RESUMO
Maternal T cells from perinatal transplacental passage have been identified in up to 40% of patients with severe combined immunodeficiency (SCID). Although engrafted maternal T cells sometimes injure newborn tissue, liver failure due to maternal T cells has not been reported. We rescued a boy with X-linked SCID who developed liver failure due to engrafted maternal T cell invasion following living donor liver transplantation (LDLT) following unrelated umbilical cord blood transplantation (UCBT). After developing respiratory failure 3 weeks postpartum, he was diagnosed with X-linked SCID. Pathological findings showed maternal T cells engrafted in his liver and hepatic fibrosis gradually progressed. He underwent UCBT at 6 months, but hepatic function did not recover and liver failure progressed. Therefore, he underwent LDLT using an S2 monosegment graft at age 1.3 years. The patient had a leak at the Roux-en-Y anastomosis, which was repaired. Despite occasional episodes of pneumonia and otitis media, he is generally doing well 6 years after LDLT with continued immunosuppression agents. In conclusion, the combination of hematopoietic stem cell transplantation (HSCT) and liver transplantation may be efficacious, and HSCT should precede liver transplantation for children with X-linked SCID and liver failure.
Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Falência Hepática , Transplante de Fígado , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X , Transplante de Células-Tronco de Sangue do Cordão Umbilical/efeitos adversos , Feminino , Humanos , Lactente , Falência Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Doadores Vivos , Masculino , Gravidez , Linfócitos T , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/genética , Doenças por Imunodeficiência Combinada Ligada ao Cromossomo X/terapiaRESUMO
BACKGROUND: We present a retrospective analysis of our experience with pediatric liver transplantation (LT), focusing on the long-term outcome of percutaneous transhepatic biliary drainage (PTBD) for post-transplant biliary strictures. METHODS: Fifty-three PTBDs were performed for 41 pediatric recipients with biliary strictures. The median ages at LT and PTBD were 1.4 and 4.4 years, respectively. The median observation period was 10.6 years. RESULTS: Post-transplant biliary strictures comprised anastomotic stricture (AS) in 28 cases, nonanastomotic stricture (NAS) in 12, anastomotic obstruction (AO) in 8, and nonanastomotic obstruction (NAO) in 5. The success rate of PTBD was 90.6%, and the 15-year primary patency rate of PTBD was 52.6%. The recurrence rate of biliary strictures after PTBD was 18.8% (9/48), and among the four NAS cases with recurrence, two underwent re-LT. The biliary obstruction rate was 27.1% (13/48). Among the eight cases with AO, five underwent the rendezvous method and three underwent surgical re-anastomosis. Among the five cases with NAO, one underwent re-LT. The recipient survival rate of PTBD treatment was 100%. CONCLUSIONS: The graft prognosis of AS by PTBD treatment is good and AO is curable by the rendezvous method and surgical re-anastomosis. However, the graft prognosis of NAS and NAO is poor.
Assuntos
Colestase/terapia , Constrição Patológica/terapia , Drenagem/métodos , Rejeição de Enxerto/terapia , Sobrevivência de Enxerto , Transplante de Fígado/efeitos adversos , Complicações Pós-Operatórias/terapia , Adolescente , Adulto , Anastomose Cirúrgica , Criança , Pré-Escolar , Colestase/diagnóstico , Colestase/etiologia , Constrição Patológica/diagnóstico , Constrição Patológica/etiologia , Feminino , Seguimentos , Rejeição de Enxerto/etiologia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/etiologia , Recidiva , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Adulto JovemRESUMO
A 56-year-old woman was diagnosed with rectal cancer and liver metastases(Stage IV), and underwent low anterior resection and laparoscopic partial hepatectomy. The patient received adjuvant chemotherapy(mFOLFOX6 for 24 weeks), but developed multiple lung metastases 11 months later. Before undergoing a pulmonary resection, the patient presented with acute small bowel obstruction. Abdominal computed tomography showed small bowel stenosis due to a tumor, and we suspected peritoneal metastases from the rectal tumor. We performed partial resection of the small intestine, and histopathological examination revealed a primary small bowel tumor. The patient was discharged to her home without complications, and later underwent pulmonary resections for bilateral lung metastases. We usually suspect that small bowel obstruction is due to peritoneal metastases in patients with advanced colorectal tumors, but must consider the rare possibility of a separate primary small bowel tumor, especially in patients with a solitary lesion. We report a rare primary small bowel tumor after FOLFOX treatment in a patient with Stage IV rectal cancer.
