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Biomedical subjects

T Westermarck

Publications and source records attributed to T Westermarck.

47 records · Page 3Linked to original sources

Two cases of an abnormal short arm of chromosome 8 (8p+) associated with mental retardation.

Morphologically similar chromosome anomalies, 8p+, were found in two inmates of an institution for the mentally retarded. Analysis of the G-and R-banding patterns showed that the first subject had a duplication of region p2 of chromosome 8; the second subject had an insertion of an unidentified chromosome segment at 8p11. Both subjects were severely mentally retarded. The physical malformations were more severe in the second subject than in the first, who had only minor signs. The dermatoglyphics are recorded.

Abnormalities, Multiple↗

Role of clozapine in the occurrence of chromosomal abnormalities in human bone-marrow cells in vivo and in cultured lymphocytes in vitro.

Bone-marrow chromosomes were examined from 38 mentally and physically retarded and two psychiatric patients who were being treated with a variety of neuropharmacologic drugs. Twenty of these patients used clozapine (Leponex). The clastogenic effects of clozapine in vitro were studied in the lymphocyte cultures of three patients--one free of hematologic disease and two who 6 months earlier had had agranulocytosis attributed to the use of clozapine. The mean frequency of cytogenetic abnormalities in the bone-marrow cells of patients who used clozapine was significantly increased (P less than 0.05). The two patients who had had agranulocytosis had a greater frequency of cytogenetic abnormalities in their cultured lymphocytes in vivo and in vitro than the patient free of hematologic disease. A clone with a 13/14 chromosome translocation was detected in one of the patients. As all patients received a number of drugs during the in vivo and in vitro studies no definite conclusions could be drawn regarding the role played by clozapine in the occurrence of chromosomal abnormalities.

Adolescent↗

Selenium content of tissues in Finnish infants and adults with various diseases, and studies on the effects of selenium supplementation in neuronal ceroid lipofuscinosis patients.

A low blood selenium level has previously been observed in healthy inhabitants of Finland (WESTERMARCK et al. 1977). In this study even lower blood selenium values were observed in patients with acrodermatitis enteropathica, dystrophia musculorum progressiva (Duchenne), infantile and juvenile type of neuronal ceroid lipofuscinosis (NCL), severe mental retardation caused by various factors, and myocardial infarction. The selenium content of the brain, heart, kidney and liver in patients of different ages was also determined. The highest selenium level was found in the kidney. The mean liver selenium concentrations in stillborn, premature and full-term neonates were 1.11 +/- 0.23 (8), 1.21 +/- 0.17 (12) and 0.93 +/- 0.16 microgram/g dry weight (12) respectively (the number of subjects in parentheses). The selenium values are considerably higher than those in infants of from one to nine months of age and adults, whose liver selenium values were 0.58 +/- 0.21 (8) and 0.67 +/- 0.08 microgram/g dry weight (8) respectively. The vitamin E levels of serum in patients with NCL, as well as in subjects with severe mental retardation (controls), were low compared with values in healthy normal subjects. Sodium selenite supplementation in patients with NCL produced at least a transitory improvement without causing any toxic effects during one year of administration.

Acrodermatitis↗

Periodontal disease associated with Down's syndrome: an orthopantomographic evaluation.

The occurrence of advanced periodontal disease with bone loss in patients with Down's syndrome was studied by orthopantomographic technique to obtain objective and quantitative data. The results were compared to a material of pairs matched according to age, sex, and the severity of mental retardation. Both groups of patients were institutionalized and subject to the same hospital conditions and dental care. Eighty-four percent of the young adults (19 to 39 years of age) with Down's syndrome showed advanced alveolar bone loss of 2.5 mm or more as compared with 27% in the matched pair contorls. The mean bone loss of the mandibular first molars in the study group was detected in 17.8%, and in 3.0% in their controls. No difference in radiographically detectable calculus, decay and/or fillings were observed between the two groups. It is concluded that Down's syndrome is associated with an increased prevalence and severity of periodontal disease, and that this is due to the basic congenital disorder rather than to institutionalization or inadequate oral hygiene.¿

Adolescent↗

Selenium content of whole blood and serum in adults and children of different ages from different parts of Finland.

