Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pneumocystis carinii characteristically causes pneumonia in patients with immunodeficiency disorders. It occurs most often in patients with malignancy or renal transplants whose immune response has been suppressed by corticosteroids or cytotoxic agents. Individuals with connective tissue disease who receive immunosuppressive drugs become susceptible to Pneumocystis. The incidence of Pneumocystis infection in connective tissue disease is low but may increase if immunosuppressive drugs are used more often. Our patient acquired Pneumocystis pneumonia after immunosuppressive therapy for polyarteritis nodosa. Prompt recognition of this condition is essential now that specific therapy is available. Untreated Pneumocystis infection is usually fatal.
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PMID:Arthritis rounds. Pneumocystis carinii associated with polyarteritis and immunosuppressive therapy. 1 12

A 13-month-old boy had a "late-onset" form of combined immunodeficiency and a fulminant Pneumocystis carinii pneumonia of one month's duration. There was no evidence of cutaneous-delayed hypersensitivity responses to diphtheria-tetanus toxoids, Candida albicans, or streptokinase-streptodornase, or of lymphocyte DNA synthesis after in vitro stimulation with the mitogens phytohemagglutinin and concanavalin A, and only 2% to 4% of peripheral blood E-rosetted T lymphocytes. The serum IgM level was normal (62 mg/dL), whereas the other immunoglobulins were markedly reduced. Despite an increased number of Ig-bearing lymphocytes, in vitro Ig secretion after pokeweed mitogen stimulation was substantially reduced. The thymus gland was dysplastic with no Hassalls' corpuscles or thymocytes, and other lymphoid organs showed depletion of T-dependent areas to a greater extent than the B-dependent areas.
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PMID:Diagnostic dilemma of a 13-month-old boy with 'late-onset' combined immunodeficiency. 8 98

Pathomorphologic findings in an 11 month old boy with severe combined immunodeficiency (case 1) and in a 4-month old boy with reticular dysgenesia (case 2) are reported. Case 1: The bone marrow exhibited regular granulo-, erythro- and thrombopoiesis. The hypoplastic thymus consisted exclusively of epithelial reticulum cells. The spleen and lymph nodes showed considerable depletion of lymphocytes in both the T- and B-cell areas. There was a complete lack of all lymphatic structures in the gastrointestinal tract and aplasia of the tonsils. Death resulted from Candida sepsis in conjunction with giant cell pneumonia closely resembling Hecht's pneumonia in measles. Case 2: The bone marrow showed a total lack of granulopoiesis. The storngly dysplastic thymus weighed only 1 g. The spleen, the lymph nodes and the gastrointestinal tract exhibited a very strange histologic structure resulting from a complete absence of lymphocytes and plasma cells. The tonsils were aplastic, the para-thyroid glands as well as the other endocrine glands were normally developed. The cause of death was Klebsiella sepsis and Pneumocystis pneumonia, the latter without the characteristic interstitial plasma cell infiltration. The importance of the immune system for activation of the nonspecific mechanisma of defense is discussed with respect to the two types of immunodeficiency states described here.
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PMID:Pathomorphologic findings in severe combined immunodeficiency and reticular dysgenesia. 81 95

Temporary extrapulmonary oxygenation may benefit selected patients with Pneumocystis carinii pneumonia who exhibit severe respiratory insufficiency. Four persons were considered candidates for extrapulmonary oxygenation with a membrane lung while under treatment for pneumocystis pneumonia. In one patient, attempts to institute membrane lung circulation were postponed until his condition was terminal. In another individual, membrane lung support was discontinued prematurely because of complications of anticoagulation. A third patient died of cardiac failure even though her oxygenation had improved during respiratory assistance. In the fourth, the membrane lung was used successfully to maintain the patient through therapy until lung recovery was adequate to resume vital function. The four cases presented are examples of the immunosuppression that creates susceptibility to pneumocystis pneumonia: In two patients, immunodeficiency was caused by lymphoma and combination chemotherapy for the underlying disease; in two others, immunosuppression was induced for the purposes of transplantation. Two patients underwent veno-venous perfusion for prepulmonary oxygenation, and one underwent venoarterial bypass with the membrane lung. Indications for, and techniques of, membrane lung bypass are reviewed. This method of extrapulmonary membrane lung support may save some patients with transient severe pulmonary insufficiency due to P, carinii pneumonia, and the membrane lung may be an adjunct to antimicrobial therapy.
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PMID:Pulmonary and extrapulmonary support for patients with Pneumocystis carinii pneumonia. 108 54

