Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 51
Filtrar
1.
Gan To Kagaku Ryoho ; 51(4): 442-444, 2024 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-38644316

RESUMO

The aim of this study was to investigate the short-term outcomes of surgery for colorectal cancer(CRC)in the elderly aged over 90 years old. We retrospectively analyzed 1,043 patients with stage Ⅰ-Ⅳ CRC who underwent curative surgery in our institutions between 2013 and 2022. The patients were divided into the super older(aged ≥90 years, 20 patients) and non-super older groups(aged 80-89 years, 243 patients). The short-term outcomes were compared between the 2 groups. There were no significant differences in tumor location, stage, surgical approach, duration of operation and blood loss. The incidence of severe postoperative complications did not differ between the 2 groups. In conclusion, our study suggested that surgery for colorectal cancer could be as safely performed in super elderly patients as in non-super elderly patients.


Assuntos
Neoplasias Colorretais , Humanos , Idoso de 80 Anos ou mais , Neoplasias Colorretais/cirurgia , Neoplasias Colorretais/patologia , Masculino , Feminino , Resultado do Tratamento , Estudos Retrospectivos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia , Estadiamento de Neoplasias
2.
Gan To Kagaku Ryoho ; 51(1): 81-83, 2024 Jan.
Artigo em Japonês | MEDLINE | ID: mdl-38247099

RESUMO

Chylous ascites is a rare post operative complication after gastrectomy, which commonly occurs in early postoperative period. Here, we successfully treated a patient with unresectable gastric cancer who occurred chylous ascites 9 months after first surgery and underwent laparoscopic surgery for chylous ascites. Since prolonged chylous ascites may cause malnutrition, surgical treatment should be considered for refractory chylous ascites.


Assuntos
Ascite Quilosa , Laparoscopia , Desnutrição , Neoplasias Gástricas , Humanos , Ascite Quilosa/etiologia , Ascite Quilosa/cirurgia , Neoplasias Gástricas/complicações , Neoplasias Gástricas/cirurgia , Gastrectomia
3.
Gan To Kagaku Ryoho ; 50(13): 1897-1899, 2023 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-38303245

RESUMO

We reported a case of an advanced gastric cancer patient with gastric outlet obstruction and malnutrition who successfully underwent neoadjuvant chemotherapy with enteral feeding. The patient is a 72-year-old man. The diagnosis was advanced gastric cancer with pyloric stenosis. Both decompression and enteral nutrition was performed with a W-ED®. Chemotherapy was markedly effective and nutritional status improved. He underwent robot-assisted distal gastrectomy(D2)and Billroth Ⅱ reconstruction under good nutritional status after neoadjuvant chemotherapy with intragastric decompression and nutritional management using a W-ED® tube. W-ED® tube is a useful tool for the proper nutritional management of patients with advanced gastric cancer who have pyloric stenosis by adequate decompression and enteral feeding.


Assuntos
Obstrução da Saída Gástrica , Desnutrição , Estenose Pilórica , Neoplasias Gástricas , Masculino , Humanos , Idoso , Nutrição Enteral , Neoplasias Gástricas/terapia , Neoplasias Gástricas/cirurgia , Terapia Neoadjuvante , Obstrução da Saída Gástrica/etiologia , Obstrução da Saída Gástrica/cirurgia
4.
Gan To Kagaku Ryoho ; 50(13): 1381-1383, 2023 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-38303281

RESUMO

We investigated the safety and efficacy of UDON(5-FU, docetaxel and nedaplatin), a similar DCF therapy, as neoadjuvant chemotherapy for advanced esophageal cancer. Twelve patients who underwent radical esophagectomy after neoadjuvant chemotherapy for esophageal cancer at our department from June 2021 to December 2022 were retrospectively evaluated. One patient had Grade Ⅲ or higher neutropenia(8%)and 2 patients had anorexia(15%)as adverse events, but they could be safely treated. Nutritional status and ADL were maintained in all patients, and surgery was performed after 2-3 courses of neoadjuvant chemotherapy. The median postoperative hospital stay was 14 days, and no severe postoperative complications were observed. The histological effect to chemotherapy was Grade 3 in 3 patients(23%). UDON therapy is a safe and effective treatment.


