T. Richter et al., CARNITINE DEFICIENCY IN CHILDREN WITH LON G-TERM TUBE-FEEDING VIA PERCUTANEOUS ENDOSCOPICALLY CONTROLLED GASTROSTOMY (PEG), Monatsschrift fur Kinderheilkunde, 144(7), 1996, pp. 716-721
Background: L(-)-Carnitine is an essential factor for the energy metab
olism of long chain fatty acids. Since about 20 years carnitine defici
ency syndromes have been described with several clinical symptoms and
marked muscular dystrophy. In infants the long term total parenteral (
carnitine-free) feeding is an important cause for the development of a
secondary carnitine deficiency syndrome. Methods: We analysed the ser
um carnitine levels of 16 children, mean age 6.2 years, with centrally
caused neurogenous dysphagia. These children exclusively received a b
alanced carnitine-free tube diet via PEG for some months. We estimated
both free and total L(-)-carnitine in serum using a radiochemical enz
ymatic method. Results: All 16 children showed a marked carnitine defi
ciency in serum (mean +/- standard deviation for free carnitine 9.5 +/
- 3.2 mu mol/l, for total carnitine 10.2 +/- 3.6 mu mol/l) after carni
tine-free diet. Those serum values are less than 20 % of the values in
healthy children. 13 of the first examined 16 children then got each
1 g L(-)-carnitine (3.3 ml Biocarn) per day. After substitution for ab
out two months the carnitine values in serum rose significantly and di
d not differ from the normal values anymore (free carnitine 43.6 +/- 1
4.5 mu mol/l, total carnitine 47.8 +/- 13.5 mu mol/l). In two children
the carnitine substitution was interrupted: After one or three months
, respectively, the serum values decreased again to the low values tha
t are characteristic for systemic carnitine deficiency. Conclusions: A
long term carnitine-free tube feeding induces a secondary systemic ca
rnitine deficiency, which is probably more frequent than the rare here
ditary primary carnitine deficiencies. The endogenous carnitine biosyn
thesis in children is not sufficient to maintain the carnitine homeost
asis. Supplementation of L(-)-carnitine to tube diet is necessary for
physiological serum carnitine levels.