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Biomedical subjects

A H Baggenstoss

Publications and source records attributed to A H Baggenstoss.

At least 19 recordsLinked to original sources
PubMed1983

Autoimmune features as determinants of prognosis in steroid-treated chronic active hepatitis of uncertain etiology.

To determine if autoimmune features influence the prognosis of severe sporadic chronic active hepatitis of uncertain etiology, 126 corticosteroid-treated patients who had been screened to exclude active hepatitis B infection and possible drug effects or transfusion-related disease were categorized according to the presence of the lupus erythematosus cell, antinuclear antibody, smooth muscle antibody, and coexistent autoimmune diseases. Patients with autoimmune markers had findings similar to those without these features and responses to therapy were comparable. Remission (77% vs. 68%), treatment failure (16% vs. 14%), progression to cirrhosis (31% vs. 39%), and death from hepatic failure (4% vs. 12%) occurred with similar frequency in patients with and without the lupus erythematosus cell or antinuclear antibody. Although patients with the lupus erythematosus cell or antinuclear antibody tended to relapse less frequently than others, 5-yr survival was not increased (92% vs. 87%). Autoimmune diseases occurred as commonly in patients without the lupus erythematosus cell or antinuclear antibody as in those with these findings (16% vs. 17%), and the presence of these conditions did not identify patients with a different clinical behavior. In severe sporadic chronic active hepatitis of uncertain etiology, a clinically and prognostically distinct subgroup reflective of a possible autoimmune disorder cannot be defined.

Adolescent↗
PubMed1983

Hemochromatosis: genetic or alcohol-induced?

To evaluate the roles of alcohol and genetic factors in hepatic iron overload, we studied prospectively 61 patients selected solely on the basis of increased stainable hepatic iron (grade 3 or 4). Independent comparisons were made between alcoholic (n = 20) and nonalcoholic (n = 41) patients, and between patients wih affected relatives (n = 25) and those without (n = 36). For the entire group, the mean value for mobilizable iron was 19.6 g and the prevalence of HLA-A3 was 69.6%, both findings compatible with genetic hemochromatosis. Subgroups were no different in clinical features (diabetes, pigmentation, cardiomyopathy, hypogonadism, or arthropaty), histologic findings (fat, inflammation, fibrosis), indexes of iron metabolism (serum iron, transferrin saturation, chelatable iron, and mobilizable iron stores), or frequency of HLA-A3 and HLA-B7. The only exception was that mean hepatic iron concentration was lower in alcoholic patients than in nonalcoholic patients (17,344 vs. 28,553 micrograms/g dry wt, p less than 0.001). Similarity between subgroups in almost all parameters examined is consistent with the hypothesis that heavy deposition of hepatic iron, as observed in our patients, is an indication of genetic hemochromatosis, regardless of alcohol consumption or the findings of affected relatives. The lower concentrations of hepatic iron in alcoholic patients, despite equal body stores in both groups, suggest that alcohol may alter the distribution of storage iron in genetic hemochromatosis.

Adult↗
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

Prognostic and therapeutic implications of extreme serum aminotransferase elevation in chronic active hepatitis.

Extreme elevation of the serum aspartate aminotransferase level typically suggests acute hepatocellular necrosis and may militate against the diagnosis of chronic active hepatitis. However, we found that 26 of 160 patients (16%) with chronic active hepatitis had aminotransferase elevations of more than 1,000 IU/liter. These patients were younger and more often jaundiced than the others, but they exhibited signs of chronic liver disease as often. In only 2 of 26 patients with extreme aminotransferase abnormality were features of chronic disease absent. Patients with extreme enzyme elevation had histologic findings of confluent necrosis (P greater than 0.005) and features associated with acute viral infection (P greater than 0.005) more often than others, but they as often had cirrhosis on biopsy specimens. Virologic markers did not distinguish the patients or correlate with viral features in liver tissue. Corticosteroids improved immediate survival (P greater than 0.005) and the likelihood of remission (P greater than 0.005). Although chronic active hepatitis may present with extreme aminotransferase elevation and histologic features associated with acute viral infection, ancillary features of chronic disease facilitate the correct diagnosis and the initiation of appropriate therapy.

Adult↗
PubMed1981

Corticosteroid-treated chronic active hepatitis in remission: uncertain prognosis of chronic persistent hepatitis.

To assess the prognosis of patients with severe chronic hepatitis after histologic examination had shown an improvement to chronic persistent hepatitis, we followed 52 such patients regularly for 54 +/- 4 months after the cessation of corticosteroid therapy. In 24 patients, the condition deteriorated 7 +/- 1 months after therapy and required further treatment with prednisone. Histologic features of chronic active hepatitis, including bridging and multilobular necrosis, were documented in all 14 patients in whom biopsies were performed. In 20 of 24 patients, the disease responded to retreatment, but 13 again had relapses, and cirrhosis developed in two. Of 28 patients who remained asymptomatic for 48 +/- 6 months, 17 retained features of chronic persistent hepatitis, and nine had improvement to normal histologic features. Cirrhosis developed in two patients without clinical manifestations of active inflammation. Findings before and after treatment did not predict outcome. We conclude that severe chronic active hepatitis that has been treated with prednisone and converted to chronic persistent hepatitis will often and unpredictably deteriorate after treatment has been stopped. Cirrhosis develops rarely but may occur with or without clinically overt chronic active hepatitis.