Assuntos
Neoplasias Esofágicas , Neutropenia , Compostos Organoplatínicos , Humanos , Docetaxel , Terapia Neoadjuvante , Estudos Retrospectivos , Fluoruracila , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias Esofágicas/tratamento farmacológico , Neoplasias Esofágicas/cirurgia , Neoplasias Esofágicas/patologia , Neutropenia/induzido quimicamente , Resultado do Tratamento , Cisplatino
5.
Gan To Kagaku Ryoho ; 48(13): 2109-2111, 2021 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-35045508

RESUMO

Mediastinoscopic esophagectomy(ME)is a minimally invasive approach without thoracotomy and pulmonary atelectasis during surgery. Here, we report the case of a 67-year-old patient who was successfully treated with thoracic esophageal cancer and severe chronic pulmonary infection using ME and home enteral nutrition therapy. Esophageal cancer patients with severe lung dysfunction have a risk of postoperative pneumoniae. ME could be a promising procedure for patients with severe lung dysfunction. We highlight the usefulness of ME as a safe approach to avoid pulmonary complications.


Assuntos
Neoplasias Esofágicas , Esofagectomia , Idoso , Neoplasias Esofágicas/complicações , Neoplasias Esofágicas/cirurgia , Humanos , Pulmão , Mediastinoscópios , Mediastinoscopia , Complicações Pós-Operatórias
6.
J Surg Res ; 245: 410-419, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31437648

RESUMO

BACKGROUND: The present study examined the impact of oxygenated machine perfusion on preservation of liver grafts donated after cardiac death by measuring sinusoidal endothelial injury and microcirculatory disturbances. MATERIALS AND METHODS: Fifteen porcine livers were retrieved 60 min after warm ischemia and allocated into three groups as follows: (1) CS group: static cold storage, (2) HMP group: oxygenated hypothermic perfusion preservation, (3) SNMP group: oxygenated subnormothermic perfusion preservation. The liver grafts donated after cardiac death were preserved for 4 h in different treatment conditions mentioned previously, then subject to ex vivo reperfusion for 2 h using diluted allogeneic blood. The hemodynamic parameters, liver function tests, tissue adenosine triphosphate (ATP) levels, and immunohistochemical findings were investigated. RESULTS: The number of sinusoidal epithelial cells and trabecular structures were maintained after 4 h of preservation in the CS, HMP, and SNMP group. Liver tissue ATP levels after 4 h of preservation in the HMP and SNMP groups were significantly higher compared with that in the CS group. The sinusoidal epithelial cells were significantly exfoliated to a more severe extent in the CS group than in the HMP and SNMP groups. Intrasinusoidal platelet aggregation occurred more frequently in the CS group than in the HMP and SNMP groups. CONCLUSIONS: The results indicated that oxygenated machine perfusion preservation was important to prevent the depletion of tissue ATP and maintain sinusoidal homeostasis regardless of the perfusate temperature. Our findings suggest oxygenated machine perfusion preservation as an effective alternative to static cold storage.


Assuntos
Transplante de Fígado , Fígado/irrigação sanguínea , Microcirculação , Preservação de Órgãos , Perfusão/métodos , Animais , Endotélio Vascular/patologia , Fígado/metabolismo , Fígado/patologia , Consumo de Oxigênio , Suínos
7.
PLoS One ; 14(12): e0226183, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31825976