Assuntos
Neoplasias Intestinais/cirurgia , Intestino Delgado/cirurgia , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Primárias Múltiplas/cirurgia , Neoplasias Retais/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Fluoruracila/administração & dosagem , Humanos , Neoplasias Intestinais/patologia , Intestino Delgado/patologia , Leucovorina/administração & dosagem , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/cirurgia , Neoplasias Pulmonares/secundário , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/tratamento farmacológico , Compostos Organoplatínicos/administração & dosagem , Neoplasias Retais/patologia , Neoplasias Retais/cirurgiaRESUMO
BACKGROUND: Long-term term survival in patients with pancreatic neuroendocrine tumors has been reported, even in patients with metastatic disease. Metastases to the spleen are extremely rare, but have been reported from a number of primary malignancies, such as breast cancer, lung cancer, melanoma and ovarian cancer. This is the first report of a splenic metastasis from a primary pancreatic neuroendocrine tumor. CASE PRESENTATION: The patient presented as a 53 years old white male with anemia and fatigue. Physical examination revealed a left upper quadrant fullness and computed tomography showed a 24 cm left upper quadrant mass with multiple liver metastases, splenomegaly and a 1 cm mass in the spleen. Resection of the primary pancreatic tumor (T4N0M1) was accompanied by gastrectomy, splenectomy and resection of adherent bowel. The spleen contained a metastatic lesion 1.0 cm in diameter, consistent with a primary neuroendocrine tumor of the pancreas. This operation was followed 8 months later, by delayed resection of liver metastases. The patient receives monthly administration of somatostatin long-acting analogue and has undergone several ablations of liver lesions with percutaneous radiofrequency ablation as well as a second liver resection. The patient is alive seven years after initial presentation, with no evidence of disease on imaging studies. CONCLUSIONS: This is the first report of a splenic metastasis from a primary pancreatic neuroendocrine tumor. The patient initially presented with synchronous multiple liver metastases and a single splenic metastasis. After resection of the primary tumor and spleen, the patient has undergone aggressive cytoreductive surgery/ablation of liver lesions and somatostatin therapy with resulting long-term survival.
Assuntos
Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Esplênicas/secundário , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Prognóstico , Neoplasias Esplênicas/cirurgiaRESUMO
Both pheochromocytoma and amniotic fluid embolism (AFE) are important causes of maternal mortality. We present a case of a 29-year-old woman who developed cardiac arrest after Caesarean section, complicated by both pheochromocytoma crisis and AFE. After resuscitation, the patient developed multiple organ dysfunction, rhabdomyolysis and disseminated intravascular coagulation (DIC). After institution of multidisciplinary interventions (including the use of an intra-aortic balloon pump, extracorporeal membrane oxygenation, continuous hemodiafiltration, and neuroprotective therapeutic hypothermia) the patient made a full recovery without any apparent neurological deficit.
Assuntos
Embolia Amniótica/terapia , Parada Cardíaca/terapia , Feocromocitoma/terapia , Neoplasias das Glândulas Suprarrenais/terapia , Adulto , Cesárea/efeitos adversos , Coagulação Intravascular Disseminada/terapia , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Humanos , Gravidez , Ressuscitação/métodosRESUMO
A 46-year-old woman was referred to our hospital because of nausea. Endoscopy revealed scirrhous gastric cancer, and abdominalcomputed tomography revealed peritonealdissemination. She was diagnosed with Stage IV gastric cancer and treated with S-1 plus CDDP combination chemotherapy. After 4 courses of chemotherapy, the primary tumor and peritoneal dissemination were considered clinically stable, but the uterus grew rapidly. She was diagnosed as having uterine metastasis based on cervicaland endometrialsmear class V cytology. As the chemotherapy was not effective for the uterine lesions, totalhysterectomy and bilateralsal pingo-oophorectomy were performed. Histological findings showed a poorly differentiated cancer with vascular emboli. Uterine metastases are an important consideration in women with scirrhous gastric cancer, and we recommend palliative hysterectomy for chemotherapy-resistant metastases if the primary tumor and other metastases are controlled.
