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Biomedical subjects

A Propst

Publications and source records attributed to A Propst.

At least 37 records · Page 2Linked to original sources

Prognosis and life expectancy on alpha-1-antitrypsin deficiency and chronic liver disease.

BACKGROUND: Alpha-1-antitrypsin deficiency is a common autosomal recessive disorder associated with early development of emphysema, liver cirrhosis, and hepatocellular carcinoma. The aim of the present study was to define prognosis and life expectancy in patients with alpha 1-antitrypsin deficiency with and without chronic liver disease. METHODS: After a follow-up of 15 years the estimated life table analysis of mortality of 160 patients with alpha 1-antitrypsin deficiency was retrospectively calculated. The survival time was estimated using the Kaplan-Meier survival curves and was compared with the life expectancy of the age- and sex-matched population of west Austria. RESULTS: Fifty-four patients with alpha 1-antitrypsin patients had evidence of chronic liver disease; of these, 78% showed positive viral markers. Of the 106 patients with alpha 1-antitrypsin deficiency without chronic liver disease none had evidence of additional viral infection. Life expectancy in patients with alpha-1 antitrypsin deficiency and chronic liver disease was significantly lower than in patients with alpha 1-antitrypsin deficiency without chronic liver disease (p = 0.001). No difference in life expectancy in alpha 1-antitrypsin deficiency without chronic liver disease was found in comparison with that of the normal population. CONCLUSIONS: We suggest that in alpha 1-antitrypsin deficiency-associated chronic liver disease it is the high coinfection rather than the inborn error of metabolism itself that is responsible for a deterioration of life expectancy or for the poor prognosis of the disease.

Chronic Disease↗

Mallory-Weiss syndrome in a patient with hemophilia A and chronic liver disease.

Eighty percent of hemophiliacs exposed to plasma products are seropositive to hepatitis B and an even higher percentage are seropositive to hepatitis C. Post-transfusion hepatitis is followed by cirrhosis in up to 25% of the cases. In the wake of portal hypertension, the development of oesophageal varices entails the risk of life-threatening hemorrhage. We report on a patient with moderate hemophilia A (factor VIII:C 4-11%) who suffered from massive hematemesis, melaena and evolving shock after excessive alcohol ingestion. The diagnosis of Mallory-Weiss syndrome and the differential diagnosis of bleeding oesophageal varices as well as prognostic consequences are discussed.

Adult↗

A case of hepatocellular adenomatosis with a follow-up of 11 years.

Hepatocellular adenomatosis is characterized by the presence of numerous (arbitrarily > 10) adenomas within an otherwise normal liver without a history of glycogen storage disease or steroid hormone therapy. Although the disease is rare, its importance lies in its tendency to produce symptoms such as abdominal pain and its potential for abdominal hemorrhages. However, the prognosis of hepatocellular adenomatosis remains uncertain. Here we describe the case of a 40-yr-old female with hepatocellular adenomatosis without evidence of serious complications, who was observed over a period of 11 yr.

Adenoma, Liver Cell↗

Decreased beta-endorphin content in peripheral blood mononuclear leukocytes from patients with Crohn's disease.

Increased activation of lymphocytes in inflammatory bowel disease is reflected by alterations of various immunological functions including enhanced spontaneous secretion of rheumatoid factor by mononuclear cells. since in rheumatic diseases increased secretion of rheumatoid factor is associated with decreased levels of beta-endorphin in circulating blood mononuclear leukocytes, we investigated levels of leukocyte beta-endorphin in inflammatory bowel disease and compared them with those in hepatobiliary disorders and in healthy subjects. Levels of beta-endorphin were measured in extracts from peripheral blood mononuclear leukocytes by radioimmunoassay. beta-Endorphin levels ranged from 0 to 67 pg/10(6) cells. Mononuclear leukocytes from ulcerative colitis patients contained as much beta-endorphin as those from healthy control subjects. In patients with Crohn's disease, levels of beta-endorphin were reduced by as much as roughly 50%. An inverse relationship was found between leukocyte beta-endorphin on the one hand and erythrocyte sedimentation rate, blood granulocyte or thrombocyte counts, and C-reactive protein levels in plasma on the other. In patients with various hepatobiliary disorders including fatty liver disease, viral hepatitis, primary biliary cirrhosis, and cryptogenic or alcoholic cirrhosis, beta-endorphin levels were not significantly different from the normal range values. Data indicate that leukocyte beta-endorphin may be involved in regulation of the systemic inflammatory activity of Crohn's disease.

Adult↗

Prevalence of hepatocellular carcinoma in alpha-1-antitrypsin deficiency.

