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The effect of intravenousl-carnitine on propionic acid excretion in acute propionic acidaemia

✍ Scribed by H. Böhles; W. Lehnert

Publisher
Springer
Year
1984
Tongue
English
Weight
255 KB
Volume
143
Category
Article
ISSN
0340-6997

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✦ Synopsis

A 6-week-old female infant presented in a severe metabolic crisis from propionic acidaemia. The condition was aggravated by pneumonia and heart insufficiency. In addition to the general supportive measures and caloric intake exclusively from glucose, intravenous L-carnitine treatment (2 $ L-carnitine/24 h) was started to enhance propionic acid excretion as a carnitine conjugate. Despite the therapeutic efforts the patient died about 48 h after admission in sudden respiratory arrest and bradycardia. Serum propionic acid concentration was increased to 0.3 gmol/ml. Propionylcarnitine excretion was measured and about 55% of the overall excretion during the 48 h treatment period was attributed to an effect of carnitine administration. 2-methylcitrate and 2methyl-3-oxovaleric acid excretion decreased during the same period. Obviously carnitine was not able to prevent metabolic deterioration but may provide some additional "buffer capacity" during long-term dietary treatment.

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The identification of (E)-2-methylglutac
The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with β-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia
✍ M. Duran; L. Bruinvis; D. Ketting; J. P. Kamerling; S. K. Wadman; R. B. H. Schut 📂 Article 📅 1982 🏛 John Wiley and Sons 🌐 English ⚖ 456 KB 👁 1 views

The identification of (E)-2-methylglutaconic acid, a 'new' metabolite of isoleucine, is described. The substance was detected in urine samples from patients with propionic acidaemia, methylmalonic acidaemia and so-called P-ketothiolase deficiency; in the majority of cases together with N-tiglylglyci