Assuntos
Adenocarcinoma Esquirroso/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Coagulação Intravascular Disseminada/etiologia , Neoplasias Gástricas/patologia , Neoplasias Uterinas/tratamento farmacológico , Adenocarcinoma Esquirroso/secundário , Adenocarcinoma Esquirroso/cirurgia , Evolução Fatal , Feminino , Gastrectomia , Humanos , Pessoa de Meia-Idade , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/cirurgia , Neoplasias Uterinas/secundárioRESUMO
Inflammation plays a key role in the pathophysiology of hepatic ischemia-reperfusion (I/R) injury. However, the mechanism by which hepatic I/R induces inflammatory responses remains unclear. Recent evidence indicates that a sterile inflammatory response triggered by I/R is mediated through a multiple-protein complex called the inflammasome. Therefore, we investigated the role of the inflammasome in hepatic I/R injury and found that hepatic I/R stimuli upregulated the inflammasome-component molecule, nucleotide-binding oligomerization domain-like receptor (NLR) family pyrin domain-containing 3 (NLRP3), but not apoptosis-associated speck-like protein containing a caspase recruitment domain (ASC). NLRP3(-/-) mice, but not ASC(-/-) and caspase-1(-/-) mice, had significantly less liver injury after hepatic I/R. NLRP3(-/-) mice showed reduced inflammatory responses, reactive oxygen species production, and apoptosis in I/R liver. Notably, infiltration of neutrophils, but not macrophages, was markedly inhibited in the I/R liver of NLRP3(-/-) mice. Bone marrow transplantation experiments showed that NLRP3 not only in bone marrow-derived cells, but also in non-bone marrow-derived cells contributed to liver injury after I/R. In vitro experiments revealed that keratinocyte-derived chemokine-induced activation of heterotrimeric G proteins was markedly diminished. Furthermore, NLRP3(-/-) neutrophils decreased keratinocyte-derived chemokine-induced concentrations of intracellular calcium elevation, Rac activation, and actin assembly formation, thereby resulting in impaired migration activity. Taken together, NLRP3 regulates chemokine-mediated functions and recruitment of neutrophils, and thereby contributes to hepatic I/R injury independently of inflammasomes. These findings identify a novel role of NLRP3 in the pathophysiology of hepatic I/R injury.
Assuntos
Proteínas de Transporte/imunologia , Fígado/imunologia , Neutrófilos/imunologia , Traumatismo por Reperfusão/imunologia , Animais , Apoptose/imunologia , Western Blotting , Proteínas de Transporte/metabolismo , Quimiotaxia de Leucócito , Citometria de Fluxo , Imuno-Histoquímica , Inflamassomos/imunologia , Fígado/metabolismo , Fígado/patologia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Proteína 3 que Contém Domínio de Pirina da Família NLR , Neutrófilos/metabolismo , Reação em Cadeia da Polimerase em Tempo Real , Traumatismo por Reperfusão/patologia , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
PURPOSE: Although improved long-term prognoses for patients with metastatic breast cancer (MBC) have been demonstrated, few reports address overall survival (OS) with sufficient follow-up. Furthermore, the relevance of immunohistological subtypes to OS in MBC has not been clarified. METHODS: We evaluated, retrospectively, the OS of patients who had been initiated on systemic therapy for MBC between 2000 and 2008. RESULTS: The subjects of this study were 527 patients with MBC treated by systemic therapy. The median survival time (MST) was 55.5 months. The MST for each immunohistological subtype was as follows: luminal, 59.9 months; luminal-HER2, not reached; triple-negative, 18.6 months; and HER2-enriched, 49.9 months. According to multivariate analysis, metastasis-free intervals of ≥2 years and treatment with anthracycline for MBC were predictive of better OS. The predictors of shorter OS included disease progression after first-line treatment for MBC, triple-negative, and all histological factors, except papillotubular carcinoma, with liver metastasis, and having three or more initial metastatic sites. CONCLUSIONS: The prognosis of the patients with MBC in this series was better than that reported before 2000, which is probably attributable to the use of novel, improved pharmacological agents. For example, luminal-HER2 tumors can be treated using both aromatase inhibitors and trastuzumab. Because of the lower toxicities, it is now possible to administer these agents for longer periods, resulting in better prognoses.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/secundário , Receptor ErbB-2/análise , Inibidores da Aromatase/administração & dosagem , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/metabolismo , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Trastuzumab/administração & dosagemRESUMO
PURPOSE: We aimed to evaluate patients who had undergone pediatric LDLT with small-for-size graft (SFSG) and identify risk factors of graft failure to establish a preoperative graft selection strategy. METHODS: The data was collected retrospectively. SFSG was used in 14LDLTs (5.7%) of 245 LDLTs performed between May 2001 and March 2014. The mean patient age and body weight at LDLT were 12.6 ± 2.0 years and 40.5 ± 9.9 kg, respectively. The graft type was left lobe in six patients, left + caudate lobe in seven patients, and posterior segment in one patient. RESULTS: The graft survival rates in SFSG and non-SFSG groups were 78.9 and 93.1%, respectively (p = 0.045). In the univariate analysis, bleeding volume during LDLT were an independent risk factors for graft failure (p = 0.011). Graft failure was caused by sepsis in all three patients and occurred at a median of 70 postoperative days 70 (range 14-88 days). Among them, two cases showed high preoperative PELD/MELD score (PELD; 19.4 and MELD; 22, respectively). CONCLUSIONS: Pediatric LDLT using SFSG had poor outcome and prognosis, especially when it accompanies the surgical infectious complications with preoperative high PELD/MELD scores.
