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Biomedical subjects

A Goldberg

Publications and source records attributed to A Goldberg.

At least 163 records · Page 9Linked to original sources
PubMed1983

Carbamazepine-induced non-hereditary acute porphyria.

Acute intermittent porphyria has hitherto been recognised as an autosomal dominant inborn error of haem metabolism characterised by a depressed activity of the enzyme uroporphyrinogen I synthase (URO.S). A case of non-hereditary acute porphyria, similar to acute intermittent porphyria, following treatment of epilepsy with carbamazepine is reported. Subsequent measurements of erythrocyte URO.S activity in a group of epileptic patients treated with various combinations of anticonvulsant drugs suggest that carbamazepine exerts a direct suppressant effect on URO.S in addition to its indirect enzyme-inducing properties.

5-Aminolevulinate Synthetase↗
PubMed1983

Immunologic measurement of elastin-derived peptides in human serum.

Chronic obstructive pulmonary disease (COPD), a major cause of morbidity and death in the smoking population, develops insidiously over many years, and usually significant impairment of lung function has occurred before the disease is diagnosed. It is likely that destruction of the elastic fiber is a prerequisite for the development of the disease, and it is possible that immunologic identification in the serum of peptides derived from lung elastin degradation might be an effective approach to the early detection and monitoring of the disease. We prepared antibodies to peptides derived from human lung parenchymal elastin and used these antibodies in an enzyme-linked immunosorbant assay to quantitate elastin-derived peptides in the serum of 39 normal control nonsmokers, 33 smokers with normal lung function, and 40 patients with COPD. On average, statistically significant higher levels of elastin-derived peptides were found in the normal smokers and COPD patients compared to the controls. Further work with larger numbers of subjects is necessary to determine whether such a test is effective in identifying those individuals who are at risk of developing COPD.

Antibodies↗
PubMed1983

Early-onset chronic renal failure as a complication of acute intermittent porphyria.

In a retrospective survey of patients who have had a proven attack of acute intermittent porphyria (AIP) in the West of Scotland a highly significant association (p less than 0.001) was observed between AIP and the development of early-onset chronic renal failure. Six patients with AIP and chronic renal failure arising in early middle-age are described. As no other cause could be attributed to the renal failure three possible causal links between these two conditions were considered, namely, enhanced susceptibility to analgesic nephropathy, porphyria-induced hypertension, and nephrotoxic effects of porphyrins and their precursors. We suggest that porphyria-induced hypertension is the most important factor in causing early-onset chronic renal failure in acute intermittent porphyria.

Acute Disease↗
PubMed1982

Oxidation of corticosteroids to steroidal 20-hydroxy-21-oic acids by mouse liver.

We have studied the enzyme catalyzed oxidation of 11-deoxycorticosterone to 20-hydroxy-3-oxo-4-pregnen-21-oic acid (pregnolic acid) in mouse liver. Enzyme activity, though variable, was higher than that of other species. Pregnolic acid was identified as the free acid, as the methyl ester by thin layer chromatography, and as the p-bromophenacyl ester by high performance liquid chromatography. With [4-14C, 21-3H]-DOC as substrate, exchange of tritium with water (interpreted as due to the reversible isomerization of the ketol to aldol form by the side chain) and the overall conversion of the ketol side chain to hydroxy acid was catalyzed by the post-microsomal supernatant fraction. Although we could not physically separate tritium exchange and acid production, pregnolic acid formation could be decreased or eliminated while tritium exchange was retained, consistent with our previous conclusion that isomerization to aldol was a precondition for acid formation. In preparations that made no acid, [4-14C]-DOC was recovered, depleted of tritium. The rate of exchange of [21S, 21-3H]-DOC with water was faster than [21R, 21-3H]-DOC. The stereochemistry of pregnolic acid at C-20 was 85-90% R (i.e.. 20 alpha-hydroxy-21-oic acid). The Km for isomerase with [21RS-21(3) H-DOC was 4.3 x 10(-5); Km for pregnolic acid formation was 8.0 x 10(-5) M. Corticosterone was oxidized to acid metabolites at 20% the rate of DOC.

Adrenal Cortex Hormones↗
PubMed1982

Screening for latent acute intermittent porphyria: the value of measuring both leucocyte delta-aminolaevulinic acid synthase and erythrocyte uroporphyrinogen-1-synthase activities.

Acute intermittent porphyria (AIP) is an autosomal dominantly inherited disorder of haem biosynthesis characterised by reduced activity of the enzyme uroporphyrinogen-1-(URO) synthase and compensatory increased activity of the rate controlling enzyme delta-aminolaevulinic acid (ALA) synthase. Subjects with the disorder should be identified as they are at risk of developing severe porphyric attacks if exposed to a variety of drugs or chemicals. We have assessed the value of measuring the activities of ALA synthase and URO synthase in peripheral blood cells as a means of identifying latent cases in affected families. In AIP subjects, ALA synthase activity was increased and URO synthase decreased compared to controls, through there was considerable overlap between the two groups when either enzyme was examined alone. When both enzymes were examined together, all but one of the 19 AIP patients had both increased ALA synthase activity (greater than 250 nmol ALA/g protein/h) and reduced URO synthase activity (less than 25.1 nmol URO/l RBC/h), whereas none of the 62 controls showed this enzyme pattern. Examination of 35 asymptomatic first degree blood relatives of AIP patients showed that 17 (49%) had the porphyric enzyme pattern with no sex bias. The combined study of these two enzymes permits accurate detection of latent cases of AIP and confirms its autosomal dominant inheritance.

5-Aminolevulinate Synthetase↗
PubMed1982

Some studies of maternal and infant lead exposure in Glasgow.

In two studies in the city of Glasgow, 236 mothers and their newly born infants and 117 mothers and their 6-weeks old children's environmental lead exposure were examined. In both studies blood lead concentrations were found to correlate significantly with the cube root of the domestic water lead concentrations. In the first study, multiple regression analyses of maternal blood lead and cord blood lead concentrations on other variables showed a significant negative correlation with gestational age. It was also noted that there was an annual fluctuation in maternal blood lead concentration with highest values in the autumn. In the second study, similar relationships were found. Although there was no association between blood lead and sex, age, place of birth or feeding method, as in the previous study, a significant association between social class and blood lead was found. This could be explained on the basis of the significant correlation between water lead and social class. In those mothers who breast fed, breast milk lead concentrations were found to correlate significantly with blood lead concentrations where breast milk lead was around one tenth of blood lead concentration. These studies emphasise the importance of water lead in the economy of environmental lead exposure to mothers and their unborn and newly born infants.

Adolescent↗
PubMed1982

Studies of lead and cadmium exposure in Glasgow, U.K.

Domestic water and whole blood samples were collected early in 1981 from two hundred volunteers living in the Glasgow area of Scotland, U.K. The concentration of lead in the water and blood samples, and of cadmium in the blood, was measured. The blood lead and cadmium concentrations were compared to those obtained in the Survey of 1979. There has been a fall in blood lead concentrations since the 1979 Survey. In contrast, the blood cadmium levels had remained similar. This diminution in blood lead concentration is attributed to a fall in water lead concentration caused by raising the pH of the water supply in the Glasgow area. The main determinant for cadmium in blood appears to be cigarette smoking habits, which had not changed.

Adolescent↗