Adolescent↗
PubMed1981

Laboratory assessment of severe chronic active liver disease during and after corticosteroid therapy: correlation of serum transaminase and gamma globulin levels with histologic features.

Serum glutamic oxaloacetic transaminase and gamma globulin levels were correlated with morphologic findings during and after corticosteroid therapy of severe chronic active liver disease to assess ability to monitor histologic change. Morphologic features were interpreted by a single observer from 441 coded biopsy specimens obtained from 69 patients. Serum glutamic oxaloacetic transaminase values were more predictive of histologic findings than gamma globulin levels (p less than 0.02), correctly reflecting morphologic status in 67% of instances during treatment and 80% after treatment. Abnormalities in either test identified chronic active liver disease with similar accuracy, but serum glutamic oxaloacetic transaminase determinations were more sensitive to active inflammation. Serum glutamic oxaloacetic transaminase elevations of more than twice normal during and after therapy were reliably associated with chronic active liver disease. Normal laboratory studies, however, did not detect chronic active liver disease in 55% of cases during treatment and 19% after therapy. The majority of lesions undetected by laboratory investigations comprised periportal necrosis while confluent necrosis was rarely missed. We conclude that serum glutamic oxaloacetic transaminase determinations provide a useful mechanism for monitoring chronic active liver disease. During therapy, serum glutamic oxaloacetic transaminase levels reliably discount resolution, but do not predict histologic inactivity. After treatment, laboratory surveillance inconsistently detects periportal necrosis, but rarely misses severe inflammation of potentially greater prognostic significance.

Adrenal Cortex Hormones↗
PubMed1980

Clinical assessment of cirrhosis in severe chronic active liver disease: specificity and sensitivity of physical and laboratory findings.

The usefulness of certain physical and laboratory findings in predicting the morphologic diagnosis of cirrhosis in severe chronic active liver disease was determined in 101 patients, 39 of whom had cirrhosis. Hypoalbuminemia (69%) and hypergammaglobulinemia (67%) were the most common findings in cirrhosis, but they lacked specificity. Thrombocytopenia, hepatic encephalopathy, and ascites, which were the most specific and sensitive abnormalities, implicated cirrhosis with 85% assurance when present and occurred in the majority of patients with cirrhosis (56%). No single feature was pathognomonic of cirrhosis, although absence of all clinical findings excluded the diagnosis; Eight or more clinical abnormalities were invariably associated with cirrhosis, but only 18% of patients had such florid manifestations. The presence of at least five abnormalities was associated with a 76% likelihood of cirrhosis and was encountered in 56% of cirrhotic patients. Patients with three or fewer findings seldom had cirrhosis (24%), and only the presence of ascites reliably implicated the lesion in these patients. We conclude that in severe chronic active liver disease, recognition of certain specific findings or a constellation of abnormalities permits confident identification of cirrhosis in the majority of patients with this disorder.

Adolescent↗
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

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

Radical pancreatoduodenectomy for cancer of the papilla of Vater.

Over a 22-year span, 87 patients with carcinoma of the papilla of Vater underwent radical pancreatoduodenectomy. No patient was lost to follow-up, and extended observation was possible in most cases: the definitive operation was at least five years earlier than this study in 87% and at least ten years earlier in 73%. Operative mortality was 11.5% among patients who had a single definitive operative procedure and 15.4% among those whose treatment involved reoperation after prior exploration elsewhere. Overall survival rates at two, five, and ten years were 56%, 34%, and 20% respectively. Factors associated with favorable survival were histologic differentiation (Broders grades 1 and 2), absence of nodal metastasis, and papillary histologic characteristics. Noteworthy is the fact that no patient having resection of an undifferentiated carcinoma (Broders grade 3 or 4) survived four years.

Adenocarcinoma, Papillary↗
PubMed1977

Severe chronic active liver disease. Prognostic significance of initial morphologic patterns.

To determine the usefulness of recognizing the different morphologic patterns of chronic active liver disease (CALD), we compared clinical and biochemical features as well as responses to treatment in 32 patients with chronic active hepatitis (CAH); 36 with subacute hepatitis and bridging necrosis (SHB); 30 with subacute hepatitis and multilobular necrosis (SHMN); and 30 with cirrhosis and active hepatitis (Cirrh). The morphological lesions did not correlate with clinical or etiologic features. Patients with CAH had less severe biochemical abnormalities, entered remission more often, and failed to respond to treatment less frequently than those with SHMN or Cirrh. SHB and SHMN resembled each other in many regards and showed greater functional changes than CAH. Cirrhosis developed more often after SHMN than CAH and was associated with a poorer prognosis than CAH. Serial liver biopsies revealed all possible histologic transitions, with reduction of inflammation usually occurring in patients treated with steroids and extension of inflammation being more frequent in those not receiving these drugs. CAH, SHB, SHMN, and Cirrh, therefore, reflect the degree and extent of disease activity at any given time in CALD, rather than representing different conditions. Identification of the initial morphologic lesion is helpful because of differences in prognosis.

