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At least 829 records · Page 46Linked to original sources
PubMed1982

Gas-chromatographic/mass spectrometric detection of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in double first cousins.

Gas chromatography/mass spectrometry was used for the detection of 3-hydroxy-3-methylglutaryl-CoA lyase (EC 4.1.3.4) deficiency in double first cousins. This enzyme is in the last step of leucine catabolism and is also involved in ketogenesis. Quantitation of urinary organic acids as their cyclohexyl esters demonstrated increased concentrations of 3-hydroxy-3-methylglutaric acid, 3-methylglutaconic acid, 3-methylglutaric acid, and 3-hydroxyisovaleric acid. The procedure is more rapid, sensitive, and specific than previously reported gas-chromatographic methods for acid quantitation. The affected children initially presented with symptoms similar to Reye's syndrome; the acids were quantitated during periods of altered intake of protein and fat. Both leucine and fat intake contributed to increased acid excretion. These studies suggest that life-threatening episodes of hypoglycemia are best prevented with a low-protein, low-fat diet.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

[Oscillographic polarography of the alkaloid dolicotheline and several related imidazole derivatives].

Oscillographic polarography in an alkaline medium has shown that the alkaloid N-isovalerylhistamine appears as an expressive wave of the adsorption character in the cathode portion of the curve. This makes it possible to detect it at concentrations up to 10(-6) g/ml. Histamine, histide, isovaleric acid, N-acetylhistamine and imidazole produce no such an effect. A definite correlation has been noted between the toxic action of the compounds studied and their behavior on a mercuric drip electrode during oscillographic polarography. In the alkaline medium the neurotoxic compounds manifest themselves by an adsorption wave in the cathode portion of the curve, while the structurally related compounds (not toxic) do not produce such an effect.

Alkaloids↗
PubMed1976

[Changes in hemostasis during epilespy treatment using dipropyl acetate. Extended study].

The influence of Dipropyl-Acetate (DPA, Ergenyl) on hemostasis parameters was examined in 12 patients. In 8 cases there was a prolongation of bleeding time, in 2 cases thrombocytopenia, in 6 out of 10 cases the platelet adhesiveness was pathologically reduced. We therefore recommend hemostasis-examinations--with emphasis on the thrombocytic system--in patients taking DPA.

Adolescent↗
PubMedDate not supplied

[Pharmacokinetic study of sodium di-n-propylacetate (usual tablet and enteric coated tablet with delayed absorption) (author's transl)].

The authors study the blood levels of DPA after ingestion of the drug in the usual tablet form and after ingestion of an enteric coated tablet with delayed intestinal absorption. They emphasize the fast and very variable absorption of the drug in the usual tablet form which makes necessary the administration of several doses each day. They find a slower absorption from the enteric coated tablet resulting incumulative effects. This form allows an easier administration schedule (one dose in the morning and one dose in the evening). Moreover, the blood levels show less variation during a twenty-four-hour period. Undoubtedly, this new form is an improvement in comparison with the usual tablet.

Adult↗
PubMed1976

[Clinical study of the normothymic effect of dipropylacetamide].

On the basis of his results, obtained during a long term study of 32 patients presenting cyclic variations of mood with a known frequency, the author concludes that dipropylacetamide permits to stabilise mood in about 70% of all cases through a supression of relapses or by reducing significantly their frequency. No biological changes were observed and the clinical tolerance of the drug was excellent in most cases. This drug constitutes a variable alternative to lithium treatment, and lacks the well known lithium side effects and limitations.

Adolescent↗
PubMedDate not supplied

[Problems posed by the therapeutic combination of phenobarbital and sodium dipropylacetate. Apropos of a case].

The authors report on the observation of an epileptic woman who showed the clinical and electro-encephalographic symptoms of an overdose of barbiturates when undergoing treatment associating Depakine and Gardenal. Resumption of the treatment results in identical symptoms. The potentialisation of this association and the mechanism by which it acts are as yet unknown.

Adult↗
PubMed1976

[Austrian complex-study on dipropylacetate. Pediatric experiences].

The clinical trial of Dipropylacetat (Convulex) demonstrates the advantages of a well organized multicenter-study. In rather short time reliable information concerning indication, compatibility and dosage can be received. The results of this project are similar to other observations dealing with DPA: Good therapeutical effect in primary generalized epilepsies independent of the seizure-type. Astonishing results influencing Lennox-Syndrome. In cases not treated before, Convulex shows at least the same effect than the "standard anticonvulsants" but less side effects, especially sedation. In those untreated "new-comers" therapeutical effect comes earlier than in cases of premedication. 20 to 50 mg/kg/day can be recommended in childhood. In rare cases the dosage was increased up to 120 mg/kg/day without problems.

Austria↗
PubMed1975

Sodium valproate in the management of intractable epilepsy: comparison with clonazepam.

Sodium valproate 400 mg.-1800 mg. daily has been used for 1-4 months in the management of 35 patients with intractable epilepsy. This preliminary report indicates that the agent is a useful addition to anti-convulsant therapy with beneficial effect to the majority of patients with gran mal, petit mal, nyoclonus and akinetic attacks. Temporal lobe epilepsy and other focal cortical seizures responded less well. There were some minor gastrointestinal and neurological side-effects which subsided with time or the reduction of dosage. The transition period while other anticonvulsants were being withdrawn was accompanied by grand mal seizures in 6 patients. It appears that sodium valproate requires 7-10 days to becoms fully active and that other anticonvulsants should be withdrawn only after the patient is established on a maintenance dosage. Comparison with clonazepam suggests that the latter is more effective in the control of petit mal and temporal lobe epilepsy but has more persistent sedative effects. Most patients transferred from other anticonvulsants to sodium valproate felt more alert and able to concentrate better.

Adolescent↗
PubMed1976

Early experience with sodium valproate.

Early experience with sodium valproate in the treatment of 20 patients with uncontrolled chronic epilepsy is described. It proved to be rapidly and markedly efficient in most of those patients with generalised types of epilepsy and spike-wave abnormalities in the electroencephalogram. In the treatment of focal epilepsies it was relatively unsuccessful. Although it is a little early to assess its full potential, this preparation is the most interesting and useful anticonvulsant to appear in recent times, and may become the drug of choice in the control of petit mal types of generalised epilepsy.

Adolescent↗