Published in J Inherit Metab Dis on January 01, 1984
Cardiac troponin I: its contribution to the diagnosis of perioperative myocardial infarction and various complications of cardiac surgery. Crit Care Med (2001) 2.26
Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients. Clin Chim Acta (1998) 1.81
Imipenem/vancomycin for Rhodococcus equi pulmonary infection in HIV-positive patient. Lancet (1991) 1.66
Recognition and management of fatty acid oxidation defects: a series of 107 patients. J Inherit Metab Dis (1999) 1.63
Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. Circulation (1999) 1.57
Monoclonal and oligoclonal gammopathies in heart-transplant recipients. Clin Chem (1991) 1.54
Simultaneous determination of serum neopterin and C-reactive protein as markers of infection in heart-transplant recipients. Clin Chem (1989) 1.52
HDL phospholipid content and composition as a major factor determining cholesterol efflux capacity from Fu5AH cells to human serum. Arterioscler Thromb Vasc Biol (1997) 1.51
Human trifunctional protein deficiency: a new disorder of mitochondrial fatty acid beta-oxidation. Biochem Biophys Res Commun (1992) 1.42
A patient with carnitine-acylcarnitine translocase deficiency with a mild phenotype. J Pediatr (1998) 1.39
Carnitine palmitoyltransferase deficiencies. Mol Genet Metab (1999) 1.34
Optimal conditions for prolidase assay by proline colorimetric determination: application to iminodipeptiduria. Clin Chim Acta (1982) 1.24
Improved fluorescent PCR-based assay for sizing CGG repeats at the FRAXA locus. Clin Chem Lab Med (1999) 1.24
A cell culture system for screening human serum for ability to promote cellular cholesterol efflux. Relations between serum components and efflux, esterification, and transfer. Arterioscler Thromb (1994) 1.19
Improved fluorometric determination of malonaldehyde. Clin Chem (1991) 1.15
Molecular characterization of 82 patients with pyruvate dehydrogenase complex deficiency. Structural implications of novel amino acid substitutions in E1 protein. Mol Genet Metab (2011) 1.10
Factors associated with increased serum levels of cardiac troponins T and I in chronic haemodialysis patients: Chronic Haemodialysis And New Cardiac Markers Evaluation (CHANCE) study. Nephrol Dial Transplant (2001) 1.08
Influence of biochemical alterations on arterial stiffness in patients with end-stage renal disease. Arterioscler Thromb Vasc Biol (1998) 1.07
Study of minimal inhibitory concentration of antibiotics on bacteria cultivated in vitro in space (Cytos 2 experiment). Aviat Space Environ Med (1985) 1.07
Role of HDL phospholipid in efflux of cell cholesterol to whole serum: studies with human apoA-I transgenic rats. J Lipid Res (1996) 1.04
Plasma prolidase activity: a possible index of collagen catabolism in chronic liver disease. Clin Chem (1984) 1.03
Carnitine-acylcarnitine translocase deficiency with severe hypoglycemia and auriculo ventricular block. Translocase assay in permeabilized fibroblasts. J Clin Invest (1993) 1.02
[In vitro activity of twenty antibiotics against Rhodococcus equi]. Pathol Biol (Paris) (1991) 1.01
Antibiotic activity in space. Drugs Exp Clin Res (1986) 0.99
Effect of lactation in a mother with galactosemia. J Pediatr (1989) 0.98
Prolidase and prolidase deficiency. Life Sci (1984) 0.97
Complete genomic structure and mutational spectrum of PHKA2 in patients with x-linked liver glycogenosis type I and II. Am J Hum Genet (1999) 0.95
Brief report: renal tubular acidosis in carnitine palmitoyltransferase type 1 deficiency. N Engl J Med (1992) 0.94
Normal serum gamma-glutamyl-transpeptidase activity identifies groups of infants with idiopathic cholestasis with poor prognosis. J Pediatr (1987) 0.94
Oxidative modifications of low-density lipoproteins (LDL) by the human endothelial cell line EA.hy 926. Experientia (1996) 0.92
Deoxyguanosine kinase mutations and combined deficiencies of the mitochondrial respiratory chain in patients with hepatic involvement. Mol Genet Metab (2005) 0.92
