Gene/Protein Disease Symptom Drug Enzyme Compound
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 1,092,456 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The replacement of genetically deficient enzymes in patients with inherited metabolic disorders by infusion of purified enzymes or by organ transplantation has had very limited success, although good results with bone marrow transplantation have been obtained in some patients with mucopolysaccharidosis, Gaucher disease and inherited immunodeficiency diseases. Genetic engineering of the patient's lymphocytes may ultimately render these approaches redundant, at least for some of these diseases. Treatment of chronic pancreatic insufficiency and of disaccharidase deficiency with oral enzymes can be very effective; therapy can be monitored in the latter by measuring the breath hydrogen excretion and in the former by a range of tests of which stool chymotrypsin assay is the most convenient. Treatment of acute myocardial infarction by intracoronary perfusion of thrombolytic enzymes can improve both cardiac function and long-term survival if given early enough. Successful reperfusion can be identified by changes in the kinetics of serum enzyme release and clearance, especially for the isoenzymes and isoforms of creatine kinase. In cancer chemotherapy, L-asparaginase has long been a useful adjunct in the treatment of acute lymphoblastic leukemia, but recent experience suggests a role in acute nonlymphoblastic leukemia as well.
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PMID:Enzymes as agents for the treatment of disease. 157 79

The Acquired Immunodeficiency Syndrome (AIDS) has involved the pediatric age group and is especially prevalent in babies born of mothers who are intravenous drug abusers or prostitutes. Approximately 30% of children born to mothers who are seropositive for the human immunodeficiency virus (HIV) will develop HIV infection. There are several important differences in children and adults with AIDS. The incubation period of the disease is shorter, and initial clinical manifestations occur earlier in children. In addition, certain infections are more common in children, and the different types of malignancy, especially Kaposi's sarcoma, are unusual in the pediatric age group. The altered immune system involves both T cells and humoral immunity and increases susceptibility to a variety of infections, particularly opportunistic organisms. In this publication the complications of pediatric AIDS involving the lungs, cardiovascular system, gastrointestinal tract, genitourinary system, and neurological system are described. The most common pulmonary complications in our experience are Pneumocystis carinii pneumonia and pulmonary lymphoid hyperplasia. The spectrum of cardiovascular involvement in pediatric AIDS includes myocarditis, pericarditis, and infectious endocarditis. Gastrointestinal tract involvement is usually due to opportunistic organisms that produce esophagitis, gastritis, and colitis. Abdominal lymphadenopathy is a common finding either due to disseminating Mycobacterium avium-intracellulare infection or nonspecific lymphadenopathy. Although cholangitis is more commonly seen in adults, it may occur in children with AIDS and, in most cases, is due to related opportunistic infections. Genitourinary infections may be the first evidence of HIV disease. Cystitis, pyelonephritis, renal abscesses, and nephropathy with renal insufficiency are complications of pediatric AIDS. A variety of neurological abnormalities may occur in pediatric AIDS. The most common cause of neurological dysfunction in children with AIDS is HIV neuropathy. We present the many complications of AIDS in children demonstrated by a variety of imaging modalities, emphasizing the importance of diagnostic imaging in children with this disease.
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PMID:Radiology of AIDS in the pediatric patient. 157 31

Infection with the human immunodeficiency virus type 1 (HIV-1) results in a variety of pathological changes culminating in the acquired immune deficiency syndrome (AIDS). While most of these changes can readily be accounted for either by direct effects of HIV-1 on the immune system or by indirect effects of secondary infectious agents as a result of faulty immune surveillance, the direct cause for a number of disease states, including some neuropathies, myopathies, nephropathy, thrombocytopenia, wasting syndromes and increased incidence of cancers (primarily lymphoma) has remained an enigma. We have recently shown that the HIV-1 protease, a viral encoded enzyme necessary for virus maturation and infectivity, can cleave a variety of host cell cytoskeletal proteins in vitro. Potential substrates for the HIV-1 protease are found in all of the cell types affected in these unexplained diseases. Recent proposals suggest that elements of the cytoskeleton may play an important role in the regulation of large scale genetic regulation. We propose that some of the degenerative changes associated with infection by HIV-1 are a direct consequence of cleavage of host cell cytoskeletal proteins, which in turn may be responsible for the increased incidence of cancer in HIV-1 infected individuals as a result of the perturbation of the regulation of gene expression by cytoskeletal components.
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PMID:Potential role of the viral protease in human immunodeficiency virus type 1 associated pathogenesis. 158 3

