Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022116 (ischemia)
91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The antiphospholipid antibody syndrome (APA) is characterized by an increased incidence of venous and arterial thrombosis. APA syndrome has some gastroenterological manifestations such as Budd-Chiari syndrome, hepatic infarction, esophageal necrosis, intestinal ischemia, pancreatitis and colonic ulceration. We report a 34-year-old man with APA syndrome complicated by hepatic venous thrombosis (Budd-Chiari) and colonic ulcers. The clinical and laboratory findings were compatible with APA syndrome that developed secondary to systemic lupus erythematosus. In order to initiate anticoagulant therapy, he was heparinized. Since lower gastrointestinal bleeding developed, heparin was discontinued and the patient was followed up with baby aspirin and steroids. This case report extends the gastroenterological manifestations of the APA syndrome to include colonic ulceration, which may outweigh the efficacy of initial anticoagulant therapy.
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PMID:A case of antiphospholipid antibody syndrome with Budd-Chiari and colonic ulcers complicated with gastrointestinal hemorrhage. 1533 24

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
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PMID:Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients. 1651 88

Several treatment options exist for the management of Budd-Chiari syndrome (BCS), yet the relative role and timing of liver transplantation (LT) remain poorly defined. Small case series published to date have not been able to delineate the impact of comorbidities and thromboembolic complications of BCS on survival after LT. To better understand the outcomes after LT for BCS, we analyzed 510 liver transplants performed for this disease in the United States between 1987 and 2006. Risk factors predicting graft loss or patient death included increased recipient age, hyperbilirubinemia, elevated creatinine, life support or hospitalization at the time of transplantation, prior transplantation, prior abdominal surgery, increased donor age, and prolonged cold ischemic time (CIT). Prior transjugular intrahepatic portosystemic shunt (TIPS) was not associated with worse outcomes. Transplantation in the Model for End-Stage Liver Disease (MELD) era was associated with significantly lower risk of graft loss (hazard ratio [HR], 0.50; 95% confidence interval [CI], 0.30-0.86; P = 0.012) and death (HR, 0.52; 95% CI, 0.29-0.93; P = 0.027). Similarly, MELD era was associated with significantly lower risk of early graft loss (odds ratio [OR], 0.35; 95% CI, 0.16-0.79, P = 0.012) and early death (odds ratio, 0.37; 95% CI, 0.14-0.95; P = 0.040). However, patients with BCS transplanted in the MELD era were less likely to have life support, hospitalization, prior transplants, and prolonged cold ischemia times. In conclusion, outcomes of LT for BCS are excellent, with further improvements since 2002 associated with a selection shift imposed by MELD-based organ allocation.
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PMID:Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. 1776 80

This tutorial survey with images examines current and possible future applications of the recently introduced 360-degree Doppler capable echoendoscopes. The better gray-scale resolution together with flow, spectral and color Doppler can improve EUS investigations in the mediastinum, abdomen and pelvis. There is no or very little EUS literature covering such important fields a Budd-Chiari syndrome, anatomic variations of the celiac trunk and hepatic artery, mesenteric artery flow determinations in the evaluation of mesenteric ischemia and arterial compression syndromes (celiac, superior mesenteric artery and hepatic artery). Additional other areas of possible future research are suggested.
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PMID:Doppler-endosonography with the GF-UE 160 electronic radial echoendoscope - current use and future potential. 1819 33

