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Biomedical subjects

J C Goldsmith

Publications and source records attributed to J C Goldsmith.

16 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

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↗
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↗
PubMed1979

Hemobilia complicating warfarin therapy.

This patient is the first case of endoscopically documented massive hemobilia exacerbated by inadvertent warfarin overdose. The true frequency of this disorder should become evident with the widespread use of endoscopy and a high index of suspicion.

Aged↗
PubMed1979

Congenital deficiency of blood clotting factors II, VII, IX, and X.

A patient congenitally deficient in factors II, VII, IX, and X has been further investigated after a follow-up of 15 yr. At birth, these factors, when determined by clotting assays, were undetectable. Following therapy with vitamin K1, the clotting activity of these factors rose but never exceeded 18% of normal. Immunologic assays revealed much higher levels of these factors than did clotting assays, thus suggesting that the vitamin-K-dependent factors were present in abnormal forms. Two-dimensional crossed immunoelectrophoresis showed that at least two forms of prothrombin were present in the patient's plasma. One form was similar to normal prothrombin; the other had the same mobility as acarboxyprothrombin. In addition, the majority of this fast-migrating peak was not adsorbable onto insoluble barium salts. These observations suggested that some molecules of the patient's prothrombin lacked the normal complement of gamma-carboxyglutamic acid residues. This observation was confirmed by a specific assay for gamma-carboxyglutamate. Since malabsorption of vitamin K, warfarin intoxication, and hepatic dysfunction were excluded as causes of this patient's syndrome, this rare congenital abnormality could represent either a defective gamma-carboxylation mechanism within the hepatocyte or faulty vitamin K transport.

Adolescent↗
PubMed1978

Purification and characterization of an abnormal factor IX (Christmas factor) molecule. Factor IX Chapel Hill.

Human Factor IX (Christmas factor) was isolated from the plasma of a patient with mild hemophilia B. The patient's plasma contained 5% Factor IX clotting activity but 100% Factor IX antigenic activity as determined by immunological assays, which included inhibitor neutralization and a radioimmunoassay for Factor IX. This abnormal Factor IX is called Factor IX Chapel Hill (Factor IXCH). Both normal Factor IX and Factor IXCH have tyrosine as the NH2-terminal amino acid. The two proteins have a similar molecular weight, a similar amino acid analysis, the same number of gamma-carboxyglutamic acid residues (10 gamma-carboxyglutamic acid residues), and a similar carbohydrate content. Both exist as a single-chain glycoprotein in plasma. The major difference between normal Factor IX and Factor IXCH is that the latter exhibits delayed activation to Factor IXa in the presence of Factor XIa and Ca2+. Thus, Factor IXCH differs from other previously described abnormal Factor IX molecules.

Adult↗
PubMed1977

Isolation and characterization of factor IX Chapel Hill: comparison to normal human factor IX.

A classification scheme for patients with hemophilia B has been devised. Assessment of factor IX antigen and factor IX coagulant activity has confirmed the genetic heterogeneity of hemophilia B and has shown that some affected patients have normal amounts of factor IX-like protein. An abnormal factor IX, factor IX Chapel Hill, has been isolated from one of these variants and compared structurally and functionally to the purified normal human factor IX molecule. The major difference between factor IX Chapel Hill and normal human factor IX appears to be defective activation of the abnormal molecule by factor XIa and calcium.

Factor IX↗