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Biomedical subjects

J C Goldsmith

Publications and source records attributed to J C Goldsmith.

At least 19 recordsLinked to original sources
PubMed1992

Coagulation factor IX: successful surgical experience with a purified factor IX concentrate.

The use of plasma-derived coagulation factor concentrates has been marked by the transmission of viral agents. Infusions of factor IX complex concentrates have been additionally complicated by inappropriate thrombosis. Use of these concentrates in the neonate, in those with liver disease, and in surgical patients results in increased risk for this complication. Twenty patients have been infused with a purified coagulation factor IX concentrate for fall-off and recovery studies. A two-compartment model indicated an initial phase half-life of 4.06 +/- 2.86 hr and a beta phase half-life of 20.0 +/- 3.8 hr following the administration of AlphaNine, Coagulation Factor IX (Human). In vivo recovery was 62.7% +/- 13.8%, with an average factor IX coagulant level of 73% +/- 16% at 15 min after the infusion of a mean dose of 45 U/kg. Thirteen previously transfused patients with hemophilia B underwent major orthopedic or general or dental surgery using this purified factor IX. Operative outcomes were excellent in all patients. No excessive bleeding was noted. There was no laboratory or clinical evidence for a disseminated intravascular coagulopathy. The excellent surgical outcomes observed in this multitransfused group with biochemical evidence for active liver disease demonstrates the utility and safety of a purified coagulation factor IX concentrate.

Adult↗
PubMed1991

Widespread dissemination of Pneumocystis carinii infection in a patient with acquired immune deficiency syndrome receiving long-term treatment with aerosolized pentamidine.

Patients with acquired immune deficiency syndrome (AIDS) may be infected with many opportunistic pathogens, the most common of which is Pneumocystis carinii. P. carinii infection typically presents as a subacute pneumonia. However, rare cases of localized, extrapulmonary, and disseminated disease have been described. Standard therapy for P. carinii is systemically administered trimethoprim-sulfamethoxazole or pentamidine. These agents, however, frequently are associated with serious adverse effects. More recently, aerosolized pentamidine has been proposed as an alternative treatment for those who cannot tolerate standard therapy and as primary and secondary prophylaxis. Inhaled pentamidine is effective, but it is not without hazards. The authors describe a patient with AIDS who received long-term treatment with aerosolized pentamidine and yet died as a result of widely disseminated P. carinii infection.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Endoscopy in the diagnosis of gastrointestinal Mycobacterium avium-intracellulare infection.

Two cases of mycobacterium avium-intracellulare (MAI) infection in association with acquired immunodeficiency syndrome (AIDS) are presented to highlight the distinctive upper gastrointestinal endoscopic appearances: 2 X 4 mm diameter, white nodules with intervening erythema and hemorrhagic erosions covered the mucosa of the second part of the duodenum. Histological evaluation of these nodules revealed diffuse expansion of the lamina propria by macrophages that contained numerous intracellular and extracellular acid-fast organisms. We conclude that endoscopy with endoscopic biopsy may represent the most rapid and sensitive diagnostic tool available in this disease.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Atypical pulmonary diseases associated with AIDS.

We present three patients with pulmonary diseases not usually associated with AIDS. Early recognition and treatment allowed a favorable outcome. The cases are discussed and the literature is reviewed.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Pneumocystis carinii pneumonia complicating multiple myeloma.

Pneumocystis carinii pneumonia complicated the course of two patients with multiple myeloma. The diagnosis was established in both cases by bronchoalveolar lavage, which demonstrated the typical pneumocysts. Clinical and roentgenographic improvement in both patients was observed following a course of trimethoprim-sulfamethoxazole. One patient had lymphocyte subsets performed with a CD4/CD8 ratio of 0.8; both patients were HIV antibody-negative by ELISA. Both patients tolerated prophylactic TMP-SMX given concurrently with the subsequent chemotherapy for myeloma. We suggest that the immune defect seen in multiple myeloma may have placed these patients at risk for opportunistic infections such as P carinii pneumonia; however, as opposed to patients with AIDS, our patients tolerated therapy with TMP-SMZ quite well.

Bronchoalveolar Lavage Fluid↗
PubMed1990

Hickman catheter-induced thoracic vein thrombosis. Frequency and long-term sequelae in patients receiving high-dose chemotherapy and marrow transplantation.

