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Biomedical subjects

J Clausen

Publications and source records attributed to J Clausen.

At least 163 records · Page 9Linked to original sources
PubMed1978

E rosette-forming lymphocytes in multiple sclerosis patients. Basic protein stimulation of rosette-forming cells.

"Total" and "active" T cells were enumerated using sheep red blood cell (SRBC) rosetting technique. The percentages of "total" and "active" T lymphocytes were not significantly different in multiple sclerosis patients as compared to other neurological disorders and/or healthy controls. However, the number of "avid" T cells binding more than three SRBC was significantly increased in MS patients. Active E rosette test has shown hypersensitization to myelin basic protein in 50% MS patients and 31% OND patients. All healthy controls studied, except one had negative response.

Adult↗
PubMed1978

Multiple sclerosis specific antigens in MS brains.

Antigens in MS and non-MS brains were investigated by means of crossed immunoelectrophoresis. Two MS specific antigens were found: a measles antigen which was present in microsomes and cytosole, and an unidentified antigen present only in cytosole.

Adult↗
PubMed1978

The enhancing effect of unsaturated fatty acids on E rosette formation.

The number of bound sheep red blood cells (SRBC) per lymphocyte of 16 healthy subjects were assayed prior and after addition of unsaturated fatty acids. Addition of fatty acids to the assay system was found to increase significantly the number of avid T lymphocytes (which bind more than 10 SRBC). It is suggested that increased fatty acid level might change lipid fluidity of the plasma membrane and increase the number of attachment points between the cell surface of the lymphocyte and SRBC. The immunoregulatory effect of fatty acids is discussed.

Arachidonic Acids↗
PubMed1978

Biochemical abnormalities in Batten's syndrome.

The present data indicate that a group of ten patients with Batten's syndrome showed reduced activity of erythrocyte glutathione (GSH) peroxidase (Px) (glutathione: H2O2 oxidoreductase, EC 1.1.1.9.) using H2O2 as peroxide donor. Assay of erythrocyte GSHPx using H2O2, cumene hydroperoxide and t-butyl hydroperoxide as donors also makes it possible biochemically to divide Batten's syndrome into two types: (1) one type with decreased values when H2O2 and cumene hydroperoxide are used, and (2) one type with increased values when t-butyl hydroperoxide is used. Furthermore an increased content of palmitic, oleic and of eicosatrienoic acid but decreased linoleic acid content was found in serum from patients with Batten syndrome. An inverse relationship between erythrocyte GSHPx and serum eicosatrienoic acid was found in the patients. Finally normal selenium levels were found in erythrocytes, but decreased values were traced in whole blood. In normal human beings a connection was found between the erythrocyte selenium content and GSHPx activity assayed by cumene hydroperoxide as a peroxide donor.

Adolescent↗
PubMed1977

Sleep patterns in mental retardation: Down's syndrome.

EEG and eye movements during two consecutive nights were recorded in 21 "normal" and 21 Down's subjects of both sexes in the 14-24-year age range. Records of most Down's subjects were characterized by longer total sleep time, lack of waking alpha, frequent awakening and movement epochs and fewer spindle bursts. Significantly longer "awake" and stage 4 percentages, but less stage 2, were found in the Down's group. They also had longer REM latency, and lower eye movement density based on number of eye movements. Density based on integrated eye activity correlated positively with IQ in the Down's subjects. Spectrum analysis for EEG frequencies from 1 to 20 c/sec showed significantly greater power in the Down's group below 9 and above 12 c/sec, but no difference in the alpha range. The greater power at low frequencies was more pronounced in "awake", stage 1 and REM.

Adolescent↗
PubMed1977

The Dyggve-Melchior-Clausen (DMC) syndrome. A 15 year follow-up and a survey of the present clinical and chemical findings.

The Dyggve-Melchior-Clausen (DMC) syndrome includes short stature, dwarfism, mental retardation, and skeletal abnormalities especially in the spine and the extremities resembling the findings in the mucopolysaccharidoses. A particular abnormality is the "lace border" found on radiological examination of the iliac crest. The three original cases have been followed for 15--20 years and the course is characterized by increasing mental retardation and motor disability whereas the "lace border" is less pronounced than before. A survey of 17 other cases is given and similarities and differencies to the mucopolysaccharidoses are pointed out. Patients with the DMC syndrome have been suggested to be deficient in an enzyme cleaving glycoprotein-acid mucopolysaccharide (AMP) linkage. We have previously found in DMC patients, an abnormal excretion of urinary AMP's of which some were undersulfated and some were oversulfated. Lysosomal acid proteinase, i.e., cathepsin D and neutral proteinases: elastase and cathepsin G were found to be normal in DMC patients. However, alfa2-macroglobulin in serum was raised. This increase may cause an inhibition of the neutral proteinases. An increased level of chondroitin sulfate N-acetylgalactosamine-6-sulfate-sulfatase and decreased enzymic levels of aryl sulphatase A and B (assayed with p-nitrocatecholsulfate as a substrate) were found in leucocytes of DMC patients. Metabolic studies have revealed an unbalanced incorporation of glycoprotein AMP-precursors in DMC lymphocytes. All in all the data suggests the DMC syndrome to be an inborn error of glycoprotein-AMP-metabolism.

Abnormalities, Multiple↗
PubMed1977

Lysosomal (leucocyte) proteinase and sulfatase levels in Dyggve-Melchior-Clausen (DMC) syndrome.