RESUMO

The recent clinical application of perfusion technology for the machine preservation of donation after cardiac death (DCD) grafts has some advantages. Oxygenation has been proposed for the preservation of DCD liver grafts. The aim of this study is to clarify whether the use of HbV-containing preservation solution during the subnormothermic machine perfusion (SNMP) of the liver graft improves the graft function of DCD porcine livers in an ex vivo reperfusion model. Pig livers were excised after 60 minutes of warm ischemic time and were preserved under one of three preservation conditions for 4 hours. The preservation conditions were as follows: 4°C cold storage (CS group; N = 5), Hypothermic machine preservation (HMP) with UW gluconate solution (HMP group; N = 5), SNMP (21°C) with UW gluconate solution (SNMP group; N = 5), SNMP (21°C) with HbVs (Hb; 1.8 mg/dl) perfusate (SNMP+HbV group; N = 5). Autologous blood perfusion was performed for 2 hours in an isolated liver reperfusion model (IRM). The oxygen consumption of the SNMP and SNMP+HbV group was higher than the HMP groups (p < 0.05). During the reperfusion, the AST level in the SNMP+HbV group was lower than that in the CS, HMP and SNMP groups. The changes in pH after reperfusion was significantly lower in SNMP+HbV group than CS and HMP groups. The ultrastructural findings indicated that the mitochondria of the SNMP+HbV group was well maintained in comparison to the CS, HMP and SNMP groups. The SNMP+HbVs preservation solution protected against metabolic acidosis and preserved the liver function after reperfusion injury in the DCD liver.


Assuntos
Hemoglobinas/química , Fígado/patologia , Modelos Animais , Preservação de Órgãos/métodos , Oxigênio/química , Adenosina/química , Alopurinol/química , Animais , Aspartato Aminotransferases/metabolismo , Feminino , Glutationa/química , Hemoglobinas/metabolismo , Artéria Hepática/fisiologia , Humanos , Concentração de Íons de Hidrogênio , Insulina/química , Ácido Láctico/metabolismo , Fígado/metabolismo , Transplante de Fígado , Mitocôndrias/ultraestrutura , Preservação de Órgãos/instrumentação , Soluções para Preservação de Órgãos/química , Oxigênio/metabolismo , Consumo de Oxigênio , Rafinose/química , Suínos , Temperatura
8.
Pediatr Transplant ; 21(3)2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28213931

RESUMO

Children with ESRD in need of RRT are commonly managed by PD due to difficulty with vascular access for HD and the relatively large extracorporeal blood volume required. Major abdominal surgery may result in injury to the peritoneum and consequent adhesion, thereby resulting in a reduction in the anatomical capacity and transport capability across the peritoneal membrane. Here, we report successful resumption of PD after LDLT in two pediatric patients. The causes of ESRD were PH1 and juvenile nephronophthisis, respectively. Both patients were managed by PD prior to LDLT. PD was converted to HD starting three days before LDLT and was continued postoperatively until resumption of PD on days 13 and 28, respectively. The PD weekly Kt/V urea was maintained before and after LDLT. The patients continued to do well on PD without complications. Meticulous intra-operative techniques during LDLT allow postoperative PD resumption by preservation of peritoneal integrity with effective transport capability and without added risk of peritonitis.


Assuntos
Falência Renal Crônica/terapia , Cirrose Hepática/cirurgia , Transplante de Fígado/métodos , Diálise Peritoneal , Criança , Drenagem , Feminino , Homozigoto , Humanos , Lactente , Período Intraoperatório , Doadores Vivos , Mutação , Peritonite/etiologia , Fatores de Tempo , Transaminases/genética , Resultado do Tratamento
9.
Pediatr Transplant ; 21(1)2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27891735

RESUMO

Ornithine transcarbamylase deficiency (OTCD) is a urea cycle disorder of X-linked inheritance, affecting the detoxification of excess nitrogen and leading to hyperammonemia (hyper-NH3 ). Living donor liver transplantation (LDLT) has been applied for the treatment of OTCD. This case series retrospectively reviewed two OTCD patients who experienced hyper-NH3 following LDLT. The first case was a 5-year-old girl who had onset of OTCD at 2 years of age. Ornithine transcarbamylase (OTC) enzyme activity was 62% for the donor and 15% for the recipient. The patient suffered from recurrence of hyper-NH3 within 2 months following LDLT. The second case was a 5-year-old girl who had onset of OTCD at 3 years of age. OTC enzyme activity was 42.6% for the donor and 9.7% for the recipient. The patient suffered hyper-NH3 for 12 days starting on the date of surgery. Both of the patients transiently required continuous veno-venous hemodialysis; however, they are currently doing well without intensive medical treatment. The use of asymptomatic OTCD heterozygous donors in LDLT has been accepted with careful examination. However, an OTCD heterozygous carrier donor should be avoided if there is another donor candidate, due to the potentially fatal condition of hyper-NH3 following LDLT.


