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)

It has recently been shown that human alveolar macrophages can be selectively activated without systemic effect by the use of aerosolized interferon-gamma (IFN gamma), a cytokine that enhances macrophage oxidative and antimicrobial activity. We report the case of a 38-yr-old man negative for human immunodeficiency virus (HIV), with silicosis and advanced cavitary lung disease due to Mycobacterium avium intracellulare (MAI), who failed to improve despite 3 yr of continuous medical therapy with three or more drugs. He received three courses of aerosolized IFN gamma (500 micrograms 3 d per week for 5 wk in two courses and 200 micrograms 3 d a week for 5 wk after a short single trial of subcutaneous IFN gamma). The numbers of MAI decreased in the sputum during therapy, but cultures of the organism remained positive at the same level for the first two treatment periods. The patients sputum became AFB smear negative and the number of colonies decreased significantly after the third course of IFN gamma therapy. Cessation of IFN gamma was associated with a rapid increase in the numbers of MAI in the sputum. Aerosolized IFN gamma can be considered as an adjuvant to conventional drug therapy, with a good tolerance, in cases of lung disease caused by resistant MAI.
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PMID:Aerosolized interferon gamma for Mycobacterium avium-complex lung disease. 766 88

Bloom's syndrome is a rare autosomal recessively transmitted disorder, the main clinical feature of which is small body size. A sun-sensitive, erythematous facial skin lesion, an excess of well-demarcated hyper- and hypopigmented skin lesions located anywhere on the body, and increased numbers of bacterial infections due to immunodeficiency are accompanying features of diagnostic value. In Bloom's syndrome, the complications are formidable: cancer, chronic lung disease, and diabetes. Cancers of the types and sites seen in the general population arise frequently and unusually early. Bloom's syndrome cells are hypermutable, and excessive numbers of somatic mutations are responsible for many of the clinical features. The clinical diagnosis is confirmed cytogenetically by demonstrating a characteristic chromosome instability.
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PMID:Bloom's syndrome. 771 53

Cytomegalovirus (CMV) infection is the most frequent systemic viral opportunistic infection in AIDS, occurring in almost 40% of patients, at a stage of profound immunodeficiency, with a CD4 cell count lower than 50/microL. The most frequent localizations are retinal and gastrointestinal. Diagnosis of retinis, which can be totally asymptomatic, is based on fundus examination, which should be performed regularly in patients with AIDS and/or low CD4 count. Diagnosis of colitis, as of other rare manifestation (oesophagitis, hepatitis, encephalitis, myeloradiculitis, pneumopathy), relies on the association of suggestive clinical symptoms and CMV inclusions in biopsy specimens and/or CMV positive culture. The 2 drugs available for treatment of CMV disease, ganciclovir and foscarnet, are administered by intravenous route, with 2 infusions per day for induction therapy (usually 2 to 3 weeks), then once a day as lifelong maintenance therapy, to lessen or delay recurrences. Active drugs which could be given orally, combination of 2 drugs, new potent drugs and the development of prophylaxis in at-risk patients should help to improve the prognosis of CMV infection in AIDS.
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PMID:[Cytomegalovirus infections in AIDS]. 775 12

All human immunodeficiency virus type 1 (HIV-1) infected adult patients referred to the Division of Pulmonary Diseases of the Centre Hospitalier de Kigali, Rwanda for evaluation of a pulmonary disease of undetermined etiology (PDUE) were investigated by fiberoptic bronchoscopy using both bronchoalveolar lavage (BAL) and transbronchial biopsy (TBB). During a 10-mo period 111 HIV-1 infected patients with PDUE were examined, of whom 47 (42%) fulfilled the World Health Organization (WHO) clinical case definition for acquired immunodeficiency syndrome (AIDS) and seven (6%) had an AIDS-defining illness. Nonspecific interstitial pneumonitis was diagnosed in 42 (38%) patients, tuberculosis in 25 (23%), cryptococcosis in 14 (13%), Kaposi's sarcoma (KS) in 10 (9%), Pneumocystis carinii pneumonia (PCP) in five (5%). The diagnosis remained undetermined in 18 (16%) patients. Chest radiograph patterns were generally nonspecific. TBB and BAL had diagnostic yields of 82 and 26% of all final diagnoses, respectively. Our study on Rwandese HIV-1-infected patients with PDUE provides evidence for a large spectrum of pulmonary diseases with relative frequencies differing strikingly from those in developed countries. Detailed investigations confirm the rarity of PCP in Africa and highlight nonspecific interstitial pneumonitis as the predominant diagnosis of PDUE. Empiric antituberculosis treatment is justified in the absence of clinical manifestations suggestive of a specific diagnosis and while awaiting the results of the diagnostic procedures. Primary prophylaxis for PCP would not be appropriate in Africa.
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PMID:Pulmonary disease associated with the human immunodeficiency virus in Kigali, Rwanda. A fiberoptic bronchoscopic study of 111 cases of undetermined etiology. 800 18

