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1.
Int J Surg Pathol ; 25(7): 585-591, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28552015

RESUMO

INTRODUCTION: Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of amphicrine tumor and first case associated with chronic pancreatitis. METHODS: Retrospective review of 3 patients who were diagnosed to have ampullary MANEC. RESULTS: All 3 patients were diagnosed preoperatively as neuroendocrine carcinoma and underwent margin negative pancreaticoduodenectomy. The histopathology revealed MANECs of small cell, mixed type in 2 patients and large cell, amphicrine type in 1 patient. The neuroendocrine component was grade 3 in all, the tumor was T3 in 2 and T2 in 1 and all had nodal metastases. Two patients received adjuvant chemotherapy and 2 of them had recurrence at 13 and 16 months. The median survival was 15 months. CONCLUSION: Ampullary MANECs are rare tumors. They are diagnosed on histopathologic examination of the resected specimen. Clinical presentation, management, and prognosis is similar to ampullary adenocarcinoma in literature.


Assuntos
Adenocarcinoma/patologia , Ampola Hepatopancreática/patologia , Carcinoma Neuroendócrino/patologia , Neoplasias do Ducto Colédoco/patologia , Tumor Misto Maligno/patologia , Doenças Raras/patologia , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/mortalidade , Adenocarcinoma/terapia , Adulto , Ampola Hepatopancreática/diagnóstico por imagem , Ampola Hepatopancreática/cirurgia , Biomarcadores Tumorais/sangue , Carcinoma Neuroendócrino/diagnóstico por imagem , Carcinoma Neuroendócrino/mortalidade , Carcinoma Neuroendócrino/terapia , Quimioterapia Adjuvante , Neoplasias do Ducto Colédoco/diagnóstico por imagem , Neoplasias do Ducto Colédoco/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/diagnóstico por imagem , Tumor Misto Maligno/mortalidade , Tumor Misto Maligno/terapia , Gradação de Tumores , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Pancreaticoduodenectomia , Prognóstico , Doenças Raras/diagnóstico por imagem , Doenças Raras/mortalidade , Doenças Raras/terapia , Estudos Retrospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios X
2.
Transpl Int ; 29(10): 1126-35, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27429066

RESUMO

This study aimed to evaluate the association of postoperative thrombocytopenia with outcome following adult living donor liver transplantation (LDLT) for end-stage liver disease (ESLD). It was a prospective study of 120 consecutive adult LDLT from September 2012 to May 2015. Preoperative platelet counts (PLTs) and postoperative PLTs were recorded at regular intervals till 3 months after LDLT. Univariate and multivariate analyses were performed. The median pretransplant PLT was 61 × 10(9) /l. The lowest median PLT after LDLT was observed on POD 3. Patients were stratified into low platelet group (n = 83) with PLT <30 × 10(9) /l and high platelet group (n = 37) with PLT ≥30 × 10(9) /l. Patients with PLT <30 × 10(9) /l had statistically significant higher grade III/IV complication (P = 0.001), early graft dysfunction (P = 0.01), sepsis (P = 0.001), and prolonged ascites drainage (P = 0.002). On multivariate analysis, PLT<30 × 10(9) /l was identified as an independent risk factor for grade III/IV complications (P = 0.005). Overall, patients survival was significantly different between two groups (P = 0.04), but this predictive value was lost in patients who survived more than 90 days (P = 0.37). Postoperative PLT of <30 × 10(9) /l was a strong predictor of major postoperative complications and is associated with early graft dysfunction, prolonged ascites drainage, and sepsis. The perioperative mortality rate was high in the thrombocytopenia group.


Assuntos
Doença Hepática Terminal/cirurgia , Transplante de Fígado/efeitos adversos , Trombocitopenia/etiologia , Adulto , Ascite/complicações , Doença Hepática Terminal/complicações , Feminino , Sobrevivência de Enxerto , Humanos , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Contagem de Plaquetas , Complicações Pós-Operatórias , Período Pós-Operatório , Curva ROC , Fatores de Risco , Sensibilidade e Especificidade , Sepse/complicações , Resultado do Tratamento
3.
Hepatol Int ; 10(4): 657-64, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26988386

RESUMO

BACKGROUND AND AIM: A thorough donor evaluation in the living donation process is mandatory to ensure a safe outcome in an otherwise healthy individual. The aim of the current study was to evaluate the reasons for not proceeding to donation and the outcome of live liver donors. METHODS: A prospective study of potential donors who underwent evaluation and proceeded to surgery from 1 April 2012 to 31 January 2015 was conducted. The process of donor selection, its outcome and peri-operative complications were recorded. RESULTS: A total of 460 donors were evaluated in a stepwise manner for 367 potential recipients. Of the 321 (69.7 %) donors not proceeding to donation, the reasons were donor-related in 63.6 % and recipient-related in the rest. Common donor-related reasons were: donor reluctance (23.5 %), negative liver attenuation index (16.2 %), anatomic variations (10.3 %), inadequate remnant liver volume (9.8 %), unacceptable liver biopsy (8.8 %), and inadequate graft volume (5.4 %). A majority of donors (82.8 %) were turned down early in the (steps 1 and 2) evaluation process. Recipient death was the most common recipient-related reason [n = 51 (43.6 %)] for not proceeding to donation. There was no donor mortality. The overall complication rate was 19.8 % and major complication rate (grade 3 or higher) was 4.4 %. CONCLUSIONS: A stringent stepwise donor evaluation process leads to early recognition of unsuitable donors and a low complication rate.


Assuntos
Seleção do Doador/métodos , Doadores Vivos/estatística & dados numéricos , Adulto , Seleção do Doador/normas , Seleção do Doador/estatística & dados numéricos , Feminino , Humanos , Índia , Transplante de Fígado , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Obtenção de Tecidos e Órgãos/normas , Obtenção de Tecidos e Órgãos/estatística & dados numéricos , Transplantados , Adulto Jovem
4.
J Clin Exp Hepatol ; 5(4): 344-7, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26900277

RESUMO

Dyskeratosis congenita is a multisystem genetic disorder. Although hepatic involvement is reported in about 7% of patients with dyskeratosis congenita, it is not well characterized and often attributed to hemochromatosis from frequent blood transfusions. A few case reports describe cirrhosis and hepatic cell necrosis in affected individuals in autosomal dominant pedigrees. Bone marrow failure and malignancies are the principal causes of death in dyskeratosis congenita. We describe the first case of living donor liver transplantation, in dyskeratosis congenita for decompensated cirrhosis with portal hypertension. The patient also had associated severe hepatopulmonary syndrome, interstitial lung disease, bilateral hip replacement for avascular necrosis of the head of femur, and a past history of bone marrow transplantation for bone marrow failure.

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