Assuntos
Hiperamonemia/complicações , Falência Hepática/complicações , Falência Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Doença da Deficiência de Ornitina Carbomoiltransferase/complicações , Doença da Deficiência de Ornitina Carbomoiltransferase/genética , Pré-Escolar , Feminino , Heterozigoto , Humanos , Hiperamonemia/etiologia , Fígado/enzimologia , Doadores Vivos , Ornitina Carbamoiltransferase/metabolismo , Doença da Deficiência de Ornitina Carbomoiltransferase/diagnóstico , Recidiva , Diálise Renal , Estudos Retrospectivos
10.
J Pediatr Surg ; 51(11): 1807-1811, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27325360

RESUMO

BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULT: 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%. CONCLUSION: 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.


Assuntos
Artéria Hepática/cirurgia , Hepatoblastoma/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Veia Porta/cirurgia , Criança , Pré-Escolar , Feminino , Hepatoblastoma/irrigação sanguínea , Hepatoblastoma/mortalidade , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Neoplasias Hepáticas/irrigação sanguínea , Neoplasias Hepáticas/mortalidade , Masculino , Taxa de Sobrevida/tendências , Resultado do Tratamento
11.
Pediatr Transplant ; 20(4): 594-596, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27029560

RESUMO

PV thrombosis following pediatric LT is a serious complication that may lead to graft loss. LDLT poses limitations with regard to the availability of vein grafts for complex PV reconstructions. We herein report a unique reconstruction of the PV inflow in a one-yr-old boy with situs inversus undergoing re-LDLT. The inflow was derived from the SPV and the RRV. A common channel was created utilizing a donor IMV and the recipient explant LHV as vascular conduits. With the application of innovative surgical reconstructions, pre-existing portomesenteric thrombosis may be amenable to re-LDLT in the pediatric population.


Assuntos
Transplante de Fígado/métodos , Veia Porta/cirurgia , Complicações Pós-Operatórias/cirurgia , Veias Renais/cirurgia , Situs Inversus/complicações , Veia Esplênica/cirurgia , Trombose Venosa/cirurgia , Anastomose Cirúrgica , Atresia Biliar/complicações , Atresia Biliar/cirurgia , Humanos , Lactente , Doadores Vivos , Masculino , Reoperação , Trombose Venosa/etiologia
12.
Pediatr Transplant ; 20(3): 401-7, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-27012966

RESUMO

LT from ABO-I donors requires preconditioning regimens to prevent postoperative catastrophic AMR. NAC for HBL is known to cause myelosuppression leading to a reduction in the number and function of lymphocytes. We investigated this chemotherapy-induced myelosuppression in HBL patients listed for LT from ABO-I donors with reference to the kinetics of B, T cells, and anti-ABO blood type isoagglutinin titers. Between 2005 and 2015, of the 319 patients who underwent LDLT at our institute, 12 were indicated for unresectable HBL. Three patients with unresectable HBL who underwent LDLT from ABO-I donors are included in this study. Immunosuppression consisted of a standard regime of tacrolimus and low-dose steroids as in ABO compatible/identical LDLT. No additional preoperative therapies for B-cell depletion were used. Absolute lymphocyte counts, lymphocyte subsets (including CD20+ B cells, CD3+CD4+ T cells and CD3+CD8+ T cells), and anti-ABO blood type isoagglutinin titers were measured before LDLT and postoperatively. The median age at diagnosis was 19 months (range, 3-31 months). The median follow-up was seven months (range, 6-15 months). The median interval from the last NAC to LDLT was 33 days (range, 25-52 days). The median interval from LDLT to adjuvant chemotherapy was 28 days (range, 22-36 days). The counts of CD20+ B cells before LDLT were depleted to median 5 cells/mm(3) (range, 0-6 cells/mm(3)). There was a transient rebound in the CD20+ B cell counts on day seven (maximum of 82 cells/mm(3)) followed by a decline starting at 14 days after LDLT that was sustained for the duration of adjuvant chemotherapy. Anti-ABO blood type isoagglutinin titers were lowered to between 1:1 and 1:16 before LDLT and remained low for the duration of follow-up in this study. All of the three patients remained in good health without either acute cellular or AMR after LDLT. The B-cell depletion that occurs after cisplatin-based chemotherapy for HBL may help accomplish safe ABO-I LDLT in children without the use of additional conditioning regimens for prevention of AMR.


