Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The present report describes an infant with severe combined immunodeficiency and cartilage-hair hypoplasia whose lymphocytes responded to thymosin in vitro. Immunologic evaluation was undertaken at 4 1/2 months of age following a history of recurrent severe infection. Family history included three cousins who died in early infancy, one from streptococcal meningitis and pneumonia, one from generalized varicella, and another from reticuloendotheliosis. Quantitative immunoglobulins were markedly depressed: IgG 141, IgA 0, and IgM 24 mg/100 ml. There was an absolute lymphopenia, multiple skin tests were negative, and in vitro lymphocyte responses to mitogens and antigens were depressed. Spontaneous E rosette determinations were 21% compared with control values of 65.7%. Erythrocyte adenosine deaminase (ADA) activity was normal. The patient's E rosette formation increased in the presence of thymosin, fraction 5, reaching a maximum of 56% with a concentration of 500 mug thymosin. Blastogenic responses to phytohemagglutinin also increased in the presence of thymosin. Transplantation of 24-week fetal thymus in Millipore diffusion chambers and subsequently transplantation of 18-week fetal thymus by intraperitoneal injection was accomplished. E rosettes increased to 35-40% and blastogenic responses to mitogens increased. Eight days after the second transplant the patient underwent a mild graft vs. host reaction which subsided after 1 week and mitogen blastogenic responses again increased to 5-8 times previous values, but still well below control ranges. Repeated episodes of pulmonary infection ensued, cor pulmonale resulted, and the clinical course was relentlessly downhill with the patient expiring from respiratory failure 5 months after transplantation.
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PMID:Severe combined immunodeficiency with cartilage-hair hypoplasa: in vitro response to thymosin and attempted reconstitution. 99 98

Twenty-six patients with hairy cell leukemia (HCL) were treated with 2-chlorodeoxyadenosine (2-CdA), a purine analogue resistant to adenosine deaminase, at 0.1 mg/kg/d for 7 days by continuous intravenous infusion. Fifteen patients were previously untreated, while 11 patients had received prior treatment with splenectomy alone (three patients), interferon alpha alone (four), splenectomy, then interferon alpha (two), or splenectomy, interferon alpha, then 2-deoxycoformycin (2-DCF) (two). Sixteen (80%) of 20 patients evaluable at 3 months achieved complete remission (CR), and four (20%) achieved partial remission (PR) following a single cycle of therapy. All four patients in PR had complete recovery of their peripheral blood counts (except one patient whose platelet count remained 84,000/microL), but had residual HCL in the bone marrow (three patients) or residual splenomegaly (one). Patients with bulky adenopathy, massive splenomegaly, and severe pancytopenia responded as well as those with only modest marrow involvement. The three patients with residual marrow disease received a second cycle of 2-CdA, and two have attained CR. Therefore, 18 of 20 (90%) achieved CR with either one or two cycles of therapy. No patient achieving CR has relapsed at a median follow-up of 12 (+/- 2.1) months. Toxicities included myelosuppression and culture-negative fever. A community-acquired pneumonia was the only infectious complication. Since a single cycle of 2-CdA induces sustained CR in the vast majority of patients with minimal toxicity, this agent is emerging as the treatment of choice for all patients with HCL.
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PMID:A single cycle of 2-chlorodeoxyadenosine results in complete remission in the majority of patients with hairy cell leukemia. 135 62

Human adenosine deaminase (ADA; EC 3.5.4.4) consists of three isoenzymes: ADA1, ADA1+CP, and ADA2. We developed an electrophoretic technique to distinguish between these three isoenzymes. The isoenzyme pattern was studied in tissue and cell homogenates, as well as in serum from normal subjects and from patients with increased serum ADA who had either hepatitis, infectious mononucleosis, tuberculosis, pneumonia, rheumatoid arthritis, or acute lymphoblastic leukemia (ALL). The highest ADA activity was found in lymphocytes and monocytes. ADA2 could be detected only in monocytes (18% of total ADA activity). It was also the predominant isoenzyme in the sera of controls and all disease groups, except for ALL--the only condition evaluated that is not of an inflammatory nature. We conclude that serum ADA reflects monocyte/macrophage activity or turnover in most diseases studied. The exception is ALL, where serum ADA most probably originates from lymphocyte precursors.
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PMID:Serum adenosine deaminase: isoenzymes and diagnostic application. 162 98

