Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.5.4.4 (adenosine deaminase)
 5,136 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate their role as a form of replacement therapy, frozen irradiated red blood cells were administered to a child with adenosine deaminase deficiency associated with severe combined immunodeficiency disease. In vitro lymphocyte responses to mitogens and allogeneic cells were restored. Subsequently, a "thymus shadow" appeared, and immunoglobulin synthesis was demonstrated. Frozen irradiated plasma, which alone had no effect on lymphocytes numbers or responses, promoted lymphocytosis when given with frozen irradiated red blood cells. The patient received the transfusions with or without irradiated plasma at four-week intervals and remained free of infection for 17 months. The patient's lymphocyte adenosine triphosphate levels were elevated before therapy, which consistently reduced them without altering the lymphocyte adenosine deaminase activity. Enzyme replacement therapy may provide a way to treat patients with adenosine deaminase deficiency associated with severe combined immunodeficiency disease who do not have histocompatible bone-marrow donors.
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PMID:Enzyme replacement therapy for adenosine deaminase deficiency and severe combined immunodeficiency. 98 79

Lymphocytes from patients with acute and chronic T-cell malignancy or chronic T gamma lymphocytosis were characterized by studying the activity of three enzymes involved in purine metabolism and by determining the isoenzyme pattern of lactate dehydrogenase (LDH) in addition to analysis of surface marker expression with monoclonal antibodies. Four clinically different types of disease were distinguished on the basis of the enzyme parameters. Lymphocytes from patients with acute lymphocytic leukemia (T-ALL) showed an enzyme profile similar to that of normal thymocytes, i.e., an elevated level of adenosine deaminase (ADA) activity as compared with normal T lymphocytes, reduced activities of purine 5'nucleotidase (5'NT) and purine nucleoside phosphorylase (PNP), and a binomial distribution of the LDH isoenzyme pattern. Cells from "null"-ALL patients had an ADA/PNP ratio that was intermediate between that of normal T cells and that of T-ALL cells or thymocytes, but their 5'NT activity and LDH isoenzyme pattern were thymocyte-like. Patients with chronic T-cell proliferation were subdivided into those with chronic T gamma lymphocytosis and those with proven chronic T malignancy. The lymphocytes from these patients had ADA and PNP activities within the ranges of those of normal T lymphocytes. However, the ADA activity and/or the ADA/PNP ratio were consistently higher in the cells from the patients with chronic T gamma lymphocytosis than in those with chronic T malignancy. The enzyme profile of the cells from the T gamma patients was similar to that of T gamma cells of normal individuals. The cells from patients with chronic T malignancies showed a heterogeneous enzyme pattern as compared with that of normal T lymphocytes. Analysis with monoclonal antibodies enabled us to distinguish null-ALL patients from the other leukemias studied, but a distinction between chronic and acute T-cell proliferation disease, for instance, was not possible with monoclonal antibodies alone. Our data demonstrate that the enzyme profiles studied provide supplementary information for classification and diagnosis of lymphoproliferative diseases to that obtained with cell surface markers alone.
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PMID:Enzyme analysis of lymphoproliferative diseases: a useful addition to cell surface phenotyping. 630 82

In order to study if enzymes of purine metabolism could be used as cell markers in B-chronic lymphocytic leukemia (B-CLL), the activities of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5'-nucleotidase (5'N) were repeatedly measured in blood mononuclear cells from B-CLL patients and were compared to those obtained in normal controls. Enzyme activities in patients were also compared to other biological parameters indicative of B-CLL to activities of ADA and PNP in erythrocytes. Results show that B-leukemic cells display abnormal enzyme patterns: subnormal ADA activity is characteristic; 5'N activity is depressed in 60% of the cases but increased in 15%. An inverse relationship between PNP activity and corresponding lymphocytosis is observed in leukemic but not in normal cells. The enzymatic anomalies seem to be linked to the presence of an unusual peripheral lymphocytic population, induced by the leukemic process. Indeed, ADA and PNP are not abnormal in erythrocytes. In untreated nonevolutive patients, the enzyme profile tends to remain stable throughout the course of the illness; normalization of enzyme patterns in treated patients occurs only when therapy induces improvement in T and B cell distribution.
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PMID:Enzymes of purine metabolism in B-chronic lymphocytic leukemia. 633 Nov 55