Assuntos
Antineoplásicos/efeitos adversos , Linfócitos B/efeitos dos fármacos , Incompatibilidade de Grupos Sanguíneos , Hepatoblastoma/cirurgia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado/efeitos adversos , Sistema ABO de Grupos Sanguíneos , Antígenos CD20/sangue , Linfócitos B/citologia , Complexo CD3/sangue , Linfócitos T CD4-Positivos/citologia , Linfócitos T CD8-Positivos/citologia , Quimioterapia Adjuvante/métodos , Pré-Escolar , Cisplatino/uso terapêutico , Infecções por Citomegalovirus/complicações , Feminino , Hepatoblastoma/sangue , Hepatoblastoma/tratamento farmacológico , Humanos , Imunidade Inata , Terapia de Imunossupressão , Lactente , Neoplasias Hepáticas/sangue , Neoplasias Hepáticas/tratamento farmacológico , Transplante de Fígado/métodos , Doadores Vivos , Subpopulações de Linfócitos , Masculino , Risco , Rituximab/uso terapêutico , Tacrolimo/uso terapêutico , Condicionamento Pré-Transplante , Resultado do Tratamento
13.
Pediatr Transplant ; 20(5): 633-9, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26857795

RESUMO

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.


Assuntos
Doença Hepática Terminal/cirurgia , Hepatectomia/métodos , Transplante de Fígado/métodos , Doadores Vivos , Doença da Urina de Xarope de Bordo , Coleta de Tecidos e Órgãos/métodos , Pré-Escolar , Seleção do Doador , Feminino , Humanos , Lactente , Japão , Masculino
14.
Pediatr Transplant ; 19(6): E142-5, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26121997

RESUMO

The management of LSRS is a crucial problem to ensure a sufficient PV flow during pediatric LT. Although several techniques have been indicated to solve this problem, a more appropriate approach to LSRS is still needed in pediatric LT. We herein present a modified surgical approach to the ligation of LSRS via the left side of the IVC for a nine-month-old boy with severe portal hypertension and a history of Kasai portoenterostomy. LSRS was identified and exposed through the left side of the IVC and the dorsal surface of the pancreas from the superior side of the body of the pancreas. The post-operative course was uneventful with an excellent PV flow. The central approach for the ligation of LSRS is worth considering as an alternative procedure for a patient with collateral vessels and a history of multiple laparotomies.


Assuntos
Atresia Biliar/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Derivação Esplenorrenal Cirúrgica/métodos , Atresia Biliar/complicações , Humanos , Hipertensão Portal/etiologia , Hipertensão Portal/cirurgia , Lactente , Masculino
15.
J Pediatr Surg ; 50(4): 559-64, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25840063

RESUMO

BACKGROUND/PURPOSE: Acoustic radiation focus impulse (ARFI) elastography is a new method for assessing the degree of tissue stiffness. We herein evaluated the degree of spleen stiffness (SS) using ARFI elastography in patients with biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: We retrospectively collected the liver stiffness (LS) and SS values on ARFI elastography from 43 patients undergoing KPE between September 2010 and November 2013. We analyzed the correlations between these values and variables related to the severity of liver dysfunction and portal hypertension (PHT). The data were expressed as the standard deviation score (z-score) relative to the previously reported normal values for the patient's age. RESULTS: The SS value was significantly associated with the spleen diameter and development of collateral vessels, in comparison to the LS value. Interestingly, there was a significant correlation between SS value and the portal vein (PV) diameter. Thirty patients (69.8%) consequently underwent LT; these patients showed higher SS values and smaller PV diameters than the patients monitored without LT. CONCLUSIONS: The degree of SS measured on ARFI elastography can be used to predict the severity of PHT in BA patients after KPE.