A study was performed to reveal possible differences in lymphocyte subpopulations from bronchoalveolar lavage (BAL) of acquired immunodeficiency patients with and without Pneumocystis carinii pneumonia. Forty-one consecutive human immunodeficiency virus-seropositive patients were studied. Pneumocystis carinii infection was detected in the BAL fluid from 18 patients. The BAL lymphocyte subpopulations were determined by surface marker analysis with the immunoperoxidase slide assay. No significant differences in the percentage of CD4+ and CD8+ lymphocytes were found between the two groups. The percentage of CD57+ natural killer (NK) cells was significantly higher in the Pneumocystis carinii-negative group than in the -positive group. Since NK cells protect from microbial infections, it is conceivable that the loss of CD57+ NK cells may be one of the phenomena leading to the immunodeficiency state that underlies the pulmonary complications characteristic of the acquired immunodeficiency syndrome.
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PMID:Phenotypic analysis of bronchoalveolar lavage lymphocytes from acquired immunodeficiency patients with and without Pneumocystis carinii pneumonia. 128 Mar 89

Current treatment options for acquired-immunodeficiency syndrome (AIDS)-related non-Hodgkin's lymphoma (NHL) are unsatisfactory because of excessive toxicity rates and frequent recurrence of lymphoma. In this phase II study, we evaluated a novel 12 week chemotherapy program with respect to feasibility, toxicity and therapeutic results. Thirty HIV-seropositive patients with intermediate grade or small non-cleaved cell NHL received a 12 week program of weekly intravenous and oral chemotherapy consisting of etoposide, adriamycin, cyclophosphamide, bleomycin, vincristine, methotrexate and prednisone as well as biweekly intrathecal cytosine arabinoside. Prophylaxis against Pneumocystis carinii pneumonia (PCP) and candida were given routinely. The overall objective response rate was 73% with 33% complete responders. The time to progression for those stable or responding was 9.4 months. Five of 10 complete responders are well and free of disease 13.2 to 24.5 months from diagnosis. Median survival for the 30 patients was 8.1 months. NHL was the most common cause of death (13/22); opportunistic infection caused only one death (cryptococcal meningitis). Only 1 case of PCP occurred. The major toxicity was neutropenia. In conclusion this regimen resulted in response rates similar to other reports with acceptable toxicity and a very low incidence of PCP. Relapse of NHL remains a major challenge, however, and further studies are needed. Routine PCP prophylaxis should be incorporated into new trials of therapy for AIDS-related NHL.
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PMID:Treatment of AIDS-related non-Hodgkin's lymphoma with a twelve week chemotherapy program. 128 56

Patients undergoing bronchoscopy for possible pneumocystis pneumonia were studied retrospectively to characterize the impact of common viral pathogens on the course of advanced human immunodeficiency virus (HIV) disease and atypical pneumonia. In 327 episodes, Pneumocystis carinii was found in 220 (67%), cytomegalovirus (CMV) in 145 (44%), and herpes simplex virus in 16 (5%). Early deterioration in oxygenation and use of intensive care was less common in CMV-positive patients. Neither CMV nor P. carinii was a predictor of mortality in multivariate analyses. CMV was not associated with an increased prevalence of later CMV disease. Isolation of CMV from the bronchoalveolar lavage fluid of these patients was not an indication for antiviral therapy. Pulmonary shedding of CMV may be associated with a decreased inflammatory response to P. carinii. The outcome of HIV-associated atypical pneumonia where no clear pulmonary pathogen is found on routine evaluation was no better than that of treated P. carinii pneumonia.
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PMID:Impact of Pneumocystis carinii and cytomegalovirus on the course and outcome of atypical pneumonia in advanced human immunodeficiency virus disease. 130 75

