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Biomedical subjects

C Keller

Publications and source records attributed to C Keller.

At least 307 records · Page 17Linked to original sources

Reduction of Lp(a) by different methods of plasma exchange.

Lipoprotein(a) has been shown to be an independent risk factor for atherosclerosis. The effect of different forms of plasmapheresis therapy on the removal of Lp(a) is examined. Plasmapheresis is successfully administered in a small number of hypercholesteremic patients who fail to respond to conventional therapy. Comparison of four different methods of plasma exchange (albumin substitution, anti-apoB antibody column, LDL precipitation, filtration) reveals significant differences in effectiveness in the ability to lower plasma Lp(a): plasma exchange with albumin und LDL precipitation seem to be the most effective, plasma filtration the least.

Adolescent↗

Unusual but reversible hepatic lesions following long-term treatment with pyridylcarbinol for familial hypercholesterolemia.

We report two cases of unusual hepatic lesions following years of monotherapy with pyridylcarbinol (timespan) in a daily dose of 1.35 to 1.5 g for familial hypercholesterolemia. Mild elevations of liver enzymes correlated to a large tumor-like mass in the gallbladder region in one case and nodular changes in the whole liver (diagnosed by ultrasound) similar to metastatic lesions. After discontinuation of pyridylcarbinol the changes in the liver regressed markedly in both cases.

Adult↗

Changes of atherosclerosis of the carotid arteries due to severe familial hypercholesterolemia following long-term plasmapheresis, assessed by duplex scan.

Three males, aged 15, 22, and 25 years, have been followed for Familial hypercholesterolemia (FH) from 1968 and 1976 onward, being treated by plasmapheresis for 2 to 5 years continuously, at 1- to 2-week intervals. Whereas two patients have been continuously treated to date, the third discontinued plasma exchange after 4 years and has been reevaluated 6 years later. Discontinuation of plasma exchange led to progression of atherosclerosis of the carotid arteries documented by duplex scan, whereas one patient exhibited marked regression of former impressive soft plaque-formation and one patient showed no significant change. Two-dimensional computer-analysis suggested a reduction of plaque mass of up to 45% in one patient.

Adult↗

Atherosclerosis of the carotid arteries in young patients with familial hypercholesterolemia.

Patients with heterozygous and homozygous Familial Hypercholesterolemia exhibit a high incidence of premature coronary heart disease, presumably due to atheromatous plaque-formation in the coronary arteries. Clinical symptoms develop when the disease has progressed to more severe stages of atherosclerosis. Aim of our study was to visualize and document early atheromatous lesions in the carotid arteries of asymptomatic patients with familial hypercholesterolemia under 30 years of age by Duplex-scan. Of 44 patients, 70% had detectable carotid plaques, while only 12% of the controls were affected. All patients with severe carotid disease had serum cholesterol levels above 350 mg/dl. In the age group 2-20 years, 66% of the patients exhibited plaques. Only 6% of the FH patients 21-30 years had normal carotid arteries. We conclude that the process of atheromatous plaque formation in patients with FH starts early in life, severity of atherosclerosis being a function of both extent and duration of hypercholesterolemia. Duplex-scan examination of the carotid arteries is efficient and precise non-invasive method suitable to visualize this process and, by measuring regression, monitor the efficacy of therapeutic measures.

Adolescent↗

Psychological and physical variables as predictors of coping strategies.

26 women and 17 men between the ages of 26 and 62 yr. of age and diagnosed as having chronic illnesses were administered four questionnaires to assess physical and psychological variables and coping strategies: the Sickness Impact Profile, the Time Reference Inventory, and the Multidimensional Health Locus of Control. The Jaloweic Coping Scale assessed coping strategies. Canonical correlation indicated a single-factor link (Wilks' lambda) between scores on the first 3 tests, and the subscales of the coping scale. A significant but small correlation of .31 between the Multidimensional Health Locus of Control subscale, Powerful Others, and the coping subscale of Emotion-focused strategies was observed, that with the subscale Chance and the coping subscale confrontive strategies was negative. A second canonical correlation deleting the Time Reference Inventory score of age extension and including the three Sickness Impact Profile subscales was significant and positive for the Composite score and the subscale Palliative of the Jaloweic Coping Scale. Such data suggest that individuals who have a locus of control orientation of Powerful Others respond to chronic illness with emotionally based behaviors. In addition, chronically ill individuals who are physically dysfunctional tend to respond with palliative coping strategies.

Activities of Daily Living↗

Atherosclerosis of the carotid arteries documented by duplex scan as a predictor of coronary artery disease in familial hyperlipidemias.

The degree of atherosclerotic plaques in the carotid arteries was evaluated in 85 patients with familial hypercholesterolemia and in 43 patients with familial combined hyperlipidemia by means of Duplex scan which recognizes early atherosclerosis. In patients older than 40 years carotid atherosclerosis of moderate degree was associated with coronary heart disease in 90% of cases. The extent of elevated cholesterol, age, cigarette smoking, and hypertension determined the degree of carotid atherosclerosis.

Adolescent↗

'Ancestral' neural mechanisms of electrolocation suggest a substrate for the evolution of the jamming avoidance response.

