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J Willeit

Publications and source records attributed to J Willeit.

At least 55 records · Page 3Linked to original sources

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). Carotid arteries free of atherosclerosis and wall thickening preserved a normal size to high ages. In contrast, common and internal carotid arteries with elevated intima-media thickness (>/=50th percentile) experienced marked age-dependent dilation that started already in the 5th decade and continuously accelerated thereafter (structural ageing). Vessel diameters were subject to complex regulation involving morphometric characteristics, sex, wall thickness, hypertension, LDL cholesterol levels, and alcohol consumption. Vascular remodelling secondary to incident or slowly progressive (mural) atherosclerosis included local compensation and a generalised dilation response of vascular segments not primarily affected. Adaptive enlargement at the site of active atherogenesis effectively preserved a near-normal lumen in most instances. The current study identified a second main type of plaque growth, characterized by episodic marked increase in lesion volume probably on the basis of plaque thrombosis. In this setting, we did not observe maximum but insufficient compensation but instead usually observed no compensation at all. Failure of vascular remodelling and marked expansion in plaque size acted synergistically in producing significant lumen compromise. The current prospective survey describes fundamental principles and various facets of arterial remodelling and vascular biology in the general population (in vivo). Vessel geometry was subject to marked temporal changes and showed a correspondingly complex (multifactorial) and dynamic regulation. Vascular remodelling emerged as an important compensatory process in human atherogenesis, which crucially contributed to the determination of lumen obstruction. Efficacy and failure of compensation primarily depended on the type and pathomechanisms of underlying atherogenesis and only in second instance on plaque size and location.

Adult↗

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. All subjects had high-resolution echo-duplex evaluation of the common and internal carotid arteries (eight regions of interest on both sides) in 1990 and 1995 to detect the change in carotid status over time. The occurrence of new plaques in previously normal segments was termed "incident nonstenotic" or "early atherosclerosis," and the occurrence of stenosis in >40% of previously normal segments was termed "incident stenotic" or "advanced atherosclerosis." In addition, we evaluated the changes in the atherosclerosis score (the sum of all plaques) during the follow-up, and we measured intimal-medial thickening (IMT) in the common carotid artery in 1995. In all subjects, several candidate risk factors were assessed: sex, age, BMI, waist-to-hip ratio, glucose, HbA1c, insulin, urate, lipids, apolipoproteins A1 and B, blood pressure, lipoprotein(a), fibrinogen, antithrombin III, factor V Leiden mutation, ferritin, leukocyte count, smoking, alcohol intake, physical activity, and socioeconomic status. Fasting plasma glucose (FPG), plasma glucose 2 hr after the glucose load (2-h PG), and HbA1c concentrations in 1990 and 1995 were averaged in each subject to obtain an estimate of long-term glucose exposure of the arterial wall. RESULTS: Linear or logistical regression analyses indicated that neither baseline glucose and HbA1c levels nor mean FPG, mean 2-h PG, or mean HbA1c in 1990 and 1995 were independently related to IMT, a 5-year change in the atherosclerotic score, incident nonstenotic (early) atherosclerosis, or incident stenotic (advanced) atherosclerosis. Likewise, subjects with FPG levels above the median and subjects in the new category of "impaired fasting glucose" did not have an increased occurrence or progression of atherosclerosis. All results were consistent before and after adjustment for other vascular risk factors and possible confounders. CONCLUSIONS: These results suggest that plasma glucose levels within the normal range (<7.8 mg/dl both at FPG and 2-h PG) are not independently related to any stage of atherosclerosis.

Adult↗

[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). The incidence of CIP in septic patients with MODS ranges from 50% to 70%. Electroneurography (ENG) and electromyography (EMG) are essential diagnostic procedures. The mortality in patients with CIP is higher than in patients without CIP. Depending on the severity of CIP, recovery of neurological function in survivors is usually relatively good.

