Cases reported "Hypertension, Portal"

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1/14. Contrast-enhanced three-dimensional magnetic resonance angiography for visualization of ectopic varices.

    We report the case of a 62-year-old male with portal hypertension and recurrent bleeding refractory to surgical intervention from varices in a sigmoid stoma. Although stomal varices were detected neither by physical examination, stomal endoscopy, nor duplex sonography, contrast-enhanced three-dimensional magnetic resonance angiography of the portal vein and its collateral branches demonstrated their presence. Surgical revisions of the stoma failed to prevent bleeding, but implantation of a transjugular intrahepatic shunt successfully prevented recurrent hemorrhage. This case indicates that contrast-enhanced, three-dimensional magnetic resonance angiography is useful to detect this rare complication of portal hypertension and helps to tailor adequate treatment in patients with recurrent bleeding from stomal varices.
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2/14. Portal hypertension develops in a subset of children with standard risk acute lymphoblastic leukemia treated with oral 6-thioguanine during maintenance therapy.

    BACKGROUND: 6-thioguanine (TG) was recently studied to determine whether TG in maintenance therapy achieves better event free survival than 6-mercaptopurine (MP) for standard risk acute lymphoblastic leukemia (ALL) on the clinical trial, CCG-1952 (5/1996-1/2000). Veno-occlusive disease was previously recognized as a complication of TG on CCG-1952. We report a newly recognized pediatric complication of TG: splenomegaly and portal hypertension (PH) developing during maintenance or after completion of therapy. PROCEDURE: Twelve patients (3-10 years) had been randomized to receive a targeted dose of 50 mg/m(2)/day of TG during maintenance phases. Actual TG dose ranged from 25 to 77 mg/m(2)/day (median 34 mg/m(2)/day). RESULTS: The initial patient, a boy who had marked thrombocytopenia and intermittent splenomegaly during maintenance therapy, was evaluated for persistent pancytopenia and progressive splenomegaly 3 months after completion of therapy. Dilated splenic vein and collaterals consistent with PH were documented by MRI/MRA. Esophagogastroduodenoscopy found esophageal varices. Liver biopsy showed periportal fibrosis and marked dilatation of veins and venules. Of the other 12 patients, 9 patients studied had abnormal MRI/MRAs with evidence of varices in 4. Eight patients had splenomegaly on physical examination. Liver biopsies in a girl after 3.3 courses of TG and a boy after 4.6 courses of TG showed periportal fibrosis and dilatation of venules and sinusoids and minimal focal fatty changes. Subsequent MRI/MRAs have been stable or improved. CONCLUSIONS: The evaluations of these 12 patients suggest that treatment with TG causes injury to the liver leading to PH and that thrombocytopenia and splenomegaly are clinical hallmarks of this toxicity.
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3/14. Delayed development of a traumatic superior mesenteric arteriovenous fistula following multiple gunshot wounds to the abdomen.

    Traumatic visceral arteriovenous fistulae are rare and pose a diagnostic and therapeutic challenge. We present the case of a 20-year-old male who sustained multiple gunshot wounds to the abdomen, injuring the inferior vena cava, duodenum, stomach, and small bowel. The patient was taken emergently to the operating room for repair of his injuries, which required primary small bowel repair, duodenal repair, and inferior vena cava ligation due to exigent hemorrhage. At the initial operation there was a normal pulse in the superior mesenteric artery at the base of the small bowel mesentery, with no evidence of hematoma or thrill in the small bowel mesentery. The patient was subsequently returned to the operating room several times for bowel exploration and abdominal wall closure with mesh. Ten days after his initial injury, the patient was noted to have an abdominal bruit on physical exam. Arteriography demonstrated a fistula between the proximal superior mesenteric artery and vein with significant portal hypertension. The patient underwent surgical repair of the superior mesenteric artery and vein with closure of the fistula. The patient had no further complications and was discharged from the hospital 1 month later, after abdominal wall skin grafting, in good condition. The patient remains in good health 12 months later. Continued vigilance and careful physical examination are important in the identification of delayed vascular injuries and allow timely treatment and avoidance of untoward long-term sequelae.
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4/14. chylous ascites as the main manifestation of left ventricular dysfunction: a case report.

    BACKGROUND: Ascites is one of the most common complications of liver diseases, even though in 15% of the cases it is related to extrahepatic diseases; 3% are of cardiac nature and they appear associated with signs and symptoms of heart failure. CASE PRESENTATION: A 70 year old man was admitted with more than one year history of abdominal distension and a weight gain of 10 kilograms. He is asymptomatic and walks 2000-3000 meters a day without angor or dyspnea. The physical examination shows moderate abdominal distension, with no hepatosplenomegaly or edema, and there is mild jugular vein distension. The studies performed (complete laboratory work up, paracentesis, liver biopsy, echocardiogram, intrahepatic pressure measurements, etc.) showed a chylous ascites related to portal hypertension, and left ventricular dysfunction was the only probable cause found. CONCLUSION: Asymptomatic heart dysfunction can mimic liver disease and should be kept in mind as a cause of chylous ascites.
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5/14. vitamin a toxicity in a physical culturist patient: a case report and review of the literature.