The genus Sternopygus, believed to reflect ancestral traits of gymnotiform electric fish, is closely related to the more 'modern' genus Eigenmannia (Mago-Leccia 1978; Fink and Fink 1981). Sternopygus is the only known genus of electric fish that does not perform a jamming avoidance response (JAR) to minimize the potentially detrimental effects of signal interference between discharging neighbors (Bullock et al. 1972, 1975), and its ability to electrolocate objects is rather immune to jamming (Matsubara and Heiligenberg 1978). By studying the responses of midbrain neurons to stimulus regimes effective in eliciting the JAR in Eigenmannia, we found that Sternopygus has neurons capable of discriminating the sign of the difference frequency between interfering electric organ discharges (EODs). These 'sign-selective' neurons, which are believed to be important elements in the control of the JAR in Eigenmannia, may, therefore, fulfill a more general function in the detection of moving objects and conspecifics but could potentially be assembled for the evolution of a JAR in Sternopygus. The relative immunity to jamming in this genus may result, in part, from a stronger reliance upon the ampullary electrosensory system which operates in the DC and low-frequency range, outside the EOD spectrum of these fish.

Action Potentials↗

Alkylating cytostatic treatment in renal amyloidosis secondary to rheumatic disease.

Fourteen consecutive patients with chronic inflammatory rheumatic disease and reactive renal amyloidosis were treated with alkylating cytostatics in 22 separate periods varying in duration between six and 30 months. Chlorambucil alone was given in 14 treatment periods, cyclophosphamide alone in six, and both alternately in two. The dosage was adjusted to attain a major suppression of the rheumatic inflammation and a blood lymphocyte level below 1.0 X 10(9)/l. Renal function improved in 12 treatment periods, renal deterioration was arrested in three periods, and in another four periods the rate of functional decline slowed down. In the remaining three treatment periods, associated with further deterioration in renal function, treatment was inadequate owing to blood dyscrasia and failure to control hypertension. Glomerular filtration rate (GFR) was followed more closely in 10 treatment periods, in all of which the falling trend was arrested or reduced. The survival rate at five years was 93%. Three patients who dropped out of the treatment programme are so far the only ones not still alive. Nine are still being followed up after 6-17 years, and the other two remaining live patients have had renal transplants for five years.

Adult↗

[Mucopolysaccharidosis 6-A (Maroteaux-Lamy disease): comparison of clinical and pathologico-anatomic findings in a 27-year-old patient].

The autopsy of the 27-year-old patient suffering from a severe form of mucopolysaccharidosis type VI-A (Maroteaux-Lamy) described in the article by Rampini et al. revealed the compressive cervical myelopathy (C1) to be due to a severe, slit-like deformity of the vertebral canal which was due to a massive thickening of the ligaments, dislocation of the posterior arch of the atlas and broadening of the dura. The massive, generalized disorder of the enchondral ossification involving mainly tubular bones and spine, led to short stature, arthrosis of large joints, and to an angular thoraco-lumbar kyphoscoliosis. Our patient had a massive V-shaped stenosis of the upper third of the trachea. The atrio-ventricular valves of the heart were thickened, and there was fibrosis of the endocardium. The cardiac conduction system displayed fibrosis and foam cell transformation of the fibroblasts in the bundle of His, a lesion not previously reported. Light and electron microscopic investigation showed a massive increase of mucopolysaccharides mainly in the matrix of the fibrous tissue and of the cartilage. Furthermore, the fibroblasts and chondrocytes as well as the hepatocytes and the ganglion cells of the central nervous system contained increased amounts of glycosaminoglycans.

Adult↗

Regression of valvular aortic stenosis due to homozygous familial hypercholesterolemia following plasmapheresis.

A 39-year-old male with homozygous familial hypercholesterolemia confirmed by tissue culture suffered from mild aortic insufficiency and valvular stenosis with a gradient of 20 mm Hg across the aortic valve. Plasmapheresis carried out every 2 weeks for 4 years resulted in a marked reduction in the serum cholesterol level and in a regression of the valvular stenosis, as shown by echocardiography and by left heart catheter.

Adult↗

Predicting the performance of daily activities of patients with chronic lung disease.

40 men and 60 women between the ages of 40 and 59 yr. of age and having the diagnosis of chronic obstructive lung disease as measured by Fev1 values of less than 70% predicted pulmonary function were administered an outcome measure of functional ability, the Sickness Impact Profile. A prediction equation for functional ability, the variables of sex, socioeconomic status, respiratory impairment, and self-reporting of symptoms for analysis. In the two-variable model socioeconomic status and self-reporting of breathing difficulty contributed to 28% of the total variability in functional ability scores. In the three-variable model, adding the third independent variable of respiratory impairment (Fev1) did not significantly increase the explained variability in the outcome measure.

Activities of Daily Living↗

Effect of plasma exchange with and without concomitant drug treatment on lipids and lipoproteins in patients with familial hypercholesterolemia confirmed by tissue culture.

Repeated plasma exchange is an effective treatment for young patients with familial hypercholesterolemia. We treated 2 homozygous and 1 heterozygous patient with very high cholesterol levels with continuous plasma exchange using human albumin solution as exchange medium. The treatment was repeated every 2 weeks in 2 patients and weekly in the third. Treatment periods of plasma exchange alone and of plasma exchange with concomitant drug therapy were compared. For drug treatment beta-pyridylcarbinol, the alcohol corresponding to nicotinic acid (0.9 g/day equivalent to 3 g of nicotinic acid) and cholestyramine (16 g/day) were used. Plasma exchange alone resulted in a decrease of all lipids by 55% and of apolipoproteins by 50-60% as compared to the plasma levels before exchange. Subsequently all lipoproteins rose again to reach pre-exchange levels within about 2 weeks. There was no difference between the homozygous and the heterozygous patients. Beta-pyridylcarbinol or cholestyramine given concomitantly did not alter the post-exchange increase of total, LDL, HDL cholesterol nor of the corresponding apolipoproteins except of apolipoprotein B.

Adult↗