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PubMed | Organ Transplantation Center and Second Military Medical University
Type: Journal Article | Journal: Journal of digestive diseases | Year: 2016

To establish a non-invasive model for the assessment of portal venous pressure (PVP) based on the magnetic resonance (MR) parameters.In this prospective study, contrast-enhanced magnetic resonance imaging (MRI) scan was performed in 109 patients indicated for upper abdominal surgeries after their written consents were obtained, and intraoperative PVP measurements were completed in 92 patients. Altogether 17 patients were excluded for not undergoing surgery or unsuccessful catheterization. A linear model was constructed for estimating PVP levels in 56 patients and further validation was conducted in the other 36 patients.The PVP levels were significantly correlated with MR parameters, including splenic volume (SV), splenic venous diameter (SVD), liver/splenic volume ratio, portal venous diameter, hepatic diameter, portal venous cross-sectional area, ascites, varices and arterial portal shunts. A linear model was established as follows: PVP (mmHg) = 2.529 + 1.572 SVD (mm) + 0.231 SV/body mass index ( 10(4) cm(5) /kg) + 3.44aspartate aminotransferase-to-platelet ratio index. This model showed excellent accuracy in the detection of portal hypertension, with the area under the receiver operating characteristic curve (AUROC) of 0.945 (95% CI 0.867-1.000), with the sensitivity and specificity of 91.7% and 93.7%, respectively. The agreement analysis revealed that the predictive value using this formula closely reflected the patients actual PVP level. Moreover, the validation confirmed the accuracy of this model for the assessment of portal hypertension [AUROC 0.935 (95% CI 0.856-1.000)].The MRI-based formula has great potential for detecting portal hypertension. As a non-invasive measurement, it may be clinically accepted for the replacement of invasive modalities after further refinement.

PubMed | National Center for Child Health and Development, Organ Transplantation Center and Tokyo Electron
Type: Journal Article | Journal: Journal of pediatric surgery | Year: 2016

Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to improve the outcomes in patients undergoing LT for unresectable HB.Twelve children received LT for unresectable HB. In 9 patients, we applied early exclusion of hepatic inflow (hepatic artery and portal vein) and creation of a temporary portocaval shunt during LT.There were differences in the duration of and the blood loss during operation as compared with previously reports. The estimated glomerular filtration rate was well preserved at 3, 6, and 12months and the latest follow-up after LT, and the recurrence-free survival was 88.9%.Early inflow control during LT for unresectable HB may benefit recurrence-free survival by minimizing blood loss and tumor dissemination, preserving renal function and allowing early adjuvant chemotherapy.

PubMed | National Center for Child Health and Development, Aichi University, Jichi Medical University and Organ Transplantation Center
Type: Journal Article | Journal: Pediatric transplantation | Year: 2016

As the priority of LD-Domino LT is the safety of the first recipient, limitations and technical difficulties in the second recipient often occur. The most technically challenging part of LD-Domino LT is the reconstruction of the vessels. For the reconstruction of HVs, the native HVs were exteriorized as far as possible using a CUSA because longer extensive HVs are essential for facilitating the reconstruction. At the back table, the HVs of the domino graft were sutured together, and the single cuff of the HVs was anastomosed to the IVC by joining the orifices. The HAs, the presence of insufficient length, and multiple vessels in the whole liver rendered the reconstruction more difficult. We determined the dividing sites of the vessels according to the preoperative 3D-CT findings obtained in two institutions. This is the first case series using grafts in DLT obtained from LDLT for patients with MSUD between two institutions. In conclusion, LD-Domino LT is a safe and feasible therapeutic option to expand the donor pool by technical refinement in the reconstruction of the second recipient. Further studies with a greater accumulation of patients and a longer follow-up will be necessary to establish LD-Domino LT using an MSUD donor.

