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Biomedical subjects

K D Lindor

Publications and source records attributed to K D Lindor.

At least 109 records · Page 6Linked to original sources
PubMed1995

Antimitochondrial antibody-negative primary biliary cirrhosis.

OBJECTIVES: We reviewed our experience with patients who had biochemical and histological features of primary biliary cirrhosis in the absence of antimitochondrial antibodies (AMA) to better understand this variant of the syndrome. METHODS: During the period between 1976 to 1992, 597 patients with clinical and histological features of primary biliary cirrhosis were seen at the Mayo Clinic. Thirty-five (5.8%) of these patients were negative for antimitochondrial antibody and had normal cholangiographic studies. The records of these patients were reviewed for this study. RESULTS: No difference was found between the two groups with respect to age, gender, or biochemical features. IgM and gamma-globulin levels were higher in the antimitochondrial antibody-positive than the antimitochondrial antibody-negative patients. What is more important, 96% of the AMA-negative patients who could be tested were positive for antinuclear antibody or anti-smooth-muscle antibodies. These tests were positive in only 56% of the antimitochondrial antibody-positive group (p < 0.05). The response of five of these patients to ursodeoxycholic acid appeared comparable to the response seen in antimitochondrial antibody-positive patients. CONCLUSIONS: Patients with histological features of primary biliary cirrhosis, whether antimitochondrial antibody positive or negative, are quite comparable with respect to clinical and biochemical features. Other autoantibodies, such as antinuclear or anti-smooth-muscle antibodies, are more common in the antimitochondrial antibody-negative group. These two conditions might be part of a spectrum that has been termed "autoimmune cholangitis" and that is characterized by chronic cholestasis, histological features of chronic nonsuppurative destructive cholangitis, and the presence of any of a variety of serum autoantibodies.

Antibodies, Antinuclear↗
PubMed1994

The role of portosystemic shunts for variceal bleeding in the liver transplantation era.

OBJECTIVE: To review our experience with portosystemic shunts during the era of liver transplantation at the Mayo Clinic to provide insight into the selection of patients for these procedures. DESIGN: We reviewed the charts of a cohort of 57 patients who underwent portosystemic shunting between 1985 and 1990 for the management of variceal bleeding. A follow-up survey by letter and telephone was also conducted. SETTING: The Mayo Clinic, a tertiary referral center. PATIENTS: These patients were not considered transplantation candidates at the time of the shunt because of active alcoholism, extensive portal vein thrombosis, coexistent myelodysplastic syndromes, or malignant neoplasms. INTERVENTION: Portosystemic shunts; the exact type was at the surgeon's discretion. OUTCOME: Survival after shunt surgery among patients with various liver diseases. RESULTS: Twenty-two patients died during follow-up after being shunted for bleeding, and one of the shunted patients subsequently required liver transplantation. Comparison of Kaplan-Meier survival curves between various groups of patients indicated that patients who were actively alcoholic had a poorer chance of survival (P < .003) than did those who were abstinent. Patients with portal vein obstruction or chronic cholestatic liver disease appeared to do better after shunt surgery than did patients with other causes of portal hypertension. Other factors such as age, Child-Pugh score, or presence of malignant neoplasms did not reliably predict outcome from portosystemic shunts. CONCLUSIONS: In well-selected patients who may not be deemed candidates for liver transplantation, portacaval shunts can be effectively employed to prevent bleeding from esophageal varices that are resistant to obliteration by sclerotherapy.

Cause of Death↗
PubMed1994

Ursodeoxycholic acid in the treatment of primary biliary cirrhosis.

