for long-segment stenosis remains challenging. We investigate the usefulness of BIOTUBE, an in-body tissue-engineered collagenous tube with a biodegradable stent, as a novel tracheal scaffold in a beagle model.

Materials And Methods:  We prepared BIOTUBEs by embedding specially designed molds, including biodegradable stents, into subcutaneous pouches in beagles. After 2 months, the molds were filled with ingrown connective tissues and were harvested to obtain the BIOTUBEs. The BIOTUBEs, cut to 10- or 20-mm lengths, were implanted to replace the same-length defects in the cervical trachea of five beagles. Endoscopic and fluoroscopic evaluations were performed every week until the lumen became stable. The trachea, including the BIOTUBE, was harvested and subjected to histological evaluation between 3 and 7 months after implantation.

Results:  One beagle died 28 days after 20-mm BIOTUBE implantation because of insufficient expansion and retention force of the stent. The remaining four beagles were implanted with a BIOTUBE reinforced by a strong stent, and all survived the observation period. Endoscopy revealed narrowing of the BIOTUBEs in all four beagles, due to an inflammatory reaction, but patency was maintained by steroid application at the implantation site and balloon dilatation against the stenosis. After 2 months, the lumen gradually became wider. Histological analyses showed that the internal surface of the BIOTUBEs was completely covered with tracheal epithelial cells.

Conclusion:  This study demonstrated the usefulness of the BIOTUBE with a biodegradable stent as a novel scaffold for tracheal regeneration.
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http://dx.doi.org/10.1055/s-0038-1673709DOI Listing
February 2019

Clinical implications of serum Mac-2-binding protein (M2BPGi) during regular follow-up of patients with biliary atresia.

Pediatr Surg Int 2018 Oct 20;34(10):1065-1071. Epub 2018 Aug 20.

Department of Pediatric Surgery, Osaka University of Graduation School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: The Mac-2-binding protein glycosylation-modified isomer (M2BPGi) is a new marker for progression of hepatic fibrosis. We examined the relationship between serum M2BPGi levels and liver histological findings in patients with biliary atresia (BA) who were not transplant candidates.

Methods: Patients with BA who were not transplant candidates with good liver function were included. We examined M2BPGi levels and histological findings in relation to other laboratory markers of liver fibrosis, including aspartate aminotransferase (AST) to platelet ratio index, fibrosis-4 index, and type IV collagen 7s domain. Liver fibrosis was evaluated based on the METVIR score.

Results: 37 patients were included. The median age was 18 years (range 3-38 years). M2BPGi values ranged from 0.3 to 6.9 cutoff index (COI) (median 1.6). The degree of liver fibrosis varied with M2BPGi level. For predicting cirrhosis (F4) and advanced liver fibrosis (≥ F3), M2BPGi had higher areas under the curve (AUCs; 0.93, respectively) with cutoff COIs of 1.84 and 1.67, respectively, than for the four conventional markers for fibrosis.

Conclusion: M2BPGi is a novel marker for liver fibrosis in patients with BA. It is especially useful for following patients with BA with a native liver and supporting liver biopsy interpretation findings.
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http://dx.doi.org/10.1007/s00383-018-4317-2DOI Listing
October 2018

Patch esophagoplasty using an in-body-tissue-engineered collagenous connective tissue membrane.

J Pediatr Surg 2018 Feb 13;53(2):223-226. Epub 2017 Nov 13.

Division of Medical Engineering and Materials, National Cerebral and Cardiovascular Centre Research Institute, Osaka, Japan.

Aim: Although many approaches to esophageal replacement have been investigated, these efforts have thus far only met limited success. In-body-tissue-engineered connective tissue tubes have been reported to be effective as vascular replacement grafts. The aim of this study was to investigate the usefulness of an In-body-tissue-engineered collagenous connective tissue membrane, "Biosheet", as a novel esophageal scaffold in a beagle model.

