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Biomedical subjects

E R Dickson

Publications and source records attributed to E R Dickson.

At least 127 records · Page 7Linked to original sources
PubMed1984

Manifestations of nonsuppurative cholangitis in chronic hepatobiliary diseases: morphologic spectrum, clinical correlations and terminology.

The features of nonsuppurative cholangitis were studied in liver biopsy specimens from 185 patients with chronic active hepatitis (CAH), 280 patients with primary biliary cirrhosis (PBC), and 55 patients with primary sclerosing cholangitis (PSC). Specimens from patients with other liver diseases in which the presence of nonsuppurative cholangitis had been recorded were also studied. We identified four types of nonsuppurative cholangitis: granulomatous cholangitis, lymphoid cholangitis, fibrous cholangitis, and pleomorphic cholangitis. Granulomatous cholangitis almost always seemed to be destructive; the other types were either destructive or nondestructive. Granulomatous cholangitis was, for all practical purposes, diagnostic of PBC and the obliterative form of fibrous cholangitis was similarly diagnostic for the hepatic manifestations of PSC in adults and paucity of intrahepatic bile ducts in infants. All other types of cholangitis were found in CAH, PBC, PSC, and other liver diseases. Thus, the term "nonsuppurative cholangitis" describes a spectrum of morphologic lesions that differ in incidence, morphogenesis, usefulness for liver biopsy diagnosis, and, probably, pathogenesis.

Bile Ducts↗
PubMed1983

Primary biliary cirrhosis and lichen planus.

Lichen planus and primary biliary cirrhosis were seen in twenty-four patients. In seventeen patients, the cutaneous eruption followed the administration of D-penicillamine. In seven patients, lichen planus developed unrelated to therapy. Three of the latter group of patients were treated with D-penicillamine and had subsequent relapse or exacerbation of their preexisting lichen planus. The presence of lichen planus in patients with primary biliary cirrhosis and the propensity to develop this type of eruption while on D-penicillamine therapy are consistent with a graft-versus-host pathogenesis of primary biliary cirrhosis. Preexisting lichen planus should be regarded as a relative contraindication to the use of D-penicillamine in patients with primary biliary cirrhosis.

Clinical Trials as Topic↗
PubMed1983

Diagnosis of Wilson's disease presenting as fulminant hepatic failure.

The clinical course, results of standard laboratory tests, parameters of copper metabolism, and hepatic morphology in 9 cases (3 of our own and 6 from the literature) of Wilson's disease presenting as fulminant hepatic failure were compared with the findings in 5 cases of idiopathic fulminant hepatic failure. Patients with Wilson's disease were usually younger, and 7 of the 9 patients had Kayser-Fleischer rings. Patients with idiopathic fulminant hepatic failure had elevated 24-h urinary copper, decreased ceruloplasmin, and low or normal serum copper. Fulminant hepatic failure with Wilson's disease differed from idiopathic fulminant hepatic failure by the following biochemical findings: (a) higher copper levels in serum, urine and liver; (b) less pronounced elevations of transaminase levels; (c) higher concentrations of total bilirubin; and (d) lower hemoglobin values. Serum copper was the most useful biochemical test in diagnosing Wilson's disease before death. At autopsy, only hepatic copper concentrations clearly separated the two groups. Serial serum copper levels (antemortem) and quantitative analysis of hepatic copper (after recovery or postmortem) in patients with fulminant hepatic failure should help to exclude Wilson's disease.

Adolescent↗
PubMed1982

Immune complexes in primary biliary cirrhosis. Higher prevalence of circulating immune complexes in patients with associated autoimmune features.