PubMed | National Center for Child Health and Development, Osaka City General Hospital, Kyoto University and Organ Transplantation Center
Type: Journal Article | Journal: Pathology international | Year: 2016

The hepatocyte paraffin 1 (Hep Par 1) antibody is widely used as a hepatocyte marker, recognizing carbamoyl phosphate synthetase 1 (CPS1), an essential component of the urea cycle. Various missense, nonsense, and frameshift mutations occur in the CPS1 gene. In neonatal patients with homozygous CPS1 deficiency (CPS1D), urea cycle defects with resulting severe hyperammonemia can be fatal, though liver transplantation provides a complete cure for CPS1D. We performed Hep Par 1 immunostaining in the explanted livers of 10 liver transplant patients with CPS1D. Seven were negative for Hep Par 1 in the hepatocytes and the other three showed normal diffuse granular cytoplasmic staining. As expected, all three Hep Par 1-positive patients had at least one missense mutation, and all four patients who had only nonsense or frameshift mutations were Hep Par 1-negative. The other three patients were unexpectedly negative for Hep Par 1, even though each had one missense mutation. These results suggest that CPS1D can be related to the loss of Hep Par 1 reactivity due to the loss of protein production, a one amino acid substitution resulting in an abortive protein product, or both. Hep Par 1 immunohistochemistry can be used as a simple method to confirm CPS1D.

Zeng Z.,Organ Transplantation Center | Huang H.F.,Organ Transplantation Center | Chen M.Q.,Organ Transplantation Center | Song F.,Organ Transplantation Center | Zhang Y.J.,Chongqing Medical University
Hepato-Gastroenterology | Year: 2010

Background/Aims: Ischemic postconditioning (Postcon) is a phenomenon that intermittent interruptions of blood flow in the early phase of reperfusion can protect organ against ischemia/reperfusion (I/R) injury. The potential application of postconditioning to liver is not available. In the present study, we investigated the effects of Postcon in liver I/R injury in rat liver transplantation models. Methodology: Adult male Sprague-Dawley rats were randomly allocated to three groups including sham group without I/R, I/R group with 24h cold ischemic donor liver before orthotopic liver transplantation, and Postcon group treated the same as I/R group and 6 cycles of 60s ischemia and 60s reperfusion at the onset of reperfusion. Peripheral blood samples were collected after reperfusion. Serum transaminases level, plasma cytokines concentration, histopathology, liver tissues malondialdehyde (MDA) level and superoxide dismutase (SOD) activity were measured. The heme oxygenase-1 (HO-1) expression levels of liver were evaluated. Results: Serum transaminases level and plasma cytokines concentration significantly decreased in Postcon group as compared to I/R group. Postcon treatment reduced MDA production and increased SOD activity compared with the I/R group. The HO-1 expression levels of liver were significantly higher in Postcon rats than in the I/R group at the end of reperfusion. Conclusions: These results indicate that Postcon ameliorates liver I/R injury. This protective effect is likely mediated by up-regulation of HO-1 expression. © H.G.E. Update Medical Publishing S.A.

To explore the efficacy and safety of autologous peripheral blood hematopoietic stem cell transplantation (APBHST) in patients with type 1 diabetes mellitus. Hematopoietic stem cells were mobilized with cyclophosphamide and granulocyte colony stimulating factor for 16 patients with type 1 diabetes mellitus who admitted to our department during November 2009 to August 2010. And then stem cells were collected from peripheral blood by leukapheresis and cryopreservation. The cells were infused intravenously after conditioning with cyclophosphamide and antithymocyte globulin. To compare the daily dose of exogenous insulin requirements, the serum levels of hemoglobin A1c (HbA1c), C-peptide, islet cell function during the mixed meal tolerance test were measured before and at different times after APBHST. Blood glucose was monitored 7 times a day before and after APBHST. And the adverse effects were recorded during and after APBHST. The median follow-up was 28 weeks (range: 8 - 44 weeks). Twelve of 16 patients stayed free from insulin at 3 - 20 days post APBHST. And islet cell function greatly improved after APBHST. Four of 16 patients required exogenous insulin but the dosage decreased. And all 4 patients had a poor level of C-peptide before APBHSCT. There were no such severe adverse effects as myelosuppression. Very encouraging results have been obtained in the patients treated with APBHST. There is definite therapeutic effects and safety in a short term. But further follow-up is necessary to confirm the duration of insulin independence and the mechanisms of action.