A parent education booklet describing Pneumocystis carinii pneumonia (PCP) was prepared by the Pediatric Branch of the National Cancer Institute. In addition to information about prophylaxis and treatment of PCP, the booklet discusses overall care of children infected with human immunodeficiency virus.
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PMID:Pneumocystis carinii pneumonia (PCP) and your child: a parent information booklet. 159 71

The records of 18 immunocompromised patients with recent onset of pulmonary disease who had fibreoptic bronchoscopy and bronchoalveolar lavage over a two year period (1989-90) were reviewed. The underlying diseases were human immunodeficiency virus (HIV) infection (n = 7), organ transplantation (n = 9), and chemotherapy for malignancy (n = 2). Four patients were receiving prophylactic therapy and 12 had been started on empirical therapy for infection. Patients proceeded to bronchoscopy either because of atypical disease presentation or failure to respond to empirical therapy. Bronchoscopy with bronchoalveolar lavage was diagnostic in 13/18 (72%) patients and provided clinically useful information in 16/18 (89%). There was one diagnostic failure (6%); Pneumocystis carinii pneumonia in an HIV positive patient receiving nebulised pentamidine prophylaxis was missed. Transbronchial biopsies were not routinely performed and provided additional diagnostic information in only 1/6 (17%) patients. Overall, the commonest diagnoses were Pneumocystis carinii pneumonia (61%) and cytomegalovirus pneumonitis (28%). There were no complications of the procedures. In this highly selected setting of diagnostic or therapeutic uncertainty, fibreoptic bronchoscopy with bronchoalveolar lavage remains an effective and safe technique for evaluating pulmonary disease in immunocompromised patients.
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PMID:Fibreoptic bronchoscopy and bronchoalveolar lavage in the investigation of the immunocompromised lung. 159 42

The association of lung cancer and infection by the human immunodeficiency virus (HIV) is uncommon. This report and critical review of the medical literature defines a clinical profile of 22 patients affected with this uncommon association. This clinical profile includes young age (median, 38 years), intravenous drug abuse (14 of 22 patients), preponderance of adenocarcinoma over other cell subtypes (11 of 22 patients), and advanced clinical stage at presentation (10 of 15 patients with staging data had Stage III or IV disease). This study also examines a possible increased risk for lung cancer in patients infected by HIV. Continued surveillance and reporting of lung tumors (other than lymphomas and Kaposi sarcomas) in patients infected by HIV should help to define the frequency of the association and the validity of the clinical profile.
Cancer 1992 Jul 15
PMID:Lung cancer in association with human immunodeficiency virus infection. 161 92

Seeking ways to improve their health, gay men with human immunodeficiency virus (HIV) infections living in San Francisco are developing information networks and patterns of self-care behavior. Drawing from a set of explanatory theories, this cross-sectional survey with retrospective elements examined patterns and potential predictors of information-seeking activity in a cohort or 162 HIV seropositive (HIV+) men, 60 of whom provided complete data sets. The study suggests that 1 year after becoming aware of an HIV+ health status, most patients have developed multifaceted information networks. The amount of tangible aid acquired from these networks and frequency of consultation is positively related to patterns of HIV self-care behaviors and "feeling calm" (p less than 0.01), suggesting that these variables may be important markers for the need of supportive-educative nursing care. Suggestions for nursing practice and research are also described.
Cancer Nurs 1992 Apr
PMID:Potential predictors of information-seeking behavior by homosexual/bisexual (gay) men with a human immunodeficiency virus seropositive health status. 161 17

