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Query: UMLS:C0000737 (abdominal pain)
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We report on the clinical presentation and diagnostic findings in 45 patients with Budd-Chiari syndrome. The diagnosis was confirmed by histology at the time of liver transplantation (n = 37) or shunt surgery (n = 8). An underlying disorder could be established only in half of the patients, oral contraceptives as predisposing factor were known in 18 cases. Clinically, abdominal pain and distension as well as hepatomegaly and ascites were most frequent findings, whereas changes in laboratory data were more or less unspecific. By use of repeated ultrasound, a definite diagnosis of a Budd-Chiari syndrome could be confirmed in all cases by obligatory demonstration of obstruction or thrombosis of at least one major liver vein. Hepatic venography revealed only one false-negative result. Celio-mesenteric arteriography plus portography, cavography and preoperative liver biopsy did not present additional diagnostic information. These techniques may contribute to treatment planning of portosystemic shunt surgery or hepatic transplantation. In conclusion, the presence of hepatosplenomegaly, ascites, abdominal pain and distension, especially in combination with a known hypercoagulable state, should alert to the possibility of a Budd-Chiari syndrome. Ultrasound is the diagnostic tool of choice. Hepatic venography should only be performed if even repeated ultrasound is not conclusive.
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PMID:The Budd-Chiari syndrome: clinical presentation and diagnostic findings in 45 patients treated by surgery. 798 58

In 24 patients with Budd-Chiari syndrome age 15-66, 19 were men, and 5 women. The patients experienced abdominal pain, hepatomegaly, splenomegaly and ascites. Imaging examinations were carried out in all the patients (16 ultrasonography, 5 Doppler, 10 CT, 18 venography, and 4 MRI). Direct features of Budd-Chiari syndrome were striking reduction in caliber, complete absence or obstruction of hepatic veins, intrahepatic inferior vena cava, "comma-shaped" or curled-tubular intrahepatic collateral veins; indirect signs included hepatomegaly, splenomegaly and ascites, extrahepatic collateral veins. Ultrasonography,CT, MRI and venography were all useful in the diagnosis of patients with Budd-Chiari syndrome. It is suggested that ultrasonography is a non-invasive procedure and MRI is the best imaging procedure of choice in the evaluation of Budd-Chiari syndrome.
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PMID:[Imaging diagnosis of Budd-Chiari syndrome: report of 24 cases]. 812 14

The clinical presentation, investigations, therapeutic modalities, prognosis and outcome of 44 patients with Budd-Chiari syndrome (BCS) were reviewed. There were 27 women and 17 men. Median age at presentation was 37 years (range 14-60). Possible aetiological factors were identified in 31 patients (70%). Myeloproliferative disorders were the commonest aetiology. Abdominal pain and swelling were the commonest presenting symptoms. Sixteen patients underwent a shunt operation (14 mesocaval, 2 mesoatrial). Ten patients had liver transplantation. Eleven patients had angioplasty/stent as their only treatment, and seven were treated medically or died before any treatment was instituted. Radiological intervention was comparable to mesocaval shunt in relieving patients' symptoms and in achieving good long-term results. Medical treatment and liver transplantation both yielded poor results. Radiological intervention in the form of balloon angioplasty or stent placement gives good results in a subgroup of BCS patients, and should be tried first to relieve the hepatic outflow obstruction. Mesocaval shunts provide good results in selected cases. Underlying haematological causes should be intensively investigated and promptly treated.
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PMID:Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. 891 49

In 1992 The International Registry of Inferior Vena Cava (IVC) Leiomyosarcomas was established to study the pathogenesis and natural history of the tumor and to support the most rational treatment. We collected 218 patients through a literature review and personal communications. We corresponded with several Authors to obtain up-to-date follow-up and any other data lacking at the initial review. The series was analyzed to identify predictive factors for clinical outcome. Tumors arose from the IVC lower segment in 80 patients, from the middle in 94 and from the upper in 41. A radical tumor resection was undertaken in 134 (61.5%) patients, 26 (11.9%) had a palliative resection and 58 (26.6%) were inoperable. An increased risk of death was associated with upper IVC segment involvement (p < 0.001), lower limb edema (p < 0.001), Budd-Chiari's syndrome (p < 0.001), intraluminal tumor growth (p < 0.001) and IVC occlusion (p < 0.001). Radical tumor resection was associated with better 5- and 10-year survival rates (49.4% and 29.5%). Tumors which arose from the middle segment fared better (56.7% and 47.3%) than those of the lower segment (37.8% and 14.2%) (p < 0.002). No palpable abdominal mass and abdominal pain were associated, in patients radically operated, with a better outcome and longer survival (p < 0.03 and p > 0.04 respectively). Despite the high rate of recurrence, radical tumor resection is the only long-term cure.
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PMID:International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. 892 Jul 90

