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Biomedical subjects

E D Gomperts

Publications and source records attributed to E D Gomperts.

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↗
PubMed1992

Arterial thrombosis and protein S deficiency.

A previously healthy boy had progressive painful discoloration of the lower extremities and was treated with exchange transfusion and anticoagulation, which were unsuccessful in arresting pedal ischemia; amputation of all of the child's toes was required. Studies of the patient and his parents resulted in a diagnosis of inherited protein S deficiency.

Amputation, Surgical↗
PubMed1992

The impact of clotting factor concentrates on the immune system in individuals with hemophilia.

All reported studies to date, whether comparative or not, have shown a tendency toward a slower decrease in CD4+ numbers in patients receiving very high-purity agents with the rapidity in fall-off being affected by level of CD4+ numbers (low CD4+ numbers decreasing more rapidly), age (older persons showing more rapid CD4+ cell fall-off), and anti-HIV therapy. Clearly, these observations need to be extended in numbers, and the high-purity agents should also be similarly compared with the very high-purity therapies.

Antigens↗
PubMed1991

In vivo recovery and survival of monoclonal-antibody-purified factor VIII concentrates.

In response to reports of discrepant in vitro assays of high-purity concentrates, a double-blind crossover study of in vivo recovery and half-life of two brands of monoclonal-antibody-purified factor VIII concentrates (Monoclate and Hemofil-M) was performed in 23 patients with hemophilia A. In vivo recoveries were close to values predicted from the labelled unitage when plasma samples were assayed by a one-stage method. When a two-stage assay was used, lower recoveries were calculated and the recovery with Hemofil-M was slightly but significantly lower than that with Monoclate. The concentrates were re-assayed in vitro by the two-stage method. Monoclate (which is assayed by the manufacturer using a two-stage method) contained 97% of the labelled potency and Hemofil-M (which is assayed by the manufacturer using a one-stage method) contained 81% of the labelled potency. Differences in in vitro and in vivo assay methods contribute to disparities between expected and observed factor VIII recovery. Clearance of Hemofil-M was significantly faster than that of Monoclate, but volume of distribution at the steady state, mean residence time, and plasma half-disappearance times of the two concentrates were not significantly different.

Adolescent↗
PubMed1990

Bone mineral density and its association with inherited protein S deficiency.

Two unrelated children with thrombotic disease associated with inherited protein S deficiency and osteopenia have been identified. Measurement of protein S yielded markedly reduced levels of free protein S (less than 12.5%) in both propositi, a normal level of total protein S (79%) in propositus #1, and markedly reduced level of total protein S (34%) in propositus #2. Bone densitometry measurements of the two children revealed trabecular vertebral bone mineral content below two standard deviations. This defect is associated with vertebral body compression fractures in propositus #2. Therefore, it is hypothesized that protein S deficiency is associated with abnormalities of bone mineral density.

Adult↗
PubMed1990

HIV infection in hemophiliac children: clinical manifestations and therapy.

The effects of human immunodeficiency virus (HIV) disease, with its progressive and multifaceted manifestations, have not yet been fully expressed in the hemophilic population. However, even at this phase of the disease, the effects of the viral illness on the growing hemophilic child are extremely complex and have only recently been recognized and studied. The management of these unfortunate children is difficult and demands understanding and insight to obtain maximum benefit from the limited therapeutic armamentarium at hand. This unsatisfactory situation will continue at least for the near future, requiring innovative research and meticulous medical care in order to help those affected. This review addresses current knowledge pertaining to hemophilic children infected with HIV-1 and covers information available on the epidemiology, clinical manifestations, and management of HIV and its complications.

Acquired Immunodeficiency Syndrome↗
PubMed1990

Age and human immunodeficiency virus infection in persons with hemophilia in California.

Thirteen hemophilia centers provide comprehensive care to approximately 90 percent of persons with hemophilia in California. For 1987, these centers reported patient human immunodeficiency virus (HIV) antibody status, age group, level of clotting factor deficiency, and hemophilia type on 1,438 persons with hemophilia A and B; HIV serologic status was known for 860 persons (59.8 percent) of whom 537 (62.4 percent) were HIV-antibody-positive. The HIV positivity rate increased with age after taking into account hemophilia type, clotting factor level and treatment center type. The three-year cumulative incidence of reported AIDS (acquired immunodeficiency syndrome) cases based on the number of HIV positive patients, was 11.6 percent. The cumulative incidence rate was 14.6 percent (54 of 370) for those patients over 20 years of age and 4.8 percent (8 of 167) for those under 21 years of age. Although a comparable distribution of the date of diagnoses of AIDS was seen by age group, there appeared to be a bimodal distribution in the rate of AIDS among the age groups, with the 6-12-year-olds and the 21 and older age groups showing higher incidence rates.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Growth failure in boys with hemophilia and HIV infection.

The growth of 22 boys with hemophilia, infection with human immunodeficiency virus (HIV), and lymphadenopathy, but not overt acquired immunodeficiency syndrome (AIDS) was evaluated. Three patients were found to have significant growth failure for 3-4 years with the onset after HIV infection. Extensive endocrine evaluation revealed that two of the three had neurodysregulation of growth hormone release with hyposomatomedinemia. None had classical growth hormone deficiency, thyroid deficiency, or evidence of malnutrition/malabsorption or other systemic illness. It appears that growth failure is not rare in boys with hemophilia and HIV infection and that this might be due to a direct effect on the physiologic secretion of growth hormone.