Two silent substitutions in the PDHA1 gene cause exon 5 skipping by disruption of a putative exonic splicing enhancer. Mol Genet Metab (2007) 0.92
Diagnostic value of serum gamma-glutamyl transpeptidase activity in liver diseases in children. J Pediatr Gastroenterol Nutr (1991) 0.92
Neonatal hyperammonemia caused by a defect of carnitine-acylcarnitine translocase. J Pediatr (1995) 0.91
Cholesterol efflux potential of sera from mice expressing human cholesteryl ester transfer protein and/or human apolipoprotein AI. J Clin Invest (1995) 0.90
Coronary calcification and its relation to extracoronary atherosclerosis in asymptomatic hypercholesterolemic men. The PCV METRA Group. Circulation (1992) 0.89
Changes in prolinase and prolidase activity during CCl4 administration inducing liver cytolysis and fibrosis in rat. Br J Exp Pathol (1987) 0.88
Human ApoA-IV overexpression in transgenic mice induces cAMP-stimulated cholesterol efflux from J774 macrophages to whole serum. Arterioscler Thromb Vasc Biol (2000) 0.88
Analysis of the relationship between triglyceridemia and HDL-phospholipid concentrations: consequences on the efflux capacity of serum in the Fu5AH system. Atherosclerosis (2001) 0.87
Very long chain acyl-coenzyme A dehydrogenase deficiency in two siblings: evolution after prenatal diagnosis and prompt management. J Pediatr (1997) 0.87
[Application of derivative spectrophotometry in biochemistry]. Ann Biol Clin (Paris) (1985) 0.87
Determination of dipeptides in urine. Clin Chim Acta (1974) 0.86
Behavior of bacteria and antibiotics under space conditions. Aviat Space Environ Med (1994) 0.86
Growth and division of Escherichia coli under microgravity conditions. Res Microbiol (1994) 0.86
Preliminary results of the "Antibio" experiment. Naturwissenschaften (1986) 0.86
Approach to the patient with a fatty acid oxidation disorder. Prog Clin Biol Res (1992) 0.86
Rapid diagnosis of long chain and medium chain fatty acid oxidation disorders using lymphocytes. Ann Clin Biochem (1995) 0.86
Identification of four novel mutations in patients with carnitine palmitoyltransferase II (CPT II) deficiency. Mol Genet Metab (1998) 0.85
A new DNA polymorphism in the 5' untranslated region of the human SREBP-1a is related to development of atherosclerosis in high cardiovascular risk population. Atherosclerosis (2001) 0.85
A cytotoxic electronegative LDL subfraction is present in human plasma. Arterioscler Thromb Vasc Biol (1996) 0.85
A method to screen for the antioxidant effect of compounds on low-density lipoprotein (LDL): illustration with flavonoids. J Pharmacol Toxicol Methods (1993) 0.84
Amino acid transport systems in the human hepatoma cell line Hep G2. Biochem Biophys Res Commun (1992) 0.84
Molecular analysis of carnitine palmitoyltransferase II deficiency with hepatocardiomuscular expression. Am J Hum Genet (1996) 0.84
A secondary respiratory chain defect in a patient with Fanconi-Bickel syndrome. J Inherit Metab Dis (2002) 0.84
[Lipoprotein lipase activity in human milk; inhibition in vitro of the glucuro-conjugation of bilirubin (author's transl)]. Clin Chim Acta (1975) 0.84
Thiamine responsive pyruvate dehydrogenase deficiency in an adult with peripheral neuropathy and optic neuropathy. J Neurol Neurosurg Psychiatry (2008) 0.84
Comprehensive cDNA study and quantitative analysis of mutant HADHA and HADHB transcripts in a French cohort of 52 patients with mitochondrial trifunctional protein deficiency. Mol Genet Metab (2011) 0.83
Lipid peroxidation abnormalities in hemodialyzed patients. Nephron (1993) 0.83
Faster estimation of reserve bilirubin binding capacity of serum from the neonate by thin-layer chromatography on sephadex. Clin Chem (1977) 0.83
Isolation and characterization of an abnormal alpha slow-moving high-density lipoprotein subfraction in serum from children with long-standing cholestasis. Clin Chem (1986) 0.82