    Excessive intake of vitamin a may produce acute or chronic toxicity. vitamin a can be consumed in foods, fortified products and supplements. We present a case of a young physical culturist man who was referred to our Unit because of chronic liver disease of unknown origin. The patient had a history of increased vitamin a intake from natural source with the addition of high dose of vitamin a supplements with the purpose of improving his muscular development. Our patient showed chronic liver disease with severe fibrosis, signs of portal hypertension and marked hyperplasia of Ito cells. In conclusion, chronic vitamin a toxicity may produce severe liver damage and should be recognized in the differential diagnosis of chronic liver diseases.
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6/14. association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations.

    To determine the clinical, laboratory and hemodynamic profile in patients with primary pulmonary hypertension and associated portal hypertension, 7 new cases and 71 previously reported cases were analyzed. There was no gender predilection and the average age at diagnosis was 41 years. liver cirrhosis was the most frequent cause of hypertension (82%) and a surgical portosystemic shunt was present in 29%. Almost invariably, portal hypertension either preceded or was diagnosed concurrently with pulmonary hypertension, favoring the hypothesis that in portal hypertension, the pulmonary vasculature may be exposed to vasoactive substances normally metabolized or produced by the diseased liver, possibly inducing vasoconstriction or direct toxic damage to the pulmonary arteries. Clinically, exertional dyspnea was the most frequent presenting symptom (81%); other symptoms, such as syncope, chest pain and fatigue, were present in less than 33%. An accentuated pulmonary component of the second heart sound (82%) and a systolic murmur (69%) were the most common physical findings. At least 75% of these patients had evidence of pulmonary hypertension on electrocardiography (right ventricular hypertrophy) or roentgenography (cardiomegaly or dilated main pulmonary arteries, or both). Hemodynamic findings included severe pulmonary hypertension (mean pulmonary artery pressure 59 /- 19 mm Hg) with normal pulmonary capillary wedge pressure and cardiac output. Treatment was basically palliative and the mean and median survival times were 15 and 6 months, respectively. In brief, on the basis of clinical presentation and laboratory features, patients with combined primary pulmonary hypertension and portal hypertension seldom represent a diagnostic challenge. Further research is needed on treatment, which remains palliative. The survival rate is poor and worse than that seen in isolated primary pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
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7/14. Functional asplenia and portal hypertension in a patient with primary splenic hemangiosarcoma.

    A 60-year-old man with primary splenic hemangiosarcoma (PSH) presented with weakness, weight loss, abdominal pain, and anemia. physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. The patient also had portal hypertension, which was confirmed by physical findings and by measurement of portal vein pressure during operation. A liver-spleen scan using Tc-99m sulfur colloid and Tc-99m labeled heat denatured erythrocytes failed to demonstrate any splenic uptake, a reliable feature of functional asplenia. Although on a total body scan with Ga-67 citrate there was no splenic uptake, there was gallium uptake in the liver, where the presence of the metastatic lesion was histopathologically verified and confirmed by operation. There was also uptake in the middle zones of the lungs. Ga-67 citrate imaging appears to be helpful in the diagnosis of metastasis of PSH, and PSH can rarely cause portal hypertension.
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8/14. Portal hypertension following renal transplantation.

    Portal hypertension and variceal hemorrhage may be found in the renal transplant patient with chronic liver disease. The development of portal hypertension was found to occur after long-term graft survival without significant rejection. The development of positive cytomegalic virus and negative hepatitis-associated antigen appeared to be common. splenomegaly and prominent venous collateral were the most frequent physical findings, while ascites and hepatomegaly were less frequent. Portasystemic decompression can be performed successfully, however, the mortality and morbidity appear to be higher for this group than for other cirrhotic patients with comparable hepatic reserve.
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9/14. Portal hypertension as an early manifestation of carcinoma of the pancreas.

    Massive hematemesis followed by 48 hours the onset of obstructive jaundice in a previously asymptomatic 38-year-old male patient. splenomegaly was the only abnormal physical finding. esophagoscopy visualized bleeding varices. The liver scan was normal. Dilated intra- and extrahepatic bile ducts and a possible retroperitoneal mass were demonstrated by ultrasonography. laparotomy disclosed the presence of a carcinoma of the head and body of the pancreas that obstructed the common bile duct and the portal vein. This case report should draw attention to the vast spectrum of rare presentations of pancreas carcinoma.
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10/14. Splenic arteriovenous fistula.

    A patient with an uncalcified splenic artery aneurysm with an arteriovenous fistula and aneurysmal dilatation of the splenic vein who underwent splenectomy and resection of the fistula is described. Splenic arteriovenous fistulas are rare and are usually associated with aneurysms of the splenic artery. patients with these fistulas frequently have signs and symptoms of portal hypertension and may often present with upper gastrointestinal hemorrhage. The patient described in this report was asymptomatic, and the only manifestation of the fistula was a loud, continuous bruit in the left flank noted on routine physical examination. Although an upper abdominal incision can be used to manage most splenic artery aneurysms, a left thoracoabdominal incision is advocated in the presence of an arteriovenous fistula, particularly in anticipation of portal hypertension or aneurysm of the splenic vein.
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