Assuntos
Hepatopatias/cirurgia , Transplante de Fígado/métodos , Fígado/anatomia & histologia , Fígado/cirurgia , Doadores Vivos , Adolescente , Criança , Feminino , Sobrevivência de Enxerto , Humanos , Masculino , Tamanho do Órgão , Seleção de Pacientes , Estudos Retrospectivos , Fatores de RiscoRESUMO
In the field of pediatric living donor liver transplantation (LDLT), physicians sometimes must reduce the volume of left lateral segment (LLS) grafts to prevent large-for-size syndrome. There are 2 established methods for decreasing the size of an LLS graft: the use of a segment 2 (S2) monosegment graft and the use of a reduced LLS graft. However, no procedure for selecting the proper graft type has been established. In this study, we conducted a retrospective investigation of LDLT and examined the strategy of graft selection for patients weighing ≤6 kg. LDLT was conducted 225 times between May 2001 and December 2012, and 15 of the procedures were performed in patients weighing ≤6 kg. We selected S2 monosegment grafts and reduced LLS grafts if the preoperative computed tomography (CT)-volumetry value of the LLS graft was >5% and 4% to 5% of the graft/recipient weight ratio, respectively. We used LLS grafts in 7 recipients, S2 monosegment grafts in 4 recipients, reduced S2 monosegment grafts in 3 recipients, and a reduced LLS graft in 1 recipient. The reduction rate of S2 monosegment grafts for use as LLS grafts was 48.3%. The overall recipient and graft survival rates were both 93.3%, and 1 patient died of a brain hemorrhage. Major surgical complications included hepatic artery thrombosis in 2 recipients, bilioenteric anastomotic strictures in 2 recipients, and portal vein thrombosis in 1 recipient. In conclusion, our graft selection strategy based on preoperative CT-volumetry is highly useful in patients weighing ≤6 kg. S2 monosegment grafts are effective and safe in very small infants particularly neonates.
Assuntos
Falência Hepática/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Seleção de Pacientes , Adolescente , Peso Corporal , Criança , Pré-Escolar , Feminino , Artéria Hepática/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Veia Porta/fisiopatologia , Período Pré-Operatório , Estudos Retrospectivos , Trombose/etiologia , Resultado do Tratamento , Trombose Venosa/etiologia , Adulto JovemRESUMO
The serum ferritin (SF) concentration is a widely available and objective laboratory parameter. SF is also widely recognized as an acute-phase reactant. The purpose of the present study was to identify the chronological changes in the recipient's SF concentration during liver transplantation (LT) and to clarify factors having an effect on the recipient's intraoperative SF level. In addition, the study retrospectively evaluated the usefulness of measuring SF during LT. Ninety-eight pediatric recipients were retrospectively analyzed. The data were analyzed and compared according to the SF level in the recipient. Patients were classified into 2 groups based on the intraoperative peak SF levels of ≤ 1000 ng/mL (low-SF group) or >1000 ng/mL (high-SF group). The SF value increased dramatically after reperfusion and fell to normal levels within the early postoperative period. The warm ischemia time (WIT) was significantly longer in the high-SF group (47.0 versus 58.5 minutes; P = 0.003). In addition, a significant positive correlation was observed between the peak SF value and WIT (r = 0.35; P < 0.001). There were significant positive correlations between the peak SF value and the donors' preoperative laboratory data, including transaminases, cholinesterase, hemoglobin, transferrin saturation, and SF, of which SF showed the strongest positive correlation (r = 0.74; P < 0.001). The multivariate analysis revealed that WIT and donor's SF level were a significant risk factor for high SF level in the recipient (P = 0.007 and 0.02, respectively). In conclusion, the SF measurement can suggest the degree of ischemia/reperfusion injury (IRI). A high SF level in the donor is associated with the risk of further acute reactions, such as IRI, in the recipient.
