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Biomedical subjects

E R Dickson

Publications and source records attributed to E R Dickson.

At least 145 records · Page 8Linked to original sources
PubMed1979

Copper stains and the syndrome of primary biliary cirrhosis. Evaluation of staining methods and their usefulness for diagnosis and trials of penicillamine treatment.

Use of Shikata's stain was found to be a sensitive method for the demonstration of copper-protein complexes, but use of the rhodamine stain may be more reliable. Interobserver agreement was slightly better with Shikata's stain. Hepatic copper increased with the histologic progression of chronic nonsuppurative destructive cholangitis (CNDC). All specimens that contained Mallory bodies and most specimens (91%) that contained bile yielded positive copper stains. No correlation was found between positive copper stains and other histologic features. A strongly positive copper stain aided in the diagnosis of CNDC. All specimens with negative copper stains contained less than 250 microgram of copper per gram of dry weight. From a strongly positive copper stain, the chemical copper content could not be predicted with certainty. Shikata's method seemed adequate to evaluate penicillamine treatment trials.

Adult↗
PubMed1978

Asymptomatic primary biliary cirrhosis. Presentation, histology, and results with D-penicillamine.

Of 103 patients with the syndrome of primary biliary cirrhosis (chronic, nonsuppurative destructive cholangitis) who entered a double-blind, randomized, controlled treatment trial with either D-penicillamine or placebo, 21 (20%) were asymptomatic with respect to their liver disease. Study of these 21 patients revealed that (1) 43% of patients with asymptomatic primary biliary cirrhosis had advanced histologic lesions (fibrosis or cirrhosis); (2) asymptomatic patients with advanced histologic lesions likely have had their disease for 10 years or more; (3) stage of primary biliary cirrhosis may remain unchanged for years; and (4) most asymptomatic patients receiving D-penicillamine, when compared with patients given placebo, had improved liver function tests at 1-year follow-up. However, the incidence of major toxicity with D-penicillamine for primary biliary cirrhosis in a maintenance dose of 1 g approximates 20%. Furthermore, one of our patients who was asymptomatic but who had advanced histologic changes died recently from D-penicillamine-associated bone marrow suppression. It remains to be determined whether the benefit-to-risk ratio of D-penicillamine in primary biliary cirrhosis justifies its use.

Adult↗
PubMed1978

Deferoxamine-chelatable iron in hemochromatosis and other disorders of iron overload.

Deferoxamine-chelatable iron was measured in 103 patients with known or suspected iron overload. All of 34 patients with untreated hemochromatosis had distinctly elevated values for deferoxamine-chelatable iron. The mean value in these cases was significantly greater than that in patients with cirrhosis, who had little or no stainable hepatic iron. In 15 patients with hemochromatosis who were tested sequentially during the course of phlebotomy therapy, deferoxamine-chelatable iron proved a reliable index of the degree of reduction of storage iron. In 22 additional patients with partially treated hemochromatosis and 14 with iron overload accompanying chronic anemia, this test correlated well with the magnitude of iron deposits in liver or bone marrow. In patients with unexplained elevations of serum iron, normal or only slightly elevated deferoxamine-chelatable iron correctly indicated that storage (hepatic) iron was not excessive. The test was more reliable than determination of serum iron or transferrin saturation as an indicator of increased storage iron. Elevated values could not be attributed to disturbed liver function. Determination of deferoxamine-chelatable iron is a safe, practical, and useful procedure for identifying persons with increased iron stores and for assessing the effect of phlebotomy therapy.

Adult↗
PubMed1978

Plasmaperfusion for the treatment of intractable pruritus of cholestasis.

A patient with primary biliary cirrhosis and intractable pruritus was treated with plasmaperfusion of charcoal-coated glass beads on two occasions. The procedures were well tolerated and resulted in the removal of about 70% (494 mumol) of the estimated chenic acid pool. There was, in addition, pronounced amelioration of the pruritus which enabled the patient to sleep through the night without awakening because of itching. The pruritus, however, did not disappear and gradually returned to its preperfusion intensity by the end of the third week after perfusion.

Cholestasis↗
PubMed1978

Circulating IgG complexes in primary biliary cirrhosis. A serial study in forty patients followed for two years.

Several antibodies are present in sera of patients with primary biliary cirrhosis (PBC). We have looked for evidence of antigen-antibody complexes in sera of PBC assuming that some of the antibodies may circulate complexed with an antigen. The Raji cell radioimmunoassay, which determines complement-bound immune complexes, was used to determine the levels of such complexes in serial samples of sera from forty patients with PBC followed for 2 years. Twenty-four patients (60%) were found to have significantly elevated levels of circulating complexes. In the majority they were detected from the beginning of the study and the high levels persisted. In seven patients whose sera initially had normal levels of complexes, the levels increased to become abnormal during the following year. These complexes sedimented at greater than or equal to 19S in the majority of patients studied. The mean level of C3 but not C4 was lower in patients with elevated complexes than in those with normal complexes. A significant correlation was observed between the presence of elevated complexes and the severity of the inflammatory cell infiltrate surrounding intrahepatic portal tracts and serum IgG and IgM levels. There was also a significant correlation with titres of antimitochondrial antibody, but not with the histological stage of disease or with the collagen and copper content of the liver. Although the method of detection of immune complexes is indirect and the antigen is unknown, the presence of such high levels of complexes suggests a possible role of immune complexes in the pathogenesis of PBC.

