James E. Bredfeldt

Increased Blood Flow Through the Portal System in Cirrhotic Rats

Portal venous pressure is the result of the interplay between portal venous blood flow and the vascular resistance offered to that flow. Whether portal hypertension is maintained only by an increased portal venous resistance or also by an increased blood flow within the portal venous system is still open to speculation. To resolve these differences, splanchnic and systemic hemodynamics were evaluated in cirrhotic rats, induced by CCl4. Blood flow and portal-systemic shunting were measured by radioactive microsphere techniques. All cirrhotic rats had portal hypertension (portal venous pressure 13.5 +/- 1.1 vs. 9.0 +/- 0.5 mmHg, in normal control rats; p less than 0.01), but portal-systemic shunting in cirrhosis (31% +/- 13% vs. 0.2% +/- 0.02%; p less than 0.05) was variable, ranging from 1% to 97%. Portal venous inflow, the total blood flow within the portal system, was increased in cirrhotic rats (5.75 +/- 0.04 vs. 4.52 +/- 0.36 ml/min per 100 g; p less than 0.05). Total splanchnic arterial resistance was reduced in cirrhotic rats (3.3 +/- 0.2 vs. 5.8 +/- 0.5 dyn X s X cm-5 X 10(5); p less than 0.01). Portal venous resistance, however, was not abnormally elevated in cirrhotic rats (4.6 +/- 0.5 vs. 4.7 +/- 0.5 dyn X s X cm-5 X 10(4), p = NS). Splanchnic hemodynamics in cirrhotic rats demonstrate that portal hypertension is maintained, at least in part, by a hyperdynamic portal venous inflow. The hemodynamic data in cirrhotic rats provided evidence that supports the role of an increased portal blood flow in portal hypertension and gives a quantitative definition of splanchnic hemodynamics in intrahepatic portal hypertension.

Portal colopathy : prospective study of colonoscopy in patients with portal hypertension

Twenty patients with portal hypertension related to a variety of causes prospectively underwent colonoscopy for hematochezia (n = 10), hemoccult positive stool and anemia (n = 9), or polyp found with screening flexible sigmoidoscopy (n = 2) (includes 1 patient with anemia/heme-positive stool). Twelve patients (60%) had previously undergone a course of sclerotherapy, and 10 (50%) had endoscopic evidence of congestive gastropathy. Colonoscopic findings included mucosal abnormalities resembling multiple vascular ectasias in 14 (70%), 4 of whom also had endoscopic features suggesting a mild, chronic colitis. Neither signs of chronic liver disease nor stigmata suggestive of more severe portal hypertension correlated with the colonoscopic findings. Two patients required heater probe therapy for actively oozing lesions resembling vascular ectasias and an additional two patients sclerotherapy for bleeding midrectal varices. Although likely an overestimate of the frequency, this study suggests that portal colopathy can occur in portal hypertension. Vascular ectasialike lesions in such settings may be associated with acute as well as chronic gastrointestinal bleeding and may require pharmacological, directed endoscopic, or portal decompressive therapy. Additional studies are required to determine not only the pathophysiology but also the true frequency of this entity.

A twenty-year experience with endoscopic therapy for symptomatic primary sclerosing cholangitis.

Goals The current study presents 1 tertiary endoscopy centers 20-year experience using endoscopic therapy to treat patients with symptomatic primary sclerosing cholangitis (PSC). Background Endoscopic therapy for patients with PSC and dominant strictures has been used for more than 20 years, but there is concern that instrumenting a sclerotic biliary tree induces risks that outweigh anticipated benefits. Study In this retrospective chart review, 117 patients with PSC were identified using ICD-9 codes. Patients had a mean age of 47 years (range: 15 to 86 y). Mean duration of follow-up was 8 years (range: 2 to 20 y). Of the 117 identified patients, 106 underwent endoscopic retrograde cholangiopancreatography on one or more occasions (for a total of 317 endoscopic retrograde cholangiopancreatographies), and a subset of 84 patients received endoscopic therapy for treatment of dominant strictures and/or deteriorating clinical status. Actual survival for endoscopically treated patients was compared with predicted survival using the Mayo Clinic natural history model for PSC. Results Our chart review revealed 23 recognized complications among the 317 procedures performed (7.3%), and no procedure-related deaths. Observed patient survival at years 3 and 4 was significantly higher than that predicted by the Mayo Clinic natural history model for PSC (P=0.021). Conclusions Patients with PSC who have a deteriorating clinical course benefited from endoscopic therapy to provide drainage of bile ducts, removal of stones, and/or temporary relief from obstructions, with acceptable procedure-related complications and higher than expected 3-year and 4-year survival.

Autoimmune chronic active hepatitis masquerading as acute hepatitis.

An unusual clinical presentation of chronic active hepatitis is the abrupt onset of symptoms and jaundice, suggesting acute viral hepatitis. In this report, six patients had the acute onset of a severe liver disease. Five of the patients were female and ranged in age from 13 to 64 years. Marked elevations in the total bilirubin (17.1 ± 11.4 mg/dl), AST (1,346 ± 352 mIU/ml), and ALT (1,043 ± 213 mlU/ml) were present (mean ± SD). Negative serologies for hepatitis A and B were found. Liver histology showed severe hepatocellular injury. A diagnosis of autoimmune chronic active hepatitis with acute features was made on the basis of high titers of antinuclear antibody and smooth muscle antibody and the presence of hypergammaglobulinemia. As immunosup-pressive therapy is a beneficial treatment of autoimmune chronic active hepatitis, an acute presentation of this liver disease should be considered as an alternative diagnosis to acute non-A, non-B hepatitis in patients with these clinical characteristics.

Sinusoidal lymphocytosis of the liver in Felty's syndrome with a review of the liver involvement in Felty's syndrome

A 68-year-old woman with Feltys syndrome had massive hepatomegaly. Liver biopsy showed diffuse infiltration of the sinusoids with mature lymphocytes (sinusoidal lymphocytosis of the liver). Nodular regenerative hyperplasia of the liver and portal fibrosis were not found. Although liver involvement is frequent in association with Feltys syndrome, sinusoidal lymphocytosis of the liver is unusual.

Idiopathic portal hypertension in a renal transplant recipient.

A renal transplant recipient developed a noncirrhotic form of portal hypertension, idiopathic portal hypertension, several years after the transplantation. The liver histology by light microscopy disclosed only chronic portal inflammation and portal fibrosis; however, a prominent collagen deposition within the space of Disse seen only by electron microscopy evolved during the course of the liver disease. This report, in association with other reports of the same disorder in renal transplant recipients, suggests that idiopathic portal hypertension may occur as a direct consequence of renal transplantation.