Serum concentrations of neuron-specific enolase (NSE) were measured in 135 patients with benign pulmonary diseases who also underwent a clinical, laboratory, and radiologic evaluation. Eleven percent of the patients as a whole and 27.3% of those who were tuberculous had abnormal serum levels of NSE. Significant differences in NSE levels were observed among the six diagnostic groups evaluated (p = 0.002). Males had higher levels than females (p = 0.003), and patients infected with the human immunodeficiency virus (HIV) had higher NSE levels than those not infected (p = 0.0026). Patients with alveolar infiltrates or an interstitial pattern on chest X-ray had higher NSE levels than those with normal radiographs (p = 0.003 and p = 0.01, respectively). In fact, only 3.6% of the patients with normal radiographs had above-normal levels of NSE. Direct damage to the neural or neuroendocrine lung cells or some degree of local hypoxia is likely to play a role in the increase in NSE in these patients. The small number and degree of abnormal values of NSE observed in this study make it unlikely that an underlying benign lung disease will substantially modify the interpretation of an increased NSE value in patients with lung cancer. However, care should be taken in interpreting a moderately abnormal NSE value in the cancer patient in the presence of lung infiltrates such as obstructive pneumonitis.
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PMID:Measurement of the serum tumor marker neuron-specific enolase in patients with benign pulmonary diseases. 802 40

Adult T cell leukaemia-lymphoma (ATL) was first discovered and reported in Japan, where it has a high incidence in the south-west region. The first human retrovirus HTLV-I (human T-cell lymphotropic virus type I) is considered to be related to its aetiology. In ATL endemic areas, HTLV-I carriers form a fairly high percentage of the population, even among healthy individuals. ATL shows diverse clinical features. It can be divided into four subtypes: acute, chronic, smouldering and lymphoma type. ATL cells originate from the CD4-positive subset of peripheral T cells; they show a characteristic notch in the nucleus and a tendency to lobulation. ATL resists chemotherapy, and patients with acute and lymphoma types have a fairly poor prognosis. A definite diagnosis of ATL is made by documenting the presence of HTLV-I proviral DNA in the DNA of tumour cells. HTLV-I infection is caused by transmission of live lymphocytes via three routes (from mother to child, from males to females, and by transfusion). Familial occurrence of ATL is frequently seen. HTLV-I infection is seen in other countries, but its incidence is highest in Japan. Infection with HTLV-I is a direct cause of ATL. Furthermore, infection with this virus can indirectly cause many other diseases via the induction of immunodeficiency, such as chronic lung disease, opportunistic lung infection, cancer of other organs, monoclonal gammopathy, chronic renal failure, strongyloidiasis, non-specific dermatomycosis, HTLV-I-associated lymphadenitis, HTLV-I uveitis and HTLV-I-associated myelopathy-tropical spastic paraparesis (HAM/TSP).
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PMID:Adult T cell leukaemia-lymphoma. 803 96

In 42 adults with recurrent respiratory infections (RRI) and common variable immunodeficiency or immunoglobulin G (IgG) subclass deficiency, the results of pulmonary function tests were related to factors apt to produce airway obstruction: serum concentration of IgG and IgG subclasses, various features of acute RRI (number/year, time from onset to diagnosis, episodes of pneumonia, etc) and type of chronic lung disease (smoking and nonsmoking related chronic bronchitis, episodic wheezing, and bronchiectasis). Compared with nonsmokers, usually less than 40 years of age, the patients above 40 had smoking-related chronic bronchitis and had obstruction (%FEV1/forced vital capacity [FVC] 55.3 +/- 8.1 vs 80.1 +/- 4.5), hyperinflation (residual volume 182.7 +/- 22.7 percent vs 109.7 +/- 8.8 percent of pred) hypoxemia (66.6 +/- 5.8 vs 83.4 +/- 4.2 mm Hg) and impaired carbon monoxide transfer (65.5 +/- 9.1 percent vs 93.3 +/- 5.8 percent). The features of acute or chronic RRI, the time from onset to diagnosis (< 10 yr in the entire group), the type of IgG deficiency or the serum concentration of the deficient protein did not correlate with substantial obstruction (FEV1/FVC < 70%). In conclusion, in adults with IgG deficiency and RRI for less than 10 yr, smokers with chronic bronchitis rather than nonsmokers develop substantial airway obstruction.
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PMID:Airway obstruction in adults with recurrent respiratory infections and IgG deficiency. 816 26

