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

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 g L-carnitin...

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Bibliographic Details
Published in:European journal of pediatrics 1984-11, Vol.143 (1), p.61-63
Main Authors: BÖHLES, H, LEHNERT, W
Format: Article
Language:English
Subjects:
Amino Acid Metabolism, Inborn Errors - blood
Amino Acid Metabolism, Inborn Errors - drug therapy
Aminoacid disorders
Biological and medical sciences
Carnitine - administration & dosage
Carnitine - analogs & derivatives
Carnitine - metabolism
Carnitine - therapeutic use
Carnitine - urine
Errors of metabolism
Female
Humans
Infant
Injections, Intravenous
Medical sciences
Metabolic diseases
Propionates - blood
Propionates - metabolism
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Summary: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 g 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 mumol/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 2-methyl-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.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF00442751