Assuntos
Atresia Biliar/cirurgia , Técnicas de Imagem por Elasticidade/métodos , Hipertensão Portal/etiologia , Portoenterostomia Hepática , Complicações Pós-Operatórias , Índice de Gravidade de Doença , Baço/diagnóstico por imagem , Pré-Escolar , Feminino , Humanos , Hipertensão Portal/diagnóstico por imagem , Lactente , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Estudos Retrospectivos , Medição de Risco , Resultado do Tratamento
16.
J Pediatr Surg ; 50(4): 688-95, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25840084

RESUMO

BACKGROUND/PURPOSE: Liver transplantation was previously indicated as a curative operation for congenital absence of portal vein. Recent advances in radiological interventional techniques can precisely visualize the architecture of the intrahepatic portal system (IHPS). Therefore, the therapeutic approach for congenital portosystemic shunt (CPS) needs to be reevaluated from a viewpoint of radiological appearances. The aim of this study was to propose the IHPS classification which could explain the pathophysiological characteristics and play a complementary role of a therapeutic approach and management for CPS. METHODS: Nineteen patients with CPS were retrospectively reviewed. The median age at diagnosis was 6.8 years old. Eighteen of these patients underwent angiography with a shunt occlusion test and were classified based of the severity of the hypoplasia of IHPS. RESULTS: The eighteen cases who could undergo the shunt occlusion test were classified into mild (n=7), moderate (n=6) and severe types (n=5) according to the IHPS classification. The IHPS classification correlated with the portal venous pressure under shunt occlusion, the histopathological findings, postoperative portal venous flow and liver regeneration. Shunt closure resulted in dramatic improvement in the laboratory data and subclinical encephalopathy. Two patients with the severe type suffered from sepsis associated with portal hypertension after treatment, and from the portal flow steal phenomenon because of the development of unexpected collateral vessels. The patients with the severe type had a high risk of postoperative complications after shunt closure in one step, even if the PVP was relatively low during the shunt occlusion test. CONCLUSION: The IHPS could be visualized by the shunt occlusion test. The IHPS classification reflected the clinicopathological features of CPS, and was useful to determine the therapeutic approach and management for CPS.


Assuntos
Veias Hepáticas/anormalidades , Sistema Porta/anormalidades , Veia Porta/anormalidades , Malformações Vasculares/classificação , Humanos
17.
Pediatr Transplant ; 19(3): E70-4, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25712501

RESUMO

PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII. PC deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC deficiency treated with LTx have been published; however, no report of DLT as a therapy for PC deficiency is available. We describe a case of a 23-month-old girl who received DLT for compound heterozygous PC deficiency. Her PC activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased-donor transplantation. We performed living DLT using the liver from a patient with MSUD. Activated PC concentrate safely supported the perioperative period. After DLT, she maintained normal PC activities and BCAA levels. This is the first case of PC deficiency successfully treated by living DLT with MSUD. We propose that DLT using liver from patients with MSUD is a treatment option for PC deficiency.


Assuntos
Transplante de Fígado/métodos , Doença da Urina de Xarope de Bordo , Deficiência de Proteína C/cirurgia , Púrpura Fulminante/etiologia , Anticoagulantes/uso terapêutico , Feminino , Heterozigoto , Humanos , Lactente , Fígado/metabolismo , Doadores Vivos , Mutação , Proteína C/metabolismo , Resultado do Tratamento
18.
Pediatr Transplant ; 19(2): 144-52, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25418834