BACKGROUND/AIMS: A double-blind, placebo-controlled trial of ursodeoxycholic acid (UDCA) was conducted in 180 patients with primary biliary cirrhosis (PBC) to define the efficacy and safety of UDCA. Efficacy was assessed by time to treatment failure defined as death; liver transplantation; histological progression; development of varices, ascites, or encephalopathy; doubling of total serum bilirubin levels; progression of fatigue or pruritus; drug toxicity; or voluntary withdrawal. METHODS: Patients with well-defined PBC underwent complete history, physical examination, liver chemistries, ultrasonography, upper endoscopy, and liver biopsy at entry as well as at 2 years. Liver chemistries were determined every 3 months. RESULTS: In patients receiving UDCA, treatment failure was delayed compared with the placebo-treated group (P = 0.0003, log rank test). Seven patients receiving UDCA died or required transplantation compared with 12 in the placebo group (P = 0.18). No patients discontinued UDCA because of side effects of toxicity. CONCLUSIONS: UDCA was extraordinarily safe and well tolerated, and its use was associated with delayed progression of the disease as defined in this study. However, the lack of effects on symptoms, histology, and the need for liver transplantation or survival indicate that further evaluation is necessary to determine the ultimate role of UDCA in the treatment of PBC.

Adult↗
PubMed1994

Effect of ursodeoxycholic acid on serum lipids of patients with primary biliary cirrhosis.

OBJECTIVE: To study the effects of ursodeoxycholic acid (UDCA) on lipid levels in patients with primary biliary cirrhosis (PBC). DESIGN: A randomized, placebo-controlled prospective trial of UDCA was conducted in 177 well-characterized patients with PBC. MATERIAL AND METHODS: The two treatment groups (placebo and UDCA) were matched at entry with respect to age, sex, histologic stage of PBC, biochemical values, and serum lipid levels. Serum cholesterol, high-density lipoprotein cholesterol, and triglyceride levels were measured at entry, 1 year, and 2 years. RESULTS: The decrease in total cholesterol level at 1 and 2 years in the UDCA-treated group was significant in comparison with that in the placebo group. Serum high-density lipoprotein cholesterol and triglyceride levels, however, were unchanged. No severe side effects from UDCA were noted. The changes in serum cholesterol levels at 2 years were directly and strongly correlated with changes in serum bilirubin concentrations (r = 0.70; P < 0.001) and inversely correlated with initial serum cholesterol levels (r = -0.86; P < 0.00001). CONCLUSION: The cholesterol-lowering effect of UDCA could be related to amelioration of the underlying liver disease or to a direct effect of the drug on the metabolism of cholesterol in patients with hypercholesterolemia.

Adult↗
PubMed1994

The natural history of abdominal pain associated with primary biliary cirrhosis.

OBJECTIVES: The aim of the present study was to determine the prevalence and natural history of abdominal pain in patients with primary biliary cirrhosis. METHODS: We studied 178 patients with well-defined primary biliary cirrhosis enrolled in a prospective randomized trial of ursodeoxycholic acid. These patients underwent upper endoscopy and upper abdominal ultrasound prior to entry, at 2 yr, and as indicated. Fourteen patients had additional evaluations including abdominal CT (four), colon x-ray (five), colonoscopy (three), endoscopic retrograde cholangiopancreatography (two), and upper gastrointestinal x-ray (two). RESULTS: Patients with abdominal pain generally presented with right upper quadrant discomfort. Thirty-one patients (17%) had pain at study entry: 33% of these had pain persisting at 1 yr, and 20% of these had pain persisting at 2 yr. The resolution of pain was not clearly affected by ursodeoxycholic acid. Evaluation with ultrasound and upper endoscopy found four patients with asymptomatic cholelithiasis, one with esophageal erosions, four with gastric erosions, one with a gastric ulcer, and two with duodenal erosions. Additional tests were unrevealing in 14 patients. Patients with pain were similar to patients without pain with regard to age, histological stage, gender, and liver biochemistries. CONCLUSIONS: We conclude that chronic right upper quadrant pain is not uncommon in patients with primary biliary cirrhosis, that it usually resolves spontaneously, and that upper endoscopy is the most important diagnostic test to use to exclude treatable causes of pain.