A longitudinal study, examining the levels of immune complexes serially for three years, in serum from 88 patients with primary biliary cirrhosis was performed by the Raji cell radioimmunoassay. Studies of the association of autoimmune features in primary biliary cirrhosis and the effect of D-penicillamine therapy in relation to the levels of complexes were carried out. Twenty-two patients (25 percent) were found to have autoimmune features, such as Sjögren's syndrome, rheumatoid-like arthritis, scleroderma, Raynaud's disease, and Hashimoto's thyroiditis. In this subset of patients with primary biliary cirrhosis, a significantly higher prevalence (86 percent) of circulating immune complexes was detected compared with those patients showing no autoimmune features (60 percent). In addition, patients with associated autoimmune features had higher mean levels of immune complexes (259.7 micrograms AHG eq/ml) compared with those without autoimmune features (202.1 micrograms AHG eq/ml). The mean levels of complement C4, reflecting activation of classic complement pathway, were significantly lower in patients with elevated immune complexes and associated autoimmune features. The mean level of immune complexes in 13 patients receiving D-penicillamine, in contrast to the placebo group, decreased at one year but subsequently was greater than the initial level. Patients who had normal levels of immune complexes and received penicillamine therapy continued to have complex levels within the normal range for up to three years of follow-up study, but patients receiving placebo showed significantly elevated levels at subsequent intervals. Thus, levels of immune complexes in primary biliary cirrhosis may reflect the association with autoimmune features.

Antigen-Antibody Complex↗
PubMed1982

Autoimmune associations in primary biliary cirrhosis.

The prevalence of autoimmune associations was determined in 113 patients with primary biliary cirrhosis who participated in a therapeutic trial of D-penicillamine. Eighty-four percent of the patients had at least one associated autoimmune disease and 41% had two or more such diseases in addition to primary biliary cirrhosis. Keratoconjunctivitis sicca, which was present in 66% of the patients, was the most commonly associated autoimmune disease. In most of the patients the autoimmune disease was recognized after the diagnosis of primary biliary cirrhosis had been made. The prevalence of autoantibodies in selected subgroups of patients ranged from 70% (rheumatoid factor) to 22% (anti-native DNA). Polyclonal elevation of serum immunoglobulins was a consistent finding, and frequently all three major isotypes were simultaneously increased. Only IgA elevations correlated with histologic progression of primary biliary cirrhosis. The prominent involvement of epithelial tissues in the autoimmune disease of primary biliary cirrhosis suggests that an autoimmune syndrome affecting the secretory immune system is associated with the pathogenesis of primary biliary cirrhosis and the coexisting autoimmune diseases.

Adult↗
PubMed1981

Nutritional status of a group of alcoholics before and after admission to an alcoholism treatment unit.

The nutritional status of the alcoholic has been presumed to be less than adequate because of the large quantity of alcohol ingested. Most reports have focused on the derelict alcoholic. In contrast, the patients in this investigation are more representative of the usual alcoholic population. The pretreatment dietary histories of 58 alcoholics were compared with their actual dietary intake while they were hospitalized for the treatment of alcoholism. Comparisons were also made between dietary intake by history and Recommended Dietary Allowances. The mean calorie, protein, fat, and carbohydrate intake of the patients was adequate and was well within the Recommended Dietary Allowances ranges. After the patients stopped drinking, they increased their intake of all major nutrients, especially carbohydrates. A subgroup of 11 patients whose recent dietary intake by history was corroborated by their urinary nitrogen excretion had no change in total caloric intake after they stopped drinking.

Adolescent↗
PubMed1981

Lichen planus-like lesions caused by penicillamine in primary biliary cirrhosis.

Six patients with primary biliary cirrhosis experienced lichen planus-like lesions while taking penicillamine. Oral lesions developed in all patients, and the lichenoid lesions also appeared elsewhere in two patients. Lichen planus-like lesions developed from four to 16 months after penicillamine therapy had been started, and, in four cases, they resolved in one to three months after use of the drug was stopped. Two patients have continued using penicillamine despite adverse cutaneous and mucosal reactions and have continued to experience lichen planus-like lesions intermittently. Other cutaneous reactions associated with penicillamine are reviewed.

Clinical Trials as Topic↗
PubMed1980

Treatment of pruritus of cholestasis by plasma perfusion through USP-charcoal-coated glass beads.