Assuntos
Ferritinas/sangue , Rejeição de Enxerto/sangue , Terapia de Imunossupressão/métodos , Transplante de Fígado/efeitos adversos , Traumatismo por Reperfusão/sangue , Biomarcadores/sangue , Criança , Pré-Escolar , Feminino , Seguimentos , Rejeição de Enxerto/epidemiologia , Rejeição de Enxerto/etiologia , Humanos , Incidência , Lactente , Japão/epidemiologia , Doadores Vivos , Masculino , Monitorização Intraoperatória/métodos , Prognóstico , Traumatismo por Reperfusão/complicações , Traumatismo por Reperfusão/prevenção & controle , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Fatores de TempoRESUMO
BACKGROUND: Although advanced esophageal squamous-cell carcinoma (ESCC) is treated using a multidisciplinary approach, outcomes remain unsatisfactory. The microenvironment of cancer cells has recently been shown to strongly influence the biologic properties of malignancies. We explored the effect of supernatant from esophageal fibroblasts on the cell growth and chemo-resistance of ESCC cell lines. METHODS: We used 22 ESCC cell lines, isolated primary human esophageal fibroblasts and immortalized fibroblasts. We first examined cell proliferation induced by fibroblast supernatant. The effect of supernatant was evaluated to determine whether paracrine signaling induced by fibroblasts can influence the proliferation of cancer cells. Next, we examined the effects of adding growth factors HGF, FGF1, FGF7, and FGF10, to the culture medium of cancer cells. These growth factors are assumed to be present in the culture supernatants of fibroblasts and may exert a paracrine effect on the proliferation of cancer cells. We also examined the intrinsic role of HGF/MET and FGFs/FGFR in ESCC proliferation. In addition, we examined the inhibitory effect of lapatinib on ESCC cell lines and studied whether the fibroblast supernatants affect the inhibitory effect of lapatinib on ESCC cell proliferation. Finally, we tested whether the FGFR inhibitor PD-173074 could eliminate the rescue effect against lapatinib that was induced by fibroblast supernatants. RESULTS: The addition of fibroblast supernatant induces cell proliferation in the majority of cell lines tested. The results of experiments to evaluate the effects of adding growth factors and kinase inhibitors suggests that the stimulating effect of fibroblasts was attributable in part to HGF/MET or FGF/FGFR. The results also indicate diversity in the degree of dependence on HGF/MET and FGF/FGFR among the cell lines. Though lapanitib at 1 µM inhibits cell proliferation by more than 50% in the majority of the ESCC cell lines, fibroblast supernatant can rescue the growth inhibition of ESCC cells. However, the rescue effect is abrogated by co-treatment with FGFR inhibitor. CONCLUSION: These results demonstrate that cell growth of ESCC depends on diverse receptor tyrosine kinase signaling, in both cell-autonomous and cell-non-autonomous manners. The combined inhibition of these signals may hold promise for the treatment of ESCC.