Methods: We prepared Biosheets by embedding specially designed molds into subcutaneous pouches in beagles. After 1-2months, the molds, which were filled with ingrown connective tissues, were harvested. Rectangular-shaped Biosheets (10×20mm) were then implanted to replace defects of the same size that had been created in the cervical esophagus of the beagle. An endoscopic evaluation was performed at 4 and 12weeks after implantation. The esophagus was harvested and subjected to a histological evaluation at 4 (n=2) and 12weeks (n=2) after implantation. The animal study protocols were approved by the National Cerebral and Cardiovascular Centre Research Institute Committee (No. 16048).

Results: The Biosheets showed sufficient strength and flexibility to replace the esophagus defect. All animals survived with full oral feeding during the study period. No anastomotic leakage was observed. An endoscopic study at 4 and 12weeks after implantation revealed that the anastomotic sites and the internal surface of the Biosheets were smooth, without stenosis. A histological analysis at 4weeks after implantation demonstrated that stratified squamous epithelium was regenerated on the internal surface of the Biosheets. A histological analysis at 12weeks after implantation showed the regeneration of muscle tissue in the implanted Biosheets.

Conclusion: The long-term results of patch esophagoplasty using Biosheets showed regeneration of stratified squamous epithelium and muscular tissues in the implanted sheets. These results suggest that Biosheets may be useful as a novel esophageal scaffold.
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http://dx.doi.org/10.1016/j.jpedsurg.2017.11.004DOI Listing
February 2018

Image-based surgical risk factors for Wilms tumor.

Pediatr Surg Int 2018 Jan 8;34(1):29-34. Epub 2017 Nov 8.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2, Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: The standard treatment for Wilms tumor (WT) is primary resection. However, in cases with unresectable tumor or tumor spillage, which are considered to have high surgical risks, more intensive chemotherapy and radiotherapy are required. In the present study, we retrospectively analyzed preoperative image parameters to identify factors associated with surgical risks.

Methods: Twenty-nine patients with WT were enrolled in this study. Data on various preoperative image parameters, such as tumor size, tumor volume, displacement of great vessels, and contralateral extension of the tumor were collected, and their relationship with surgical factors, including operative time, hemorrhage, tumor spillage, and unresectability were analyzed.

Results: Patients with unresectable tumor or with tumor spillage (surgical high-risk group) more frequently demonstrated displacement of great vessels and contralateral tumor extension. Operative time and blood loss were also significantly related to tumor size, area, volume, displacement of great vessels and contralateral extension.

Conclusion: Besides tumor size, displacement of great vessels and contralateral extension were significantly associated with surgical risks. These factors are easily determined using CT images and are, therefore, useful to decide whether preoperative chemotherapy should be started instead of primary tumor resection for large localized WTs.
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http://dx.doi.org/10.1007/s00383-017-4210-4DOI Listing
January 2018

Clinicopathological features of neuroblastic tumors with opsoclonus-myoclonus-ataxia syndrome: Follicular structure predicts a better neurological outcome.

Pathol Int 2017 Oct;67(10):503-509

Department of Pediatric Hematology and Oncology, Osaka City General Hospital, Miyakojima-ku, Osaka, Japan.

Neuroblastic tumors (NT) with opsoclonus-myoclonus syndrome (OMS) display characteristic histological features, such as lymphocytic infiltration with lymphoid follicles, indicating an underlying immune response. We retrospectively assessed NT patients from 2001 to 2016. Five cases of NT with OMS and 76 cases of NT without OMS were histopathologically reviewed in this study. The grade of lymphocytic infiltration was evaluated. The number of follicles was counted and the presence or absence of lymphoid follicles was recorded for each case. We also confirmed the presence or absence of follicular dendritic cells (FDCs). We investigated the relationship between the histopathological and clinical findings of NT with OMS. Lymphocytic infiltration was observed in all cases; however, the precise follicular structure was occasionally unclear. Patients with clear follicular structures displayed germinal centers including tingible body macrophages and FDCs. All patients without neurological sequelae demonstrated a clear follicular structure with a FDC meshwork pattern. The interval between OMS onset and the detection and initial treatment of NT was typically longer in patients with neurological sequelae compared to those without neurological sequelae. Early detection and treatment of NT with OMS at the phase of a clear follicular formation with multiple FDC may provide favorable neurological outcomes.
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http://dx.doi.org/10.1111/pin.12591DOI Listing
October 2017

The role of pulmonary metastasectomy for hepatoblastoma in children with metastasis at diagnosis: Results from the JPLT-2 study.