We measured the activity of serum adenosine deaminase (ADA) in paired sera from 171 military conscripts with radiographically verified pneumonia. Patient serum samples were selected on the basis of serologic analyses identifying as single etiologic agents Streptococcus pneumoniae in 29 patients, Haemophilus influenzae in 7, Mycoplasma pneumoniae in 43, adenovirus in 24, influenza A or B in 12, and parainfluenza in 5 patients. In 14 patients Neisseria meningitidis and in 31 Chlamydia spp were considered the main etiologic agent. Compared with a control group of 45 healthy men, the ADA activity in patients with pneumonia was significantly higher (p less than 0.001) in all patient groups except those with meningococcal pneumonia. The highest ADA levels were seen in patients with pneumonia caused by M pneumoniae (27.4 +/- 9.7 U/L), Chlamydia spp (26.3 +/- 9.1 U/L), and adenovirus (28.5 +/- 10.9 U/L) compared with the controls (11.1 +/- 3.0 U/L). In patients with meningococcal pneumonia, the ADA activity was significantly decreased (p less than 0.001). Serum ADA activity probably reflects differences in cellular immune response to different infectious agents. The ADA determinations may give corroborative information on the etiologic agent of pneumonia.
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PMID:Serum adenosine deaminase in viral and bacterial pneumonia. 189 19

Bone marrow transplantation provides an important modality for "enzyme replacement" and the immune reconstitution of patients with adenosine deaminase (ADA) deficiency and severe combined immunodeficiency disease. We report a patient with ADA deficiency who develops severe varicella pneumonia 6 years after successful bone marrow transplantation and immune reconstitution. Marked abnormalities in T-cell mitogen responsiveness and pokeweed mitogen-induced polyclonal immunoglobulin synthesis occurred. Coculture experiments suggested the presence of increased suppressor activity. T-cell phenotyping showed decreased T3 and T4 subsets. These abnormalities slowly resolved over several months as the patient recovered from the varicella infection. ADA enzyme levels and metabolite concentrations in urine and erythrocytes remained unchanged. These findings, together with the chromosome and immune studies, suggested that the bone marrow graft remained intact. These studies indicate that immunologically reconstituted ADA-deficient patients may be at higher risk for complications related to varicella infection and suggest that the institution of preventive measures is important.
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PMID:Varicella pneumonia in a bone marrow-transplanted, immune-reconstituted adenosine deaminase-deficient patient with severe combined immunodeficiency disease. 298 24

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

Absent or severely reduced adenosine deaminase (ADA) activity produces inherited immunodeficiency of varying severity, with defects of both cellular and humoral immunity. We report somatic mosaicism as the basis for a delayed presentation and unusual course of a currently healthy young adult receiving no therapy. He was diagnosed at age 2 1/2 years because of life-threatening pneumonia, recurrent infections, failure of normal growth, and lymphopenia, but he retained significant cellular immune function. A fibroblast cell line and a B cell line, established at diagnosis, lacked ADA activity and were heteroallelic for splice-donor-site mutation in IVS 1 (+1GT-->CT) and a missense mutation (Arg101Gln). All clones (17/17) isolated from the B cell mRNA carried the missense mutation, indicating that the allele with the splice-site mutation produced unstable mRNA. In striking contrast, a B cell line established at age 16 years expressed 50% of normal ADA; 50% of ADA mRNA had normal sequence, and 50% had the missense mutation. Genomic DNA contained the missense mutation but not the splice-site mutation. All three cell lines were identical for multiple polymorphic markers and the presence of a Y chromosome. In vivo somatic mosaicism was demonstrated in genomic DNA from peripheral blood cells obtained at 16 years of age, in that less than half the DNA carried the splice-site mutation (P < .002, vs. original B cell line). Consistent with mosaicism, erythrocyte content of the toxic metabolite deoxyATP was only minimally elevated. Somatic mosaicism could have arisen either by somatic mutation or by reversion at the site of mutation. Selection in vivo for ADA normal hematopoietic cells may have played a role in the return to normal health, in the absence of therapy.
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PMID:Somatic mosaicism for a newly identified splice-site mutation in a patient with adenosine deaminase-deficient immunodeficiency and spontaneous clinical recovery. 802 52

