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Biomedical subjects

B Mattsson

Publications and source records attributed to B Mattsson.

At least 91 records · Page 5Linked to original sources
PubMed1984

Completeness of the Swedish Cancer Register. Non-notified cancer cases recorded on death certificates in 1978.

Of the death certificates issued in Sweden in 1978 and stating cancer as the underlying or contributory cause of death, 1634 cases were unrecorded in the national cancer register. In 62 per cent of the cases the criteria for cancer registration were fulfilled. The non-reported cases represented a total deficit of 4.5 per cent calculated on cancer deaths in 1978. The factors responsible for the deficit were investigated. When the diagnosis had been histologically/cytologically confirmed the deficit was less than 2 per cent but was about 30 per cent when the diagnostic basis was only clinical. More than half of the non-notified cancer patients were older than 75 years. Exclusion of this age group and of myeloma and leukaemia cases gave a cancer-register deficit of 2.3 per cent. Non-notification to the Swedish cancer registry can be diminished by supplementation with data from death certificates, as practised in other Nordic countries. On regional basis these death certificates will now be collected and used as a supplement to the cancer notification in Sweden.

Adolescent↗
PubMed1983

Glutathione synthetase deficient human fibroblasts in culture.

Cultured skin fibroblasts from patients with 5-oxoprolinuria caused by hereditary deficiency of glutathione synthetase have decreased levels of the corresponding enzyme as well as of glutathione. Fibroblasts from the same patients accumulated gamma-glutamyl cysteine, but the levels were lower than those of glutathione in control fibroblasts. The uptake of [35S]cystine was equally rapid in control and patient fibroblasts. In the acid-soluble fraction gamma-glutamyl-[35S]cysteine accumulated in fibroblasts from patients but not from controls. Appreciable turnover of gamma-glutamyl cysteine and glutathione in the respective cell strains was observed, the half-lives of these pools being approximately 5 hours. The growth rate of mutant fibroblasts in culture was significantly slower than that of control fibroblasts. There was no significant accumulation of 5-oxoproline in the culture medium.

Cell Division↗
PubMed1981

5-oxoprolinuria due to hereditary 5-oxoprolinase deficiency in two brothers--a new inborn error of the gamma-glutamyl cycle.

Two brothers, aged 16 and 11 years, had recurrent episodes of vomiting, diarrhoea and abdominal pain, starting in infancy. In spite of extensive investigations no cause of their enterocolitis could be established. After several years symptomatic treatment was discontinued without any recurrence of symptoms. Their father and several paternal relatives have had kidney stones. Both boys developed urolithiasis and an oxalate-containing stone was removed from the elder brother's kidney. He had no hypercalciuria. His glomerular and tubular function tests were normal. Gas chromatography of urine from both brothers revealed massive excretion of L-5-oxoproline (pyroglutamic acid). Glutathione levels in erythrocytes of both patients were normal. The activities of enzymes of the gamma-glutamyl cycle were analysed in erythrocytes, leukocytes and cultured skin fibroblasts. The level of glutathione synthetase was normal, as was the affinity of this enzyme for its substrate gamma-glutamyl-cysteine. Feedback inhibition of gamma-glutamyl-cysteine synthetase by glutathione was also normal. Both patients had a specific deficiency of 5-oxoprolinase, the activity of which was 2-4% of that of control subjects. Their parents had intermediate 5-oxoprolinase activities in fibroblasts, indicating a recessive mode of inheritance. Thus, 5-oxoprolinuria in these two patients was due to a lack of 5-oxoprolinase, i.e., a new inborn error in the gamma-glutamyl cycle.

Adolescent↗
PubMed1978

The comprehesive psychopathological rating scale--CPRS--in patients with schizophrenic syndromes. Inter-rater reliability and in relation to Mårtens' S-scale.

In the course of a multicenter controlled trial of the effects of neuroleptic drugs on patients with schizophrenic or paranoid syndromes a comparison was made between the Swedish symptom rating scale--Mårtens' S-scale especially designed for patients with schizophrenic syndromes--and a new rating scale--the Comprehensive Psychopathological Rating Scale--CPRS. The Spearman rank correlation coefficient between the two scales was found to be 0,48 and as the inter-rater reliability for both scales was found to be quite satisfactory the validity of the scales is discussed. The CPRS scale was found to be easy to handle even for untrained doctors and in a separate study of inter-rater reliability where 5 doctors saw 16 patients a quite satisfactory reliability rk = 0,70--0.97, was found for 33 out of 39 items. In some items, espically those concerning different aspects of affective disturbances a lower inter-rater reliability was found but these items have been revised in later versions of the scale.