Patients with the DMC syndrome have been suggested to possess a specific sulfatase abnormality and/or to be deficient in a proteinase cleaving glycoprotein-acid mucopolysaccharide (AMP) linkage. We have previously found in DMC patients an abnormal excretion of urinary AMPs of which hyaluronic acid and chondroitin sulfate (A + C) were oversulfated and keratosulfate and heparan sulfate were undersulfated. Lysosomal acid proteinase, i.e. cathepsin D (EC 3.4.23.5) and neutral proteinase : elastase (EC 3.4.21.11) and cathepsin G were found to be normal in DMC patients. However, alpha 2-macroglobulin in serum was raised. This increase may be associated with a complex formation of alpha 2-macroglobulin with a neutral proteinase released from the cells. Increased levels of chondroitin sulfate N-acetylgalactosamine-6-sulfate sulfatase and sulfamidase and decreased enzymic levels of arylsulfatase A and B (EC 3.1.6.1) were found in leucocytes of DMC patients. The sulfatase activities assayed in the present study support our theory that a specific sulfatase abnormality may exist in the DMC syndrome.

Adenosine Monophosphate↗
PubMed1977

Erythrocyte glutathione perioxidase deficiency in multiple sclerosis.

The present study demonstrates a significant decrease in glutathione peroxidase activity in erythrocytes of 24 patients with multiple sclerosis (MS) when the data are expressed as enzymic units per mg hemoglobin and compared to data from normal controls without known family history of demyclinating diseases. Since selenium is an essential part of glutathione peroxidase, this study also compares the topographic differences in selenium availability (expressed as selenium content of forage) withage) iwth the prevalence and death rates of MS in the USA. The comparison cannot exclude the possibility of a relationship between low selenium content and high prevalence as MS. The data are discussed in relationship to current theories on the pathogenesis of MS.

Adult↗
PubMed1977

Juvenile neurolipidosis of Bernheimer-Seitelberger's type. Histopathological and biochemical findings.

Two children are reported, who became diseased at the age of 4 and 2 years, respectively, with progressive upper motor neuron symptoms leading to severe disability within a few years. The boy died at age 10 and autopsy disclosed a severe gangliolipidosis throughout the central nervous system, maximal in the brain stem and cerebrellum, corresponding with his early and dominantly bublar symptoms. The girl is still living, although severly disabled. The etiology was confirmed through biochemical studies which showed lower activity of galactosaminidase and galactosidase in frontal lobe tissue from the boy, in liver tissue from the girl. The activity of galactosaminidase A isoenzyme was more than 50 per cent lower in serum from the girl and in serum from healthy relatives of the boy, the father, mother and one of two brothers. Furthermore, the galactosidease activity was within or below the lower normal range limit in leucocytes from relatives of the boy with exception of the father. This abnormality was also encountered in serum from our girl patient.

Adolescent↗
PubMed1977

Social remedial measures for multiple sclerosis patients in Denmark.

A total of 57 patients admitted to the Danish Multiple Sclerosis Society's treatment and nursing home in Haslev have been interviewed about their socio-medical situation concerning their course of disease, physical, psychic and economic status, family relationships, and different assistance needs. Sixty per cent lived together, and of the solitary only 9 were unmarried. None were employed fulltime and as a whole they received higher allowances than other disabled persons. All suffered from walking impairment. 17 had no wheelchair, 21 had a wheelchair and limited walking ability, 19 could move around only by wheelchair. None were bedridden. The distribution of housing corresponded to that of other disabled persons but was inferior to that of the population as a whole. 54 per cent had got modifications to housing paid for by the public authorities and 42 per cent had a car for the disabled. 47 per cent alleged that their need for transportation remained uncared for. None of the patients were lonely to a severe degree. Expenses for medicine and transportation varied greatly, showing inequality of grant administration. The results of the investigation are discussed.

Adult↗
PubMed1977

Cutting fluids and their effects on the skin of mice. An experimental study with special reference to carcinogenicity.

Reports of skin malignancies due to occupational exposure have decreased since the introduction of solvent-refining of mineral oil fraction in the manufacture of oil based cutting fluids. Commercial mineral oil based cutting fluids caused local and general pathological changes after repeated application to the skin of mice in the present study. Forty-eight per cent of the mice exposed to oils showed severe dysplasia or malignancy of the skin on histological examination. The corresponding figure for the control group, where various additives were used was 8 per cent. The frequency of papillomas was also increased in the mice exposed to oils. The systemic lesions included focal necrosis of the liver, associated with amyloid deposition, as well as amyloidosis of the skin, spleen and kidneys. The substances responsible for these apparent carcinogenic properties of the complex mixtures may be polycyclic hydrocarbons; the latter are still present in the commercial products despite solvent refining; on the other hand the carcinogens may be additives to the cutting oils the composition of which is generally a trade secret.

Amyloidosis↗
PubMed1977

Heavy metal pollution among autoworkers. I. Lead.

Lead pollution was evaluated in 216 individuals working in 10 garages on the Island of Funen, Denmark and related to data from biochemical and medical examinations. Clinical symptoms were recorded by means of a questionnaire. Increased blood test lead levels were foun in 59% with 9% having above 80 microgram lead/100 ml (3-86mumol/1) whole blood. Mechanics in eight out of ten garages had significantly increased blood lead levels. A decrease in delta-aminolevulinic acid dehydratase (ALAD) activity was associated with increased blood lead levels but the latter were not related to haematological changes, tobacco consumption or to length of service in the trade. Particulate lead air pollution was not the sole cause of increased blood lead levels. Raised lead values were maximal among diesel engine workers who are exposed to high pressure-resistant lubricants containing lead naphthenate. As these workers complained of skin damage, lead absorption may have occurred through the skin. Assay of lead content showed 9290 ppm in gear oil and 1500-3500 ppm in used motor oils. The data are discussed in relation to the occupational risks in auto repair shops.

Adolescent↗