Assuntos
Carcinoma de Células Escamosas/metabolismo , Resistencia a Medicamentos Antineoplásicos , Neoplasias Esofágicas/metabolismo , Fatores de Crescimento de Fibroblastos/metabolismo , Fibroblastos/metabolismo , Fator de Crescimento de Hepatócito/metabolismo , Quinazolinas/farmacologia , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/patologia , Técnicas de Cultura de Células , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Células Cultivadas , Meios de Cultivo Condicionados/farmacologia , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Neoplasias Esofágicas/tratamento farmacológico , Neoplasias Esofágicas/patologia , Carcinoma de Células Escamosas do Esôfago , Esôfago/citologia , Fatores de Crescimento de Fibroblastos/genética , Fatores de Crescimento de Fibroblastos/farmacologia , Fibroblastos/citologia , Fator de Crescimento de Hepatócito/genética , Humanos , Lapatinib , Dados de Sequência Molecular , Comunicação Parácrina/efeitos dos fármacos , Proteínas Proto-Oncogênicas c-met/metabolismo , Pirimidinas/farmacologia , Receptores de Fatores de Crescimento de Fibroblastos/antagonistas & inibidores , Receptores de Fatores de Crescimento de Fibroblastos/metabolismoRESUMO
Previous studies have demonstrated the safety of ABO-incompatible pediatric LDLT using preoperative plasmapheresis and rituximab; however, no reports have described the timing and dosage of rituximab administration for pediatric LDLT. This study aimed to describe a safe and effective dosage and timing of rituximab for patients undergoing pediatric ABO-incompatible LDLT based on the experience of our single center. A total of 192 LDLTs in 187 patients were examined. These cases included 29 ABO-incompatible LDLTs in 28 patients. Rituximab was used beginning in January 2004 in recipients older than two yr of age (first period: 375 mg/m(2) in two cases; second period: 50 mg/m(2) in two cases; and 200 mg/m(2) in eight cases). Two patients who received 375 mg/m(2) rituximab died of Pneumocystis carinii pneumonia and hemophagocytic syndrome. One patient who received 50 mg/m(2) rituximab required retransplantation as a consequence of antibody-mediated complications. All eight patients administered 200 mg/m(2) survived, and the mean CD20(+) lymphocyte count was 0.1% at the time of LDLT. In the preoperative management of patients undergoing pediatric ABO-incompatible LDLT, the administration of 200 mg/m(2) rituximab three wk prior to LDLT was safe and effective.
Assuntos
Sistema ABO de Grupos Sanguíneos/imunologia , Incompatibilidade de Grupos Sanguíneos/imunologia , Imunossupressores/uso terapêutico , Transplante de Fígado/métodos , Rituximab/uso terapêutico , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Falência Hepática/cirurgia , Doadores Vivos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Plasmaferese , Pneumonia por Pneumocystis/diagnóstico , Período Pós-Operatório , Reoperação , Fatores de Tempo , Resultado do TratamentoRESUMO
CONTEXT: Insulinomas, which are generally smaller than 2 cm, may be difficult to detect by routine imaging modalities including abdominal ultrasonography, computed tomography, and magnetic resonance imaging. Although preoperative detection of insulinomas is essential for operative planning, it is often challenging due to their small size. While arterial stimulation and venous sampling has been used in patients with insulinomas it has been largely supplanted by early-phase thin-slice computed tomography. CASE REPORT: We report three patients with insulinomas, which were not detected by routine computed tomography scan, but were successfully imaged using early-phase thin-slice computed tomography. Enucleation was performed in all patients based on preoperative imaging. All three patients had an unremarkable postoperative course. CONCLUSION: Early-phase thin-slice computed tomography is recommended for the preoperative identification of insulinomas. This non-invasive imaging technique should be considered before performing arterial stimulation and venous sampling.
RESUMO
PURPOSE: We hypothesized that a reduction in the size of the lymph nodes after neoadjuvant therapy for locally advanced rectal carcinoma would be associated with decreased lymph node metastases and/or a better prognosis. METHODS: Between March 2006 and April 2012, 71 patients with primary rectal cancer received neoadjuvant chemoradiation therapy (CRT). For all lymph nodes 5 mm or larger in size, the major and minor axes were measured on CT scan images, and the product was calculated. The lymph node size was determined before and after CRT. The patients were divided into three groups based on the lymph node size before and after treatment. Group A exhibited a reduction in size of 60% or more, Group B a reduction of less than 60% and Group C had no lymph node enlargement before treatment. RESULTS: The incidence of lymph node metastases on pathological examination was 15% in Group A and 50% in Group B (p = 0.006). The five-year disease-free survival in Group A was 84% compared with 78% in Group B (log rank p = 0.34). The five-year overall survival in Group A was 92% compared with 74% in Group B (log rank p = 0.088). CONCLUSIONS: A reduction in the size of enlarged lymph nodes after neoadjuvant therapy may be a useful prognostic factor for recurrence and survival.
