Urinary Excretion of 17-Oxosteroids in Hereditary Coproporphyria

1975 ◽  
Vol 49 (5) ◽  
pp. 441-444
Author(s):  
J. W. Paxton ◽  
M. R. Moore ◽  
A. D. Beattie ◽  
A. Goldberg

1. Urinary 17-oxosteroid conjugates were measured by gas-liquid chromatography in five patients with hereditary coproporphyria. 2. Three patients were in an acute attack and showed significantly increased excretion of sulphate or glucuronide conjugates of aetiocholanolone. There was increased excretion of several other related steroids but no consistent pattern was apparent. 3. In the two patients in remission, excretion of urinary 17-oxosteroids was not increased. 4. The ratio of total urinary aetiocholanolone to androsterone (5β:5α) was found to be significantly elevated for the three patients in an acute attack. Serial measurements were made in two of these patients and showed a highly significant linear correlation between this ratio and the urinary content of δ-aminolaevulic acid and porphobilinogen. 5. These observations suggest the involvement of the 17-oxosteroids, especially aetiocholanolone, in the pathogenesis of hereditary coproporphyria.

1970 ◽  
Vol 48 (12) ◽  
pp. 817-823
Author(s):  
M. P. Stefanovic ◽  
H. S. Bayley ◽  
S. J. Slinger

Two boars were fed normally to 19 weeks of age and then underfed so that their growth was severely restricted. Two other boars were underfed to 19 weeks of age and thus did not grow; they were then normally fed and the experiment was continued until they reached the same weight as the first two boars. The excretion of 17-ketosteroids (17-KS) in the urine of the four boars was measured on 2 days each week by separating their trimethylsilyl ethers by gas–liquid chromatography. The excretion of 17-KS reached a maximum of 9.0 mg per day for the boars which were fed normally in the first part of the experiment. Reducing their allocation of feed did not have an immediate effect on the excretion of total 17-KS, but after 4 weeks the excretion declined to about 1.0 mg per day. The 17-KS excretion of the boars which were underfed initially remained less than 0.5 mg per day, but increased when the feed allocation and hence growth rate of the boars increased. However, the excretion of 17-KS by these boars did not reach the levels recorded for the boars which had grown normally from the start of the experiment.


1974 ◽  
Vol 20 (12) ◽  
pp. 1543-1547 ◽  
Author(s):  
Teruo Kitagawa

Abstract Gas—liquid chromatographic methods are described for measuring phenylalanine metabolites in urine and serum. These were studied in controls and in various types of hyperphenylalaninemics. The plasma phenyllactic acid concentration increased as the serum phenylalanine concentration increased. The 24-h urinary excretion of both phenylalanine and phenyllactic acid depends upon their respective concentrations in serum.


1977 ◽  
Vol 6 (6) ◽  
pp. 586-590
Author(s):  
J Wüst

By quantitative gas-liquid chromatography of glucose-containing blood cultures at the moment of first signs of growth, a presumptive diagnosis of anaerobic bacteremia could be made in 24 out of 26 cultures yielding obligate anaerobes upon subsequent culture. With Bacteroides sp. (20 strains isolated), elevated levels of isovaleric acid (greater than or equal to 0.1 mumol/ml) and/or succinic acid (greater than or equal to 5 mumol/ml) were detected in the medium used. An exception to this were two strains of Bacteroides thetaiotaomicron that did not produce sufficient quantities of these acids. In the case of butyrate-producing gram-positive cocci (four strains), butyric acid in amounts of greater than or equal to 0.8 mumol/ml was detected. Propionibacteria (five strains) produced propionic acid in amounts of greater than or equal to 3.9 mumol/ml. No false positive results were found in 103 blood cultures with growth of aerobic or facultatively anaerobic bacteria only.


1977 ◽  
Vol 52 (1) ◽  
pp. 51-65 ◽  
Author(s):  
J. A. Summerfield ◽  
Julia Cullen ◽  
S. Barnes ◽  
Barbara H. Billing

1. The bile acids and bile acid sulphates in the urine, serum and bile of eight cholestatic patients were studied quantitatively by gas-liquid chromatography and gas-liquid chromatography/mass spectrometry. 2. The primary bile acids (cholic acid and chenodeoxycholic acid) comprised on average 94% of the total bile acids in bile, 70% in the serum and 64% in urine. 3. The percentage composition of bile acids in bile was relatively constant and was not influenced by the degree of cholestasis. In contrast, in the serum only the primary bile acids were increased, the concentrations of the secondary bile acids (deoxycholic acid and lithocholic acid) and the minor bile acids remaining constant. 4. The data do not support the hypothesis that monohydroxy bile acids accumulate in cholestasis and are related to the pathogenesis of this syndrome. 5. The pattern of bile acid urinary excretion was similar to that in the serum. But in one patient, 3α,7β,12α-trihydroxy-5β-cholan-24-oic acid was a principal urinary bile acid, although very low concentrations of the compound were found in that patient's serum, suggesting that some of the minor bile acids in urine may originate by epimerization in the kidney. 6. In bile only a small proportion of the bile acids was sulphated (range 2·1–4·6%) and in serum the degree of sulphation was very variable (9–50%). However, in urine, sulphate esters accounted for a large proportion of the total bile acids (33–72%). 7. The output of bile acid sulphate in the urine was related to the urine total bile acid output but the serum concentration of bile acid sulphate remained relatively constant. Consequently, in contrast to the non-sulphated bile acids, whose renal clearance was relatively constant, the renal clearance of sulphated bile acids was directly related to the urine total bile acid output. This finding is inconsistent with the earlier hypothesis that their predominance in urine was due to a high renal clearance. It may indicate renal synthesis of some of the bile acid sulphates in the urine and/or inhibition of active renal tubular reabsorption of sulphated bile acids by non-sulphated bile acids.