J Pediatr Surg 2017 Dec 4;52(12):2051-2055. Epub 2017 Sep 4.

Japan Children's Cancer Group (JCCG) liver tumor committee (JPLT).

Background/purpose: The purpose of this study was to clarify the role of pulmonary metastasectomy in hepatoblastomas with lung metastasis at diagnosis. We reviewed cases enrolled in the JPLT-2 study.

Methods: A total of 360 cases with hepatoblastoma were enrolled. The clinical courses and outcome of 60 cases with pulmonary metastasis at diagnosis were reviewed, focusing on metastasectomy.

Results: Induction chemotherapy resulted in eradication of nodules in 26, residual nodules in 33, and early treatment-related death in one. Of the 33 cases with residual nodules, 11 underwent complete resection of the lung lesions, and among these, progression was reported in five. Complete resection of the liver tumor was not achieved in two of these. Three underwent incomplete resection of lung nodules, eventually leading to progression. Twelve cases with incomplete or no liver tumor resection progressed regardless of the status of lung lesions. Contrarily, among patients who underwent complete resection of the liver tumor, half were cured without metastasectomy.

Conclusions: Metastasectomy for residual pulmonary nodules after induction chemotherapy is effective provided that the liver tumor could be completely resected.

Type Of Study: Prospective Cohort Study.

Level Of Evidence: Level II.
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http://dx.doi.org/10.1016/j.jpedsurg.2017.08.031DOI Listing
December 2017

Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia.

Pediatr Surg Int 2017 Oct 4;33(10):1053-1057. Epub 2017 Sep 4.

Department of Pediatric Surgery, Osaka University of Graduation School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients.

Methods: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH.

Results: The study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8-26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied.

Conclusion: The onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.
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http://dx.doi.org/10.1007/s00383-017-4136-xDOI Listing
October 2017

Learning curve for the thoracoscopic repair of esophageal atresia with tracheoesophageal fistula.

Asian J Endosc Surg 2018 Feb 18;11(1):30-34. Epub 2017 Jul 18.

Department of Pediatric Surgery, Kindai University Nara Hospital, Nara, Japan.

Aim: Thoracoscopic repair (TR) of esophageal atresia with tracheoesophageal fistula (EA/TEF) remains a considerable challenge, even for the most experienced pediatric surgeons. The aim of this study is to report the outcomes of our experience with TR of EA/TEF and to determine the learning curve for this procedure.

Methods: Eleven consecutive cases that had undergone TR of EA/TEF at our institutes were included in this study. The medical charts were reviewed retrospectively. To determine the learning curve for TR of EA/TEF, a logarithmic curve-fitting analysis was performed. The data were expressed as medians with ranges.

Results: The median age and birth weight were 1 day (range, 1-3 days) and 2.8 kg (range, 2.5-3.7 kg), respectively. TR was completed in all cases without any complications. The median operative time was 230 min (range, 164-383 min). There were no cases of anastomotic leakage. One patient with a long gap required repeated balloon dilatation for refractory anastomotic stricture. No mortality or recurrence of tracheoesophageal fistula occurred. The operative time was significantly longer in patients with a long gap (>20 mm) than in those with a shorter gap. Once the three cases with a long gap had been excluded, the operative time decreased as the number of treated cases increased. The relationship between the operative time and case number fit a logarithmic function curve well (operative time in minutes = 300 - 62 × log (case number), R  = 0.8359, P = 0.0015).