Critical Care↗

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. At baseline (1990) carotid arteries were evaluated by duplex sonography and plasma samples were obtained for testing antioxidant capacity by two different test systems. One assay system used neutrophils from healthy volunteers as a source of oxygen free radicals activating the non-fluorescent dichlorofluorescin diacetate in the presence of antioxidant containing plasma from study subjects. In the other test system, total plasma antioxidants were detected colorimetrically by using 2,2'-azino-di-(3-ethylbenzthiazoline sulphonate), metmyoglobin and superoxide in the presence of plasma. Carotid arteries were re-evaluated for the development of new plaques 5 years later (1995). RESULTS: Increased baseline total antioxidant capacity of plasma was significantly associated with the development of new atherosclerotic lesions during a period of 5 years. CONCLUSIONS: Endogenous antioxidant capacity of plasma is increased in patients with active atherosclerotic disease. As scavenging of oxygen free radicals is thought to protect from atherogenesis, elevated antioxidative capacity may represent an adaptive mechanism.

Adult↗

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. Participation and follow-up were more than 90% complete. Changes in carotid atherosclerosis between the 1990 baseline and the first follow-up in 1995 were monitored by high-resolution duplex ultrasonography. Alcohol intake was quantified with a standardized questionnaire and prospective diet records. RESULTS: Alcohol consumption less than once a week (occasional drinking) had no effect on atherogenesis. The association between regular alcohol intake and incident carotid atherosclerosis (early atherogenesis) was J-shaped, with light drinkers facing a lower risk than either heavy drinkers or abstainers. Protection offered by alcohol consumption of <50 g/d appeared to act through inhibition of the injurious action of high levels of low-density lipoprotein (LDL) cholesterol. Excess risk of incident atherosclerosis observed among heavy alcohol consumers (> or =100 g/d) clearly surpassed the risk burden afforded by heavy smoking. The association between regular alcohol intake and incident carotid stenosis (advanced atherogenesis) was U-shaped. Odds ratios were generally shifted toward protection and did not rely on LDL cholesterol levels. We failed to find any differential effects of alcohol from various sources. All associations remained independently significant when we adjusted for lifestyle, coincidental smoking, and the metabolic complex associated with drinking. CONCLUSIONS: Our findings support the view that adverse and beneficial effects of alcohol on arterial disease are mediated in part by a dose-dependent promotion or deceleration of atherogenesis. The protection afforded by light drinking may possibly be attributed to antithrombotic effects and inhibition of the atherogenic action of high levels of LDL cholesterol.

Adult↗

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). Insulin resistance was estimated by homeostasis model assessment (HOMA(IR)), preliminarily validated against a euglycemic-hyperinsulinemic clamp in 85 subjects. The lower limit of the top quintile of HOMA(IR) distribution (i.e., 2.77) in nonobese subjects with no metabolic disorders (n = 225) was chosen as the threshold for insulin resistance. The prevalence of insulin resistance was 65.9% in IGT subjects, 83.9% in NIDDM subjects, 53.5% in hypercholesterolemia subjects, 84.2% in hypertriglyceridemia subjects, 88.1% in subjects with low HDL cholesterol, 62.8% in hyperuricemia subjects, and 58.0% in hypertension subjects. The prevalence of insulin resistance in subjects with the combination of glucose intolerance (IGT or NIDDM), dyslipidemia (hypercholesterolemia and/or hypertriglyceridemia and/or low HDL cholesterol), hyperuricemia, and hypertension (n = 21) was 95.2%. In isolated hypercholesterolemia, hypertension, or hyperuricemia, prevalence rates of insulin resistance were not higher than that in nonobese normal subjects. An appreciable number of subjects (n = 85, 9.6% of the whole population) was insulin resistant but free of IGT, NIDDM, dyslipidemia, hyperuricemia, and hypertension. These results from a population-based study documented that 1) in hypertriglyceridemia and a low HDL cholesterol state, insulin resistance is as common as in NIDDM, whereas it is less frequent in hypercholesterolemia, hyperuricemia, and hypertension; 2) the vast majority of subjects with multiple metabolic disorders are insulin resistant; 3) in isolated hypercholesterolemia, hyperuricemia, or hypertension, insulin resistance is not more frequent than can be expected by chance alone; and 4) in the general population, insulin resistance can be found even in the absence of any major metabolic disorders.

