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Biomedical subjects

G Geoffroy

Publications and source records attributed to G Geoffroy.

At least 73 records · Page 4Linked to original sources
PubMed1976

Hemodynamic findings in Friedreich's ataxia.

Thirteen patients with classical Friedreich's ataxia underwent cardiac catheterization with recordings of retrograde cardiac pressures, measurements of cardiac output and calculation of the left ventricular volumes and mass. The cardiomyopathy in Friedreich's ataxia falls into the hypertrophic group of cardiomyopathies with decreased compliance of ventricular myocardium, varying degrees of concentric and asymmetric hypertrophy and outflow tract obstruction. Although there is no clear parallel between the degree of abnormal hemodynamic findings and the degree of neurological impairment, severely handicapped patients may present a diffusely hypertrophied and hypokinetic left ventricular myocardium.

Adolescent↗
PubMed1976

Cardiac angiographic findings in Friedreich's ataxia.

Angiograms of 12 patients with typical Friedreich's ataxia were analyzed. The results corroborate previous reports and justify the conclusion that the cardiomyopathy is of the hypertrophic type. In 10 of 12 cases, the hypertrophy is concentric, and non obstructive. Less frequently (2 cases), this hypertrophy is accompanied by diffuse hypokinesis and depressed ejection fraction.

Angiocardiography↗
PubMed1976

Clinical laboratory findings in Friedreich's ataxia.

All clinical laboratory tests carried out in 4 groups of patients with the diagnosis of typical or atypical Friedreich's ataxia have been found to be within the normal range. In this prospective study of 50 patients, a number of findings previously reported to be abnormal in the literature, have not been confirmed.

Alanine Transaminase↗
PubMed1976

Glucose and insulin metabolism in Friedreich's ataxia.

Our prospective survey of 50 ataxic patients confirms the previous finding of frequent clinical or chemical diabetes in Friedreich's ataxia. Eighteen percent of our typical cases have clinical diabetes and 40% at least an abnormal glucose tolerance curve. However, this finding does not appear to be specific to that form of ataxia. Furthermore, we have shown that most patients with ataxia have normal or low fasting insulin levels, but a hyperinsulinic response to a glucose load.

Blood Glucose↗
PubMed1976

Amino acid metabolism in Friedreich's ataxia.

A study of amino acids determined by sequential Multi-sample Amino Acid Automatic Analyzer in plasma, urine and cerebrospinal fluid (CSF) in patients with Friedreich's ataxia and control subjects has revealed a number of mathematically significant variations from normal. Of practical physiological importance are the following: a high urinary excretion of alanine with slightly elevated plasma levels; a low plasma and CSF concentration of aspartic acid in the presence of normal urinary values and finally a low CSF concentration of taurine accompanied by normal plasma levels, but elevated urinary output and renal clearance rates. We postulate that the modifications in alanine and aspartic acid are less specific and probably secondary, but there could be a genetic defect in the membrane transport of taurine and the other beta-amino acids in Friedreich's ataxia.

Alanine↗
PubMed1976

Pyruvate metabolism in Friedreich's ataxia.

Friedreich's ataxia patients show evidence of an abnormally elevated and prolonged response of pyruvate and lactate to a glucose load, with normal fasting levels. However, ther is a bimodal distribution of this response with high and low pyruvate responders. This trait appears to be determined genetically, However, although in vivo tests suggest low oxidation of pyruvate, we were unable to confirm any in vitro impairment of each of the components of the pyruvate dehydrogenase (PDH) complex. We conclude that the defect is in the metabolic regulation of PDH, probably at the E3 (lipoamide dehydrogenase) step.

Alanine Transaminase↗
PubMed1975

Subacute sclerosing panencephalitis (SSPE).

Subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the central nervous system occurring in children and adolescents. The measles virus, or a virus closely associated, plays an important role in the disease. SSPE appears years after these children have had the measles but still a measles-like virus can be found in their brain and in other organs. A certain deficit, still to be defined of the cell-mediated immunity as a chronic stimulation of the antibody response against the measles virus is documented in SSPE pointing to a possible immunologic imbalance in these patients. Studying the possibility of the presence of immune complexes in SSPE, immunofluorescent studies of kidney biopsy in SSPE patients have been performed. Complement deposits in the glomerular basement membrane have been found in most patients. The possible role of immune complexes in SSPE is still to be defined.

Antibody Formation↗
PubMedDate not supplied

Spinal cord paralysis following sclerotherapy for esophageal varices.

A child with cryptogenic cirrhosis underwent a third session of elective sclerotherapy. Endoscopic therapy consisted of intravascular injection of ethanolamine oleate in varices newly developed at the midesophagus level. Irreversible paraplegia was documented within 8 hr postoperatively. Two years later she eventually died from gastrointestinal bleeding. Autopsy findings were compatible with an infarct of the spinal cord secondary to an occlusion of the anterior spinal artery. Various hypotheses which might explain the passage of the sclerosing material from the esophagus to the anterior spinal artery include: arterial occlusion secondary to venous thrombosis and spinal cord necrosis, accidental injection in an intercostal artery or azygous vein through the esophageal wall, the presence of a congenital arteriovenous fistula or the opening of arteriovenous shunt. Paravasal injection of dilute sclerosing agent might protect against this unusual but dramatic complication.

Arteries↗