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Biomedical subjects

M Cooper

Publications and source records attributed to M Cooper.

At least 289 records · Page 16Linked to original sources
PubMed1982

Combined vitamin A and E therapy prevents retinal electrophysiological deterioration in abetalipoproteinaemia.

Eight patients with abetalipoproteinaemia had the typical ocular, systemic, and laboratory findings of this disease. Combined therapy with vitamins A and E was administered, starting as early as the first day of life and as late as 26 years of age. The patients were followed up for 2-6 years. Electroretinography was undertaken in all cases and electrooculography in some. After initiation of vitamin A and E therapy no progression of disturbed visual function could be detected in any patient. These objective tests of retinal function demonstrated that the combined vitamin A and E therapy may be useful in arresting retinal deterioration in abetalipoproteinaemia.

Abetalipoproteinemia↗
PubMed1982

Abnormal high density lipoproteins of abetalipoproteinemia: relevance to normal HDL metabolism.

We investigated high density lipoprotein (HDL) subfractions in abetalipoproteinemia (ABL) using rate zonal ultracentrifugation. In ABL, HDL2 is the major subfraction, 65% of total mass compared to less than 10% in normal subjects with similar HDL levels. HDL2 and HDL3 in ABL (n = 3) are larger and lighter than in normals (n = 3), with mean diameters of 136 +/- 19 A and 100 +/- 12 A, respectively (as compared to 113 +/- 12 A and 86 +/- 11 A), and contained more apoprotein E. ABL-HDL2 and HDL3 particles contain 2- to 2.5-fold more cholesteryl ester molecules than normals. ABL-HDL can be modified towards normal HDL by allowing VLDL triglycerides to exchange for ABL-HDL cholesteryl esters, followed by addition of lipoprotein lipase and hydrolysis of the triglycerides. In addition, ABL plasma contains a previously undescribed small and spherical (61 +/- 8 A) protein-rich (63% by weight) HDL fraction, which we call ABL-HDL4. Our data suggest that absence of cholesteryl ester transfer to triglyceride-rich lipoprotein in ABL causes accumulation of abnormally large cholesteryl ester-rich particles.

Abetalipoproteinemia↗
PubMed1980

Gallium scan as a prognostic indicator in neuroblastoma.

Gallium-67 scan results were correlated with the long-term clinical course in ten children with neuroblastoma. The four patients whose primary tumor did not accumulate gallium have exhibited a significantly better survival time (mean duration 68.2 months) as compared to the six patients with gallium-positive tumors (mean duration 16.6 months, P < 0.001). This difference in survival was not explainable on the basis of accepted prognostic indicators, such as age of patient or stage of disease at initial diagnosis. These results indicate that gallium uptake may be a useful prognostic indicator in children with neuroblastoma, independent of other variables.

Adolescent↗
PubMed1979

Mexiletine. Use in control of chronic drug-resistant ventricular arrhythmia.

The antiarrhythmic efficacy of mexiletine hydrochloride was studied in ten selected patients with stable resistant ventricular ectopic activity in whom lidocaine, procainamide, and other antiarrhythmic agents were of no avail. Continuous Holter ECG tapes were analyzed before, during, and after mexiletine. Orally administered mexiletine was found to be very effective in treatment of drug-resistant ventricular arrhythmias, with total abolition of arrhythmia in six and satisfactory control in two additional patients. Long-term follow-up confirmed maintenance of antiarrhythmic efficacy, while biochemical and hematological screening demonstrated no abnormalities. The long therapeutic half-life of the drug allowed convenient dosage schedule and good compliance. Its efficacy, low frequency of side effects, and long half-life make it an agent of notable value in suppressing ventricular arrhythmias.

Adult↗
PubMed1979

The role of gallium-67 scanning in the clinical staging and preoperative evaluation of patients with carcinoma of the lung.

Gallium-67 scanning was evaluated in 100 patients with proved carcinoma of the lung. It was valuable in separating primary from secondary lung tumors, determining the extent of contralateral hilar or mediastinal lymph node involvement, and detecting distant organ metastases. In addition to multiplane whole-body Ga-67 tomographic scanning, colloid liver scans, bone scans, and computerized axial tomography scans of the brain were obtained to determine the presence of distant metastasis. The gallium scan detected 11 of 12 occult metastases and identified 7 of 7 liver, 9 of 14 brain, 4 of 4 soft tissues, 1 of 4 contralateral lung, and 9 of 11 bone metastases. The whole-body gallium scan accurately detected or excluded extrathoracic metastatic disease in 11 of 12 patients examined postmortem within three months of a gallium scan. An approach is recommended using gallium scanning along with chest roentgenograms for clinical staging and preoperative evaluation of patients with carcinoma of the lung. Specific organ scans should be reserved for the occasional symptomatic patient with a negative gallium scan or for clarification of an indeterminate gallium scan.

