Search PubMed⌕ Search

Biomedical subjects

P M Blatt

Publications and source records attributed to P M Blatt.

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↗
PubMed1979

Detection of an abnormal plasma clot structure by a simple rigidity assay.

We report here on a patient whose abnormal fibrin clot was detected via the measurement of clot rigidity with a simple buoyant inner cylinder elastometer. The patient's clinical coagulation studies were all within normal limits except for prolonged thrombin and reptilase clotting times and high level of fibrin split products. The measured rigidity of the patient's clot was approximately ten times lower than that of a clot formed from normal pooled plasma. Light scattering studies indicated that this modified structure was not caused by a gross change in gel fiber morphology. Antithrombin activity was eliminated as a possible cause of the altered clot structure; this suggests the possibility of a modified fibrinogen. Abnormalities in the reptilase time and fibrinogen levels in two siblings support the hypothesis that the modification is an inherited defect. We suggest that the simple measurement of rigidity can be used rountinely to detect abnormalities in plasma clot structure. The screening for such disorders should be of importance to clinician, patient, and biochemical researcher.

Aged↗
PubMed1979

Thrombotic thrombocytopenic purpura: a pathology review.

A retrospective analysis of 12 patients with the diagnosis of thrombotic thrombocytopenic purpura was undertaken with a review of pathological material. The gingival biopsy was found to be a poor method of diagnosing thrombotic thrombocytopenic purpura. Pathological review also verified chronicity of disease. The pancreas was found to be the most severely involved organ, although serum amylase values did not always reflect pancreatic disease.

Adrenal Glands↗
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↗
PubMed1977

Treatment of anti-factor VIII antibodies.

Bleeding episodes in patients with hemophilia A with anti-factor VII antibodies are frequently difficult to treat. Factor VIII concentrates administered by continuous infusion or prothrombin complex concentrates (PCC) have been used for treatment. Hemophilia A patients with inhibitors who respond to factor VIII concentrates generally have low to moderate inhibitor titers (generally less than 20 Bethesda units). Those patients who receive PCC are quite difficult to evaluate but promising clinical responses have clearly been observed. This paper describes our experience with both modalities of therapy and will offer specific guidelines for such therapy.

Adult↗
PubMed1977

Liver biopsy in hemophilia A.

Hepatitis is a significant complication of the treatment of hemophilia A with factor VIII concentrates. Chronic liver disease in these patients is infrequently documented in the literature. The results of percutaneous liver biopsy, under the coverage of glycine-precipitated factor VIII, in six patients with hemophilia A who had the persistence of abnormal liver-function tests for at least 6 months, are described. Three patients had chronic active hepatitis, and three had chronic persistent hepatitis. No complications were encountered as a result of the biopsy procedure. These results suggest that percutaneous liver biopsy should be considered in patients with hemophilia A with continuously abnormal liver-function tests to establish a histologic diagnosis and to guide further therapy.

Acute Disease↗
PubMed1976

Antihemophilic factor concentrate therapy in von Willebrand disease. Dissociation of bleeding-time factor and ristocetin-cofactor activities.

Treatment of von Willebrand disease with two plasma antihemophilic factor (AHF) concentrates, cryoprecipitate and glycine-precipitated AHF, was compared. Both concentrates were equally effective in immediately raising the plasma levels of factor VIII, the factor VIII-related antigen, and the ristocetin-related von Willebrand factor (vWF) and in stimulating a secondary rise in plasma factor VIII. Given either concentrate, the vWF activity, the antigen, and factor VIII levels were normalized in a patient with von Willebrand disease. However, correction of the prolonged bleeding time and control of bleeding occurred only with the cryoprecipitate. The bleeding-time corrective factor and the ristocetin-related vWF or platelet-aggregating factor are dissociable, distinct activites.

Antigens↗
PubMed1976

Intravascular coagulation with use of human prothrombin complex concentrates.

Prothrombin complex concentrates are used in the treatment of the congenital bleeding disorders associated with Factors II, VII, IX, and X deficiencies. They have also been extensively used to treat acquired coagulation abnormalities secondary to vitamin K deficiency, warfarin ingestion, and various types of liver disease. The reported complications of prothrombin complex concentrates administration include hepatitis, anaphylaxis, and thrombosis. This paper documents the development of disseminated intravascular coagulation in association with the administration of prothrombin complex concentrates to patients with liver disease.

Adult↗
PubMed1976

Hemolysis caused by factor VIII concentrates.

Immune hemolytic anemia is a recognized complication of the use of factor VIII concentrates. Hemolysis is obscured often by the presence of active bleeding. Where hemolysis has been demonstrated, red blood cell (RBC) destruction has been attributed to anti-A antibodies found in the transfused material. We present two episodes of hemolysis associated with the use of factor VIII concentrate. In the first, a high titer of "immune" anti-A (1:256) was present in the factor VIII. In the second, the patient's RBCs were group B, and the hemolysis was caused by anti-B antibody in the factor VIII concentrate. In addition, the antibody titer in the material that was received was much lower than previously described. The RBC destruction presumably occurred because of the massive dosage of factor VIII concentrate administered on order to overcome a factor VIII inhibitor.

Adolescent↗
PubMed1975

Dominant inheritance of hemophilia A in three generations of women.

A bleeding diathesis is described which is phenotypically indistinguishable from hemophilia A and which has been transmitted as a dominant trait in three generations of women in a North Carolina kindred. The abnormal phenotype is characterized by clinical mildness and slightly abnormal clotting time, prothrombin consumption, and partial thromboplastin time. Bleeding time, platelet count, clot retraction, tourniquet test, and prothrombin time are normal. Concentration of factors I, II, V, VII, IX, X, and XII are normal, while factor VIII activity is reduced to 2%-5% of control values. De novo synthesis of factor VIII does not occur after transfusion; factor VIII-related antigen is normal; patients' plasmas aggregate platelets normally in the presence of ristocetin, and a typical protein pattern is seen when a chymotryptic digest of cryoprecipitate of the proband is examined by SDS-polyacrylamide gel electrophoresis. Six possible genetic explanations are entertained. Balanced X-autosomal translocation of hemophilia A heterozygotes has been excluded by cytogenetic analysis of metaphase chromosomes. Classes von Willebrand's disease (vWd) is probably excluded on the basis of the laboratory data, and extreme lyonization of hemophilia A heterozygotes on probabilistic grounds. The genetic possibilities which cannot be excluded include a previously unrecognized variant mutation at the vWd locus, a dominant mutation at the hemophilia A locus on the X chromosome, and dominant mutation at a hypothetical fourth locus involved in factor VIII synthesis and control.

Animals↗