PubMed HealthSearch

Biomedical subjects

J T Meredith

Publications and source records attributed to J T Meredith.

10 recordsLinked to original sources

A case of glutaric acidemia type II (severe multiple acyl-CoA dehydrogenation disorder) with subsequent prenatal exclusion in a sibling.

A case of severe multiple acyl-CoA dehydrogenation disorder is described. This is the second such case reported to have had an elevated maternal serum alpha-fetoprotein and normal amniotic alpha-fetoprotein. The child's 3-day extrauterine life was characterized by intractable acidosis, respiratory distress, and ventricular fibrillation. The characteristic biochemical, morphologic, and microscopic findings of this condition are reviewed. A subsequent pregnancy was evaluated using chorionic villus sampling and analysis of cultured trophoblasts. The trophoblasts were biochemically normal, and a normal child was subsequently delivered. Since the manifestations of this disorder developed in utero, prenatal diagnosis and therapy offer the only hope for a more prolonged survival.

Acidosis

Cyanosis due to pulmonary arteriovenous malformation.

Cyanosis is frequently encountered in the neonate or infant. Most often it is due to congenital heart disease or primary lung disease. Pulmonary arteriovenous malformation is an unusual cause of cyanosis. Polycythemia and clubbing are associated findings. The treatment is excision or embolization if the disease is not too extensive.

Adult

Gastrointestinal arterial fibromuscular dysplasia of childhood.

An unusual case of a progressive, noninflammatory stenosing vasculopathy, arterial fibromuscular dysplasia is presented. The distinctive features of this particular case include onset in early childhood with a predominant involvement of the gastrointestinal system, sparing of the renal arteries, lack of hypertension, and no cutaneous features of progressive systemic sclerosis. We discuss the clinical history over a decade and the pathologic features, including routine and electron microscopic findings of biopsy and autopsy tissues, and review the literature.

Adolescent

Toxoplasmosis of the central nervous system.

Toxoplasmosis is usually an asymptomatic infection, but symptomatic cases are becoming more common with the rise in AIDS cases and the increased number of patients receiving immunosuppressive therapy. Toxoplasmosis of the central nervous system occurs most often in immunocompromised hosts. Brain biopsy may be necessary for diagnosis. Pyrimethamine and sulfadiazine are active against T. gondii, but they do not affect the encysted organisms.

Adult

Lipoprotein-X.

Jaundice can occasionally present a medical dilemma. Is the jaundice due to a surgically correctable or medical condition? One method to resolve this problem is the determination of levels of serum lipoprotein-X. A review of the literature concerning lipoprotein-X and pertinent clinical trials suggests that the determination of levels of serum lipoprotein-X, by itself, is of little value, but measurement after a two-week course of cholestyramine therapy can provide significant information regarding the presence of extrahepatic obstruction.

Cholestasis, Extrahepatic

Perimetry, visual evoked potentials and visual evoked spectrum array in homonymous hemianopsia.

Confrontation field examination, perimetry, visual evoked potentials (VEPs) to pattern hemi-field stimulation and visual evoked spectrum array to flash stimulation were compared in 50 patients with homonymous hemianopsia. Visual field could be examined by confrontation in all patients and demonstrated the field defect in 96% of cases. Goldmann perimetry could only be performed in 60% of patients, but always quantitatively defined the margins of the defect. VEPs could only be tested in 77% of patients and demonstrated an abnormality in 79% of cases. VEPs were absent to stimulation of the affected hemifield in every case of homonymous field defects with macular splitting. Visual evoked spectrum array could be tested in 95% of patients and revealed abnormalities in 67% of cases. The relative value of each test is discussed.

Adult

Pattern-reversal visual evoked potentials and retinal eccentricity.

The effect of stimulation of discrete areas of the retina on visual evoked potentials (VEP) was studied in 16 normal volunteers. The stimulus consisted of a constant luminance 2 degrees 18' field containing checks of 34'30' reversing at a frequency of 500 msec. The amplitude of the VEP was highest at the fixation point and inside the 2 degrees isopter. Rapid amplitude decrement was noted with stimuli located within the 2-4 degrees isopters. No identifiable response was obtained outside the 4 degrees or 6 degrees isopter with a 2 degrees 18' stimulus. VEP could, however, be elicited by increasing the size of the stimulus. The smallest size of a field required to evoke a detectable response also varied in relations to retinal eccentricity. Stimulation at 0 degree, 8 degrees and 14 degrees horizontal eccentricities with fields of a size estimated to activate an equivalent amount visual cortex produced VEPs of similar amplitude.

Electroencephalography