Conclusions: Our results suggest that TR of EA/TEF is a safe procedure. It has a considerable learning curve, but requires advanced endoscopic surgical skills.
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http://dx.doi.org/10.1111/ases.12411DOI Listing
February 2018

Magnetic compression anastomosis for postoperative biliary atresia.

Pediatr Int 2017 Jun;59(6):737-739

Department of Pediatric Surgery, Graduate School of Medicine, Osaka University, Suita, Osaka, Japan.

We report a case of successful magnetic compression anastomosis (MCA) for obstructed cyst-jejunostomy in a young woman who had undergone surgery for type 1 biliary atresia (BA) on day 78 of life. A 16-year-old girl was admitted with obstructive jaundice. Jaundice resolved with percutaneous trans-hepatic cholangiodrainage (PTCD) but contrast medium injected from the PTCD tube did not flow through the anastomosis. Magnets were placed on each side of the anastomosis, in the cyst and the jejunum, to compress the partition. On postoperative day (POD) 6, the anastomosis was recanalized and the PTCD tube placed trans-anastomotically until POD 245. The patient remained free from jaundice after removal of the PTCD tube. MCA can be a useful and less invasive procedure for treating biliary tract anastomotic obstruction in patients with BA.
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http://dx.doi.org/10.1111/ped.13295DOI Listing
June 2017

Long-term morbidity in adolescents and young adults with surgically treated esophageal atresia.

Surg Today 2017 Jul 27;47(7):872-876. Epub 2016 Dec 27.

Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodocho, Izumi, Osaka, 594-1101, Japan.

Purpose: To investigate the long-term morbidity of surgically treated esophageal atresia (EA) in adolescents and young adults and establish whether these long-term morbidities are affected by the type of EA.

Patients And Methods: We reviewed the medical records, including backgrounds and associated conditions, of 69 long-term survivors of EA, aged >15 years. The long-term morbidities included neurodevelopmental abnormality, nutritional impairment (short height <-2SD, low BMI <18.5), subjective symptoms, and musculoskeletal deformities. Comparisons of the results were made between Gross A-type EA (n = 6) and Gross C-type EA (n = 63).

Results: All patients underwent esophageal anastomosis without esophageal replacement. Cardiac anomalies and long gap were present in 26 and 18%, respectively. Esophageal dilatation, fundoplication, and aortopexy were performed in 40, 34, and 18%, respectively. The incidence of long gap and esophageal stenosis was higher in Gross A-type EA than in Gross C-type EA. The long-term morbidities included neurodevelopmental abnormality (13%), nutritional impairment (62%: as short height in 34% and as low BMI in 46%), subjective symptoms (14%), and musculoskeletal deformities (59%). There were no differences in the long-term morbidities between Gross A and Gross C.

Conclusions: The incidence of the long-term morbidities is high in adolescents and young adults, regardless of the type of EA. Early detection of morbidity is important to improve the long-term outcomes of EA.
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http://dx.doi.org/10.1007/s00595-016-1462-xDOI Listing
July 2017

Implantable central venous access device in infants: Long-term results.

Pediatr Int 2016 Oct 8;58(10):1027-1031. Epub 2016 Sep 8.

Department of Pediatric Surgery, Osaka City General Hospital, Osaka, Japan.

Background: The management of central venous access device (CVAD) is extremely difficult in babies and small infants (BSI). We therefore compared the long-term results and complications of CVAD in BSI with those in children.

Methods: One hundred and twenty patients were divided into two groups as follows: age <1 year or weight <10 kg (group A; 25 patients); and age ≧1 year and weight ≧10 kg (group B; 95 patients). The clinical results were retrospectively compared between the groups.

