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A new patient with medium-chain dicarboxylic aciduria and suberyl glycinuria during an attack of acute illness is reported. When, inadvertently he was given medium-chain triglycerides for 2 days, the excretion of abnormal metabolites of medium-chain fatty acids increased and hepatomegaly became more pronounced. During remission a low excretion of the metabolites were observed. After 16 h of fasting hypoglycaemia was accompanied by an increase of urinary dicarboxylic acids and psi-hydroxyacids similar to that found on admission. Interestingly this urinary organic acid pattern persisted 8 h after intravenous administration of glucose. In a blood sample obtained after 16 h of fasting there was hypoketonaemia and increased levels of total free fatty acids, octanoic, decanoic and cis-4-decenoic acids. These biochemical data suggest the existence of a deficiency at the level of medium-chain acyl-CoA dehydrogenase.
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
April 1994,
European journal of pediatrics,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
August 1991,
Lancet (London, England),
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
January 1990,
Progress in clinical and biological research,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
January 1998,
Ryoikibetsu shokogun shirizu,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
January 2001,
Ryoikibetsu shokogun shirizu,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
January 1992,
Archives of disease in childhood,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
May 1989,
The New England journal of medicine,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
May 1989,
American family physician,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
April 1988,
Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme,
J A Del Valle, and
M J Garcia, and
B Merinero, and
C Pérez-Cerdá, and
F Roman, and
A Jimenez, and
M Ugarte, and
M Martínez-Pardo, and
C Ludeña, and
C Camarero
January 2015,
Maternal health, neonatology and perinatology,