Antibodies↗
PubMed1977

Pigmented corneal rings in non-Wilsonian liver disease.

Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet's membrane that have been deemed pathognomonic of Wilson's disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson's disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.

Ceruloplasmin↗
PubMed1977

Effect of D-penicillamine on copper retention in patients with primary billiary cirrhosis.

As part of a double blind, randomized trial evaluating D-penicillamine in primary biliary cirrhosis, we monitored urinary copper excretion and hepatic copper concentration during the 1st year of therapy in 46 patients with this disease. The retention of copper in primary biliary cirrhosis was confirmed by finding abnormally high levels of standard copper measurements in almost all patients before treatment. The hepatic copper was elevated in 43 of 45 patients, the urinary copper in 42 of 46, and the ceruloplasmin in 46 of 46. Urinary copper excretion correlated with the hepatic copper concentration (r = 0.68, P less than or equal to 0.001). No significant correlation occurred between hepatic copper and ceruloplasmin. Hepatic copper concentrations greater than 400 microng per g of dry weight were found almost exclusively in patients with advanced histological disease (P less than or equal to 0.01). Therapy with D-penicillamine and a low copper diet sustained increased urinary copper excretion for 1 year in almost all patients (P less than or equal to 0.001). Among patients taking placebo, the median hepatic copper concentration increased 13 microng per g of dry weight after 1 year. In contrast, among the patients taking D-penicillamine, the median hepatic copper concentration decreased 99 microng per g of dry weight (P less than or equal to 0.02). Continued observation of this therapeutic trial may help to clarify the relationship of copper retention and liver injury in primary biliary cirrhosis.

Ceruloplasmin↗
PubMed1976

Splenosis: autotransplantation of splenic tissue.

Splenosis is the autotransplantation of splenic tissue that usually follows traumatic rupture of the spleen. Generally, the splenic implants are numerous and are located within the peritoneal cavity; however, extra-abdominal splenosis does occur. The implants are rarely clinically significant and are incidental findings at autopsy or at abdominal operation, unrelated to and distant from the splenic trauma and splenectomy. Splenic implants retain their ability to function, and recurrence of a hematologic disease for which the spleen was previously removed should alter the clinician to the possibility of splenosis. The differential diagnosis includes accessory spleens, endometriosis, hemangiomas and metastatic cancer. We report three cases, two of which represent unusual complications of splenosis: a twisted pedicle of a splenic implant, which apparently caused abdominal pain, and recurrent Felty's syndrome associated with splenosis and an accessory spleen.

Adolescent↗
PubMed1976

Alpha1-antitrypsin phenotypes in chronic active liver disease and primary biliary cirrhosis.

Alpha1-antitrypsin concentrations and phenotypes were determined in groups of patients with chronic active liver disease and primary biliary cirrhosis. The concentrations of alpha1-antitrypsin were above normal values in both groups; the patients with primary biliary cirrhosis had higher concentrations than those with chronic active liver disease. The prevalence of common phenotypes in these two groups did not differ from that in a sample of healthy blood donors from this institution of from a large Norwegian sample. We interpret our data as disputing the view that alpha1-antitrypsin phenotypes, other than Z. significantly predispose adults to hepatic cirrhosis.

Adolescent↗
PubMed1976

Lymphocyte cytotoxicity and inhibition studied with autologous liver cells: observations in chronic active liver disease and the primary biliary cirrhosis syndrome.

A method is described for determining the cytotoxicity of normal and autologous lymphocytes for 51Cr-labeled isolated parenchymal liver cells in a low aggressor to target cell ratio. Results were compared from patients with chronic active liver disease (CALD), chronic persistent hepatitis (CPH), miscellaneous liver diseases, or primary biliary cirrhosis (PBC). In 53% of CALD patients, lymphocytes showed greater cytotoxicity for hepatic cells than did normal allogenic lymphocytes, but in 32% there was significantly less 51Cr release than normal; in the remainder, results were in the normal range. Lymphocyte cytotoxicity was greater in patients with disease of short duration and less in those treated with corticosteroids. In untreated CALD, decreased 51Cr release was associated with the presence of plasma factor(s) inhibiting phytohemagglutinin (PHA)-induced transformation of normal lymphocytes. Lymphocytes from approximately 50% of the patients with PBC exhibited cytotoxicity for hepatic cells but 25% showed less 51Cr release than controls and the remaining patients had results in the normal range. Lymphocyte cytotoxicity was also greater during the earlier stage of PBC. In contrast to CALD, decreased 51Cr release was not associated with the presence of plasma factor(s) inhibiting PHA-induced transformation of normal lymphocytes. Our findings support the hypothesis of in vivo lymphocyte-mediated liver cell damage in CALD and PBC, suggesting a potentially important role for lymphocyte suppression in the pathogenesis of both diseases.

Adult↗
PubMed1975

Pigmented corneal rings in a patient with primary biliary cirrhosis.

A patient with primary biliary cirrhosis who had high hepatic, serum, and urine copper levels was found by slit-lamp biomicroscopy to have pigmented corneal rings similar to Kayser-Fleischer rings. Wilson's disease was excluded on the basis of the elevated serum ceruloplasmin and copper levels, the lack of neurological disease despite advanced liver disease, a negative family history, and the typical clinical features of primary biliary cirrhosis. Although the exact chemical nature of the corneal rings is unknown, the cornea may be another tissue associated with elevated tissue copper levels in primary biliary cirrhosis.

Cornea↗