Clinical Conditions Associated with Urinary Excretion of 2-Hydroxybutyric Acid

1975 ◽  
Vol 35 (3) ◽  
pp. 259-266 ◽  
Author(s):  
S. Landaas ◽  
J. E. Pettersen
PEDIATRICS ◽  
1959 ◽  
Vol 23 (4) ◽  
pp. 815-815
Author(s):  
JOHN H. MENKES

In the course of further investigations on the excretory products in maple syrup disease the strong likelihood has arisen that the defect is more extensive than is indicated in my previous article (Pediatrics, 23:348, 1959). Initially, the presence of high urinary levels of alpha-ketoisocaproic acid together with the excretion of the keto analogues of valine and isoleucine suggested an abnormality in the metabolism of branched-chain amino acids. This postulate, however, does not explain the excretion of a cyclic ester of alpha-hydroxybutyric acid, which, as first suggested by A. J. Smith and L. B. Strang (Arch. Dis. Childhood, 33:109, 1958), is responsible for the maple syrup odor. This compound could well arise as a degradation product of methionine, of which the urinary excretion is also high (Brit. M. J., 1:91, 1959), by way of alpha-ketobutyric acid.


1985 ◽  
Vol 226 (1) ◽  
pp. 51-57 ◽  
Author(s):  
M K Qato ◽  
M D Maines

Non-human primates were used as a model of human neonatal hyperbilirubinaemia and its chemotherapeutic suppression. High levels of haem oxygenase activity were detected in the liver and the spleen of neonatal rhesus (Macaca mulatta) and cynomolgus (Macaca irus) monkeys. When 1-day-old neonatal animals were given a single injection of Zn-protoporphyrin (40 mumol/kg, subcutaneously), serum bilirubin levels declined to nearly normal adult levels within 24 h and remained suppressed throughout the postnatal period (12 days). This treatment inhibited the activities of haem oxygenase and biliverdin reductase in the liver and the spleen, without affecting that of the brain. Zn-protoporphyrin treatment did not alter the activity of brain biliverdin reductase or increase brain bilirubin levels. The biological disposition of Zn-protoporphyrin was examined by measuring the biliary and urinary excretion of the metalloporphyrin complex, as well as its uptake and deposition in blood cells and tissues. Biliary excretion of the metalloporphyrin was minimal (0.12% over a 28 h period), and no evidence was detected for the urinary excretion of Zn-protoporphyrin. However, the concentration of metalloporphyrin in erythrocytes increased over the duration of the experiment (11 days) to such an extent that 46% of the administered compound was taken up by the cells. It appeared that the molecular basis for the sustained suppression of haem oxygenase activity and bilirubin production by Zn-protoporphyrin involved the release of the metalloporphyrin in the normal process of the degradation of fetal erythrocytes. The scope of the biological activity of Zn-protoporphyrin to alter haem-dependent processes appeared limited in nature, insofar as the microsomal contents of cytochrome P-450 and b5, as well as the aniline hydroxylase, were similar to those of the control animals. Also, the concentration of glutathione in the liver was unchanged. These findings suggest the potential usefulness of Zn-protoporphyrin in experimental and perhaps clinical conditions in which hyperbilirubinaemia occurs.


1981 ◽  
Vol 111 (2-3) ◽  
pp. 169-178 ◽  
Author(s):  
Cornelis Jakobs ◽  
Monika Bojasch ◽  
Eberhard Mönch ◽  
Dietz Rating ◽  
Hartmut Siemes ◽  
...  

1965 ◽  
Vol 10 (3) ◽  
pp. 170-179 ◽  
Author(s):  
T. D. Lovegrove ◽  
E. V. Metcalfe ◽  
G. E. Hobbs ◽  
J. A. F. Stevenson

The 24-hour excretions of adrenaline, noradrenaline, and 17-hydroxycortico-steroids were measured in normals and in a) acute and b) chronic schizophrenics, (c) non-schizophrenic psychotics, d) psychoneurotic disorders, and e) a miscellaneous group composed primarily of personality disorders. Some patients were studied over a period of time and the changes in hormone excretion and in clinical conditions compared. On comparison between groups, no difference was found in adrenaline and noradrenaline output. Similarly, when the patients were grouped by dominant emotional reaction at the time of test, no significant differences were observed. The acute schizophrenics, however, did show a greater output of free corticoids, but not of total corticoids compared to the normals. When the same patients were studied over a period of time, there appeared to be a relationship between catecholamine excretion and emotional tension and/or stages of illness. Depressed patients showed a decreased adrenaline and noradrenaline output on admission to hospital but only that of adrenaline was significant. Both adrenaline and noradrenaline showed a marked, but not significant decrease in acute schizophrenics after recovery. In the chronic schizophrenics, there was, after four months of withdrawal of tranquillizers, a significant rise in the output of both; the total and free corticoids showed a similar increase which, however, was not significant. Although no significant differences in hormone excretion were observed among the groups studied, other than increased excretion of free corticoids in the acute schizophrenics, the longitudinal studies in several groups suggest significant correlations may occur between changes in clinical condition and/or emotional state and the excretion of some of the hormones measured.


2001 ◽  
Vol 53 (3) ◽  
pp. 399-402 ◽  
Author(s):  
P. V. Kavanagh ◽  
P. Kenny ◽  
J. Feely

1964 ◽  
Vol 45 (4_Suppl) ◽  
pp. S99-S124 ◽  
Author(s):  
Svend G. Johnsen

ABSTRACT Quantitative measurements of the urinary excretion of gonadotrophins and androgen metabolites have been correlated with the functional condition of the germinal epithelium in various forms of male hypogonadism. Data on the influence of Leydig-cells, Sertoli-cells and the spermatogenetic stages on the gonadotrophin production are given. The finding of some, previously undescribed, lesions in spermatogenesis in certain clinical conditions is discussed. It is concluded that the testicular-hypophyseal feed-back mechanism in man depends on a very late stage in spermatogenesis. The suggestion is put forward that the cytoplasm which is split off from the spermatid immediately before liberation of the mature spermatozoon produces the hypophyseal inhibitor.


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