Abdominal Pain↗
PubMed1994

Methionine-enkephalin concentrations correlate with stage of disease but not pruritus in patients with primary biliary cirrhosis.

OBJECTIVE: Opiate antagonist agents ameliorate pruritus in some patients with cholestatic liver disease, suggesting that endogenous opioids may mediate pruritus in cholestasis. However, the endogenous opioids potentially responsible for pruritus remain unknown. Methionine-enkephalin is an endogenous opioid synthesized in numerous tissues including the gastrointestinal tract, and released into the portal circulation. Serum methionine-enkephalin concentrations are elevated in patients with chronic liver disease, making this opioid a candidate for opioid-mediated pruritus in cholestatic liver disease. The aim of our study was to determine whether elevated circulating methionine-enkephalin concentrations are associated with pruritus. METHODS: Serum concentrations of methionine-enkephalin were measured in 54 consecutive, untreated patients with primary biliary cirrhosis and 20 healthy controls. There was a direct correlation between methionine-enkephalin concentrations and total serum bilirubin (r = 0.42, p < 0.05) and the Mayo risk score (r = 0.5, p < 0.005). Methionine-enkephalin concentrations were significantly higher in histological stages 3 and 4 (48 +/- 4 pg/ml, n = 44), compared with stages 1 and 2 (29 +/- 12 pg/ml, n = 10, p < 0.05). Methionine-enkephalin concentrations in both histological groups were significantly higher than those in 20 healthy controls (16 +/- 4 pg/ml, p < 0.005). RESULTS: There was no significant correlation between methionine-enkephalin concentrations and the presence of pruritus (46 +/- 25 pg/ml with pruritus vs. 40 +/- 22 pg/ml without pruritus, n = 20). CONCLUSIONS: Increased serum methionine-enkephalin concentrations in patients with primary biliary cirrhosis were not associated with pruritus.

Bilirubin↗
PubMed1994

Hepatic osteodystrophy in primary biliary cirrhosis: effects of medical treatment.

OBJECTIVES: Osteoporosis is a frequent extrahepatic complication of primary biliary cirrhosis. Although histologically similar to the osteoporosis commonly seen in postmenopausal females, the pathogenesis and management of bone disease in patients with primary biliary cirrhosis is poorly understood. The experience with a subgroup of patients with primary biliary cirrhosis treated with vitamin D, calcium, and estrogen supplementation was reviewed to determine the effects of medical treatment on hepatic osteodystrophy. METHODS: The records of 203 women with the diagnosis of primary biliary cirrhosis were reviewed retrospectively for lumbar spine bone mineral density, menopausal status, and supplementation with vitamin D, calcium, and estrogen. RESULTS: The 16 postmenopausal patients treated with estrogen replacement had a statistically significant increase in the lumbar spine bone mineral density at 1 yr (+0.014 +/- 0.049 vs. -0.03 +/- 0.046 g/cm2, p < 0.038), without a significant change in the serum bilirubin or alkaline phosphatase. In treated patients, vitamin D and calcium supplementation did not lead to significant improvement in lumbar spine bone mineral density. CONCLUSIONS: Calcium and vitamin D supplementation, even in the presence of vitamin D deficiency, do not improve lumbar spine bone mineral density in patients with primary biliary cirrhosis. Estrogen replacement in postmenopausal patients, however, does appear to improve lumbar spine bone mineral density without increasing clinical or biochemical cholestasis, a potential complication reported in animal studies. This study should serve as an impetus for a controlled trial of estrogen replacement in postmenopausal patients with primary biliary cirrhosis.

Adult↗
PubMed1993

Outcome of patients hospitalized for complications after outpatient liver biopsy.

