Reversible severe myopathy of respiratory muscles due to adult-onset type III glycogenosis.

Subacute severe myopathy of the respiratory muscles developed in a 47-year-old woman after a 3-week period of strict fasting. Histological and biochemical work-up of muscle biopsy specimens permitted the diagnosis of debrancher deficiency. The course of the disease was characterized by subacute respiratory failure and prolonged mechanical ventilation.

Association of serum antibodies to heat-shock protein 65 with carotid atherosclerosis : clinical significance determined in a follow-up study.

Background: Previous work has proved that increased titers of antibodies against heat-shock protein (hsp) 65 are associated with atherosclerotic lesions independently of other established risk factors. The present follow-up study was designed to further scrutinize the association of hsp antibodies and atherosclerosis and evaluate the possible predictive value of these antibodies for the development and/or progression of lesions in the same population.

Methods And Results: A total of 750 subjects 45 to 74 years old were recruited, and the rate of participation was 93.

Role of lipoprotein(a) and apolipoprotein(a) phenotype in atherogenesis: prospective results from the Bruneck study.

Background: Experimental studies have suggested both atherogenic and thrombogenic properties of lipoprotein(a) [Lp(a)], depending on Lp(a) plasma concentrations and varying antifibrinolytic capacity of apolipoprotein(a) [apo(a)] isoforms. Epidemiological studies may contribute to assessment of the relevance of these findings in the general population.

Methods And Results: This study prospectively investigated the association between Lp(a) plasma concentrations, apo(a) phenotypes, and the 5-year progression of carotid atherosclerosis assessed by high-resolution duplex ultrasound in a random sample population of 826 individuals.

Plasma glucose within the normal range is not associated with carotid atherosclerosis: prospective results in subjects with normal glucose tolerance from the Bruneck Study.

Objective: There is substantial evidence that glucose intolerance is associated with an increased risk of cardiovascular disease. However, it is not well established whether plasma glucose is independently related to atherosclerosis when glucose tolerance is normal and, if so, to which stage of the complex atherosclerotic process.

Research Design And Methods: We prospectively examined the status of carotid arteries in 625 subjects aged 40-79 years who were randomly selected from the general population and had normal glucose tolerance (according to World Health Organization criteria) both at baseline and at 5 years of follow-up.

Different clinical aspects of debrancher deficiency myopathy.

Objective: To characterise the main clinical phenotypes of debrancher deficiency myopathy and to increase awareness for this probably underdiagnosed disorder.

Methods: The diagnosis of debrancher deficiency was established by laboratory tests, EMG, and muscle and liver biopsy.

Results: Four patients with debrancher deficiency myopathy were identified in the Tyrol, a federal state of Austria with half a million inhabitants.

The natural course of atherosclerosis. Part II: vascular remodeling. Bruneck Study Group.

Arterial remodelling is a potentially important component in atherogenesis aimed at delaying the development of significant lumen compromise. Current knowledge on this phenomenon is mainly restricted to experimental evaluations and a few postmortem studies. We used high-resolution duplex ultrasound to study 5-year changes (1990 to 1995) in vessel geometry in a large random sample of the general population (Bruneck Study).

The natural course of atherosclerosis. Part I: incidence and progression.

The natural course of early atherogenesis is not well established. The current prospective survey was designed to monitor 5-year changes in carotid atherosclerosis in a large, stratified random sample of the general population using high-resolution duplex ultrasound (Bruneck Study). Incidence rates of carotid atherosclerosis ranged from near zero to 184 per 1000 person-years.

Endothelial cytotoxicity mediated by serum antibodies to heat shock proteins of Escherichia coli and Chlamydia pneumoniae: immune reactions to heat shock proteins as a possible link between infection and atherosclerosis.

Background: Growing evidence suggests that an immunological reaction against heat shock proteins (HSPs) may be involved in atherogenesis. Because HSPs show a high degree of amino acid sequence homology between different species, from prokaryotes to humans, we investigated the possibility of "antigenic mimicry" caused by an immunological cross-reaction between microorganisms and autoantigens.

Methods And Results: Serum antibodies against the Escherichia coli HSP (GroEL) and the 60-kDa chlamydial HSP (cHSP60) from subjects with atherosclerosis were purified by use of affinity chromatography.

Poor response to activated protein C as a prominent risk predictor of advanced atherosclerosis and arterial disease.

Background: The potential role of activated protein C (APC) resistance in arterial thrombosis and disease is a matter of ongoing controversy.

Methods And Results: In the present population-based survey, a random sample of 826 men and women underwent high-resolution duplex ultrasound scanning of the carotid and femoral arteries. Response to APC was expressed in APC ratios.

Association of high plasma antioxidant capacity with new lesion formation in carotid atherosclerosis: a prospective study.

Background: In atherosclerosis, both reductions and elevations in plasma levels of antioxidants have been reported. This study investigated total antioxidant capacity of plasma from subjects with atherosclerotic disease.

Materials And Methods: The study population consisted of 48 men with or without carotid atherosclerosis.

Prevalence of insulin resistance in metabolic disorders: the Bruneck Study.

The prevalence of insulin resistance in the most common metabolic disorders is still an undefined issue. We assessed the prevalence rates of insulin resistance in subjects with impaired glucose tolerance (IGT), NIDDM, dyslipidemia, hyperuricemia, and hypertension as identified within the frame of the Bruneck Study. The study comprised an age- and sex-stratified random sample of the general population (n = 888; aged 40-79 years).

Human and bovine endothelial cell monolayers are differentially activated for neutrophil transmigration by human plasma.