RESUMO

The immune function (ImmuKnow) assay is a measure of cell-mediated immunity based on the peripheral CD4+ T cell ATP activity. The efficacy of ImmuKnow in pediatric LDLT is not well documented. The aim of this study was to assess the correlations between the ImmuKnow and the clinical status in pediatric LDLT recipients. A total of 716 blood samples were obtained from 60 pediatric LDLT recipients (one month to 16 yr of age). The recipient's status was classified as follows: stable, infection, or rejection. The ImmuKnow values in the pediatric LDLT recipients with a clinically stable status had a lower immune response (IQR 85-297 ATP ng/mL) than that previously reported in adults. Meanwhile, the ImmuKnow values of the stable patients were not correlated with age. Furthermore, a significant difference was found in the ImmuKnow values between the bacterial or fungal infection and stable groups, but not between the CMV or EBV infection and stable groups. The ImmuKnow levels in the pediatric LDLT were lower than those observed in the adult LDLT. The proposed reference value is between 85 and 297 ATP ng/mL in pediatric LDLT recipients. We conclude that the ImmuKnow assay could be helpful for monitoring pediatric LDLT recipients with bacterial or fungal infections.


Assuntos
Falência Hepática/imunologia , Falência Hepática/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Trifosfato de Adenosina/química , Adolescente , Linfócitos T CD4-Positivos , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto , Humanos , Sistema Imunitário , Imunidade Celular/imunologia , Lactente , Recém-Nascido , Masculino , Reação em Cadeia da Polimerase , Valores de Referência , Estudos Retrospectivos , Linfócitos T/citologia
19.
Pediatr Transplant ; 19(1): E1-6, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25323048

RESUMO

Several transplant strategies for PH1 have been proposed, and LT is performed to correct the metabolic defects. The patients with PH1 often suffer from ESRD and require simultaneous LKT, which leads to a long wait due to the shortage of suitable organ donors. Five patients with PH1 underwent LDLT at our institute. Three of the five patients were under dialysis before LDLT, while the other two patients were categorized as CKD stage 3. An isolated LDLT was successfully performed in all but our first case, who had complicated postoperative courses and consequently died due to sepsis after retransplantation. The renal function of the patients with CKD stage 3 was preserved after LDLT. On the other hand, our second case with ESRD underwent successful LDKT six months after LDLT, and our infant case is waiting for the subsequent KT without any post-LDLT complications after the early establishment of PD. In conclusion, a two-step transplant strategy may be needed as a life-saving option for patients with PH1 and may be possible even in small infants with systemic oxalosis. While waiting for a subsequent KT, an early resumption of PD should be considered from the perspective of the long-term requirement of RRT.


Assuntos
Hiperoxalúria Primária/complicações , Hiperoxalúria Primária/cirurgia , Hiperoxalúria/etiologia , Hiperoxalúria/prevenção & controle , Transplante de Rim/métodos , Insuficiência Renal/complicações , Adolescente , Criança , Progressão da Doença , Feminino , Humanos , Lactente , Masculino
20.
Pediatr Transplant ; 18(8): 860-7, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25311536

RESUMO

An IL-2 receptor antagonist, basiliximab, decreases the frequency of ACR in liver transplant (LT) recipients as induction therapy. The aim of this study was to evaluate the effectiveness of basiliximab against SRR as rescue therapy in pediatric LT patients with ALF. Forty pediatric ALF patients underwent LT between November 2005 and July 2013. Among them, seven patients suffering from SRR were enrolled in this study. The median age at LT was 10 months (6-12 months). SRR was defined as the occurrence of refractory rejection after more than two courses of steroid pulse therapy. Basiliximab was administered to all patients. The withdrawal of steroids without deterioration of the liver function was achieved in six patients treated with basiliximab therapy without patient mortality, although one patient developed graft loss and required retransplantation for veno-occlusive disease. The pathological examinations of liver biopsies in the patients suffering from SRR revealed severe centrilobular injuries, particularly fibrosis within one month after LT. We demonstrated the effectiveness and safety of rescue therapy consisting of basiliximab for SRR in pediatric LT recipients with ALF.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Rejeição de Enxerto/tratamento farmacológico , Imunossupressores/uso terapêutico , Transplante de Fígado , Proteínas Recombinantes de Fusão/uso terapêutico , Adolescente , Basiliximab , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Falência Hepática Aguda , Masculino , Prednisolona/uso terapêutico , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...