Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a report of a long term prospective study of 13 seriously ill patients with Budd-Chiari syndrome as a result of occlusion of the hepatic veins who were treated by side to side portacaval shunt from four to 78 weeks after the onset of symptoms and who were under observation for three to 16 years. The patient population was young, ranging in age from 19 to 45 years; seven were men and six were women. The presumed cause was the use of oral contraceptives in three, polycythemia rubra vera in two, Behcet disease in one patient and unknown in seven patients. All of the 13 patients had abdominal pain, marked ascites, hepatosplenomegaly, wasting and disturbed liver function. Diagnosis was based on the symptoms and signs: angiographic demonstration of hepatic vein occlusion and a patent inferior vena cava; pressure measurements that showed an inferior vena caval pressure that was normal or within the usual range for patients with massive ascites and an elevated wedged hepatic vein pressure that was much higher than the inferior vena caval pressure, and the results of biopsy of the liver showing centrilobular congestion and necrosis. Side to side portacaval shunt was very effective in decompressing the liver, reducing the mean corrected portal pressure from 240 millimeters of saline solution before to 7 millimeters of saline solution after the shunt. Operative survival rate was 92 per cent, and the long term survival rate for three to 16 years is 85 per cent. All of the survivors are free of ascites without requiring diuretic therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long term results of treatment of Budd-Chiari syndrome by side to side portacaval shunt. 290 30

We present a case of Budd-Chiari syndrome (BCS) having two risk factors, Behcet's disease (BD) and oral contraceptive (OC) usage. A 33-year-old woman with BD was admitted to the Emergency Unit with nausea, vomiting, abdominal pain, abdominal distention, and confusion started 12 days ago before admission. Since the patient was in a shock state, she was taken to the Intensive Care Unit (ICU) with the suspicion of abdomen-originated sepsis. Abdominal ultrasound showed massive hepatosplenomegaly and moderate ascites. Abdominal MRI revealed an inferior vena cava (IVC) obstruction starting above the renal veins and diffuse thrombosis of the right and medial hepatic veins. An extensive thrombosis of the IVC and the hepatic veins (BCS) which led to shock was diagnosed. In addition to BD, the unnotified OC usage for a year by the patient without her doctor's knowledge was recognized as possible precipitating factor of BCS. Pulse methylprenisolone was started for three consecutive days to treat active BD-induced vasculitis. IVC digital subtraction angiography (DSA) showed occlusion of the IVC below the hepatic veins with extensive collateral circulation originating at the occlusion level suggesting that obliteration had a subacute or chronic course. Since intralesional thrombolytic therapy failed, the patient was transferred to a liver transplantation center. While waiting for an appropriate donor, the patient died due to hepatic failure. Since BCS is mortal and deemed multi-factorial, every patient with a thrombotic risk factor such as BD should be questioned for other possible causes of thrombosis.
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PMID:A case of Budd-Chiari syndrome with Behcet's disease and oral contraceptive usage. 1757 62