The aim of the present study was to determine the prevalence of hepatocellular carcinoma in adults with heterozygous alpha 1-antitrypsin deficiency and to assess the presence of possible co-risk factors for the development of hepatocellular carcinoma. Two hundred and forty patients with cirrhosis of different aetiologies and 130 patients with alpha 1-antitrypsin deficiency without evidence of chronic liver disease were investigated. Out of the 240 patients with cirrhosis, 61 patients (25%) were found to have alpha 1-antitrypsin deficiency, 36 patients (15%) had chronic hepatitis C infection, 50 (21%) had chronic hepatitis B and 24 (10%) had hepatitis C and hepatitis B infection. Thirty patients (12%) had cryptogenic cirrhosis and 39 (16%) alcoholic cirrhosis. The prevalence of hepatocellular carcinoma in patients with alpha 1-antitrypsin deficiency-associated cirrhosis was comparable to that of hepatocellular carcinoma in patients with cirrhosis of other aetiologies. Positive viral markers were found in 67% of the patients with alpha 1-antitrypsin deficiency-associated cirrhosis with hepatocellular carcinoma. In contrast, in the group of 130 patients with alpha 1-antitrypsin deficiency but without clinical and laboratory signs of chronic liver disease, none was found to have hepatocellular carcinoma (p = 0.001). Our results indicate that heterozygous alpha 1-antitrypsin deficiency-associated cirrhosis is a risk factor for hepatocellular carcinoma, but this is due to chronic liver disease and not due to the metabolic disorder itself.

Adult↗

Do therapist experience, diagnosis and functional level predict outcome in short term psychotherapy?

The purpose of this study was to investigate the effects of clinical diagnosis, functional level and therapist experience on the outcome of brief psychotherapy. Patients (N = 123) were clinically diagnosed and assigned to either a psychiatrist, psychiatry resident, family practice resident or medical student. Global Assessment of Functioning (GAF) scores and the Global Severity Index (GSI) of the SCL-90 were rated at baseline, at the end of therapy and at six month follow-up. The Client Satisfaction Questionnaire was also scored after therapy. All groups of patients improved significantly. Neither therapist type and diagnostic category nor their interaction were related to outcome GAF or to GSI. Patients improved irrespective of their baseline symptom severity. Satisfaction with therapy was highly related ot increased functioning and decreased symptom severity. The number of therapy sessions attended by patients was modestly related to outcome and patient satisfaction. The results suggest that many diagnostic groups benefit from brief psychotherapy administered by therapists of varying experience. Furthermore, the results support the practice of having medical students conduct psychotherapy under supervision during their training.

Adjustment Disorders↗

[Prognosis and life expectancy in primary biliary cirrhosis].

The aim of the present study was to investigate prognosis and life expectancy in patients with primary biliary cirrhosis. We retrospectively analysed 59 patients from western Austria over 15 years (mean 6 years). The results of the present study were compared with the average life expectancy of the population of western Austria and with the results of the Mayo study published in 1989. The mean survival time in our study was 112.7 months, 25% were dead by 132 months. Kaplan-Meier analysis showed a 95% probability to survive 1 year and a 84% probability to be alive at 5 years. Asymptomatic patients had a significantly better prognosis than symptomatic patients. In comparison with the Mayo study the patients in our study had a better prognosis and in comparison with the normal population a significantly worse life expectancy.

Adult↗

Early diagnosis of gastric lymphoma: gene rearrangement analysis of endoscopic biopsy samples.

The diagnosis of gastric lymphoma in endoscopic biopsy specimens remains difficult despite the emergence of accepted criteria for the histologic diagnosis of lymphomas originating from mucosa-associated lymphoid tissue (MALT). The sensitivity and validity of immunoglobulin (Ig) gene rearrangement analysis of mucosal biopsies for the diagnosis of malignant B-cell lymphoma were investigated in comparison with conventional histology and immunohistology. Biopsy specimens from 34 different endoscopies of 20 patients with a previous history, or tentative diagnosis of gastric lymphoma, and 12 control samples were analyzed for the presence of clonal Ig gene rearrangements. A clonal B-cell population was detected by Southern blot analysis in all patients with a definitive histologic diagnosis of lymphoma. In addition, in two patients the detection of clonal rearrangements in biopsy specimens preceded by several months the histologic diagnosis of lymphoma, and clonality was confirmed in three further patients where histology remained inconclusive. In some cases of low-grade MALT-lymphoma, discrete spreading of malignant cells within chronically inflamed mucosa was suggested by the presence of identical clonal rearrangements in all simultaneously obtained biopsies, with or without histologically detectable involvement by lymphoma. Our results show that immunoglobulin gene rearrangement studies of endoscopic biopsy samples are an additional powerful tool for the diagnosis of gastric lymphoma, especially for detecting early recurrence, and improve the preoperative assessment of the extent of mucosal involvement.

Biopsy↗

Spontaneous bacterial peritonitis is associated with high levels of interleukin-6 and its secondary mediators in ascitic fluid.