Author(s):  
L. Ellis ◽  
R. R. McSwiney ◽  
Susan M. Tucker

The urinary excretion of N-acetyl-β-D-glucosaminidase (NAG) has been claimed to be of high diagnostic value in indicating acute renal graft rejection. Similar claims have been made for the significance of urinary lysozyme excretion. Serial measurements of urinary lysozyme and NAG have been made in 35 patients after renal transplantation, and during 52 suspected rejection episodes. A close correlation has been found between these two parameters although they did not consistently change in parallel. The use of both assays may give a better indication of impending rejection than either assay alone.


1979 ◽  
Vol 83 (1) ◽  
pp. 95-100 ◽  
Author(s):  
B. W. MUJAJI ◽  
K. J. TOUMBA ◽  
R. E. OAKEY

SUMMARY The urinary excretion of oestrone, oestradiol-17β and oestriol was measured by gas–liquid chromatography in 24 h urine specimens, collected after 34 weeks of gestation from each of eight women whose pregnancies were complicated by steroid sulphatase deficiency and from each of 21 pregnant women without this complication. Mean (±s.d.) values μmol/24 h, sulphatase deficient; normal) were: oestrone, 0·7 ± 0·2; 2·5 ± 1·3; oestradiol-17β, 0·5 ± 0·2; 1·6 ± 0·5: oestriol, 2·0 ± 0·9; 54·4 ± 19·9. Thus, mean oestrone and oestradiol-17β excretions were only 30% of normal whilst mean oestriol excretion was reduced by more than 90%. Consequently, the mean ratio of urinary oestriol/(oestrone + oestradiol-17β changed from 13·7 ± 2·3 in normal pregnancy to 1·6 ± 0·6 in pregnancies complicated by steroid sulphatase deficiency. The greater fall in oestriol excretion in this condition, in comparison with that of oestrone and oestradiol-17β, emphasizes the importance of conjugated 16-oxygenated C19 steroids in the biosynthesis of oestriol in human pregnancy. The change in the relative proportions of the three urinary oestrogens implies that in the precursor pool of normal pregnancy the concentration of 16-oxy-C19 steroids relative to that of the 16-deoxy-C19 steroids, is much greater in the sulphate fraction than it is in the unconjugated fraction. The measurement of the urinary oestriol/(oestrone+oestradiol-17β) ratio may have the potential to make it possible to distinguish, before birth, between pregnancies complicated by steroid sulphatase deficiency and those with foetal adrenal hypoplasia.


1972 ◽  
Vol 55 (3) ◽  
pp. 570-572
Author(s):  
Damon Larry

Abstract Six samples of carbonated beverage were spiked at levels from 0 to 400 p pm with foodgrade ammonium glycyrrhizinate and sent to 7 participating laboratories for collaborative study. To facilitate the analysis of samples containing microquantities of ammonium glycyrrhizinate, slight modifications were made in the published GLC method. Recoveries of ammon ium glycyrrhizinate were 116.4, 102.0, 93.4, 102.3, and 105.8% for 35, 100, 200, 300, and 400 ppm levels, respectively. The method has been adopted as official first action.


1974 ◽  
Vol 24 (11) ◽  
pp. 645-654 ◽  
Author(s):  
F. Alan Barbour ◽  
Richard V. Barbour ◽  
J. Claine Peterson

1974 ◽  
Vol 75 (4) ◽  
pp. 647-652 ◽  
Author(s):  
G. Rannevik ◽  
J. Thorell

ABSTRACT Eight amenorrhoeic women were given 100 μg synthetic LRH (Hoechst) iv and im, respectively, at an interval of 2 weeks. Four of the women received the iv injection first and four the im injection. The urinary excretion of oestrogens and pregnanediol was low and unaltered throughout the test weeks. The effects of LRH were compared by serial measurements of the plasma LH and FSH during 8 h. The initial response of LH for up to 25 min and that of FSH for up to 60 min were equal whether LRH was given iv or im. The difference appeared later. Four hours after the injection the mean increase of LH to iv injection was 0.5 ng/ml (N. S.), while that to im injection was 1.9 ng/ml (P < 0.01). The corresponding values for FSH were 1.3 (P < 0.05) and 3.2 (P < 0.001). The effect of LRH administration im was thus found to be larger and more prolonged.


1971 ◽  
Vol 67 (2) ◽  
pp. 303-315 ◽  
Author(s):  
A. J. Moolenaar ◽  
A. P. van Seters

ABSTRACT The 17-oxosteroids were estimated in the urine of 27 patients with Cushing's syndrome by gas-liquid chromatography (G. L. C.). The values of the various steroid fractions are compared with those of normal subjects, patients with thyrotoxicosis and obese subjects. The effect of the age of the patients on the diagnostic value of the invidual 17-oxosteroids and their ratios is discussed.


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