Lin J.,Organ Transplantation Center | Zeng Z.,Organ Transplantation Center
World Chinese Journal of Digestology | Year: 2010

Liver transplantation is the only effective treatment for end-stage liver diseases. Ischemia-reperfusion injury remains a major cause of post-transplantation liver dysfunction and even failure. Ischemic postconditioning is defined as rapid intermittent periods of reperfusion and ischemia in the early phase of repefusion after long ischemia of the tissues and organs. Many investigations have demonstrated that ischemic postconditioning has a protective effect against hepatic ichemia-reperfusion injury. Ischemic postconditioning exerts protective effects through many possible mechanisms such as oxygen free radicals, calcium overload, polymorphonuclear neutrophils, cytokines, cell apoptosis and mitochondria.

Kim S.H.,Organ Transplantation Center | Lee S.D.,Organ Transplantation Center | Kim Y.K.,Organ Transplantation Center | Park S.-J.,Organ Transplantation Center
World Journal of Gastroenterology | Year: 2014

Living donor right hepatectomy (LDRH) is currently the most common donor surgery in adult-to-adult living donor liver transplantation although the morbidity and mortality reported in living donors still contradicts the Hippocratic tenet of "do no harm". Achieving low complication rates in LDRH remains a matter of major concern. Living donor surgery is performed worldwide as an established solution to the donor shortage. The aim of this study was to assess the current status of LDRH and comment on the future of the procedure; assessment was made from the standpoint of optimizing the donor selection criteria and reducing morbidity based on both the authors' 8-year institutional experience and a literature review. New possibilities have been explored regarding selection criteria. The safety of living donors with unfavorable conditions, such as low remnant liver volume, fatty change, or old age, should also be considered. Abdominal incisions have become shorter, even without laparoscopic assistance; upper midline laparotomy is the primary incision used in more than 400 consecutive LDRHs in the authors' institution. Various surgical techniques based on preoperative imaging technology of vascular and biliary anomalies have decreased the anatomical barriers in LDRH. Operative time has been reduced, with low blood loss. Laparoscopic or robotic LDRH has been tried in only a few selected donors. The LDRHspecific, long-term outcomes remain to be addressed. The follow-up duration of these studies should be long enough to address possible late complications. Donor safety, which is the highest priority, is ensured by three factors: preoperative selection, intraoperative surgical technique, and postoperative management. These three focus areas should be continuously refined, with the ultimate goal of zero morbidity. © 2014 Baishideng Publishing Group Inc. All rights reserved.

Kasahara M.,Organ Transplantation Center
Nihon Geka Gakkai zasshi | Year: 2013

We reviewed our initial experience of deceased split liver transplantation in National Center for Child Health and Development and report the results herein. We have listed 50 patients with end-stage liver disease for deceased liver transplantation, and done 6 cases of split liver transplantation during October 2010 through October 2012. Variables including indication of liver transplantation outcome were reviewed. The indication for split liver transplantation was acute liver failure in 4, re-transplantation in 1 and primary sclerosing cholangitis in 1. The age of recipient ranged 17 days-15 yeras and body weight ranged 2.4 kg-55 kg. The split grafts were hyper-reduced left lateral segment in 1, left lateral segment in 3, left lobe in 1 and right lobe in 1. Although case #2 received retransplantation with living donor, all the patients are currently doing well with acceptable liver function with minimum follow-up of 2 months. Satisfactory result can be achieved on initial experience of split liver transplantation in National Center for Child Health and Development. The follow-up period was too short to make definitive conclusion, however, split liver transplantation could be expand potential donor pool and may reduce the waiting list mortality without alterations of the results.

PubMed | Organ Transplantation Center
Type: | Journal: Pediatric transplantation | Year: 2016

Ornithine transcarbamylase deficiency (OTCD) is a urea cycle disorder of X-linked inheritance, affecting the detoxification of excess nitrogen and leading to hyperammonemia (hyper-NH

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