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Biomedical subjects

W Bush

Publications and source records attributed to W Bush.

9 recordsLinked to original sources
PubMed2003

A model of mucopolysaccharidosis IIIB (Sanfilippo syndrome type IIIB): N-acetyl-alpha-D-glucosaminidase deficiency in Schipperke dogs.

Mucopolysaccharidosis III (MPS III) is characterized by lysosomal accumulation of the glycosaminoglycan (GAG) heparan sulphate (HS). In humans, the disease manifests in early childhood, and is characterized by a progressive central neuropathy leading to death in the second decade. This disease has also been described in mice (MPS IIIA and IIIB), dogs (MPS IIIA), emus (MPS IIIB) and goats (MPS IIID). We now report on dogs with naturally occurring MPS IIIB, detailing the clinical signs, diagnosis, histopathology, tissue enzymology and substrate levels. Two 3-year-old Schipperke dogs were evaluated for tremors and episodes of stumbling. Examination of the animals found signs consistent with cerebellar disease including dysmetria, hind limb ataxia and a wide-based stance with truncal swaying. There were mildly dystrophic corneas and small peripheral foci of retinal degeneration. Magnetic resonance imaging of the brain and skeletal radiographs were normal. Intracytoplasmic granules were found in the white cells of peripheral blood and cerebral spinal fluid, and in myeloid lineages in bone marrow. Electrophoresis of urinary GAGs indicated the presence of HS, while assays of cultured fibroblasts found N-acetyl-alpha-D-glucosaminidase (Naglu) activity of between 4.3% and 9.2% of normal. Owing to neurological deterioration, both dogs were euthanized, and post-mortem examinations were performed. Biochemical studies of liver and kidney from both animals demonstrated profound deficiency of Naglu activity and abnormally high GAG levels. Pathology of the brain included severe cerebellar atrophy, Purkinje cell loss, and cytoplasmic vacuolation in neurons and perithelial cells throughout the central nervous system. Pedigree analyses and Naglu levels of family members supported an autosomal recessive mode of inheritance. Using an obligate heterozygote, a breeding colony has been established to aid in understanding the pathogenesis of MPS IIIB and testing of potential therapies.

Acetylglucosaminidase↗
PubMed1988

Results of renal artery balloon angioplasty limit its indications.

Percutaneous transluminal balloon angioplasty (PTA) of 83 renal artery lesions in 55 patients was done because of renal failure in eight patients, hypertension in 35, renal failure and hypertension in 11, and polycythemia in one patient. Early results in 38 patients with arteriosclerosis showed five (13%) were worse and 13 (34%) were unchanged. Twenty patients (53%) with arteriosclerosis were initially cured or improved; however, seven of these patients had recurrence in 4 to 48 months. Ultimately, only 13 of 38 patients with arteriosclerosis (34%) were cured or improved (mean follow-up 22 months). Nine patients with fibromuscular dysplasia required 17 dilatations of arteries (three bilateral and five repeat), resulting in eight patients (89%) who were cured or improved. Selection of patients with hypertension by medical failure while receiving three or more hypertension medications or by lateralizing renal vein renin values yielded benefit in 17 of 26 patients (65%). Five of six patients with transplant stenosis of the renal artery and hypertension were cured or improved at mean follow-up of 18 months. Overall technical results of 83 artery dilatations were as follows: good, 58 (69%); fair, 10 (12%); poor or unsatisfactory, 16 (19%); these were judged with a blinded radiologic review. No patient suffered main renal artery thrombosis. There were 16 patients with complications of dilatation (morbidity rate of 29%). Nine patients subsequently had renal artery surgery from the same day to 64 days later with good results in all patients except one.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1980

Traumatic bronchial rupture.

Traumatic injury to the tracheobronchial tree requires prompt, accurate diagnosis for optimum surgical treatment. The radiologist is in a pivotal position either to suggest this diagnosis or to initiate further investigation to establish it. Three cases of traumatic bronchial rupture illustrate the spectrum of radiologic findings and document the value of tomography in confirming this diagnosis.

Adolescent↗
PubMed1979

Improved liquid-chromatographic determination of propranolol in plasma, with fluorescence detection.

We describe an analysis for propranolol in plasma, with use of reversed-phase "high-pressure" liquid chromatography and fluorescence detection. Pronethalol is used as the internal standard. The procedure, which involves extraction into an organic solvent, evaporation, and clean-up by micro-scale back extraction from hexane into an aqueous phase, is specific and sensitive. The detection limit is less than 6 microgram/L (2.3 X 10(-8) mol/L). Within-day and between-day coefficients of variation are 1.8 and 4.4%, respectively. Commonly used drugs, including procainamide, N-acetylprocainamide, and quinidine, do not interfere.

Chromatography, High Pressure Liquid↗
PubMed1978

Mammographic parenchymal patterns as a risk indicator for prevalent and incident cancer.

Different mammographic parenchymal patterns have been found to be associated with significantly different rates for the development of breast cancer in a screening program of self-referred women. These differences are qualitatively similar but of lesser magnitude than those in previous reports by Wolfe which were based on symptomatic women who had had a previous negative mammogram. In addition, this report indicates a small difference in the rate of breast cancer at first mammographic examination, using the same parenchymal classifications. These findings, coupled with other risk factors, may permit the concentration of mammographic screening on a smaller segment of the population at risk, thus improving the benefit to risk ratio.

Adult↗
PubMed1978

Diffuse interstitial pneumonitis. Clinicopathologic correlations in 20 patients treated with prednisone/azathioprine.

Twenty patients with diffuse interstitial pulmonary disease diagnosed by open lung biopsy received combined prednisone/azathioprine therapy. Twelve patients demonstrated improvement with therapy. Each patient's clinical presentation, roentgenologic features and pathologic findings were correlated with their therapeutic response. Patients with an illness of one year's duration or less had a more favorable response to therapy than patients with a greater than two year duration of illness. Patients with associated extrathoracic abnormalities (anemia, glomerulitis, hepatopathy) exhibited a better therapeutic response that those with only pulmonary disease. The biopsy material from each patient was quantitatively graded on 20 morphologic variables. Statistical analysis using multiple linear regression revealed that a single variable, degree of interstitial fibrosis, was more that 90 per cent accurate in separating those responsive to therapy from those who failed to respond. Patients who respond to treatment had less interstitial fibrosis. Neither the amount of alveolar septal inflammation nor intra-alveolar cellular reaction was discriminatory in predicting response to therapy. A beneficial response to therapy was reflected in both improved lung volumes and gas exchange. Eight patients appeared to have a selective beneficial effect from azathioprine.

Adult↗