Results: Mean age and weight were 12.5 ± 5.9 months and 8.2 ± 1.2 kg in group A, and 78.8 ± 58.9 months and 20.9 ± 13.6 kg in group B. Operation time was 57 ± 29 min in group A and 52 ± 21 min in group B (P = 0.38). The catheter was advanced with difficulty into the central vein in five and in 16 patients (P = 0.77), and surgical complications occurred in one and in seven patients in groups A and B, respectively (P > 0.99). The CVAD remained in place for 627 ± 494 and 550 ± 414 days (P = 0.47) and was removed before treatment completion in five and in 14 patients in groups A and B, respectively (P = 0.54).

Conclusion: The clinical results for CVAD in BSI did not differ from those in children. CVAD are useful and safe for the treatment of BSI with serious diseases.
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http://dx.doi.org/10.1111/ped.12945DOI Listing
October 2016

Early Detection and Treatment of Neuroblastic Tumor with Opsoclonus-Myoclonus Syndrome Improve Neurological Outcome: A Review of Five Cases at a Single Institution in Japan.

Eur J Pediatr Surg 2016 Feb 26;26(1):54-9. Epub 2015 Sep 26.

Department of Pediatric Hematology and Oncology, Osaka City General Hospital, Osaka, Japan.

Introduction: Opsoclonus-myoclonus syndrome (OMS) is a paraneoplastic neurological disorder associated with neuroblastic tumor (NT) in childhood. Half of patients have neurological sequelae after the neurological and oncological treatment. We reviewed the neurological and oncological outcomes of NT with OMS, and discussed whether the treatment of NT would contribute to improving the neurological prognosis.

Methods: We retrospectively assessed NT patients with OMS from January 2001 to December 2013 at a single institution in Japan. Demographic data, neurological and oncological status, histopathology, treatments, prognosis, and diagnosis and treatment timing were retrospectively reviewed from the records. The timings assessed were the interval between OMS onset and NT detection, initial NT therapy, and initial OMS therapy, the interval between NT therapy and OMS remission, and duration of OMS.

Results: A total of 73 patients with NT were treated during the study period, and 5 of 73 patients were diagnosed as having NT with OMS. The median age at onset of OMS was 22 months (range, 18-30 months). The median age at detection of NT was 29 months (range, 21-33 months). Three of five cases showed no uptake on meta-iodobenzylguanidine scintigraphy. The tumor histopathology was neuroblastoma in two patients, ganglioneuroblastoma in two patients, and ganglioneuroma in one patient. Primary resection was performed in three cases. All patients survived. Two of five cases presented with atypical neurological symptoms without opsoclonus. The initial neurological therapy was started within a mean of 20 days (range, 3-76 days) from the onset of OMS in all cases. Four patients received intravenous immunoglobulin, and one with persistent neurological problems received rituximab. Neurological symptoms resolved in three cases. The mean interval between the onset of OMS and the detection of NT in case without neurological sequelae was 57 days (range, 25-113 days), while in case with neurological sequelae it was 365 days (range, 271-458 days). The mean interval between onset of OMS and initial therapy for NT in case without neurological sequelae was 88 days (range, 47-145 days), while in case with neurological sequelae it was 389 days (range, 292-486 days).

Conclusion: The interval between the onset of OMS and the detection and initial therapy of NT tended to be longer in patients with neurological sequelae than in those without neurological sequelae. This study suggested that early detection and treatment of NT with OMS might improve the neurological outcomes.
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http://dx.doi.org/10.1055/s-0035-1564714DOI Listing
February 2016

Fibroadenoma in Beckwith-Wiedemann syndrome with paternal uniparental disomy of chromosome 11p15.5.

Pediatr Int 2014 Dec;56(6):931-934

Division of Molecular Genetics and Epigenetics, Department of Biomolecular Sciences, Faculty of Medicine, Saga University, Saga, Japan.