Assuntos
Adenocarcinoma/terapia , Quimiorradioterapia Adjuvante , Linfonodos/patologia , Neoplasias Retais/terapia , Adenocarcinoma/mortalidade , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Linfonodos/diagnóstico por imagem , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Pelve , Prognóstico , Neoplasias Retais/mortalidade , Neoplasias Retais/patologia , Neoplasias Retais/cirurgia , Reto/cirurgia , Estudos Retrospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios XRESUMO
Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are primary pancreatic neoplasms that can act as precursors to invasive adenocarcinoma of the pancreas. The peritumoral stroma has been increasingly recognized to play an important role in many types of tumors. Therefore, to investigate the clinicopathological significance of the peritumoral stroma in IPMNs, we examined the histological features of the peritumoral stroma in each subtype and histological grade of IPMNs. Eighty-two patients with IPMN, who underwent surgical resection, were reviewed clinicopathologically. Intraductal papillary mucinous neoplasms (86 lesions in total) were histologically subclassified into gastric (n = 51), intestinal (n = 22), pancreatobiliary (n = 11), and oncocytic (n = 2) subtypes. Peritumoral histological features between the gastric and intestinal subtypes were evaluated by each histological grade. The results showed that subepithelial edema and inflammatory cell infiltration were more commonly observed in the gastric subtype (74% and 79%, respectively) than in the intestinal subtype (12% and 25%, respectively) of low-grade IPMNs. On the other hand, mucus lakes were more commonly observed in the intestinal subtype (100%) than in the gastric subtype (0%) of high-grade IPMNs. In addition, pancreatobiliary subtype IPMNs tended to exhibit acute inflammation such as neutrophil predominance. This study showed that peritumoral histological features differed among subtypes of IPMNs from low-grade tumors. These differences suggest the possibility that each subtype of IPMNs has a distinct mechanism from an early stage of tumor progression, which is reflected in the properties of the peritumoral stroma.
Assuntos
Adenocarcinoma Mucinoso/patologia , Adenocarcinoma Papilar/patologia , Carcinoma Ductal Pancreático/patologia , Neoplasias Pancreáticas/patologia , Adenocarcinoma Mucinoso/classificação , Adenocarcinoma Mucinoso/metabolismo , Adenocarcinoma Mucinoso/cirurgia , Adenocarcinoma Papilar/classificação , Adenocarcinoma Papilar/metabolismo , Adenocarcinoma Papilar/cirurgia , Idoso , Biomarcadores Tumorais/metabolismo , Carcinoma Ductal Pancreático/classificação , Carcinoma Ductal Pancreático/metabolismo , Carcinoma Ductal Pancreático/cirurgia , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/classificação , Neoplasias Pancreáticas/metabolismo , Neoplasias Pancreáticas/cirurgia , Estudos RetrospectivosRESUMO
Some studies have found that gender mismatch between donors and recipients are related to poor graft prognosis after liver transplantation. However, few studies have investigated the impact of gender mismatch on acute cellular rejection (ACR) in pediatric living donor liver transplantation (LDLT). This retrospective study investigated the clinical significance of these factors in ACR after pediatric LDLT. Between November 2001 and February 2012, 114 LDLTs were performed for recipients with biliary atresia (BA) using parental grafts. We performed univariate and multivariate analyses to identify the factors associated with ACR. The donor-recipient classifications included mother donor to daughter recipient (MD; n = 43), mother to son (n = 18), father to daughter (FD; n = 33), and father to son (n = 20) groups. The overall incidence rate of ACR in the recipients was 36.8%. Multivariate analysis showed that gender mismatch alone was an independent risk factor for ACR (P = 0.012). The FD group had a higher incidence of ACR than the MD group (P = 0.002). In LDLT, paternal grafts with gender mismatch were associated with a higher increased incidence of ACR than maternal grafts with gender match. Our findings support the possibility that maternal antigens may have an important clinical impact on graft tolerance in LDLT for patients with BA.