AIDS-Related Complex↗
PubMed1987

Assessment for evidence of non A-non B hepatitis in patients given n-heptane-suspended heat-treated clotting factor concentrates.

Nineteen patients, (2 adults, 17 children) with inherited bleeding disorders were infused with n-heptane-suspended-heated clotting factor concentrates. Twelve of the nineteen were previously untreated. Six patients were infused with Profilnine Heat-Treated and 13 with Profilate Heat-Treated. Five separate centers participated and were given various lots of concentrates for use. Blood from the seventeen children was sampled prior to entry, at infusion, 2 weeks, 6 weeks, 12 weeks and 6 months after the first infusion. The two adults were sampled every 2 weeks. Twelve of the 19 patients were followed beyond six months. Three patients demonstrated a rise in ALT during the first six months of observation with levels above 2.5 times the upper limit of normal. One of these patients showed a parallel increment in a-CMV IgM titer and a second patient, an adult, had previously received many units of single donor blood components. During the second 6 month observation interval, two patients showed a rise in ALT. One of these patients had been exposed to only one lot of concentrate with no other viral cause being determined. Two additional patients had a moderate increase in ALT up to 98 U/L (normal less than 50). No patients were clinically ill or showed jaundice during these episodes. The hepatitis episode at 11 months in the patient using one lot of concentrate, might suggest a non-viral mechanism in this instance. This study indicates that these concentrates may be associated with episodes of ALT above 2.5 times the upper limit of normal in approximately 20% of the patients treated, but the etiology of the raised ALT may not always be Non A-Non B hepatitis.

Adult↗
PubMed1986

PROPLEX vs. PROPLEX SX: a controlled double blind study of the effectiveness in treating acute hemarthroses in hemophilia A patients with inhibitors to factor VIII.

A blinded randomized multicenter trial of two non-activated prothrombin complex concentrates was carried out to determine the clinical effectivity in the treatment of acute hemarthrosis in hemophiliac patients with inhibitors. The one product was prepared via DEAE Sephadex chromatography, while the second was fractionated via various precipitation procedures including polyethylene glycol. Equivalence of the two products was established with less than 15% difference in efficacy rates.

Blood Coagulation Factors↗
PubMed1986

Procedures for the inactivation of viruses in clotting factor concentrates.

A considerable body of data has accumulated on the viral inactivated clotting factor concentrates. This information strongly indicates considerable safety and expected efficacy of these products. Probably most procedures are capable of inactivating large amounts of the LAV/HTLV-III virus, but there is considerable variability between products in the ability to inactivate the hepatitis viruses and perhaps also the LAV/HTLV-III. This variability is probably related to the mechanism of viral inactivation, the time of exposure to heat or chemical additives, and also the presence and type of stabilizer. So-called "second-generation" products will probably show improved elimination of more resistant viruses including the non-A, non-B forms.

Animals↗
PubMed1985

LAV/HTLV III presence in peripheral blood lymphocytes of seropositive young hemophiliacs.

Recent studies indicate a high prevalence of seropositivity to the lymphadenopathy-associated virus/human T-lymphotropic virus (type III) among individuals with hemophilia exposed to clotting factor concentrates prepared from large donor pools. The peripheral blood lymphocytes of 19 young seropositive patients with inherited bleeding disorders were examined for the presence of this virus by coculture with phytohemagglutinin-stimulated lymphocytes. Viral isolates were obtained from six of 19 patients. While none of these patients have developed the acquired immunodeficiency syndrome (AIDS) or AIDS-related complex, five of them had lymphadenopathy in two noncontiguous areas, and two showed clinically symptomatic enlarged tonsils and adenoids. Of the 13 patients in whom virus was not demonstrated, five were judged clinically normal and five had mild lymphadenopathy in one anatomical area. These results suggest that as many as 33% of hemophiliacs (six of 19 patients studied), who have circulating antibodies to mature viral proteins, have viral-infected peripheral blood lymphocytes capable of infecting other lymphocytes in vitro.

Adolescent↗
PubMed1984

Abnormal T-cell subsets and mitogen responses in hemophiliacs exposed to factor concentrate.

The acquired immune deficiency syndrome (AIDS) has been reported in hemophiliacs . We examined lymphocyte numbers and functions in 28 patients with hemophilia and 16 healthy adults. Healthy hemophiliacs who had received fewer than 150 commercial factor concentrate (CFC) infusions had normal T-cell numbers, T-cell subsets, and mitogen responses. Healthy hemophiliacs who had received more than 250 CFC infusions had a decreased number of Leu 3+ "helper" T cells, a decreased Leu 3-Leu 2 ratio, and decreased pokeweed mitogen responses. Hemophiliacs who had received greater than 250 CFC infusions and who had persistent lymphadenopathy had decreased Leu 3+ cells, increased Leu 2+ "suppressor" T cells, a decreased Leu 3-Leu 2 ratio, and decreased phytohemagglutinin responses. Abnormal results of T-cell studies were found in 67% of healthy hemophiliacs who had a high degree of CFC exposure and 100% of clinically abnormal hemophiliacs. These findings suggest that hemophiliacs are at risk for the development of the immunologic changes that characterize AIDS and that this risk is related to CFC exposure.

Acquired Immunodeficiency Syndrome↗