OBJECTIVE: To determine the safety of outpatient liver biopsies by analyzing the outcome of patients hospitalized for complications. DESIGN: Retrospective review. SETTING: Large clinic referral center. PATIENTS: All patients admitted after outpatient liver biopsy at the Mayo Clinic from 1 April 1989 to 1 April 1991. RESULTS: During this period, 405 outpatients underwent biopsy. Of the 405 patients, 13 (3.2%) were admitted with complications after biopsy. Five patients (38%) were admitted with persistent localized pain, five (38%) with orthostatic hypotension, one (8%) with both pain and hypotension, one (8%) with peritoneal signs, and one (8%) with lightheadedness but no orthostatic changes. All complications were noted within 3 hours after the biopsy. Bleeding, potentially the most serious complication, was radiographically defined in 5 of the 13 patients (38%) admitted. Only two patients, however, required blood transfusions. No patient required invasive management such as surgery or chest tube placement. The average length of the hospital stay was 1.5 days. CONCLUSION: Complications after outpatient liver biopsy occur early and rarely require invasive management. Outpatient liver biopsy is safe when done on carefully selected patients in a setting that provides close observation for at least 3 hours after liver biopsy.

Ambulatory Care↗
PubMed1993

Immunohistochemical characterization of hepatic lymphocytes in primary biliary cirrhosis in comparison with primary sclerosing cholangitis and autoimmune chronic active hepatitis.

We analyzed the immunophenotypes of hepatic cellular infiltrates by quantitative immunohistochemical methods in biopsy specimens from 20 patients with primary biliary cirrhosis (PBC), 19 with primary sclerosing cholangitis, and 11 with autoimmune chronic active hepatitis. Specifically, we sought to identify activated T cells, interferon-gamma-producing cells, and natural killer cells. The portal cellular infiltrate in PBC contained a preponderance of CD4 cells in comparison with CD8 cells, with a CD4/CD8 ratio of 2.45:1. The cellular infiltrate in areas of piecemeal necrosis contained mostly CD8 cells. Infiltrating CD8 cells in PBC had the surface phenotype of cytotoxic (CD8-positive, CD11b-negative) cells. Approximately 4% of T cells expressed interleukin 2 receptors. Interferon-gamma-staining cells were rarely identified (in less than 2%). Cells that expressed the natural killer cell markers CD16, CD56, or CD57 were infrequent, constituting approximately 5% of the cellular infiltrate. The composition of the infiltrates was similar in patients with PBC and chronic active hepatitis. Natural killer cells were twice as common in patients with primary sclerosing cholangitis (P < 0.05) as in those with PBC. The inflammatory infiltrates in areas of piecemeal necrosis were similar in the three diseases and differed from those found within the portal area, in that CD8 cells were preponderant. In all three liver diseases, almost 90% of bile ducts expressed class II HLA antigens. These findings support the hypothesis that cytotoxic T cells of either the CD4 or CD8 immunophenotype but not natural killer cells may be involved in the pathogenesis of PBC and chronic active hepatitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Management of osteopenia of liver disease with special emphasis on primary biliary cirrhosis.

Bone disease occurring in the setting of chronic liver disease is being recognized increasingly often. The osteopenia may be an important cause of morbidity in these patients, particularly as effective treatments become available for the liver diseases and especially if these treatments actually worsen the coexistent bone disease. Although no specific treatments are of proven benefit for the bone disease in most instances, adequate exercise and calcium intake as well as vitamin D supplementation when deficiencies are present are recommended for all patients. Information is lacking about specific treatment for patients with PSC, hemochromatosis, and alcoholic liver disease. For patients with PBC, other than the above general measures, preliminary information suggests that supplemental estrogens in postmenopausal women and the use of calcitonin may offer promise; both measures are deserving of further study in this group of patients.

Bone Diseases, Metabolic↗
PubMed1993

High-volume postobstructive choleresis after transhepatic external biliary drainage resolves with conversion to internal drainage.

