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Biomedical subjects

E D Gomperts

Publications and source records attributed to E D Gomperts.

At least 55 records · Page 3Linked to original sources
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↗
PubMed1984

Induction of tolerance to factor VIII in a child with a high-titer inhibitor: in vitro and in vivo observations.

High dose factor VIII concentrate was infused over 12 months in a 3-year-old child with hemophilia A and a high-titer inhibitor. This regimen was successful in producing clinical tolerance to the factor VIII and was associated with a fall of inhibitor titer from 85 BU to less than 1 BU. Subsequently, a rise in inhibitor to peak at 3.5 BU correlated with a decrease in infusion dose. In vitro studies using a microdroplet assay for immunoglobulin synthesis in the presence of varying concentrations of factor VIII was carried out prior to the clinical trial. This system indicated that the patient's peripheral blood mononuclear cells were stimulated to release IgG by 0.01 U/ml factor VIII coagulant activity and inhibited at 0.9 U/ml FVIIIc. Lymphocyte subsets were monitored during the course of therapy. The ratio of Leu-3a+/Leu-2a+ (helper/suppressor) varied considerably, fluctuating between a peak of 4.4 and low point of 0.6, with the initial pretrial ratio being 1.2.

Antibodies↗
PubMed1983

Antithrombin III progressive function: a biochemical analysis.

Antithrombin III (AT III) was isolated by two procedures using polyethylene glycol-400 (PEG) precipitation as the first stage. The PEG supernatant (PEG-sup) was applied to a heparin-affinity chromatographic system and AT III-heparin cofactor (AT III-HCF) was isolated. The PEG precipitate (PEG-ppt) was separated by a Sephacryl S-200 column. Fractions were collected and those demonstrating maximum AT III antigen and progressive thrombin inhibition were pooled and reapplied to the washed Sephacryl S-200 column. Fractions were again collected and assayed via specific antisera for AT III, alpha 1-antitrypsin (alpha 1 AG), alpha 2-macroglobulin (alpha 2 M), and alpha 1-acid glycoprotein (alpha 1 AT). AT III antigen (AT III AGN) and progressive function were confined primarily to one peak containing virtually no alpha 2 M, a low level of alpha 1 AT, and moderate quantities of alpha 1 AG. The PEG-sup, PEG-ppt, AT III-HCF, and the fraction obtained after two passes across Sephacryl S-200 (S#2) were similar in that they showed reactivity with specific AT III antisera and demonstrated heparin cofactor activity. They differed, however, in that the PEG-sup and AT III-HCF demonstrated considerably reduced progressive antithrombin function assessed over 30 min. This function was present in the PEG-ppt and S#2 fractions and this progressive activity was potentiated by EDTA. AT III two-dimensional immunoelectrophoresis (2-DIE) in the presence of heparin of both the Sephacryl and heparin-affinity purified components were very different, with the Sephacryl-purified AT III AGN showing both a fast peak and a very prominent slow-moving hump. The AT III heparin affinity fraction showed primarily a fast component. Dilution of the S#2 and PEG-ppt fractions resulted in considerable loss of the progressive activity and also the slow-moving component on 2-DIE. On the basis of these observations, it is postulated that AT III purified by PEG precipitation is in an aggregated form and that aggregate formation and dissolution is associated with AT III progressive activity.

Antithrombin III↗
PubMed1981

Hepatocellular enzyme patterns and hepatitis B virus exposure in multitransfused young and very young hemophilia patients.

Thirty-eight children with severe hemophilia A, 11 years of age and under, were evaluated by initial and follow-up liver function tests (LFTs) in relation to age of onset of transfusion therapy. Each child had at least two complete evaluations within one year for a follow-up period of at least one year. The mean number of exposure days was 36 with a mean of 275 units of factor VIII per exposure day prior to initial LFTs. At initial testing, 30% of patients demonstrated antibody to HBsAg and 39--51% at least one abnormal serum enzyme level (AST, ALT, LDH). During an average follow-up period of 34.8 months, two children developed HBsAg-positive icteric hepatitis. Of those initially serologically negative for HBsAg or antibody, 44% became antibody-positive. Intermittent abnormalities of at least one serum enzyme were observed in 79% of the patient group, with 13% and 8% being persistently normal and abnormal. Eleven children born after January 1976, receiving only third-generation RIA-tested products for HBsAg, constituted a subgroup. Although only one child at first assessment had evidence of hepatitis B virus exposure, 55% had elevated ALTs, indicating considerable frequency of non-A, non-B hepatitis in this very young group.

Child↗
PubMed1981

Dialysis and renal transplant in a hemophiliac.

Hemodialysis was initiated in a mild-moderate hemophiliac at 15 years of age. Hematuria had been a frequent and persisting feature from the age of five years without documented cause. Anemia and proteinuria was first detected at 13 years. A cadaver donor renal transplant was carried out after three months of hemodialysis. Massive intravesical bleeding complicated the immediate post-transplantation period. The allograft rejected after three months and the patient was maintained for eight years on home hemodialysis. A second cadaver donor allograft was carried out at 23 years of age. Again, massive intravesical hemorrhage was a problem post-transplant. The allograft is currently functioning 27 months post-transplant. Factor VIIIc activities have fluctuated between 5% and 40% in the absence of factor infusions.

Adolescent↗
PubMed1979

An acquired inhibitor to factor XIII A case report.

An elderly woman presented with a postoperative bleeding problem, and a presumptive diagnosis of factor XIII (fibrin-stablilizing factor) deficiency was made. However, on further testing, an inhibitor to factor XIII was detected. She was on isoniazid (INH) therapy at the time. INH has been implicated in other patients who have developed inhibitors to factor XIII, and it was felt that this was the reason for this patient developing the inhibitor. The patient subsequently died from a cerebral haemorrhage, a well-described complication of factor XIII deficiency.

Aged↗