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)

ATL is a unique T-cell malignancy first described by Takatsuki and colleagues in 1970s. We estimate that more than 300 patients a year have been detected in the endemic areas of Kyushu, Japan. The surface phenotype of ATL cells characterized by monoclonal antibodies is T3+, T4+, T8-, T11+ and Tac+. In all cases the serum is positive for anti-HTLV-I antibodies and the ATL cells contain the proviral DNA of HTLV-I. Variations in the clinical features of atypical ATL suggested a division of the spectrum of ATL into five types: acute; chronic; smoldering; crisis; and lymphoma. Typical ATL takes an acute course. The survival time is short, with 50% mortality within approximately 5 months. In general a poor prognosis is indicated by the elevation of serum lactate dehydrogenase, calcium, and bilirubin, as well as by high WBC. Smoldering ATL is characterized by the presence of a few abnormal cells (0.5%-3%) in the peripheral blood over a long period. Crisis in chronic or smoldering ATL means the progression of the disease to acute ATL. The lymphoma type of ATL is considered to be a form of T-cell-type non-Hodgkin's lymphoma in which malignant cells contain proviral DNA of HTLV-I. Screening of the sera from healthy adults for presence of the anti-HTLV-I antibodies revealed that 3.6% of healthy individuals in Kumamoto Prefecture, which is located in the middle of Kyushu, were HTLV-I carriers. Family studies showed that the routes of natural infection of HTLV-I are from mother to child and also from husband to wife. The borderline between the healthy carrier state and smoldering ATL remains unclear. Smoldering ATL is frequently diagnosed in patients with fungus infection of the skin, chronic lymphadenopathy, interstitial pneumonitis, chronic renal failure and strongyloidiasis. Five patients with ATL refractory to conventional chemotherapeutic agents were treated with 2'-deoxy-coformycin (DCF), a potent inhibitor of adenosine deaminase. Two patients showed a good response, and three were resistant to DCF. In addition our experiences with a concurrence of lymphoma-type ATL in three sisters and spontaneous remissions in a patients with chronic ATL will be referred.
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PMID:[Overview of ATL (adult T-cell leukemia) research]. 288 29

A first-born baby boy presented at age 3 months with persistent diarrhoea, failure to thrive, and recurrent bacterial and fungal infections. Severe combined immunodeficiency was demonstrated. A deficiency of adenosine deaminase (ADA) activity was suggested by the presence of extensive skeletal abnormalities, and the ADA activity in erythrocyte and leucocyte lysates was < 0.005 nmol/h per mg protein. Culture of ADA-negative peripheral blood mononuclear cells, together with purified calf ADA, did not alter the absent phytohaemagglutinin response. Treatment with immunoglobulin, pentamidine, and co-trimoxazole was started and a programme of ADA enzyme replacement, with infusions of plasma and frozen irradiated erythrocytes, was begun at age 4 months and achieved blood ADA levels in excess of 30 nmol/h per mg haemoglobin. Although resolution of the interstitial pneumonitis and skeletal abnormalities was observed, there was no evidence of immunological reconstitution. The patient died at age 17 months after a parainfluenza pneumonitis. Features of importance in predicting lack of benefit from enzyme replacement by erythrocyte infusion in ADA-negative severe combined immunodeficiency appear to be early clinical presentation with associated severe skeletal abnormalities, a very low level of residual ADA activity in peripheral blood mononuclear cells, and lack of effect of exogenous ADA on the absent in vitro mitogen response of ADA-negative blood mononuclear cells.
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PMID:Severe combined immunodeficiency and adenosine deaminase deficiency: failure of enzyme replacement therapy. 743 84

Measurement of the activity of adenosine deaminase (ADA) in pleural effusions has been reported to be useful in the diagnosis of tuberculous pleuritis. To determine whether ADA activity can also be used to diagnose miliary tuberculosis, it was measured in bronchoalveolar lavage fluid from 64 subjects: 6 patients with miliary tuberculosis, 21 patients with sarcoidosis, 15 patients with idiopathic interstitial pneumonia, 15 patients with other diseases, and 7 healthy controls. Mean ADA activity was 5.02+/-3.75 IU/L (mean+/-SD) in patients with miliary tuberculosis; 1.06+/-0.99 IU/L in patients with sarcoidosis; 0.21+/-0.43 in patients with idiopathic interstitial pneumonia; and 0.30+/-0.51 IU/L in healthy controls. The level in patients with miliary tuberculosis differed significantly from the others (p<01.01). All 6 patients with miliary tuberculosis, 3 patients with sarcoidosis, 1 patient with Wegener's granulomatosis, and 1 patient with lymphangitic carcinomatosis had levels of ADA activity in bronchoalveolar lavage fluid that exceeded 2.0 IU/L. With a cut-off value of 2.0 IU/L the sensitivity of this test to miliary tuberculosis is higher than that of other diagnostic aids. Measurement of ADA activity in bronchoalveolar lavage fluid may be of great value in the early diagnosis of miliary tuberculosis.
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PMID:[Measurement of adenosine deaminase activity in bronchoalveolar lavage fluids as a tool for diagnosing miliary tuberculosis]. 862 68

A 64-year-old woman, afflicted with rheumatoid arthritis, consulted our hospital because of her clinical deterioration. Her doctor started treating her with etanercept and prednisolone 10 mg/day but without preventive chemotherapy for tuberculosis, because her chest CT showed only mild interstitial pneumonia, and her tuberculin test showed a slightly-positive reaction. Her symptoms improved, but her chest X-ray showed infiltration after two and a half months treatment, and right pleural effusion after four and a half months treatment. A diagnosis of pulmonary tuberculosis and tuberculous pleuritis was made because of an increase of adenosine deaminase in pleural effusion. She was treated with isoniazid, rifampicin, and ethambutol, resulting in a successful clinical course. Her sputum culture was positive, and a nucleic-acid amplification of Mycobacterium tuberculosis complex was positive. When prescribing etanercept, we should pay close attention to the possibility of tuberculosis.
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PMID:[A case of pulmonary tuberculosis and tuberculous pleuritis during treatment with etanercept for rheumatoid arthritis]. 1926 May 44