We evaluated soluble interleukin-2 receptors (sIL-2R), neopterin and adenosine deaminase (ADA) in pleural effusions from 93 patients with tuberculosis, malignancies, uremia, pneumonia and other kinds of pleurisy. There were significantly elevated ADA (102.7 +/- 47 U/l) and sIL-2R (8,238 +/- 4,117 U/ml) values in tuberculous (TB) pleural fluids as compared with other non-TB pleural fluids (p < 0.005). The neopterin levels in pleural fluid were significantly lower in the cancer group (17.3 +/- 7.8 nmol/l; p < 0.005) and most strikingly elevated (309.4 +/- 112.2 nmol/l; p < 0.0001) in patients with uremic pleural effusions. Using cut-off values of 60 U/l in ADA and 5,000 U/l in sIL-2R, 92.0 and 86.9% of pleural effusions were TB in origin. Eighty-four percent of patients with malignant pleural effusions had neopterin levels less than 25 nmol/l.
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PMID:Neopterin, soluble interleukin-2 receptor and adenosine deaminase levels in pleural effusions. 804 18

A 64-year-old male consulted our hospital with a 6-day history of malaise, body temperature to 38 degrees C, anorexia, and light headedness. The chest radiograph showed consolidation in the left lower lung area. Chest CT and ultrasonography revealed left pleural fluid. The pleural fluid was exudative (yellowish in color, protein 3.0 g/dl, Rivalta reaction positive, adenosine deaminase 19.4 U/L), and had a total cell count of 4.7 x 10(6)/ml with 45% lymphocytes, 40% histiocytes, and 15% polymorphonuclear leukocytes. He had kept a budgerigar, but we could not isolate Chlamydia from the pleural fluid or the pet bird. Transbronchial lung biopsy from left S10 revealed an increase of mononuclear leukocytes within the interstitial spaces, and the alveolar spaces contained polymorphonuclear leukocytes, fibrin, and organized alveolar exudate. Bronchoalveolar lavage cellular constituents were 50% lymphocytes, 27% neutrophils, and 23% macrophages. Serologic studies demonstrated C. trachomatis specific IgM antibody titers at 1:20 in a serum sample and at 1:10 in pleural fluid. We report a case of community-acquired pneumonia caused by C. trachomatis diagnosed by serologic studies.
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PMID:[A case report of pneumonia due to Chlamydia trachomatis with pleural effusion]. 831 7

A prospective study was undertaken to assess the usefulness of serum adenosine deaminase (ADA) activity in the aetiological diagnosis of 75 patients (mean age 58 years) with community-acquired pneumonia who required hospitalization. Measurements of ADA were also carried out in 35 healthy subjects (mean age 52 years). The serum ADA activity in patients with typical bacterial pneumonia (TBP) was 21 +/- 7 IU/l and in controls 22 +/- 9 IU/l. In 43 patients with atypical pneumonia (AP), ADA levels (43 +/- 23 IU/l) were significantly higher than in the previously related groups (p < 0.001). Analysis within the group of atypical pneumonia showed significant differences for infections caused by Coxiella burnetii (61 +/- 19 IU/l, p < 0.001), Mycoplasma pneumoniae (44 +/- 26 IU/l, p < 0.001) and Legionella pneumophila (39 +/- 15 IU/l, p < 0.05), as compared with patients with bacterial pneumonia and normal control subjects. We conclude that serum ADA in patients with community-acquired pneumonia requiring hospitalization may provide useful additional diagnostic information on the aetiology of pulmonary infection.
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PMID:Adenosine deaminase activity in the aetiological diagnosis of community-acquired pneumonia. 925 91


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