We describe our experience with a modification of the piggyback (PB) technique for orthotopic liver transplantation in which the donor infrahepatic vena cava is used as the venous outflow tract. From May 1997 to January 2006, a total of 109 cases using this technique were performed in 101 patients. Collected data included recipient demographics and diagnosis, warm ischemia time, use of venovenous bypass or temporary portacaval shunt and complications related to the venous outflow and graft, and patient survival. Data were compared with the patients undergoing standard PB technique during the same period. The reasons for using the technique were grouped according to whether there was a problem with the recipient hepatic veins or a concern about the length or diameter of the donor suprahepatic vena cava. These included the presence of a transjugular intrahepatic portosystemic shunt (eight cases), retransplantation (22 cases), thin-walled, friable hepatic veins (32 cases), Budd-Chiari syndrome (two cases), domino liver procurement (six cases), reduced or split liver grafts (five cases), and graft inferior vena cava to recipient hepatic veins size discrepancy (34 cases). There was no graft loss. The warm ischemia time was 39.65 minutes compared with 37 minutes in the standard PB group. The long-term graft and patient survival rates were similar in the two groups. Infrahepatic vena cavocavostomy is a useful variation of the standard PB technique.
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PMID:Infrahepatic vena cavocavostomy, a modification of the piggyback technique for liver transplantation. 1979 Jan 69

Focal nodular hyperplasia (FNH) is a relatively common benign liver tumor with rare indications to surgery. Early after pregnancy, a 35-year-old woman developed right upper quadrant abdominal pain with fever. A large abdominal mass was palpable. Abdominal CT scan showed a 18-cm FNH substituting all liver segments but S6 and S7, compressing middle and left hepatic vein near their origin, displacing and compressing right hepatic vein, with ascites. Surgery consisted of a left hepatectomy extended to S5-S8 and S1. Main technical challenge was the preservation of the right hepatic vein. Intermittent pedicle clamping was performed, associated with hepatic vascular exclusion with preservation of caval flow; total duration of ischemia was 210 min. The postoperative course was uneventful, except for a transient fall in prothrombin time, and the formation of a sub-diaphragmatic serous collection, which was percutaneously drained. The patient is well 25 months after the operation. To our knowledge, this is the second reported case requiring surgery for a FNH causing a Budd-Chiari syndrome. In these peculiar cases a cumbersome operation may be required, maximizing all precautions to perform a risk-free procedure.
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PMID:Giant focal nodular hyperplasia determining Budd-Chiari syndrome: an operative challenge requiring 210 min of liver ischemia. 2192 17

Splanchnic vein thrombosis (SVT) is a broad term that includes Budd-Chiari syndrome and occlusion of veins that constitute the portal venous system. Due to the common risk factors involved in the pathogenesis of these clinically distinct disorders, concurrent involvement of two different regions is quite common. In acute and subacute SVT, the symptoms may overlap with a variety of other abdominal emergencies while in chronic SVT, the extent of portal hypertension and its attendant complications determine the clinical course. As a result, clinical diagnosis is often difficult and is frequently reliant on imaging. Tremendous improvements in vascular imaging in recent years have ensured that this once rare entity is being increasingly detected. Treatment of acute SVT requires immediate anticoagulation. Transcatheter thrombolysis or transjugular intrahepatic portosystemic shunt is used in the event of clinical deterioration. In cases with peritonitis, immediate laparotomy and bowel resection may be required for irreversible bowel ischemia. In chronic SVT, the underlying cause should be identified and treated. The imaging manifestations of the clinical syndromes resulting from SVT are comprehensively discussed here along with a brief review of the relevant clinical features and therapeutic approach.
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PMID:Imaging Diagnosis of Splanchnic Venous Thrombosis. 2660 Aug 1

Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident. Recent data support that in BCS liver fibrosis could arise from chronic microvascular ischemia. A reasoning model of BCS physiopathology is that impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development and causes hepatic fibrosis and liver failure through chronic ischemic damage. On this assumption is the concept that relieving liver congestion could ameliorate liver function and prevent development of BCS complications. Recently, early interventional treatment with TIPS for BCS has been reported to be effective. Early TIPS seems to be the best option for BCS management. Future multicenter controlled studies should compare the outcome of BCS treated with early interventional treatment compared with stepwise strategy.
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PMID:Timing of Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: An Italian Hepatologist's Perspective. 2934 Feb 75


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