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.
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PMID:[Budd-Chiari syndrome]. 933 72

The case of a patient with sudden onset of abdominal pain, hepatomegaly and laboratory findings which indicated necrosis of a parenchymal organ is reported. Patient died soon after the admission to hospital after fulminant disease development. At the time of admission diagnosis of hepatic vein occlusion, i.e., Budd-Chiari syndrome was made based on ultrasonographic finding. Obduction confirmed this finding showing lung adenocarcinoma with liver metastases as well as occlusion of all hepatic vein branches with thrombi. This report emphasizes the role of ultrasonographic diagnostics in the early diagnosis of Budd-Chiari syndrome.
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PMID:[The Budd-Chiari syndrome--case report of a patient with hepatic vein occlusion and adenocarcinoma of the lung]. 937 21

We report a case of a 37-year-old female who suffered from upper abdominal pain, progressive abdominal distention, shortness of breath, palpitation and pitting edema of lower legs for more than one month. Abdominal sonography showed small caliber of hepatic veins, mild hepatosplenomegaly and moderate ascites. Computed tomography of abdomen disclosed extensive thrombi in bilateral femoral veins, ovarian veins and inferior vena cava. Ascites was transudate with normal cell count. Laboratory data showed hypoalbuminemia, mild elevation of total bilirubin and iron deficiency anemia. Anti-cardiolipin antibody was positive and antinuclear antibody was negative. The histopathological features, including sinusoidal dilatation with atrophic change of adjacent hepatocytes, slight congestion and hemosiderin-like material within the cytoplasm of Kupffer cells, were compatible with the criteria of Budd-Chiari syndrome. Heparin was intravenously administered immediately to prevent further progression of thrombosis. The ascites was successfully controlled with diuretics (spironolactone and furosemide). After a two-week course of treatment, she was discharged in good condition and on warfarin anti-coagulant medication.
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PMID:Anticardiolipin antibody-related Budd-Chiari syndrome: report of a case. 951 90

A 31-year-old male patient had a transjugular intrahepatic portal systemic shunt (TIPS) placed for acute Budd-Chiari syndrome secondary to paroxysmal nocturnal hemoglobinuria (PNH). Post-procedure, he was anticoagulated for his underlying paroxysmal nocturnal hemoglobinuria. After 11 days, he complained of upper abdominal pain and underwent magnetic resonance imaging (MRI). On immediate post-gadolinium spoiled-gradient-echo (SGE) images, active extravasation of gadolinium was depicted in one of two intrahepatic hematomas. Progression of layering of high signal gadolinium was shown from early to later phase post-gadolinium images. The active arterial bleeding was confirmed by conventional angiography performed immediately following the magnetic resonance imaging.
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PMID:Active intrahepatic gadolinium extravasation following TIPS. 981 Nov 50

A 26-year-old woman, after cesarean section in the 33rd week of gestation, developed after delivery thrombosis of the popliteal vein, pulmonary embolism and thrombosis of the portal vein. After completion of a six month period of oral anticoagulation, laboratory investigations revealed diminished levels of plasminogen and free protein S antigen as well as APC-resistance due to heterozygous FV R506Q mutation. After six uneventful years, abdominal sonography and magnetic resonance examination, performed because of abdominal pain, showed liver cirrhosis with Budd-Chiari syndrome. Additional hematological investigations led to the diagnosis of polycythemia vera. Association of myeloproliferative disorders, mainly polycythemia vera, with splanchnic venous thrombosis is well known and should always be looked for.
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PMID:[A 26-year-old woman with splanchnic vein thrombosis as the initial manifestation of polycythemia vera]. 1051 20

Behcet's disease (BD) is a multisystem, chronic, relapsing vasculitis of unknown origin that affects nearly all organs and systems. While recurrent oral ulcerations are a "sine qua non" of BD, the frequency of extra-oral parts of the gastrointestinal involvement varies widely in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. The liver (except with Budd-Chiari syndrome), pancreas, and spleen are rarely involved. The symptoms associated with these extra-oral manifestations of BD are abdominal pain, nausea, vomiting, diarrhea with or without blood, and constipation. The lesions typically are resistant to medical treatment and frequently recur with surgical treatment. We review the literature regarding the gastrointestinal and hepatobiliary systems in BD. Also, we present a patient who had BD complicated with radiologically-proven hepatic veins involvement (Budd-Chiari syndrome) and complete occlusion of hepatic portion of inferior vena cava and who had a good response to colchicine and penicillin treatment.
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PMID:Gastrointestinal manifestations of Behcet's disease. 1073 Sep 19


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