Adolescent↗
PubMed1978

Childhood cancer in Sweden, 1958-1974. I. Incidence and mortality.

All cases of tumours and tumour-like conditions in children 0-14 years reported to the Swedish cancer registry during the period 1958-74 have been studied. The material consists of 3797 individuals on file in this registry. The most common cancer diagnoses in children 0-14 years are leukemia and tumours of the central nervous system (together constituting approximately 58% of all cases). Almost half of the cancers affect children below five years of age. The lowest incidence occurs in the ages 7-8 years, and the highest occurs during the first year of life. The types of tumours below one year of age show a different distribution than in any other age groups. A significant increase in the incidence of childhood cancer occurred, while the mortality rates showed a slight decrease during the period studied. A remarkable increase in the incidence figures was noted concerning tumours of the nervous system, especially in boys. The decrease in the mortality rates was most obvious regarding Wilms' tumour, and leukemia in children 0-4 years of age.

Adolescent↗
PubMed1977

Breast cancer following irradiation of the breast.

During the period 1927--1957 1,115 women were treated at Radiumhemmet, Sweden with ionizing radiation for different non-neoplastic conditions of the breast. The average follow-up period was 31.5 years. The number of breast cancers occurring after irradiation was found to be four times the expected number and the breast cancer incidence rate in the irradiated breasts is dependent on the age of the patient at the first irradiation.

Adolescent↗
PubMed1977

Changes in psychopathology in relation to EEG variables and visual averaged evoked responses (V.AER) in schizophrenic patients treated with penfluridol or thiothixene.

In a study of 28 schizophrenic in-patients treated with penfluridol or thiothixene, patients were followed with clinical ratings, EEG variables, the mean integrated amplitude (MIA) on both the left and right sides--both with filters with frequency ranges from 7.5 to 13.5 and 0.5 to 25 Hz--as well as its within-patient variance (WPV) on both sides and with both filters, and also with visual averaged evoked responses (V.AER). Moreover, determinations of plasma levels of the drugs were conducted in a search for possible objective measurements of the effects of the treatment, but also to try to find measurements that would make it possible to predict the outcome of treatment. MIA left/right and WPV left/right were found to be the most promising variables to follow the effect of treatment, which were correlated to factors 1 and 2 of the Mårten's S-scale. WPV left/right before treatment was correlated to changes in factor 4 of the S-scale during the trial.

Adult↗
PubMed1976

On the mechanism of 5-oxoproline overproduction in 5-oxoprolinuria.

The primary metabolic defect in 5-oxoprolinuria (pyroglutamic aciduria) is the lack of glutathione synthetase. The mechanism of the concomitant overproduction of 5-oxoproline was studied using cell-free extracts of erythrocytes from control individuals and from patients with 5-oxoprolinuria. Such extracts catalyzed the synthesis of 5-oxoproline from L-glutamate. Addition of ATP, Mg ions and alpha-aminobutyrate was needed for optimal activity. The conversion of glutamate to 5-oxoproline occurred in two steps, catalyzed by gamma-glutamyl-cysteine synthetase and gamma-glutamyl cyclotransferase, respectively. Extracts of erythrocytes from control subjects and patients with 5-oxoprolinuria had identical capacity to synthesize 5-oxoproline. The conversion of glutamate to 5-oxoproline was markedly inhibited by reduced glutathione, which exerted its effect on the gamma-glutamyl-cysteine synthetase step. The following mechanism is postulated for the overproduction of 5-oxoproline in 5-oxoprolinuria: the deficiency of glutathione synthetase causes a lack of glutathione which is an essential feed-back inhibitor in the initial step of its biosynthesis. Therefore gamma-glutamyl-cysteine is produced in excessive amounts and it is subsequently converted to 5-oxoproline (and cysteine) by gamma-glutamyl cyclotransferase. This overproduction of 5-oxoproline exceeds the capacity of the 5-oxoprolinase and 5-oxoproline accumulates in body fluids.

Amino Acid Metabolism, Inborn Errors↗