We report high-volume postobstructive choleresis in two patients who underwent transhepatic external drainage for malignant biliary obstruction. Excessive loss of bicarbonate-rich biliary fluid (up to 6.5 L/day) caused orthostatic hypotension, prerenal insufficiency, hyponatremia, and a decrease in serum bicarbonate. Therapy with isotonic fluids containing sodium, chloride, lactate, bicarbonate, and potassium was based on measurement of biliary fluid volume and electrolyte concentrations. Biliary fluid loss was terminated by conversion to internal biliary drainage. The reason for this rare complication of external drainage of biliary obstruction is unknown, but such patients must be closely monitored for volume loss. When high-volume choleresis occurs, biliary fluid and electrolyte losses should be precisely measured and replaced, and external biliary drainage converted to internal drainage.

Adenocarcinoma↗
PubMed1993

Prevalence of peritonitis and the ascitic fluid protein concentration among chronic liver disease patients.

The prevalence of spontaneous bacterial peritonitis (SBP) or its variants, bacterascites (BA), and culture-negative neutrocytic ascites (CNNA), may vary depending on the underlying liver disease and protein content of ascites. In this study, we compared the frequency of peritonitis (SBP, BA, CNNA) upon admission in alcoholic (ALD), cholestatic (CLD), and hepatocellular liver disease (HLD); determined the relationship between Child's class and prevalence of peritonitis; and assessed ascitic total protein as a risk factor for peritonitis. Between January 1989 and April 1991, 113 consecutive patients were admitted with chronic liver disease and ascites (49, ALD; 22, CLD; and 42, HLD). All had admission paracentesis. SBP was defined as polymorphonuclear cell count (PMN) > or = 250 mm3 with a positive culture, BA as PMN < 250/mm3 and positive culture, and CNNA as PMN > or = 250/mm3 with negative culture. No patients with obvious intraabdominal source for infection (i.e., secondary peritonitis) were included in the analysis. The prevalence of peritonitis was 8/113 (7%); four patients had SBP, one BA, and three CNNA. The occurrence of peritonitis was independent of the type of liver disease (ALD, 8%; CLD, 9%; HDL, 5%). Neither ascitic fluid total protein nor the severity of liver disease (Child's class) predicted the occurrence of peritonitis. We conclude that the occurrence of peritonitis is unrelated to the type of liver disease, and severity of liver disease did not predict the presence of peritonitis. Also, ascitic fluid total protein < 1.0 g/dl may not be a sensitive predictor of risk of peritonitis.

Ascitic Fluid↗
PubMed1992

Hypercholesterolemia and atherosclerosis in primary biliary cirrhosis: what is the risk?

Hypercholesterolemia is commonly associated with primary biliary cirrhosis. In the general population, elevated serum cholesterol is associated with an increased risk of atherosclerosis. The relative risk has been poorly defined in primary biliary cirrhosis patients with hyperlipidemia. In addition, the hyperlipidemic state seen with primary biliary cirrhosis has not been well studied. We prospectively observed 312 patients with primary biliary cirrhosis for a median of 7.4 yr. During this period, 128 patients died. The incidence of atherosclerotic death in patients with primary biliary cirrhosis was not statistically different when compared with an age-matched and sex-matched U.S. control population. A similar group of 50 consecutive PBC patients had detailed serum lipid profiles. Findings included progressive increases in total cholesterol and low-density lipoprotein cholesterol with an increasing histological stage or severity of disease. High-density lipoprotein cholesterol was elevated in all stages, with the highest levels in histological stage 2 and 3 disease. Triglycerides were normal or slightly elevated in all stages. Apoprotein A-I was elevated in all but histological stage 4 disease. Our study suggests the hyperlipidemia associated with primary biliary cirrhosis does not place these patients at risk for atherosclerotic death. In light of the limitations imposed by our relatively small sample size, however, additional patients should be studied. Furthermore, an examination of the pathophysiological mechanisms leading to hypercholesterolemia should be the topic of further study.

Adult↗
PubMed1992

A randomized prospective trial comparing a defined formula diet, corticosteroids, and a defined formula diet plus corticosteroids in active Crohn's disease.