Adult↗

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. Serum insulin was measured at fasting (n = 888) and 2 h (n = 811, known diabetic subjects were excluded) after an oral glucose load. CHD was ascertained by an abnormal electrocardiogram and/or a history of angina or myocardial infarction. RESULTS: Subjects were stratified according to serum insulin quintiles at fasting or 2 h after glucose loading. After adjustment for sex, age, BMI, smoking, physical activity, alcohol intake, and socioeconomic status (analysis of covariance), cardiovascular risk factors clustered in subjects of the top insulin quintile. Multiple logistic regression analysis, including sex and age in model 1, sex, age, BMI, glucose tolerance, socioeconomic status, and behavioral variables in model 2, or this set of variables together with triglycerides and apoproteins A1 and B, fibrinogen, and blood pressure status in model 3, revealed a significant association between high serum insulin and CHD when median insulin quintile was used as the reference class. Moreover, low serum insulin levels, such as those found in subjects of the lowest quintile, were independently related to CHD. These results were found either before (model 1) or after (models 2 and 3) adjusting for several covariates. Consistent results were found in men and women, as well as in younger and older subjects. CONCLUSIONS: Results of the present study suggest that both hyperinsulinemia and "hypoinsulinemia" are independent indicators of CHD. Furthermore, it is proposed that the relationship between CHD and fasting insulin is U-shaped, whereas that between CHD and postglucose insulin may be J-shaped.

Adult↗

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. Transendothelial migration of neutrophils was tested employing endothelial cell-covered Transwell cell culture chamber inserts with micropore filters of 5 microns pore size. Results showed that human plasma induces neutrophil transmigration of both human umbilical vein and bovine pulmonary artery endothelial cell monolayers. Plasma samples from 50 to 69 year old men with and without carotid arteriosclerosis (N = 152) stimulated endothelial cells of human origin significantly stronger for transmigration of neutrophils than endothelial cells of bovine origin. Analyses of limits of agreement and of correlation of the two methods indicated that bovine pulmonary artery and human umbilical vein endothelial cells cannot replace each other in assays of plasma-induced activation of transmigration. Plasma levels of soluble adhesion molecule P-selectin, which are elevated in patients with arteriosclerotic diseases, are inversely related to plasma-induced activation of neutrophil transmigration of endothelial monolayers of human origin but not of bovine origin (N = 65). As rates of transmigration that were induced by plasma from subjects without carotid arteriosclerosis (N = 73) were significantly related between human and bovine endothelium, elevated plasma levels of soluble P-selectin may thus affect results of assays for plasma-induced activation of neutrophil transmigration of endothelial monolayers in a species-specific manner.

Adult↗

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. Serum ferritin was one of the strongest risk predictors of overall progression of atherosclerosis. The main part of this association appeared to act through modification of the atherogenic potential of LDL cholesterol (OR [95% CI] for a 1-SD unit increase in ferritin at LDL levels of 2.5, 3.6, and 4.9 mmol/L: 1.55 [1.30 to 1.85], 1.77 [1.40 to 2.24], and 2.05 [1.50 to 2.80]; P=.0012 for effect modification). Changes in iron stores during the follow-up period modified atherosclerosis risk, in that a lowering was beneficial and further iron accumulation exerted unfavorable effects. All these findings applied equally to incident atherosclerosis and the extension of preexisting atherosclerotic lesions. The significance of prominent iron stores in the development of carotid stenosis was clearly less pronounced. Finally, ferritin and LDL cholesterol showed a synergistic association with incident cardiovascular disease and death (n=59). CONCLUSIONS: The present study provided strong epidemiological evidence for a role of iron stores in early atherogenesis and suggests promotion of lipid peroxidation as the main underlying pathomechanism. This hypothesis could in part explain the sex difference in atherosclerotic vascular disease.