Herein is described a case of breast fibroadenomas in a 16-year-old girl with Beckwith-Wiedemann syndrome (BWS) and uniparental disomy (UPD) of chromosome 11p15.5. She was clinically diagnosed with BWS and direct closure was performed for an omphalocele at birth. Subtotal and 90% pancreatectomy were performed for nesidioblastosis at the ages 2 months and 8 years, respectively. Bilateral multiple breast fibroadenomas were noted at the age of 16 and 17 years. In this case, paternal UPD of chromosome 11p15.5 was identified on microsatellite marker analysis. The relevant imprinted chromosomal region in BWS is 11p15.5, and UPD of chromosome 11p15 is a risk factor for BWS-associated tumorigenicity. Chromosome 11p15.5 consists of imprinting domains of IGF2, the expression of which is associated with the tumorigenesis of various breast cancers. This case suggests that fibroadenomas occurred in association with BWS.
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http://dx.doi.org/10.1111/ped.12406DOI Listing
December 2014

Familial Currarino syndrome associated with Hirschsprung disease: two cases of a mother and daughter.

J Pediatr Surg 2013 Jan;48(1):233-8

Department of Pediatric Surgery, Osaka City General Hospital, Osaka, Japan.

Currarino syndrome with Hirschsprung disease (CS-HD) is extremely rare. We present the first family with CS-HD. Case 1: A 28-year-old woman was admitted with severe abdominal distension and dyspnea. She was diagnosed with anal stenosis, hemisacrum, anterior sacral meningocele (ASM), tethered cord (TC), and short-segment aganglionosis. She underwent the modified Duhamel operation after meningocele repair and cord detethering. A bicornuate uterus, bilateral ovarian dermoid cysts, and small rectal duplication were also noted intraoperatively. Case 2: The daughter of case 1 was admitted for abdominal distension and anal stenosis at the age of 17 days. Studies revealed a hemisacrum, ASM, TC, presacral mass, atrial septal defect, polyp in the right nasal cavity, right vesicoureteral reflux, and short-segment aganglionosis. She underwent the modified Soave operation at the age of 1 year and 4 months after meningocele repair, cord detethering, and resection of the presacral mass (epidermoid cyst). In both cases, the aganglionic segments were confirmed by preoperative rectal suction biopsy and postoperative pathological examination on full-thickness rectal specimens. Some causal genes for Currarino syndrome (CS) and Hirschsprung disease (HD) are currently investigated. Thus far, 10 CS-HD cases have been reported, including 6 cases of familial CS. However, all the patients had sporadic HD. Recent reports suggest that anomalies of the enteric nerve system contribute to postoperative constipation in CS cases.
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http://dx.doi.org/10.1016/j.jpedsurg.2012.10.061DOI Listing
January 2013

Trial using pig cells with the H-D antigen knocked down.

Surg Today 2013 Jul 5;43(7):782-6. Epub 2012 Aug 5.

Division of Organ Transplantation (E9), Department of Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Purpose: This report describes an attempt to reduce the expression level of Hanganutziu-Deicher (H-D) antigens by small interfering RNA (siRNA) for pig cytidine monophospho-N-acetylneuraminic acid hydroxylase (pCMAH).

Methods: A pig endothelial cell (PEC) line, and PEC and fibroblasts from an α1,3galactosyltransferase knockout (GalT-KO) piglet were used. Real-time PCR was used to evaluate the degradation of mRNA by siRNA. The H-D antigen was stained, and then the cells were incubated with human serum for the FACS analysis. The extent of lysis in human serum was next calculated using an LDH assay.

Results: Suppression of the mRNA of pCMAH by each siRNA was first determined. The mixture of siRNAs for pCMAH reduced the expressions of the H-D antigen on the PEC and fibroblasts to a considerable extent. The further reduction in the xenoantigenicity for human serum of the GalT-KO cells was then confirmed. In addition, the PEC line showed a significant downregulation in complement-dependent cytotoxicity by the siRNAs, thus indicating that the anti-H-D antigen in human serum is capable of causing lysis of the pig cells.

Conclusion: pCMAH silencing by siRNA reduced the expression of the H-D antigen and its antigenicity, thus confirming that the H-D antigen is one of the major non-Gal antigens in this situation.
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http://dx.doi.org/10.1007/s00595-012-0274-xDOI Listing
July 2013