Endothelial cells used in transendothelial migration assays have been derived from different sources including human umbilical vein and bovine pulmonary artery. As plasma-induced activation of endothelial cells for enhanced transmigration of leukocytes may be pathophysiologically relevant, the role of species differences between plasma and endothelial cells was studied. The effects of human plasma on human umbilical vein and bovine pulmonary artery endothelial cells with regard to transmigration of neutrophils were compared.

[Polyneuropathy in the critically ill patient--critical illness polyneuropathy].

Polyneuropathy developing in critically ill patients ("critical illness polyneuropathy"--CIP) is diagnosed with increasing frequency in intensive care units. CIP is an axonal polyneuropathy leading to difficulties in weaning from artificial ventilation and symmetrical flaccid tetraparesis. Pathogenetically CIP is considered to be part of the multiple organ dysfunction syndrome (MODS) in the course of sepsis or systemic inflammatory response syndrome (SIRS).

Alcohol consumption and atherosclerosis: what is the relation? Prospective results from the Bruneck Study.

Background And Purpose: Potential effects of regular alcohol consumption on atherogenesis are still controversial mainly due to the lack of prospective population-based studies.

Methods: The Bruneck Study is a prospective population-based survey of atherosclerosis and its risk factors. The study population comprises a sex- and age-stratified random sample of men and women aged 40 to 79 years.

U-shaped and J-shaped relationships between serum insulin and coronary heart disease in the general population. The Bruneck Study.

Objective: To evaluate the relationship existing between serum insulin and coronary heart disease (CHD) in the general population.

Research Design And Methods: In a cross-sectional survey on atherosclerosis and its risk factors, 500 men and 500 women aged 40-79 years were randomly selected from the population of Bruneck, Italy. Clinical, biochemical, and behavioral risk factors of atherosclerosis were assessed in the 936 subjects who participated in the study.

Body iron stores and the risk of carotid atherosclerosis: prospective results from the Bruneck study.

Background: Fe2+ released from tissue iron stores may accelerate lipid peroxidation by virtue of its pro-oxidant properties and thus promote early atherogenesis.

Methods And Results: The present prospective survey addresses the potential association between serum ferritin concentrations and the 5-year progression of carotid atherosclerosis as assessed by ultrasonographic follow-up evaluations. The study population comprises a random sample of 826 men and women 40 to 79 years old.

Relationship between insulin and carotid atherosclerosis in the general population. The Bruneck Study.

Background And Purpose: Although several studies have investigated the association between insulin and coronary heart disease, the relationship between this hormone and carotid atherosclerosis is not well established.

Methods: As a part of a population-based survey on atherosclerosis and its risk factors, serum insulin was measured at fasting (n = 888) and at 2 hours after an oral glucose load (n = 811; known diabetic subjects were excluded). The study population comprised an age- and sex-stratified random sample of men and women aged 40 to 79 years.

The role of insulin in age-related sex differences of cardiovascular risk profile and morbidity.

Metabolic changes and shifts in vascular risk profiles during and after menopause may partly explain the loss of premenopausal protection against cardiovascular disease (CVD). The current population-based survey addresses changes in risk factors and insulin levels across an age range of 40-79 years in men and women. Population recruitment was performed as part of the Bruneck Study from July to November 1990.

Free neurovascular transfer of latissimus dorsi muscle to the bladder. I. Experimental studies.

Objectives: Experimental studies were undertaken to investigate the practicability of a microneurovascular transfer of latissimus dorsi muscle to the bladder, and to look for possible techniques enhancing evacuation of a reservoir such as the bladder by striated muscle.

Materials And Methods: Twelve dogs were used for the studies. They were divided into 3 groups.

Insulin sensitivity and regular alcohol consumption: large, prospective, cross sectional population study (Bruneck study).

Objectives: To assess the relation between regular alcohol consumption and insulin sensitivity, and to estimate the importance of insulin in the association of alcohol with multiple vascular risk factors and cardiovascular disease.

Design: Prospective and cross sectional study of a large randomly selected population sample.

Setting: Part of the Bruneck study 1990-5 (Bolzano province, Italy).

Polyneuropathies in critically ill patients: a prospective evaluation.

Objective: To determine the incidence, severity and course of polyneuropathies in patients with sepsis or systemic inflammatory response syndrome combined with multiple organ failure.

Design: Prospective study.

Setting: Division of Intensive Care Medicine of the Department of Anesthesiology and Intensive Care and Intensive Care Unit of the Department of Neurology, University Hospital Innsbruck, Austria.

Myasthenia gravis and primary biliary cirrhosis. Common immunological features and rare coincidence.

A 43-year-old woman with recently diagnosed primary biliary cirrhosis developed antibody-negative myasthenia gravis. She did not receive D-penicillamine therapy. Clinical and immunological features of this patient are discussed.

Autoantibodies against heat shock protein 60 mediate endothelial cytotoxicity.

Stress or heat shock proteins (hsp) are a family of approximately two dozen proteins with a high degree of amino acid sequence homology between different species, ranging from prokaryotes to humans, and are representative of a generalized response to environmental and metabolic stressors. Our previous studies showed increased expression of human hsp60 on endothelial cells of arterial intima with atherosclerotic lesions, and elevated levels of serum antibodies (Ab) against hsp65/60 in subjects with carotid atherosclerosis. To investigate the possible involvement of anti-hsp65/60 Ab in endothelial injury, specific hsp-Ab were isolated from human high titer sera by affinity chromatography and probed on heat-shock human umbilical vein endothelial cells.