The accumulation of bile acids has been implicated in the pathogenesis of pruritus of cholestasis. To study the effect of bile-acid depletion on pruritus, 8 patients with chronic cholestasis and intractable pruritus which had not responded to cholestyramine and/or phenobarbitone underwent plasma perfusion through USP-charcoal-coated glass beads. DSuring twenty-two perfusions, between 957 and 6100 ml of plasma were perfused through charcoal columns, resulting in the removal of 126-795 mumol of bile acids. The mean extraction of the perfused bile acids was 81%. After the perfusions, all patients had prompt relief of their pruritus, lasting from 24 h to 5 months. Although the clinical effect may be due to the removal of unidentified pruritogens other than bile acids, charcoal plasma perfusion may improve the quality of life of incapacitated patients with servere intractable pruritus of cholestasis.

Adult↗
PubMed1980

Preciitating antibodies to mitochondrial antigens in patients with primary biliary cirrhosis.

Sera of patients with primary biliary cirrhosis (PBC) were examined for the presence of precipitating antibodies to sonicated rat liver mitochondrial (M) fraction. Three distinct precipitating systems observed in double immunodiffusion were identified and called M-A, M-B and M-C. Unsonicated mitochondria did not form precipitin lines. Precipitating system M-A was found in nineteen of twenty (95 percent) sera from PBC. The mitochondrial antigen of M-A system had the unusual property of being resistant to enzymatic digestion with deoxyribonuclease (DNase), ribonuclease (RNase) and trypsin under standard conditions. The titres of antibody to M-A antigen correlated (P less than 0.05) with titres of mitochondrial immunofluorescence staining on unfixed mouse kidney sections. Precipitating systems M-B and M-C were present in seven of twenty ribonuclease and trypsin but resistant to ribonuclease indicating that it could be DNA-protein complex. The M-C antigen was destroyed by trypsin suggesting its protein character, but it was difficult to determine if nucleic acids might also be associated with antigenicity. The antibodies to mitochondrial antigens were not present in normals (fifteen health adults), systemic lupus erythematosus (forty patients), rheumatoid arthritis (fifteen patients) and chronic liver diseases (fifteen patients). The antibodies did not show identity with antibodies to ribosomal ribonucleo-protein and other known nuclear antigens previously reported. The data confirm previous reports concerning the heterogeneity of mitochondrial antibodies present in sera of patients with PBC. The antibody to M-A antigen appeared to be a diagnostically useful immunological marker since it was present in the majority of patients with PBC.

Adult↗
PubMed1980

Glucose-6-phosphate dehydrogenase variants: reexamination of G6PD Chicago and Cornell and a new variant (G6PD Pea Ridge) resembling G6PD Chicago.

Two large and unrelated families were investigated for hereditary nonspherocytic hemolytic anemia associated with deficiency of erythrocyte glucose-6-phosphate dehydrogenase (G6PD). In both families, the kinetic and electrophoretic features of the G6PD variants resembled those of G6PD Chicago. Further investigation revealed that members of one of these families previously had been characterized as having the G6PD variants Chicago and Cornell. However, it is clear that each of these terms has been applied to the same variant in this single large kindred. In the second family, we describe a newly identified variant with unique characteristics, which we have designated G6PD Pea Ridge. G6PD Pea Ridge resembles G6PD Chicago but differs in electrophoretic mobility and in a few kinetic parameters. It exhibits an unusually high Ki for NADPH and thus appears to be insensitive to product inhibition. As other cases previously considered to be the Chicago variant become more fully characterized, this probably will be shown to be a heterogeneous group of variants.

Adult↗
PubMed1980

Plasmaperfusion and studies of copper metabolism in benign recurrent cholestasis: novel procedures in a rare syndrome.