Adult↗
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

Responses to treatment can differentiate chronic active liver disease with cholangitic features from the primary biliary cirrhosis syndrome.

Of 125 patients fulfilling preestablished criteria for severe chronic active liver disease (CALD) and enrolled in a prospective trial of treatment, 15 (12%) presented with morphological features of liver biopsy consistent with the diagnosis of both CALD and primary biliary cirrhosis (PBC) syndrome. Customary clinical, biochemical, and immunoserological studies failed to distinguish fully between these conditions. By contrast, early response to treatment with prednisone and/or azathioprine identified two different groups of patients. Five patients failed to respond, whereas 10 improved and this was followed by resolution of all clinical, biochemical, and morphological evidence of disease activity. Analysis of the initial chemical findings and cumulative bile duct counts from multiple biopsies correlated failure to respond with biochemical and morphological features more consistent with PBC than CALD. Responses to treatment can therefore be utilized when indicated for differentiating CALD with cholangitic features from PBC.

Adolescent↗
PubMed1975

Liver dysfunction and histologic abnormalities in neonatal hypopituitarism.

Pituitary aplasia or hypoplasia occurs in anencephalic and normocephalic infants. Two infants with hypopituitarism are described; in each the diagnosis was made clinically. The infants had frank clinical and histologic evidence of the neonatal hepatitis syndrome. There may be more than a chance relationship between the clinicopathologic findings of neonatal hypopituitarism and hepatitis.

Growth Hormone↗
PubMed1975

Cancer of the bile ducts associated with ulcerative colitis.

Thirteen patients with bile duct cancer (excluding gallbladder) and associated chronic ulcerative colitis (CUC) were seen at the Mayo Clinic from 1935 through 1973. Most patients had initial symptoms of severe diarrhea and bleeding, followed by a pattern of mild-to-moderate disease with exacerbations and remissions. Three patients had especially severe symptoms and underwent total colectomy (1 patient) or proctocolectomy (2 patients) an average of 15.7 years from onset of CUC symptoms. Anorexia, followed rapidly by the development of progressive jaundice (or a sudden deterioration when liver disease was already present), marked the onset of symptoms of bile duct cancer in the 13 patients. The overall mean duration from onset of CUC to development of symptoms of bile duct cancer was 19 years. The patients in whom colitis was managed by proctocolectomy or total abdominal colectomy developed symptoms of bile duct cancer an average of 9.4 years after colectomy. When cancer was diagnosed, the tumor had spread beyond the bile ducts in 10 patients. The tumors were difficult to identify and often infiltrated the hepatic hilus. The present series and review of the literature suggest that the relationship between CUC and bile duct cancer is more than a chance occurrence. The carcinoma has an onset approximately 3 decades earlier than does carcinoma of the bile ducts without CUC. Surgical removal of the diseased colon and mode of medical management of the unresected colon have no relationship to the subsequent development of carcinoma of the bile ducts; neither does the extent or severity of the colonic disease. The prognosis of carcinoma of the bile ducts unfortunately continues to be dismal.

Adenocarcinoma↗
PubMed1975

Prednisone for chronic active liver disease: dose titration, standard dose, and combination with azathioprine compared.

Among 120 consecutive patients with chronic active liver disease (CALD) randomized to different treatments, those receiving maintenance doses of prednisone 20 mg daily (Pred), prednisone in doses given on alternate days and titrated to secure resolution of clinical and biochemical abnormalities (Pred-Titrad), or a combination of prednisone 10 mg and azathioprine 50 mg daily (Comb) survived and underwent resolution of clinical and biochemical features of disease more often than a control group receiving placebo or azathioprine 100 mg daily. Histological remission occurred significantly more often with Pred and Comb than with other regimens. Major side-effects of therapy were commoner with Pred than with Comb or Pred-Titrad, which did not differ. We conclude that Comb is the initial treatment of choice for CALD, since clinical, biochemical, and histological resolution of disease activity occurs as often as with Pred, whereas early side-effects are significantly less frequent.

Adolescent↗
PubMed1975

Influence of primary bile acid feeding on cholesterol metabolism and hepatic function in the rhesus monkey.

In three healthy rhesus monkeys fed chenodeoxycholic (chenic) acid, there was no consistent increase in the total exchangeable cholesterol pool or input to the cholesterol pool. In three similar monkeys fed cholic acid, the total exchangeable pool increased in all animals and input to the cholesterol pool increased in two. Serum glutamic-pyruvic transaminase (SGPT) increased transiently in two animals in each group. Morphologic abnormalities (triaditis with atypical ductular proliferation) were noted in one animal; this animal was ingesting chenic acid but had normal liver test results at the time of biopsy. Biliary bile acids contained 8 to 14 percent lithocholic acid in the chenic acid group and 48 to 72 percent deoxycholic acid in the cholic acid group.

Alanine Transaminase↗