The human immunodeficiency virus type I (HIV-1) regulatory gene, tat III, is a powerful trans-activator of gene expression from the viral long terminal repeat and is essential for HIV replication. In addition, tat III protein has been shown to be immunosuppressive as indicated by the inhibition of antigen mediated T-cell proliferation. To further test whether tat III might play a direct role in the immunosuppressive effects of HIV-1 in addition to its role in virus replication, we examined the regulation of interleukin 4 (IL-4) receptors on a human B-lymphoblastoid cell line (Raji) transfected with HIV-1 tat gene (Raji-tat III). We used radioligand receptor binding analysis for cell surface expression and Northern blot analysis for the expression of human IL-4 receptor gene in Raji-tat III cells. Control Raji cells expressed 1383 +/- 361 (SE; n = 3) IL-4 binding sites/cell with a dissociation constant (Kd) of 144 +/- 27 pM (n = 3). However, Raji-tat III cells expressed about three times higher IL-4 receptors (4000 +/- 633 IL-4 binding sites/cell; P less than 0.03 compared to Raji cells) with a similar Kd of 273 +/- 90 pM (n = 3; P greater than 0.05 compared to Raji cells). Whereas both Raji and Raji-tat III cells exhibited a single mRNA species (approximately 4 kilobases) of IL-4 receptors by Northern blot analysis, the mRNA level was about 3-fold higher in Raji-tat III cells compared to Raji cells. Cycloheximide inhibited the expression of IL-4 receptors by 50% in about 2 h in both cell types indicating both the half-life of IL-4 receptors and the requirement for protein synthesis for the tat III up-regulation of IL-4 receptors. Since IL-4 under certain circumstances has been shown to be immunosuppressant, our observation that the HIV-1 tat gene up-regulates IL-4 receptors suggests the possibility that the immunosuppressive effects of HIV-1 are mediated at least in part through IL-4 receptors.
Cancer Res 1992 Jul 01
PMID:Human immunodeficiency virus type 1 tat gene up-regulates interleukin 4 receptors on a human B-lymphoblastoid cell line. 161 47

The lectin-like protein analogous to bovine conglutinin was purified from human serum. The carbohydrate-binding ability of conglutinin-like protein was inhibited by D-mannose, N-acetylglucosamine and L-fucose as well as by mannan-containing oligosaccharides. By applying a lectin-based ELISA system it was demonstrated that conglutinin-like protein binds to human immunodeficiency virus-1 (HIV-1) glycoprotein 120 (gp120) via its carbohydrate binding site. In vitro experiments with T-lymphoblastoid CEM cells revealed that conglutinin-like protein abolishes infection by HIV-1; a 50% cytoprotective concentration of 23.9 micrograms/ml was measured. These findings demonstrate that human conglutinin-like protein binds to HIV-gp120 and inhibits, under the described in vitro conditions, CEM cell infection.
Jpn J Cancer Res 1992 May
PMID:Inhibition of human immunodeficiency virus-1 infection by human conglutinin-like protein: in vitro studies. 161 96

Serological markers of hepatitis A, B, and Delta and human immunodeficiency viruses were studied in 25 children receiving cancer chemotherapy. Eighty-eight percent had pre-existing HAV immunity which was unaltered by chemotherapy. HDV infection was observed in 8% while HIV was conspicuous by its absence. Active HBV infection, observed in 76% of the children, was asymptomatic in the majority and was accompanied by a high incidence of HBe antigenaemia (57.9%) and its persistence. Pre-existing anti-HBs failed to prevent HBV infection recurrence, which was, however, transient and self-limiting. Multiple blood transfusions and repeated parenteral exposures appeared to be the possible sources of HBV acquisition. Transmission to close contacts was also observed. The study suggests that although HBV vaccine might not be protective against HBV infection in patients receiving cancer chemotherapy, it may prevent its persistence and thereby help in reducing chronic liver disease-related morbidity and a highly infectious reservoir. Strict HBV screening of blood donors, exclusive use of disposable equipment, and vaccination of close contacts of cancer patients is recommended, particularly in HBV endemic third-world countries.
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PMID:Infection with hepatitis A, B, delta, and human immunodeficiency viruses in children receiving cycled cancer chemotherapy. 162 14


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