Pneumocystis carinii pneumonia (PCP) occurs frequently in individuals infected with the HIV virus. Malignancy, immunosuppressive drugs, and congenital immune deficiency may be associated with PCP. We describe a patient with stage 1 testicular carcinoma who developed hypoxemic respiratory failure two days after retroperitoneal lymph node dissection. Pneumocystis carinii organisms were demonstrated by catheter lavage samples and confirmed on bronchoalveolar lavage. Testing for HIV antibody by ELISA and the Western blot test were negative; HIV viral culture and polymerase chain reaction were also negative. Pneumocystis carinii pneumonia is unusual in localized surgically cured malignancies without obvious immunodeficiency and, to our knowledge, has not been described as a cause of postoperative respiratory failure.
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PMID:Postoperative respiratory failure secondary to Pneumocystis carinii pneumonia. 131 51

The outpatient management of patients infected with human immunodeficiency syndrome is reviewed. Patients with CD4+ cell counts of greater than 0.5 x 10(9)/L (500/mm3) require no specific intervention except vaccination against influenza, pneumococcus, and possibly hepatitis B. They should have a follow-up examination every 3 to 6 months. Because of its success in preventing the progression of the disease, zidovudine (AZT), 100 mg five times per day, is recommended for patients with CD4+ cell counts of less than 0.5 x 10(9)/L (500/mm3). During this stage of the disease, a patient should be seen every 1 to 3 months and monitored for drug toxicity and disease progression. Patients with CD4+ counts of less than 0.2 x 10(9)/L (200/mm3) are at high risk of developing Pneumocystis carinii pneumonia. Prophylaxis with oral trimethoprim-sulfamethoxazole (one double-strength tablet three times weekly) or dapsone (100 mg three times weekly) is recommended. Treatment costs for the patient with CD4+ cells less than 0.5 x 10(9)/L (500/mm3) are at least $3000 per year.
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PMID:Outpatient management of patients infected with human immunodeficiency virus. 134 66

In 1989, the United States Public Health Service convened a Task Force of experts to consider the expanding knowledge base about prevention of Pneumocystis carinii pneumonia (PCP) among adults and adolescents (greater than or equal to 13 years of age) with human immunodeficiency virus (HIV) infection. This Task Force concluded that the morbidity, mortality, and cost due to PCP could be substantially reduced by appropriate use of antipneumocystis prophylaxis in subgroups of HIV-infected patients known to be at high risk, and developed recommendations for the administration of prophylactic regimens (1). The recommendations state that CD4+ T-lymphocyte counts should be monitored prospectively at 3- to 6-month intervals and prophylaxis should be instituted when patients become immunologically susceptible to PCP. Susceptibility was defined by a CD4+ T-lymphocyte count less than 200 cells/microliters or less than 20% of total circulating lymphocytes, or the occurrence of a previous episode of PCP. The goal of this approach was to reduce the frequency both of initial episodes of PCP (primary prophylaxis) and of relapses or recurrences (secondary prophylaxis). Either oral trimethoprim-sulfamethoxazole (TMP-SMX) or aerosol pentamidine was recommended for prophylaxis, but because direct comparative data were lacking, neither regimen was endorsed as "preferred." Since the recommendations were issued in 1989, additional information has become available about the efficacy and safety of aerosol pentamidine and oral TMP-SMX. A trial sponsored by the National Institute of Allergy and Infectious Diseases AIDS Clinical Trials Group compared these two regimens in a prospective randomized study; in August 1991, this study was terminated by an independent data and safety monitoring board because statistically significantly fewer recurrences of PCP were observed in the oral TMP-SMX group than in the aerosol pentamidine group (2). On the basis of this finding and other studies assessing PCP prophylaxis, the Task Force was reconvened on October 5, 1991. This report contains the revised recommendations issued by the Task Force.
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PMID:Recommendations for prophylaxis against Pneumocystis carinii pneumonia for adults and adolescents infected with human immunodeficiency virus. 134 43


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