A 21-year-old woman with recurrent epidsodes of pruritus and jaundice since childhood was diagnosed as having benign recurrent cholestasis, a rare and poorly understood disease. Plasmaperfusion was successfully employed for therapy of the severe pruritus, and various studies of copper metabolism were performed in an attempt to clarify the pathophysiology of this obscure syndrome.

Adult↗
PubMed1980

Depletion of bile acids and bilirubin in dogs with biliary obstruction by plasmaperfusion of USP-charcoal-coated glass beads.

Patients with severe, inoperable cholestasis and intractable pruritus not responding to conventional medical therapy might benefit from a depletion of their bile acid pool by sorbent perfusion since accumulated bile acids are possible responsible for their itching. In vitro, USP-charcoal-coated glass beads removed bile acids from human plasma far better than any other sorbent tested. In order to demonstrate the safety and efficacy and charcoal plasmaperfusion in vivo, five dogs underwent plasmapheresis one week following cholecystectomy and bile duct ligation, and 2.2 +/- 0.2 (mean +/- SD) times their plasma volume was passed over a column containing 400 ml of charcoal-coated glass beads prior to reinfusion. During the procedure the plasma bile acid concentration was reduced by 40.2 +/- 4.0% and the bilirubin level by 48.2 +/- 3.3%. The columns, whose capacity was far from being saturated, retained 1.3 +/- 0.4 times the pre-perfusion plasma pool of bile acids (1.1 +/- 0.2 for bilirubin) suggesting that substantial amounts of bile acids and bilirubin were mobilized from tissue stores. The procedure was well tolerated by the animals and might have promising clinical applications.

Animals↗
PubMed1979

Removal of bile acids and bilirubin by plasmaperfusion of U.S.P. charcoal-coated glass beads.

Removal of protein-bound cholephilic substances such as BA by sorbent perfusion might improve the quality of life of patients with severe inoperable cholestasis where the accumulation of BA is thought to be responsible for the often distressing pruritus. Since hemoperfusion is associated with hematological side effects, plasmaperfusion which, in addition, allows the use of novel, possibly more efficient sorbents, might be preferable, provided a substance with a high affinity for these protein-bound anions could be found. A number of sorbents suitable for plasma-perfusion were therefore tested in vitro as to their BA-adsorbing capacity. U.S.P. charcoal coated onto glass beads in order to facilitate perfusion removed BA from human plasma far better than conventional charcoal suitable for hemoperfusion, charged and uncharged resins, and immobilized albumin. The extraction of BA by the coated glass beads exceeded 90% up to a load of 0.18 mumol BA per milliliter of sorbent, and the BA-adsorbing capacity was 20.8 mumol/ml. In addition to BA, the U.S.P. charcoal columns almost quantitatively removed uric acid, creatinine, and corticosteroids and retained substantial amounts of BR and thyroxine, whereas electrolytes, clotting factors, and plasmaproteins were adsorbed only initially.

Adsorption↗
PubMed1979

Chronic active liver disease with common variable hypogammaglobulinemia.

Progressive liver injury in chronic active liver disease is usually associated with elevation of serum immunoglobulin levels. However, the role of immunoglobulins in the pathogenesis of this disease is still obscure. We report here the case of a 41-year-old man with hypogammaglobulinemia since at least 1964 in whom chronic active liver disease later developed. From 1954 he had had frequent respiratory tract infections, and these continued, along with diarrhea, despite regular gamma-globulin therapy. Studies in 1969 showed absent serum IgA and IgM and an abnormally low level of IgG. In 1974, liver enzyme abnormalities were recorded and a diagnosis of chronic active liver disease was made. A liver biopsy showed cirrhosis with active hepatitis. Lymphocyte function studies revealed that the T cells suppressed B-cell maturation and production of immunoglobulins. He was treated with azathioprine and prednisone, and this therapy has been associated with a decrease in both the elevation in liver enzymes and the frequency of infections. This case suggests that liver cell injury in chronic active liver disease is independent of the elevation of immunoglobulins and that immunosuppressive therapy may be well tolerated by patients with hypogammaglobulinemia.

Adult↗