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J Fair

Publications and source records attributed to J Fair.

5 recordsLinked to original sources

Factor XI deficiency acquired by liver transplantation.

Factor XI deficiency (the Rosenthal syndrome), an autosomal recessive genetic defect, was transmitted to a patient after orthotopic liver transplantation. The deficiency was manifested by an isolated prolonged activated partial thromboplastin time (aPTT) after surgery. Hematologic evaluation using specific factor analysis revealed an absolute deficiency of factor XI. Stored serum obtained from the organ recipient before transplantation showed normal factor XI levels. When the liver donor's family was questioned, it was discovered that he was of Ashkenazi Jewish descent and that he had a history of bleeding after dental procedures. Before his death from intracerebral bleeding, he was documented to have an isolated prolonged aPTT value. This case shows that potentially morbid genetic defects can be transmitted by organ transplantation. It also provides evidence confirming that the liver is the only site of factor XI production.

Factor XI Deficiency

Reduction of morbidity and mortality from biliary complications after liver transplantation.

Over a 4-yr period that began October 1, 1986, 103 orthotopic liver transplants were performed on 91 patients at the Johns Hopkins Hospital. Biliary reconstruction at the time of transplantation was performed in standard fashion by an appropriately trained member of the surgical team. Six (7%) patients developed biliary complications, which included three cases of common bile duct stricture and one case each of bile duct obstruction caused by biopsy-related hemobilia, biloma and a retained fragment of a T tube after removal. Five of the six patients were treated successfully by nonoperative interventional radiological procedures performed under local anesthesia with light intravenous sedation. Reoperative surgery (to remove the T-tube fragment from subcutaneous tissue) was required for only one patient, and no deaths (0 of 91) were attributable to biliary complications. All six patients are alive and well 6 to 33 mo after the operation with excellent liver function. Our findings would suggest that most biliary complications of orthotopic liver transplants are avoidable and that the few that do appear can usually be managed both safely and effectively by an interventional radiological approach.

Adult

New methodologies for studying the prevention of atherosclerosis.

To determine if multiple risk factor modification favorably alters the rate of progression of coronary atherosclerosis, 300 patients with established atherosclerosis have been randomized into a clinical trial; 155 to usual care and 145 to special intervention. All patients have medical/risk examinations at baseline and annually for 4 years. The special intervention patients undergo aggressive risk factor management with emphasis on lipoprotein modification, dietary management, smoking abatement, blood pressure control, weight loss and increased physical activity. To measure progression of atherosclerosis, a quantitative, computer-assisted coronary arteriographic system was developed to analyze the baseline and 4-year follow-up arteriograms. This procedure uses a catheter with a metallic calibration cylinder at its tip to determine absolute artery size and automated computer edge detection techniques to define the internal border of the artery. The analysis system detects artery borders using changes in cine film density and measures distances between these borders. For each segment the minimum, maximum and mean diameters are measured and percent stenosis and atheroma area calculated. This system provides precise and reproducible measures of coronary artery segment diameter. Using this technique, we estimate a 33% reduction in the rate of coronary artery progression over 4 years, defined as mean segment diameter, can be detected at a power of 0.80 and an alpha of 0.05 (one tailed test) with a sample size of 120 in each of 2 groups.

Coronary Angiography

Exercise management.

Aerobic exercise is receiving increasing emphasis as an important health promoting behavior. This paper provides a model for assessment and management of the healthy exercising adult. A data base is presented that enables the practitioner to determine the presence of risk factors and/or disease, to identify health problems which might modify exercise prescription and to collect information to individualize the prescription. The remainder of the paper details the elements of exercise prescription with a specific focus on patient education.

Adult