Abnormalities of lipid metabolism and arterial rigidity in young subjects with borderline hypertension. Clin Invest Med (1994) 0.82
Cell density affects prolidase and prolinase activity and intracellular amino acid levels in cultured human cells. Clin Chim Acta (1985) 0.82
Plasma prolidase and prolinase activity in alcoholic liver disease. Clin Chim Acta (1988) 0.82
Defective galactose oxidation in a patient with glycogen storage disease and Fanconi syndrome. Pediatr Res (1983) 0.82
[The intermittent variant of leucinosis (study of a new case)]. Arch Fr Pediatr (1971) 0.82
Influence of HDL subfractions on erythrocyte aggregation in hypercholesterolemic men. PCVMETRA Group. Arterioscler Thromb (1994) 0.82
Carnitine/acylcarnitine translocase deficiency (neonatal phenotype): successful prenatal and postmortem diagnosis associated with a novel mutation in a single family. Mol Genet Metab (2001) 0.82
Preliminary results of Cytos 2 experiment. Acta Astronaut (1985) 0.82
A patient with lethal cardiomyopathy and a carnitine-acylcarnitine translocase deficiency. J Inherit Metab Dis (1995) 0.82
Fractionation of charge-modified low density lipoproteins by fast protein liquid chromatography. J Lipid Res (1991) 0.81
Biochemical diagnosis of hepatic glycogen storage diseases: 20 years French experience. Clin Biochem (1991) 0.81
Lens hexitols and cataract formation during lactation in a woman heterozygote for galactosaemia. J Inherit Metab Dis (1989) 0.81
Homocysteine decreases endothelin-1 production by cultured human endothelial cells. Eur J Biochem (1999) 0.81
[Hepatic glycogenoses. Introduction]. Ann Gastroenterol Hepatol (Paris) (1988) 0.81
Influence of uremia on polymorphonuclear leukocytes oxidative metabolism in end-stage renal disease and dialyzed patients. Nephron (1991) 0.81
[Biological and physiopathological aspects of hepatic glycogenoses]. Ann Gastroenterol Hepatol (Paris) (1988) 0.80
Association between plasma homocysteine concentrations and cardiac hypertrophy in end-stage renal disease. J Nephrol (1999) 0.80
[Congenital galactosaemia: an unusual presentation]. Arch Pediatr (2005) 0.80
Evaluation of FibroTest-ActiTest in children with chronic hepatitis C virus infection. Gastroenterol Clin Biol (2009) 0.80
[Study of the metabolism of tryptophane in various hepato-intestinal diseases. II. Frequency of anomalies in neoplastic diseases]. Rev Fr Etud Clin Biol (1967) 0.80
Endothelin and cardiovascular remodelling in end-stage renal disease. Nephrol Dial Transplant (1998) 0.80
Structural changes of high-density-lipoprotein apolipoproteins following incubation with human polymorphonuclear cells. Eur J Biochem (1994) 0.79
Use of platelets, mononuclear and polymorphonuclear cells in the diagnosis of glycogen storage disease type VI. J Inherit Metab Dis (1988) 0.79
Effects of alpha-tocopherol on antioxidant enzyme activity in human fibroblast cultures. Int J Vitam Nutr Res (1993) 0.79
[Value of derivative spectrophotometry for the determination of plasma and urinary hemoglobin. Comparison with the method using Allen's correction]. Ann Biol Clin (Paris) (1986) 0.79
Charge heterogeneity of LDL in asymptomatic hypercholesterolemic men is related to lipid parameters and variations in the ApoB and CIII genes. Arterioscler Thromb Vasc Biol (1998) 0.79
Metabolic effects of galactose on human HepG2 hepatoblastoma cells. Metabolism (1994) 0.79
[Effect of cycloleucine on renal uptake of dibasic amino acids and cystine]. Biomedicine (1976) 0.79
Diagnosis of carnitine acylcarnitine translocase deficiency by complementation analysis. J Inherit Metab Dis (1994) 0.79
High-density lipoprotein 3 physicochemical modifications induced by interaction with human polymorphonuclear leucocytes affect their ability to remove cholesterol from cells. Biochem J (1996) 0.79
Transport of L-histidine by human diploid fibroblasts in culture. In Vitro (1983) 0.79