One hundred sixty-eight bone marrow transplant recipients and 49 patients who received high-dose chemotherapy were evaluated for symptomatic thrombosis after Hickman catheter placement. The timing of thrombotic complications was different between these two groups, with the transplant group having a significantly lower thrombus-free survival by 28 days after catheter placement. By 100 days after placement the thrombus-free survival rates of the two groups were similar. The platelet count at time of catheter placement was significantly lower in the nontransplant group, and the thrombus-free survival was longer in patients whose catheter was placed when their platelet count was less than 150,000, suggesting that thrombocytopenia delays thrombotic complications. Placement of two Hickman catheters resulted in a 12.9% thrombosis rate (21 of 162 patients) and was significantly more likely to be associated with thrombosis than placement of one catheter. Long-term follow-up evaluation of patients treated without successful fibrinolytic therapy showed no residual symptoms of venous obstruction. In those patients presenting with concomitant catheter obstruction resulting from thrombosis, low-dose fibrinolytic therapy was successful in restoring catheter function 70% of the time. Placement of two Hickman catheters is associated with an inordinate incidence of thrombosis. Thrombocytopenia at the time of catheter placement may delay this complication. Thrombotic catheter obstruction can be treated successfully with low-dose fibrinolytic therapy. Even without fibrinolytic therapy, catheter-induced subclavian vein thrombosis rarely causes long-term disability.

Bone Marrow Transplantation↗
PubMed1990

Aspergillus infection of the central nervous system in patients with acquired immunodeficiency syndrome.

Infections of the central nervous system in patients with the acquired immunodeficiency syndrome are common. Of the many microorganisms that have been implicated, infection with Aspergillus is rare. We describe three patients with Aspergillus infection of the nervous system. Two patients had cerebral lesions due to Aspergillus flavus, and one patient had Aspergillus fumigatus infection of the spinal cord. Diagnosis of the infections was difficult, and therapy appeared to be ineffective.

Acquired Immunodeficiency Syndrome↗
PubMed1989

The use of purified clotting factor concentrates in hemophilia. Influence of viral safety, cost, and supply on therapy.

Treatment of hemophilia, although greatly improved in recent years, continues to be problematic owing to infectious complications of blood product replacement therapy. This report examines the therapeutic options presently available for the treatment of hemophilia, focusing on the potential for repeated viral exposure to influence the progression of infectious disease, decreased risks of viral transmission with blood products produced using newer viral inactivation procedures, higher economic costs of newer blood products, and the current inadequate supply of blood products in the United States.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Reversible agranulocytosis related to azidothymidine therapy.

Hematopoietic toxicity from Zidovudine (AZT) is fairly common, resulting in a requirement for red cell transfusions in up to 25% of patients. Reversible agranulocytosis occurred following approximately 1 week of AZT therapy in a man with AIDS. He recovered from the episode without incident. There was no evidence for underlying bone marrow dysfunction or an adverse drug reaction. Careful monitoring of AZT therapy continues to be of great importance.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Abnormal fibrinolysis in healthy male cigarette smokers: role of plasminogen activator inhibitors.

The extrinsic fibrinolytic system and its response to cigarette smoking was studied in five healthy male smokers 35-45 years old. Tissue plasminogen activator (t-PA) release in response to venous occlusion was intact both at 8:00 A.M. and 3:00 P.M. Acutely smoking two cigarettes neither stimulated fibrinolysis nor changed levels of t-PA or plasminogen activator inhibitors. Functional plasminogen activator inhibitor (PA-I) levels and euglobulin lysis times were higher in the smoking group than in a control group matched for age, sex, and body mass. Antigenic levels of PA-I 1, the PA-I derived from vascular endothelial cells and platelets, were similar in both groups. While smoking did not acutely alter fibrinolysis in chronic smokers, these individuals had a high frequency of abnormal fibrinolysis characterized by high levels of PA-I activity. This abnormality is due to either high specific activity of PA-I 1 or to the presence of other antigenically distinct plasminogen activator inhibitors. Abnormal fibrinolysis may be one mechanism contributing to the thrombotic diathesis of cigarette smokers.

Adult↗
PubMed1989

Fatal pericarditis due to Mycobacterium avium-intracellulare in acquired immunodeficiency syndrome.

A 27-year-old white male homosexual with AIDS presented 19 months after the initial diagnosis with persistent fever, marked dyspnea at rest, and severe substernal pain in the chest. A pericardial friction rub was auscultated, and an effusion was demonstrated echocardiographically. Pericardiocentesis yielded 220 ml of serosanguinous fluid. Special stains of the fluid for microorganisms were negative. A mycobacterial infection was suspected, and therapy with multiple antimycobacterial agents was initiated. Cultures of the fluid eventually yielded MAI. Despite therapy, cardiac function declined, and the patient died two months after presentation. Autopsy confirmed the diagnosis of chronic pericarditis due to MAI. Pericarditis due to MAI should be included in the differential diagnosis of cardiac dysfunction in patients with AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Pulmonary emboli in patients receiving chemotherapy for non-Hodgkin's lymphoma.