Adult↗

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. In brief, of 1000 subjects randomly selected for inclusion 936 participated, with insulin measurements available in a random subgroup of 880 men and women, 60 of whom were excluded due to manifest diabetes mellitus. Insulin concentrations were assessed according to Hales and Randle and by a human insulin-specific radioimmunoassay. A rise in insulin concentrations with advancing age in women (5th-8th decade, 10.5-14.4 mU/l or +1.2%/year) contrasts with a marked gradual decline in insulin levels in men (5th-8th decade, 12.5-5.9 mU/l or -2.4%/year). Age trends of insulin concentrations in sexes emerged as independent of age-related changes in body weight, type of fat distribution, alcohol consumption, cigarette smoking, social status, fasting glucose, and physical activity (P < 0.001 for sex-specific difference in the regression slopes). Insulin levels in pre- and postmenopausal women of equal age differed significantly (10.1 vs. 13.9 mU/l, P = 0.003), thus advocating that variations of insulin observed may in part be related to shifts in sex hormone status. Levels of virtually all vascular risk attributes were lower in premenopausal women than in men of equal age, but the opposite was true for the elderly. The switch in the sex preponderance of vascular risk factors may be crucially involved in closing the CVD incidence gap between genders after menopause. The analysis suggests that variations in insulin levels are a common metabolic basis for sex/age trends in fasting glucose, apolipoprotein B, total cholesterol, total cholesterol/HDL cholesterol ratio, LDL density (LDL cholesterol/apolipoprotein B), triglycerides and systolic blood pressure.

Adult↗

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. Atherosclerosis in the common and internal carotid arteries was assessed twice (in 1990 and 1995) by duplex sonography. Progression during the 5-years follow-up was defined by an increase in the atherosclerosis score of more than the doubled measurement error (> 27%) or by the occurrence of new plaques. Subjects were stratified in quintiles according to baseline serum insulin at fasting or 2 hours after glucose loading. RESULTS: Logistic regression analysis revealed a significant association of carotid atherosclerosis with both low and high insulin (U-shaped relation). This finding was found before and after adjustment for several covariates (sex, age, body mass index, glucose tolerance, triglycerides, apolipoproteins Al and B, fibrinogen, blood pressure status, behavioral variables, and socioeconomic status). This relation applied equally to fasting and postglucose insulin and was more pronounced in the prospective analysis than in the cross-sectional analysis. CONCLUSIONS: We conclude that both "hypoinsulinemia" and hyperinsulinemia are independent risk indicators of carotid atherosclerosis.

Adult↗

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. Group I: Evacuation proficiency of a re-configured latissimus dorsi under tension was hydrodynamically tested after 6 months. Group II: Microneurovascular latissimus dorsi transposition anastomosing thoracodorsal vessels and nerve to circumflexa ilium profunda vessels and a lumbar plexus motor nerve, respectively, was performed to cover the bladder devoid of up to 50% of the detrusor muscle. Group III: Microneurovascular latissimus dorsi transposition was used to reconstruct the bladder after supratrigonal cystectomy. Urodynamic and radiographic in vivo studies were done after 6 and 9 months. RESULTS: Stimulation of the thoracodorsal nerve of geometrically reconfigurated non-transposed latissimus dorsi reservoirs after 6 months yielded average intraluminar pressures of 190 cm. H2O at maximum capacity, and 35 cm. H2O at a minimum capacity of 10 to 15 cc. Stimulation of latissimus dorsi transposed to the bladder resulted in a visible and measurable contraction of the transplanted muscle after 9 months. Mean bladder volumes in group II animals pre- and postoperatively were 267.5 and 270 cc, respectively. During cystography the bladder outline was smooth both during filling and voiding. Light and electron microscopic examination confirmed viable, re-innervated muscle. CONCLUSIONS: The "tension-torsion" reconfiguration produced intraluminal pressures which should enable stimulated striated muscle to evacuate bladder-like reservoirs successfully. Microneurovascular free transfer of latissimus dorsi muscle resulted in reinnervated, functional muscle in the pelvis. Augmentation cystoplasty using the latissimus dorsi in conjunction with a free omental flap for substitution of an accompanying mucosal defect was unsuccessful. The concept of microneurovascular transfer of latissimus dorsi muscle to the pelvis may be used for complicated fistula of the lower urinary tract, and may be a possible solution for the therapy resistent acontractile bladder.

