Background Budd-Chiari syndrome （BCS） is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava （IVC）. Angiography is the ＂golden standard＂ for diagnosis...Background Budd-Chiari syndrome （BCS） is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava （IVC）. Angiography is the ＂golden standard＂ for diagnosis, but it is an invasive examination. To assess the diagnostic value of a fresh blood imaging （FBI） relative to BCS, we used a magnetic resonance angiography （MRA） with an FBI sequence for a preoperative evaluation of the BCS patients in this study. Methods Fifty patients who were suspected of having BCS after they had been checked by a B-ultrasound were studied. 2D and 3D FBI were performed on a 1.5T superconductive MR scanner. Original images were rebuilt using a maximal intensity projection （MIP） method on the console. Two doctors reviewed all images before they learned of the angiography results. We then compared the diagnoses obtained from the FBI and angiography results to evaluate the diagnostic value of the FBI.Results Forty-one patients were diagnosed as BCS and 9 as non-BCS based on an angiography. The FBI correctly diagnosed 38 patients, incorrectly diagnosed 1 patient, and missed diagnosis in 3 patients. Thus, the diagnostic sensitivity of the FBI is 93% （38/41）, the specificity is 89% （8/9） and the accuracy is 92% （46/50）. The FBI images of the 13 membranous stenoses of the IVC showed a sudden stenosis of the post-liver segment of the IVC. The Images of the 5 patients with a membranous obstruction of the IVC showed IVC thickening and an absence of blood signals in the post-hepatic segment of the IVC. The images of the 4 patients with the segmental thrombosis of the IVC showed abnormal and intermittent signals in the IVC. The images of the 6 patients with a simple hepatic vein obstruction showed obstructive hepatic veins. The images of the 6 patients with the stenosis of both the IVC and the hepatic veins showed the stenosis of the IVC, the thickening of the hepatic veins and the formation of a compensatory circulation within the liver. Lastly, the images of the 7 patients showed a combination of the IVC thrombosis with stenosis or with the obstruction of one or two hepatic veins. Conclusions An FBI can show a membranous stenosis, and an obstruction and thrombosis of the IVC. In addition, it can also demonstrate the thickening of the flexural hepatic vein and the development of intra-hepatic compensatory branches with slow blood flow. Thus, it can guide the puncturing and opening of the hepatic vein involved in an interventional therapy for BCS patients.展开更多
Transfusions of blood products are common in medical practice and can be lifesaving in certain situations. Potentially life threatening reactions could occur and physicians should be alerted. Here we describe a case o...Transfusions of blood products are common in medical practice and can be lifesaving in certain situations. Potentially life threatening reactions could occur and physicians should be alerted. Here we describe a case of thrombocytopenia that was induced by transfusion of fresh frozen plasma (FFP). A 52 years old male presented to the emergency department after two episodes of hematochezia that resolved spontaneously. Since he was anticoagulated for atrial fibrillation he was given a unit of FFP to reverse a slightly elevated INR. Within 6 hours from the administration of the FFP he developed an acute decrease only in his platelet count. He was managed conservatively and his thrombocytopenia started resolving gradually. After excluding other causes the potential diagnosis was fresh frozen plasma induced thrombocytopenia. The pathophysiologic mechanism is postulated to be immune mediated by passive transfer of antibodyies from the donor to the recipient. The antibodies that are described in the literature are anti-HPA-1a and anti-CD-36. We reported the event to the American Red Cross. Interestingly a male was the donor of the plasma while in all cases in the literature the donors are females with a prior history of pregnancy. Therefore this is the first reported case of a male blood donor whose blood product caused immune mediated thrombocytopenia post transfusion.展开更多
文摘Background Budd-Chiari syndrome （BCS） is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava （IVC）. Angiography is the ＂golden standard＂ for diagnosis, but it is an invasive examination. To assess the diagnostic value of a fresh blood imaging （FBI） relative to BCS, we used a magnetic resonance angiography （MRA） with an FBI sequence for a preoperative evaluation of the BCS patients in this study. Methods Fifty patients who were suspected of having BCS after they had been checked by a B-ultrasound were studied. 2D and 3D FBI were performed on a 1.5T superconductive MR scanner. Original images were rebuilt using a maximal intensity projection （MIP） method on the console. Two doctors reviewed all images before they learned of the angiography results. We then compared the diagnoses obtained from the FBI and angiography results to evaluate the diagnostic value of the FBI.Results Forty-one patients were diagnosed as BCS and 9 as non-BCS based on an angiography. The FBI correctly diagnosed 38 patients, incorrectly diagnosed 1 patient, and missed diagnosis in 3 patients. Thus, the diagnostic sensitivity of the FBI is 93% （38/41）, the specificity is 89% （8/9） and the accuracy is 92% （46/50）. The FBI images of the 13 membranous stenoses of the IVC showed a sudden stenosis of the post-liver segment of the IVC. The Images of the 5 patients with a membranous obstruction of the IVC showed IVC thickening and an absence of blood signals in the post-hepatic segment of the IVC. The images of the 4 patients with the segmental thrombosis of the IVC showed abnormal and intermittent signals in the IVC. The images of the 6 patients with a simple hepatic vein obstruction showed obstructive hepatic veins. The images of the 6 patients with the stenosis of both the IVC and the hepatic veins showed the stenosis of the IVC, the thickening of the hepatic veins and the formation of a compensatory circulation within the liver. Lastly, the images of the 7 patients showed a combination of the IVC thrombosis with stenosis or with the obstruction of one or two hepatic veins. Conclusions An FBI can show a membranous stenosis, and an obstruction and thrombosis of the IVC. In addition, it can also demonstrate the thickening of the flexural hepatic vein and the development of intra-hepatic compensatory branches with slow blood flow. Thus, it can guide the puncturing and opening of the hepatic vein involved in an interventional therapy for BCS patients.
文摘Transfusions of blood products are common in medical practice and can be lifesaving in certain situations. Potentially life threatening reactions could occur and physicians should be alerted. Here we describe a case of thrombocytopenia that was induced by transfusion of fresh frozen plasma (FFP). A 52 years old male presented to the emergency department after two episodes of hematochezia that resolved spontaneously. Since he was anticoagulated for atrial fibrillation he was given a unit of FFP to reverse a slightly elevated INR. Within 6 hours from the administration of the FFP he developed an acute decrease only in his platelet count. He was managed conservatively and his thrombocytopenia started resolving gradually. After excluding other causes the potential diagnosis was fresh frozen plasma induced thrombocytopenia. The pathophysiologic mechanism is postulated to be immune mediated by passive transfer of antibodyies from the donor to the recipient. The antibodies that are described in the literature are anti-HPA-1a and anti-CD-36. We reported the event to the American Red Cross. Interestingly a male was the donor of the plasma while in all cases in the literature the donors are females with a prior history of pregnancy. Therefore this is the first reported case of a male blood donor whose blood product caused immune mediated thrombocytopenia post transfusion.
