N-carbamyl glutamate treatment in hyperammoniemia decompensated propionic acidaemia;Tratamiento con N-carbamil glutamato en acidemia propiónica descompensada con hiperamoniemia

Anales de Pediatria

Volumn 71, Issue 6, 2009, Pages 579-580

  Fernandez De Miguel S ^a   Gimeno Diaz De Atauri A ^a   Torres Peral, R ^a   Fernandez Carrion F ^a   Serrano Ayestaran O ^a  

^a HOSPITAL UNIVERSITARIO DE SALAMANCA   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

ARGININE; BENZOIC ACID; BIOTIN; CARGLUMIC ACID; CARNITINE; GLUCOSE; HYDROXOCOBALAMIN; INSULIN; ISOLEUCINE; METRONIDAZOLE;

CASE REPORT; DEHYDRATION; DERMATITIS; FEEDING DISORDER; HEMODIAFILTRATION; HOSPITAL ADMISSION; HUMAN; HYPERAMMONEMIA; LETTER; MALE; MUSCLE HYPOTONIA; NEWBORN; PROPIONIC ACIDEMIA; PROTEIN RESTRICTION;

GLUTAMATES; HUMANS; HYPERAMMONEMIA; INFANT, NEWBORN; MALE; PROPIONIC ACIDEMIA;

EID: 70450245378     PISSN: 16954033     EISSN: 16959531     Source Type: Journal    
DOI: 10.1016/j.anpedi.2009.08.007     Document Type: Letter

References (5)

1. C. Bachmann, S. Krähenbühl, and J.P. Colombo N-acetylglutamate synthetase deficiency: A disorder of ammonia detoxification N Engl J Med 304 1981 543 544
2. B. Gebhardt, S. Dittrich, S. Parbel, S. Vlaho, O. Matsika, and H. Bohles N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia J Inherit Metab Dis 28 2005 241 244
3. V. Levrat, S. Blanc, M. Schiff, C. Vianey-Saban, S. Boyer, and N. Guffon N-carbamoyl-L-glutamic acid (NCGA): A beneficial effect in the treatment of acute decompensation of organic acidurias (methylmalonic aciduria and propionic aciduria) with hyperammonemia? J Inherit Metab Dis 28 2005 81
4. V. Valayannopoulos, K. Mention, V. Guyot, D. Rabier, F. Lesage, L. Dupic, et al. Experience with carbamylglutamate treatment for neonatal hyperammoniemia J Inherit Metab Dis 28 2005 81
5. Jones S, Reed CA, Vijay S, Walter JH, Morris AA. N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia. J Inherit Metab Dis. Epub ahead of print 2008.