Assuntos
Atresia Biliar/cirurgia , Rejeição de Enxerto/prevenção & controle , Transplante de Fígado/métodos , Doadores Vivos , Doença Aguda , Adolescente , Adulto , Criança , Pré-Escolar , Pai , Feminino , Sobrevivência de Enxerto/imunologia , Humanos , Tolerância Imunológica , Lactente , Masculino , Mães , Estudos Retrospectivos , Fatores de Risco , Caracteres Sexuais , Adulto JovemRESUMO
The use of donors with coagulation FIX deficiency is controversial, and there are no current protocols for peri-transplant management. We herein describe the first reported case of a pediatric LDLT from an asymptomatic donor with mild coagulation FIX deficiency. A 32-yr-old female was evaluated as a donor for her 12-month-old daughter with biliary atresia. The donor's pretransplant coagulation tests revealed asymptomatic mild coagulation FIX deficiency (FIX activity 60.8%). Freeze-dried human blood coagulation FIX concentrate was administered before the dissection of the liver and 12 h afterwards by bolus infusion (40 U/kg) and was continued on POD 1. The bleeding volume at LDLT was 590 mL. On POD 1, 3, 5, and 13, the coagulation FIX activity of the donor was 121.3%, 130.6%, 114.6%, and 50.2%, respectively. The donor's post-transplant course was uneventful, and the recipient is currently doing well at 18 months after LDLT. The FIX activity of the donor and recipient at nine months after LDLT was 39.2% and 58.0%, respectively. LDLT from donors with mild coagulation FIX deficiency could be performed effectively and safely using peri-transplant short-term coagulation FIX replacement and long-term monitoring of the plasma FIX level in the donor.
Assuntos
Atresia Biliar/cirurgia , Hemofilia B , Transplante de Fígado/métodos , Doadores Vivos , Adulto , Doenças Assintomáticas , Feminino , Humanos , LactenteRESUMO
A 54-year-old Japanese woman was referred with a gallbladder tumor. Based on the results of the computed tomography scan, endoscopic retrograde cholangiopancreatography, and magnetic resonance cholangiopancreatography, a mucin-producing neoplasm of the gallbladder associated with pancreaticobiliary maljunction was diagnosed. Extended cholecystectomy, extrahepatic bile duct resection, and choledochojejunostomy were performed, and she remains free of recurrence 24 months after resection. Histopathological examination revealed that the papillary component of the lesion was an intracystic papillary neoplasm with diverse characteristics of pancreaticobiliary epithelium and intestinal epithelium including mucin. In this component, most of the papillary lesion was a high-grade intraepithelial neoplasm, but also showed slight invasion into the muscular layer. The nodular component consisted of both poorly differentiated biliary type adenocarcinoma and large cell neuroendocrine carcinoma. We report a rare case of a mixed adenoneuroendocrine carcinoma arising from an intracystic papillary neoplasm associated with pancreaticobiliary maljunction. As for the histogenesis of this tumor, based on the histopathologic appearance, transdifferentiation from poorly differentiated biliary type adenocarcinoma to large cell neuroendocrine carcinoma is considered the most possible histogenesis of this tumor.
Assuntos
Sistema Biliar/anormalidades , Biomarcadores Tumorais/metabolismo , Carcinoma Neuroendócrino/patologia , Carcinoma Papilar/patologia , Neoplasias da Vesícula Biliar/patologia , Pâncreas/anormalidades , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/metabolismo , Carcinoma Papilar/metabolismo , Diagnóstico Diferencial , Feminino , Neoplasias da Vesícula Biliar/diagnóstico , Neoplasias da Vesícula Biliar/metabolismo , Humanos , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
PURPOSE: The purpose of this study was to evaluate the clinical features, pathology, and etiology of adenocarcinoma in patients with anal fistulae. METHODS: We identified seven patients diagnosed with adenocarcinoma associated with anal fistulae from a retrospective chart review. RESULTS: Five patients were diagnosed with primary adenocarcinoma associated with anal fistulae. Two patients were diagnosed with secondary adenocarcinoma associated with anal fistulae originating from rectal cancer on the proximal side. The primary adenocarcinomas included cancers arising from long-standing anal fistulae fulfilling established diagnostic criteria in two patients, and cancer arising from short-duration anal fistulae in three patients. Excision of the fistula was performed based on the initial diagnosis of the anal fistula for all five patients. Increased suspicion of cancer was due to the existence of gelatinous material in the anal fistula in three patients and induration in the resected specimens in two patients. The etiologies of the secondary adenocarcinomas associated with anal fistulae included implantation in the anal fistula from rectal cancer and fistula formation originating due to the progression of rectal cancer. CONCLUSION: Anal fistulae are commonly seen in the coloproctology clinic, but special attention to similar conditions associated with malignant disease is needed.