Combination chemotherapy has dramatically improved the prognosis of patients with intermediate and high grade histologic subtypes of non-Hodgkin's lymphomas. Treatment-related complications, however, are considerable, and a common problem encountered is respiratory distress or respiratory insufficiency. Usually these difficulties have been attributed to infectious etiologies or to chemotherapy-induced interstitial fibrosis, most often involving bleomycin. We describe five patients presenting with respiratory problems several weeks after the initiation of chemotherapy. These patients, who represent 3 percent of all patients treated with a single bleomycin-containing regimen for intermediate or high grade non-Hodgkin's lymphoma, were all initially thought to have chemotherapy-induced interstitial fibrosis but were found on subsequent evaluation to have pulmonary emboli. Of the three patients in whom pulmonary emboli were diagnosed antemortem, two had symptoms suggestive of pulmonary emboli and all were successfully treated and remained well and free of lymphoma for over 24 months. Two additional patients were diagnosed at autopsy. We suggest that pulmonary emboli may contribute significantly to the morbidity and mortality of patients undergoing chemotherapy for non-Hodgkin's lymphoma and recommend that patients presenting with respiratory difficulties be evaluated for pulmonary emboli.

Aged↗
PubMed1986

HTLV-III/LAV antibodies and virus in hemophilia patients in Nebraska: survey and initiation of a prospective study.

Thirty-nine patients from the Nebraska Regional Hemophilia Center were studied for the prevalence and titers of antibodies to HTLV-III/LAV, and for clinical symptoms of possible progression toward the acquired immunodeficiency syndrome (AIDS). Fourteen of 26 (54%) patients with hemophilia A were positive for HTLV-III/LAV antibodies as determined by immunofluorescence and Western blotting. Retrovirus was detected in cultured lymphocytes of two out of three seropositive individuals by reverse transcriptase assay and molecular hybridization with cloned HTLV-III/LAV probe. Of the twelve factor VIII-deficient patients who were seronegative, one had received only heat-treated factor VIII concentrates, six only cryoprecipitate or fresh frozen plasma, two prothrombin complex concentrates, one had not been transfused since 1983, and two had never been transfused. None of the patients treated only with factor IX concentrates, volunteer donor plasma, or cryoprecipitate had HTLV-III/LAV antibodies. In 12 of 14 seropositive hemophiliacs, titers of serum antibodies to HTLV-III/LAV ranged from 1:1,280 to 1:10,240, indicating a strong immune response against HTLV-III/LAV antigens and/or persistent infection with the virus. In spite of the possible exposure to HTLV-III/LAV during the past four years, none of the patients in this group had AIDS. Whether or not this group of patients has developed immunity to the AIDS retrovirus remains to be determined.

Antibodies, Viral↗
PubMed1979

A new treatment strategy for hemophilia B: incorporation of factor IX into red cell ghosts.

Although patients with severe hemophilia B have lifelong spontaneous hemorrhage and crippling hemarthroses, patients with 0.01--0.03 U/ml Factor IX activity have a milder disease state. The clinical condition of severely affected individuals could potentially be improved by prolonging the half-life of transfused Factor IX. The feasibility of incorporating Factor IX into red cell ghosts was suggested by resealing experiments with similar sized molecules such as albumin. We have prepared resealed red cell ghosts containing human Factor IX and X. Human red cells were subjected to hypotonic lysis at 0 degrees C, pH 6.0. Commercial prothrombin complex concentrate was dissolved in the lysing medium immediately prior to the addition of the red cells. After being returned to isotonicity, the red cell ghosts were annealed at 37 degrees C for 30 minutes and then washed extensively. When intact red cell ghosts were tested, no Factor IX or X activity could be demonstrated. After disruption of the red cell ghost membranes with 3M urea or 2% Triton X-100, the procoagulants could be quantitatively recovered. Similar recovery of the clotting factors could be demonstrated from the lysate and the early wash samples. Red cells from Factor IX and X deficient patients served equally well as those from normal subjects. Red cell ghosts prepared in similar fashion but not exposed to the procoagulants had negligible clotting activity. We have demonstrated that human clotting factors can be incorporated into red cell ghosts. The ability of this system to prolong the biological half-life of Factor IX is under investigation.

Erythrocyte Membrane↗