Bone Neoplasms↗
PubMed1979

Carcinoembryonic antigen: increased plasma levels in recessive epidermolysis bullosa.

Circulating plasma levels of the oncofetal antigen, carcinoembryonic antigen, were examined in 18 patients with various forms of epidermolysis bullosa. Carcinoembryonic antigen was markedly elevated in the plasma of 4 of 6 patients with recessive dystrophic epidermolysis bullosa and in 1 of 2 patients with recessive epidermolysis bullosa letalis. In contrast, patients with dominantly inherited forms of the disease, dominant epidermolysis bullosa simplex and dominant dystrophic epidermolysis bullosa, had normal levels of antigen. In the recessive patients, the plasma levels of carcinoembryonic antigen appeared to correlate with the severity of cutaneous involvement. Alternatively, it is possible that expression of carcinoembryonic antigen is genetically linked to certain forms of recessive epidermolysis bullosa or is part of a pleiotropic effect of the gene coding for the disease.

Adolescent↗
PubMed1979

Altered erythrocyte pyrimidine activity in vitamin B12 or folate deficiency.

The net activity of orotidylic pyrophosphorylase and decarboxylase, sequential enzymes which catalyse the formation of uridine monophosphate from orotic acid in de novo pyrimidine biosynthesis, has been evaluated in erythrocytes of patients with folate or cobalamin deficiency. In patients with normoblastic haemopoiesis and normal cobalamin and folate status a direct relationship exists between the maturity of the peripheral blood erythrocyte population, as indicated by G6PD activity, and net orotidylic activity. In contrast in cobalamin or folate deficiency this co-ordinate relationship is not observed and net orotidylic activity is relatively reduced. Fractionation of erythrocytes by centrifugation demonstrates that this inordinately low orotidylic activity consistently occurs in the young erythrocyte population and is reversed by specific replacement therapy. In vitamin B12 or folate deficiency an impressive array of evidence now exists to implicate altered folate metabolism for the observed alterations in purine and pyrimidine metabolism (Das & Herbert, 1976). Of these changes the cornerstone defect resulting in megaloblastic maturation is impaired methylation of deoxyuridine monophosphate to thymidine monophosphate (Hoffbrand et al, 1976). In this context the reduced serum uridine plus uracil levels in patients with vitamin B12 deficiency (Parry & Blackmore, 1976) and the haematological response of these patients to orotic acid therapy (Rundles & Brewer, 1958) are not readily explicable. Since the conversion of orotic acid to uridine monophosphate depends on the integrity of the coupled activities of orotidylic pyrophosphorylase and decarboxylase, this study has quantitated this capacity in peripheral blood erythrocytes in patients with vitamin B12 or folate deficiency.

Erythrocytes↗
PubMed1979

Swallowing-induced supraventricular tachycardia.

A rare form of paroxysmal supraventricular tachycardia (SVT), associated with swallowing solid food, is described. Repeated triggering of the arrhythmia during esophagoscopy was produced by mechanical stimulation of the anterior esophageal wall, 30 cm from the incisors. Treatment with verapamil abolished this tachycardia. Esophagoscopic examination, the first of its kind in this setting, aided the investigation.

Deglutition↗
PubMed1979

Hepatic clearance mechanism of Tc-99m-HIDA and its effect on quantitation of hepatobiliary function: Concise communication.

Parameters affecting the hepatobiliary clearance of Tc-99m N(2,6-dimethylphenyl carbamoylmethyl) iminodiacetic acid (Tc-HIDA) were evaluated in dogs. Competitive clearance studies, were performed with Tc-HIDA after infusion to plasma saturation levels of an anion, sodium sulfobromophthalein (BSP), and a cation, oxyphenonium. The results demonstrated that Tc-HIDA is transported through hepatocytes by a carrier-mediated organic-anion pathway. The data are consistent with an alteration of the elimination kinetics of Tc-HIDA induced by elevations in the serum bilirubin level, and it is predicted that serum bilirubin at some increased concentration will dominate the distribution and elimination kinetics of Tc-HIDA independently of hepatobiliary status. A quantitative description of liver function in terms of regional distribution and elimination rate constants will require either a pharmacokinetic model that expressly includes the effects of bilirubin, the development of new anionic hepatobiliary agents capable of displacing endogenous bilirubin from transport binding sites, or the development of new hepatobiliary agents that use a different clearance mechanism from that used by bilirubin.

Animals↗