Although defined formula diets may be useful for initial episodes of Crohn's disease, the effects of these diets on subsequent attacks of Crohn's disease or in conjunction with corticosteroids are unknown. To evaluate these issues, we studied 27 patients in a randomized prospective trial. Ten patients received only prednisone (group I), nine received only a defined formula diet (Vital HN [high nitrogen]) (group II), and eight received a combination of prednisone and Vital HN (group III). At the time of entry into the study, the groups were similar with respect to age, sex, Crohn's Disease Activity Index, previous and current treatments, anatomic site of disease, and nutritional status. After 1 month of treatment, we noted seven successes (70%) and three failures in group I (prednisone only), three successes (33%) and six failures in group II (Vital HN only), and six successes (75%) and two failures in group III (combination therapy). Four patients randomized to receive only Vital HN were unable or unwilling to tolerate the defined formula diet. Of the five patients who were able to take the defined formula diet for 1 month, however, three (60%) were successfully treated. The patients who received prednisone (groups I and III) responded better than did the patients who received only the defined formula diet. These results may be attributable to the use of a nonelemental diet or the treatment of patients who were not experiencing an initial attack of Crohn's disease or who had previously received corticosteroids. The expensive and often poorly tolerated defined formula diets should not be considered as a substitute for standard therapy with corticosteroids in Crohn's disease.

Adult↗
PubMed1992

Increased intracranial pressure and hepatic encephalopathy in chronic liver disease.

Increased intracranial pressure is present in more than 80% of patients with fulminant hepatic failure. However, patients with encephalopathy secondary to chronic liver disease are thought not to develop elevated intracranial pressure. We report two patients with chronic liver disease in hepatic coma with raised intracranial pressure documented by an epidural intracranial pressure monitor. One patient rapidly deteriorated to coma over a period of 4 h. The other patient progressively worsened following intravenous sedation administered during upper endoscopy. Both patients had generalized tonic-clonic seizures, and one demonstrated decerebrate posturing and papilledema. Although all metabolic and structural abnormalities should be excluded in patients with hepatic encephalopathy, if the etiology remains in question, the possibility of increased intracranial pressure should be considered in patients with chronic liver disease.

Brain Edema↗
PubMed1991

Successful orthotopic liver transplantation in a patient with refractory biliary candidiasis.

The authors present a nonimmunocompromised patient who developed refractory fungal (Candida albicans) cholangitis and who subsequently underwent successful orthotopic liver transplantation for progressive destruction of intrahepatic and extrahepatic bile ducts. Liver biopsy specimens showed periductal abscesses, and cholangiograms showed rapidly progressive destruction of intrahepatic and extrahepatic bile ducts. Standard immunosuppressive therapy and perioperative amphotericin B were administered. At 6 months, the patient was clinically well with no evidence of recurrent biliary candidiasis. The authors suggest that orthotopic liver transplantation may be a reasonable therapeutic option for patients with refractory biliary candidiasis.

Candidiasis↗
PubMed1991

Ursodeoxycholic acid for the treatment of home parenteral nutrition-associated cholestasis. A case report.

Severe cholestasis associated with long-term home parenteral nutrition is rare, and no treatment is known to be effective. This study shows a case of a patient who developed jaundice while receiving long-term home parenteral nutrition. Causes of jaundice, other than the patient's parenteral feedings, were excluded. The patient's jaundice did not respond to alterations in his parenteral feeding program or to metronidazole. Ursodeoxycholic acid (600 mg/day) led to a prompt and sustained improvement in his hyperbilirubinemia. When ursodeoxycholic acid was stopped, the patient again became jaundiced, but this resolved with reinstitution of ursodeoxycholic acid. This case suggests that ursodeoxycholic acid may be an effective treatment for home parenteral nutrition--associated cholestasis and should be evaluated further in patients with cholestasis associated with parenteral feeding.

Adult↗