An open prospective clinical, microbiological, and molecular analysis of a national molecular diagnostic service for tuberculous meningitis (TBM) using an in-house IS6110-targeted PCR for molecular "Fastrack" diagnosis was carried out. Between April 1997 and June 1998. Consecutive cerebrospinal fluid (CSF) samples from 131 patients were assessed. Against a culture on the same sample, PCR had a sensitivity of 75% and a specificity of 94%. Of samples from patients classified as definite or probable TBM cases based on clinical criteria, 81% had raised CSF protein levels and 73% had a lymphocytosis, although 57% of all submitted samples showed a raised lymphocyte count. While only 46% had a CSF glucose level below the normal range, the CSF glucose level was significantly lower (P = 0. 0281) than in cases of meningitis of other etiologies. Levels of tumor necrosis factor alpha were also found to be significantly raised in definite or probable TBM cases (P = 0.028), while adenosine deaminase levels were not. The study showed IS6110-targeted PCR to be a rapid, sensitive, and specific test in routine use for the diagnosis of TBM.
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PMID:Role of IS6110-targeted PCR, culture, biochemical, clinical, and immunological criteria for diagnosis of tuberculous meningitis. 1097 Mar 48

We report a case of tuberculous pleurisy that required differentiation from pleurisy caused by Mycoplasma infection. A 28-year-old woman presented to a clinic with fever and pain on the left side of her chest. A chest radiograph revealed pleural effusion in the left thorax, and the condition was diagnosed as bacterial pleurisy. The patient was referred to our hospital because of an increase in the pleural effusion despite antibiotic treatment. Mycoplasma infection was suspected because the patient was young, the white blood cell count was not elevated, and the result of the ImmunoCard Mycoplasma test (IC) for Mycoplasma pneumoniae-specific IgM antibodies was positive. However, the fever persisted even after treatment with azithromycin and pazufloxacin. The left pleural effusion was exudative, with lymphocytosis and high adenosine deaminase (ADA) levels. The results of the QuantiFERON test were positive. Therefore, tuberculous pleurisy was diagnosed, and the effusion subsided after treatment with standard anti-tuberculosis chemotherapy. Although detection of Mycoplasma infection using the IC is rapid and simple, the accuracy of this test is poor. The patient was first diagnosed with pleurisy of Mycoplasma origin because of a single high-particle agglutination titer of 1: 320 and because of the presence of exudative pleural effusion with lymphocytosis and elevated ADA levels, which has been reported in patients with Mycoplasma infection. The results of the IC test and the ADA level of the pleural effusion might not be reliable when distinguishing between tuberculous pleurisy and pleurisy caused by Mycoplasma infection.
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PMID:[Case of tuberculous pleurisy distinguished from pleurisy caused by Mycoplasma infection]. 2381 19

We present a case of tuberculous meningitis (TBM), wherein pleural effusion developed as a manifestation of paradoxical reaction during anti-tuberculosis therapy. An 87-year-old diabetic man was referred to our clinic for fever and impaired consciousness. He did not obey vocal commands. No ocular motor deficit, facial palsy, or limb weakness was observed. He had hyponatremia due to inappropriate antidiuresis. Examination of the cerebrospinal fluid revealed lymphocytosis and high adenosine deaminase (ADA) activity, suggestive of TBM. He was treated with isoniazid, rifampicin, and pyrazinamide, after which his symptoms quickly resolved. Lymphocyte count, ADA activity, and protein concentration in the cerebrospinal fluid decreased. However, approximately 30 days after the initiation of therapy, he developed mild hypoxemia. A chest CT scan revealed pleural effusion. The pleural fluid was exudate with elevated ADA activity, which was consistent with tuberculous pleural effusion. Shortly after the use of a herbal medicine, Goreisan extract, hyponatremia and hypoproteinemia improved, and the pleural effusion was reduced. Approximately one-third of patients with TBM are reported to develop a paradoxical reaction, such as tuberculoma, hydrocephalus, and optochiasmatic and spinal arachnoiditis. The present case suggests that extra-central nervous system manifestations, including pleural effusion, should be considered when treating TBM.
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PMID:[A case of tuberculous meningitis with pleural effusion as a manifestation of a paradoxical reaction during anti-tuberculosis therapy]. 3134 Nov 31