A low level of selenium (Se) in the blood and serum of Finns was demonstrated. Inhabitants in the central, eastern and southeastern districts of the country had particularly low levels selenium. However, the Se level of Lapps and the people of the Aland archipelago seemed to be significantly higher than the level of other Finns. In general, the Se levels in th serum were lower than the values for whole blood. Furthermore, the levels were lower in children than in adults. The significance of these findings in the pathogenesis of some human diseases is discussed.

Adult↗

Selenium, vitamin E and copper in multiple sclerosis.

There has been accumulation of the nutritional muscular dystrophy of the cattle in a certain western district of Finland where the prevalence of multiple sclerosis (MS) is also highest. This animal disease is due to lack of selenium (Se) and vitamin E. The Se content of whole blood was low (52.6 +/- 11.3 ng/ml) in MS patients from this high-risk area compared to the controls (68.8 +/- 11.0). The data for serum failed to confirm this tendency. All Se values appeared to be lower than international values suggested. The values for both vitamin E and copper were within the international normal range.

Copper↗

Anti-oxidant supplementation decreases TBA reactants in serum of elderly.

An "anti-oxidant cocktail" consisting of betacarotene, vitamins B6, C, E, zinc, and selenium or corresponding placebos were given for one y as daily dietary supplements to 45 elderly residents of a nursing home. Initially, the serum TBA reactant levels were higher (2.7 +/- 0.7 mumol/L) than those of an ad hoc control group of healthy younger adults (2.3 +/- 0.6 mumol/L), p less than 0.01. After three mo supplementation, the levels among the verum elderly had decreased to 2.2 +/- 0.6 mumol/L, and they remained at this lower level until the end of the study period, whereas the placebo group showed no change. A significant inverse correlation (r = -0.428, p less than 0.01) existed between the concentrations of serum TBA reactants and whole blood selenium (B-Se), but only B-Se levels above 200 micrograms/L were associated with a decrease in serum lipid peroxides. Serum alpha-tocopherol concentration also correlated inversely with serum TBA reactants but this correlation (r = -0.273, p less than 0.76) was not as strong as that of B-Se. Deficient vitamin B6 status, in biochemical terms, was observed in 25% of the elderly; a daily supplement of 2 mg B6 fully cured all cases of deficiency. The verum group improved slightly in several psychological tests, whereas subjects on placebo remained unchanged or deteriorated during the follow-up period. Clinical amelioration among the verum subjects was reported by the nurses; no toxic side effects were reported. In conclusion, the elderly benefited biochemically and clinically of dietary antioxidant supplements.

Aged↗

Serum cholesterol and vitamins A and E in juvenile chronic arthritis.

Serum total cholesterol is decreased during acute infections and in adults with rheumatoid arthritis, probably partly because of enhanced lipid peroxidation. Oxidative stress also causes augmentation of inflammation and tissue damage in arthritic synovium. Therefore, concentrations of serum total cholesterol and the antioxidant vitamins A and E were studied in 125 children with juvenile chronic arthritis. Total serum cholesterol was significantly lower in the patients than in healthy children in most age groups and correlated with the markers of disease activity, haemoglobin and the erythrocyte sedimentation rate. In age- and sex-adjusted stepwise multiple linear regression, serum zinc had a significant predictive value for cholesterol. The vitamin A concentrations in the sera of the patients was virtually the same as in the healthy controls, though serum vitamin E concentrations were low (22.8 +/- 15.2 vs 30.5 +/- 4.3 mumol/l, p less than 0.001). The deficiency in vitamin E was not compensated for by another lipoperoxide antioxidant, glutathione peroxidase. Only serum cholesterol had an independent explanatory significance for vitamin E in multiple linear regression analysis (partial correlation 0.554, p less than 0.001). It is suggested that low vitamin E and impairment of the anti-oxidant protection further contribute to low serum cholesterol values in JCA.

Adolescent↗