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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↗
PubMed1986

Delayed complications from fine-needle biopsies of solid masses of the abdomen.

A prospective study was conducted from 1977 through 1983, to determine the incidence of complications, particularly bleeding, after fine needle biopsy for suspected malignancy of the abdomen and pelvis. Hematocrits before and after the biopsy procedure, medical record follow-up, and review by a coagulation specialist were used to identify bleeders. We performed 395 biopsies on 360 patients. Thirteen percent of the patients had bothersome pain either during or after the biopsy. Hematocrit drops of 3% or more were found in 51 (12.7%) of the patients; nine were determined to be a direct result of the biopsy procedure. Our results and review of the literature suggest that there are complications, including delayed bleeding, yet few are life threatening. We caution, however, that adequate follow-up to identify bleeders is very important in patients having fine-needle biopsies of solid masses of the abdomen.

Abdominal Neoplasms↗
PubMed1986

Lupus anticoagulants.

Explore the source record for details and available documents.

Blood Coagulation Disorders↗
PubMed1984

Spontaneously acquired anti-factor VIII antibodies: report of a patient with adenocarcinoma of the prostate.

Anti-factor VIII antibodies, inherited or acquired inhibitors of the factor VIII molecule, have not been reported previously in the urological literature. Although more common in hemophiliac patients who have received multiple transfusions, this anticoagulant may be the cause of severe hemorrhage in nonhemophiliac patients. We describe a patient with carcinoma of the prostate and an unsuspected anti-factor VIII antibody, who experienced excessive postoperative bleeding and prolonged hospitalization following a vesicolithotomy and bilateral orchiectomy. A prolonged partial thromboplastin time and a significant decrease in measurable factor VIII clotting activity in a patient with no history of bleeding problems are essential clues in making the diagnosis of a factor VIII inhibitor. This coagulation defect is treated best with prothrombin complex concentrates, which contain vitamin K dependent clotting factors.

Adenocarcinoma↗
PubMed1983

Soft-tissue hemorrhage in hemophiliac patients. Computed tomography and ultrasound study.

Fifty-four hemophiliac patients underwent a total of 94 studies using computed tomography (CT), ultrasound, or both. Not only common bleeding sites such as the iliopsoas muscles but also several unusual sites were encountered: these included the iliac bone, bowel wall, mesentery, rectus abdominis muscle, retroperitoneum, bladder wall, and scrotum. Both modalities gave comparable results, and each was helpful in (a) establishing the diagnosis, (b) evaluating the extent of bleeding and its effect on adjacent organs, and (c) demonstrating regression after treatment.

Abdomen↗
PubMed1983

Hereditary dysfibrinogenemia in a patient with thrombotic disease.

A new case of congenital dysfibrinogenemia, in which the patient has severe thrombotic disease, is reported. The abnormal fibrinogen molecules are characterized by normal fibrinopeptide release with thrombin and defective polymerization in the formation of fibrin. Clotting times with ancrod and reptilase are significantly prolonged. All other coagulation tests (except those for fibrinogen function) are normal, and the patient has no other underlying disease. The apparent paradox of defective fibrinogen, which clots abnormally and is yet associated with thrombotic disease, can be explained by further analysis of the patient's fibrinogen. The two important functional properties of this fibrinogen are: (1) it forms fibrin gels that are extremely rigid, and (2) the fibrin is highly resistant to lysis by plasmin. Thus, although the abnormal fibrinogen forms defective clots, the fibrin that is formed cannot be removed by the fibrinolytic system. These results provide a molecular explanation for the thrombotic disease in this patient. This abnormal fibrinogen appears to have unique characteristics and has been designated as fibrinogen Chapel Hill Ill.

Adolescent↗
PubMed1983

Treatment of a high titer anti-factor-VIII antibody by continuous factor VIII administration: report of a case.

Daily administration of large doses of factor VIII concentrate in a hemophiliac with a high titer factor VIII inhibitor resulted in marked reduction in the titer and response of the inhibitor to factor VIII administration and made possible elbow and bilateral knee replacements under conventional factor VIII coverage. Studies performed during the course of treatment indicated that the reduction in the inhibitor was the result of specific tolerance to factor VIII.

Adult↗
PubMed1983

Autoplex versus proplex: a controlled, double-blind study of effectiveness in acute hemarthroses in hemophiliacs with inhibitors to factor VIII.

In view of uncontrolled observations and anecdotal reports suggesting that the activated PCC, Autoplex, was much more effective than standard (non-activated) PCC in controlling bleeding in hemophiliacs with inhibitors, a controlled double-blind study was designed to compare the effectiveness of Autoplex and Proplex. Acute hemarthrosis was chosen for study as this common but non-life-threatening lesion lends itself well to controlled study. A single dose of "unknown" product (Autoplex 75 FECU/kg; Autoplex 50 FECU/kg; or Proplex 75 FIX U/kg) was given, and effectiveness was judged at 6 hr. By all criteria of efficacy, there were no significant differences between the products. It is noteworthy that a single dose of PCC was judged effective in 50% of episodes, a figure that is consistent with other published clinical trials. In this model, no additional benefit was derived from using the activated PCC, Autoplex, in either dosage.

Blood Coagulation Factors↗
PubMed1983

The management of musculoskeletal problems in hemophilia. Part I. Principles of medical management of hemophilia.

Musculoskeletal bleeding in general and arthropathy in particular are the central problems among many in the two major forms of hemophilia: classic hemophilia, caused by factor VIII deficiency, and Christmas disease, caused by factor IX deficiency. Currently available replacement therapy, if properly used in a multidisciplinary setting, should provide significant benefit to hemophilic patients despite its cost and occasional complications.

Anemia, Hemolytic↗
PubMed1983

Obstetric care of the affected carrier of hemophilia B.

Hemophilia B (factor IX deficiency) is an X-linked recessive clotting disorder similar to hemophilia A. In both diseases, phenotypic expression of the disorder may occur in significant numbers of heterozygous carriers. This is presumably due to random inactivation of one of the two X chromosomes of the female carrier as described by the Lyon hypothesis. Described herein is the obstetric care of a severely affected heterozygous carrier of hemophilia B. Predelivery automated plasma exchange was used to raise factor IX levels successfully. Commercial factor concentrates were avoided. The Lyon hypothesis is discussed in detail, and recommendations are made for the care of the clotting factor-deficient gravid woman.

Adult↗
PubMed1982

Factor VIII inhibitors: a clinical overview.

There is much evidence to indicate that inhibitors to Factor VIII in patients with classical hemophilia are the result of an immunological response to exposure to material (VIII:C or VIII:CAg) that is absent or present in reduced amounts in these patients. The inhibitor is an antibody that is usually restricted in immunochemical composition and in many instances contains predominantly or exclusively gamma G3 or gamma G4 heavy chains. Exposure to Factor VIII in many inhibitor patients leads to typical anamnestic responses with marked increases in the level of the inhibitor. The tendency to develop inhibitors and the clinical characteristics of the inhibitor may be affected by genetic factors, basal levels of Factor VIII:C and/or VIII:CAg, and the nature and amount of the "immunizing" material. Currently accepted therapeutic modalities are aimed primarily at the management of acute bleeding episodes.

Adolescent↗
PubMed1982

Amyloidosis and factor X deficiency.

The association of amyloidosis and factor X deficiency has been reported 18 times. We have added a 19th case and compiled a summary of all previously reported cases. The pathogenesis of this association is discussed and therapeutic recommendations are offered based on the accumulated experience.

Adult↗