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Literature DB >> 7449803

Successful carnitine treatment in a non-carnitine-deficient lipid storage myopathy.

R C Sengers, J A Bakkeren, J M Trijbels, A M Stadhouders, G J Egberink, H J ter Laak, H J Jaspar.

Abstract

An 18-month-old boy presented with general hypotonia, decreased muscle strength, retarded motor development and stunted growth. The excretion of dicarboxylic acids was enhanced. EMG was normal. A muscle biopsy revealed a lipid storage myopathy. Oral daily supplementation with 2 g D, L-carnitine resulted in: (1) an increase of the growth velocity; (2) increased muscle strength, and (3) a decrease in the lipid fraction of the fibre volume. The carnitine content of the muscle biopsied prior to treatment appeared to be normal.

Entities: Chemical Disease Species

Mesh：

Substances：
Carnitine

Year: 1980 PMID： 7449803 DOI： 10.1007/bf00441643

Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183

16 in total

1. Fatal lipid storage myopathy with deficiency of cytochrome-c-oxidase and carnitine. A contribution to the combined cytochemical-finestructural identification of cytochrome-c-oxidase in longterm frozen muscle.

Authors: J Müller-Höcker; D Pongratz; T Deufel; J M Trijbels; W Endres; G Hübner
Journal: Virchows Arch A Pathol Anat Histopathol Date: 1983

1 in total

Successful carnitine treatment in a non-carnitine-deficient lipid storage myopathy.

1. Carnitine deficiency: clinical, morphological, and biochemical observations in a fatal case.

2. Microdetermination of (-)carnitine and carnitine acetyltransferase activity.

3. Muscle carnitine deficiency. Genetic heterogeneity.

4. Oral treatment of carnitine myopathy.

5. Letter: Inborn error of carnitine metabolism ("carnitine deficiency") in man.

6. Metabolic myopathies associated with stunted growth.

7. The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features.

8. [Clinical, morphological and biochemical studies on muscle carnitine deficiency (author's transl)].

9. Practical stereological methods for morphometric cytology.

10. Carnitine deficiency: acute postpartum crisis.

1. Fatal lipid storage myopathy with deficiency of cytochrome-c-oxidase and carnitine. A contribution to the combined cytochemical-finestructural identification of cytochrome-c-oxidase in longterm frozen muscle.