We investigated 37 patients with ascites and liver cirrhosis in order to examine whether on the basis of correlation of cytokines and acute phase proteins of the ascitic fluid, prognosis of spontaneous bacterial peritonitis can be made. Significantly enhanced levels of interleukin-6, as well as acute phase reactants alpha-1-antitrypsin and C-reactive protein were found in the ascitic fluid of patients with spontaneous bacterial peritonitis. The levels of tumour necrosis factor alpha (TNF-alpha), neopterin, interleukin 2-receptor and granulocyte-macrophage colony stimulating factor were higher in patients with spontaneous bacterial peritonitis, but without statistical significance, whereas no differences were found between the interferon gamma, interleukin-2 and interleukin-1 levels. In addition, interleukin-6, TNF-alpha and neopterin levels were found to correlate significantly with the outcome of the disease. These findings show that acute phase reaction occurs in the ascitic compartment in correlation with the development of spontaneous bacterial peritonitis.

Acute-Phase Proteins↗

High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease.

OBJECTIVE: To determine the prevalence of chronic liver disease in adults with homozygous (Pi ZZ) and heterozygous (Pi Z) alpha 1-antitrypsin deficiency and to assess the presence of other possible risk factors for the development of chronic active hepatitis and cirrhosis of the liver in these patients. DESIGN: Cross-sectional study. SETTING: A referral-based university hospital. PATIENTS: Consecutive patients (164) with the Pi ZZ and Pi Z phenotype with and without chronic liver disease. MEASUREMENTS: The presence of antibody to hepatitis C virus (anti-HCV) was determined using an assay incorporating synthetic peptide antigen from capsid protein (United Biomedical [UBI] assay) and a second-generation enzyme immunoassay (Abbott test); the presence of antibody to hepatitis B virus (anti-HBV) was determined using radioimmunoassays incorporating hepatitis B surface antigen (HBsAg) and hepatitis B core antigen (HBcAg); assays for antinuclear antibody and antimitochondrial antibody (M2 subtype) were also done, and alcohol abuse was assessed by history. RESULTS: Among patients with cirrhosis (32%), 62% were anti-HCV positive by the Abbott test (P = 0.006), and 41% were anti-HCV positive by the UBI assay (P = 0.007). Thirty-three percent of patients with cirrhosis had hepatitis B virus (HBV) infection (P = 0.01); 41% had a history of alcoholism; and 12% had features of autoimmune liver disease. Only five patients (9%) with cirrhosis had no other risk factor for chronic liver disease. Among patients with chronic active hepatitis (7%), 80% were anti-HCV positive by the Abbott test (P = 0.002), and 75% were anti-HCV positive by the UBI assay (P less than 0.001). Thirty percent of patients with chronic active hepatitis had HBV infection (P = 0.023); 18% had autoimmune hepatitis; and 8% abused alcohol. Only two patients (17%) had no additional risk factor for the development of chronic active hepatitis. Among patients with steatosis of the liver (48%), 5% were anti-HCV positive by the Abbott test, and none were anti-HCV positive by the UBI assay; 18% had serologic evidence of past HBV infection, and 28% abused alcohol. Among patients without chronic liver disease (13%), no viral infection could be found; 9% were alcoholics. CONCLUSIONS: Chronic liver disease in patients with alpha 1-antitrypsin deficiency is associated with a high prevalence of viral infection; this infection, rather than alpha 1-antitrypsin deficiency alone, may be the cause of the liver disease in such patients.

Adolescent↗

Successful treatment of recurrent hepatic Candida infection with fluconazole after chemotherapy for acute leukemia: a case report.

Hepatic candidiasis represents an increasingly difficult problem in immunocompromised patients. It has been recognized as a variant of disseminated candidiasis after a period of neutropenia. Establishment of diagnosis is difficult since liver biopsy as well as culture may be negative, in spite of the presence of fungus. Hepatic lesions are generally not detectable by ultrasound examination or computed tomography until the neutrophil count has returned to normal. The aim of this case report is to describe the problems related to the diagnosis and treatment of hepatic candidiasis following chemotherapy for acute leukemia. The report also provides further evidence that fluconazole, a new broad-spectrum antifungal drug, can be safely and effectively used for the long-term treatment of deep-seated hepatic candidiasis.

Adult↗

The effects of teaching medical students psychotherapy skills in the outpatient department.

A psychiatric clerkship which emphasizes a clinical exposure to the conduct of outpatient psychodynamic psychotherapy is described and evaluated. Compared to more traditional inpatient based clerkships, an outpatient psychotherapy-oriented clerkship attracts significantly greater numbers of students. In addition, medical students taking such a rotation rate its educational value on its completion significantly more positively compared to other clerkship sites. Finally, students taking the psychotherapy oriented clerkship opt for psychiatry as a specialty significantly more frequently than students taking the more traditional clerkships. The impact of such a learning experience on interpersonal skills with non-psychiatric, medical patients is also discussed.

Attitude of Health Personnel↗