Animals↗

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). SUBJECTS: 820 health, non-diabetic women and men aged 40-79 years. MAIN OUTCOME MEASURE: Concentrations of fasting and post-glucose insulin, cholesterol, apolipoproteins, triglycerides, Lp(a) lipoprotein glucose, fibrinogen, and antithrombin III; blood pressure; insulin resistance estimated by the homeostasis model assessment. RESULTS: Fasting insulin concentrations in those who did not drink alcohol and subjects reporting low (1-50 g/day), moderate (51-99 g/day), and heavy (> or = 100 g/day) alcohol intake were 12.4, 10.0, 8.7, and 7.1 mU/l (P < 0.001). Likewise, post-glucose insulin concentrations and estimates for insulin resistance assessed by the homeostasis model assessment decreased significantly with increasing amounts of regular alcohol consumption. These trends were independent of sex, body mass index, physical activity, cigarette smoking, medication, and diet (P < 0.001). Regular alcohol intake predicted multiple changes in vascular risk factors over a five year period including increased concentrations of high density lipoprotein cholesterol and apolipoprotein A I; higher blood pressure; and decreased concentration of antithrombin III. These associations were in part attributable to the decrease in insulin concentrations observed among alcohol consumers. CONCLUSIONS: Low to moderate amounts of alcohol, when taken on a regular basis, improve insulin sensitivity. Insulin is a potential intermediate component in the association between alcohol consumption and vascular risk factors (metabolic syndrome).

Adult↗

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. PATIENTS: Twenty-two patients between 23 and 77 years old with sepsis or systemic inflammatory response syndrome combined with multiple organ failure fulfilling strict inclusion and exclusion criteria. INTERVENTIONS: Clinical neurologic examination and electro myography/nerve conduction velocity measurements during the stay on the Intensive Care Unit and 2-3 months later. MEASUREMENTS AND RESULTS: In 9 of the 22 patients signs of polyneuropathy were found at the initial clinical investigation and in 7 patients at the follow-up investigation. Electrophysiologic investigation revealed signs of polyneuropathy in 18 patients initially and in 11 patients 2-3 months later. CONCLUSION: In our patient population the frequency of the development of polyneuropathy was high (81.8%). Electrophysiologic investigation is superior to clinical neurologic examination in the detection of polyneuropathies.

Activities of Daily Living↗

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. In previous reports on an association between primary biliary cirrhosis and myasthenia D-penicillamine treatment was an obligate causal linkage between both disorders.

Adult↗

Copper-induced acute rhabdomyolysis in Wilson's disease.

Wilson's disease is a lethal defect in copper metabolism causing a continual increase in tissue copper concentrations that become toxic to the liver, brain, kidney, eye, skeletal system, and several other tissues and organs. The liver is unique among these in being both the site of the etiologic biochemical abnormality and the organ that is always affected by copper toxicosis. Although myocardial muscle involvement has been reported in association with Wilson's disease, copper deposits in peripheral muscle tissue have not yet been described. A case of a young patient with Wilson's disease who developed recurrent episodes of acute rhabdomyolysis is presented, and the accumulation of copper in muscle tissue as a possible complication is discussed.

Acetates↗