Abstract
A 1-year and 11-month-old female infant with bilateral lesions of the thalamus, basal ganglia, cerebellar and brainstem disease died from heart failure 9 days after being administered a measles vaccination. She had a high fever, hypocarnitinemic and non-ketotic hypoglycemia, serum levels of total carnitine 7.4 μmol/L, free carnitine 5.6 μmol/L, acylcarnitine 1.8 μmol/L and glucose 13 mg/dL. Due to feeding difficulty, the patient, however, had been administered parenteral elementary nutrition through a feeding tube since early infancy. The commercially available parenteral nutrition solutions do not contain carnitine. A secondary carnitine deficiency followed by non-ketotic hypoglycemia-related heart failure may readily develop even in a patient without valproic acid, during high fever.
Key words
Carnitine - measles vaccination - severe motor and intellectual disabilities - hypocarnitinemia - parenteral elementary nutrition
References
1
American Academy of Pediatrics Committee on Infectious Diseases .
Recommended childhood and adolescent immunization schedule-United States, January-June 2004.
Pediatrics.
2004;
113
142
2
Coulter DL.
Carnitine deficiency: a possible mechanism for valproate hepatotoxicity.
Lancet.
1984;
24
689
3
Feller AG, Rudman D, Erve PR, Johnson RC, Boswell J, Jackson DL, Mattson DE.
Subnormal concentrations of serum selenium and plasma carnitine in chronically tube-fed patients.
Am J Clin Nutr.
1987;
45
476-483
4
Igarashi N, Sato T, Kyouya S.
Secondary carnitine deficiency in handicapped patients receiving valproic acid and/or elemental diet.
Acta Pediatr Jpn.
1990;
32
139-145
5
Krageloh-Mann I, Helber A, Mader I, Staudt M, Wolff M, Groenendaal F, DeVries L.
Bilateral lesions of thalamus and basal ganglia: origin and outcome.
Dev Med Child Neurol.
2002;
44
477-484
6
Matsuo M, Saiki K, Momota T, Ishida A, Kanazawa K, Murakami R. et al .
Transient carnitine-responsive medium-chain dicarboxylic aciduria in an infant with cholestasis, hypoglycemia and cardiac failure.
Acta Pediatr Jpn.
1989;
31
211-215
7
Plioplys AV, Bagherpour S, Kasnicka I.
L-carnitine as a treatment of lethargy in children with chronic neurologic handicaps.
Brain Dev.
1994;
16
146-149
8
Schafer J, Riechmann H.
Subnormal carnitine levels and their correction in artificially fed patients from a neurological intensive care unit: a pilot study.
J Neurol.
1990;
237
213-215
9
Stanley CA, Hale DE, Coates PM, Hall CL, Corkey BE, Yang W. et al .
Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels.
Pediatr Res.
1983;
17
877-884
10
Vianey-Liaud C, Divry P, Gregersen N, Mathieu M.
The inborn errors of mitochondrial fatty acid oxidation.
J Inherit Metab Dis.
1987;
10
159-200
Correspondence
K. Okanari
Department of Brain and Nerve Science, Pediatrics
Faculty of Medicine
Oita University
1-1 Idaigaoka
Hasama
Oita
879-5593 Yufu
Japan
Telefon: +81/97/586 58 33
Fax: +81/97/586 58 39
eMail: o-kazuo@med.oita-u.ac.jp