The single most important respiratory pathogen in infancy and early childhood is respiratory syncytial virus (RSV). Approximately 40% of primary RSV infections in children result in lower respiratory tract disease. Approximately 1% of RSV-infected children require hospitalization. Especially in high-risk children, primary RSV infection results in significant morbidity and, sometimes, death. This high-risk group includes children with bronchopulmonary dysplasia, children with congenital heart disease, premature infants less than 6 months of age, and children with immunodeficiency diseases. It has been estimated that, in the United States, 14,000 infants with chronic lung disease and 16,400 infants with heart disease will be identified by 12 months of age. More than 91,000 children are hospitalized annually with lower respiratory tract disease caused by RSV, and 4500 deaths occur. In 1985 a report from the Institute of Medicine calculated that the annual hospitalization costs attributable to RSV infection were $300 million. Data collected at the New England Medical Center in 1991 show that the average cost of hospitalization of a child with RSV was $808 each day. Because of difficulty in developing a safe and effective RSV vaccine, attention is now focused on passive immunization using an RSV immune globulin. On the basis of a recently completed multiinstitutional trial, RSV immune globulin appears to be a safe and cost-effective option for prevention of severe RSV disease in high-risk children.
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PMID:Economic impact of viral respiratory disease in children. 816 53

Alveolar macrophages (AMs) harvested from 32 HIV-infected patients with respiratory problems (opportunistic pulmonary infections, n = 12; other lung disease, n = 20) and 13 healthy controls were stained with a panel of 15 monoclonal antibodies directed against surface antigens implicated in cell function. Antigen expression was quantified by flow cytometry and expressed as relative linear median fluorescence intensity (RLMFI). On AMs of patients, as compared with controls, there was a significant enhancement of HLA DP (12.1 +/- 1.5 vs 6.5 +/- 0.9, p = 0.01, M +/- SEM, RLMFI units), CD11b (3.4 +/- 0.5 vs 1.7 +/- 0.4, p = 0.014), CD11c (8.9 +/- 1.0 vs 4.8 +/- 0.8, p = 0.0046), CD14 (2.1 +/- 0.3 vs 1.0 +/- 0.2, p = 0.0009), and CD33 (1.7 +/- 0.1 vs 1.0 +/- 0.2, p = 0.0093). No significant differences could be established for HLA-DR (36.9 +/- 5.8 vs 30.9 +/- 7.5, NS), HLA-DQ (3.4 +/- 0.3 vs 3.1 +/- 0.6, NS), CD54 (1.9 +/- 0.3 vs 1.2 +/- 0.1, NS), CD13 (2.5 +/- 0.6 vs 1.5 +/- 0.3, NS), CD36 (1.4 +/- 0.2 vs 0.9 +/- 0.3, NS), CD71 (10.3 +/- 1.9 vs 8.9 +/- 1.8, NS), CD25 (0.8 +/- 0.0 vs 0.9 +/- 0.1, NS), 27E10 (1.1 +/- 0.1 vs 0.8 +/- 0.3, NS), RM3/1 (1.9 +/- 0.4 vs 1.5 +/- 0.4, NS), and CD4 (1.5 +/- 0.3 vs 1.0 +/- 0.0, NS). The expression of CD14 and CD11b, but not of HLA class II antigens and CD71, was increased in the smaller cell population compared with the larger, thus suggesting monocyte recruitment. The increased expression of HLA-DP, CD11c, CD14, and CD33 on the patients' AMs was independent of smoking habits. The degree of immunodeficiency as indicated by the absolute peripheral CD4 count, the character of HIV-related pulmonary disease, and the prophylactic use of pentamidine or zidovudine did not significantly modify the antigen expression pattern. It is concluded that HIV infection may lead, most probably indirectly, to enhanced expression of surface antigens by local upregulation and/or recruitment of monocytes from the peripheral circulation. The functional significance of enhanced marker expression requires further clarification.
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PMID:Expression of surface markers on alveolar macrophages from symptomatic patients with HIV infection as detected by flow cytometry. 818 14

Pulmonary disease is a frequent manifestation in the terminal stages of acquired immune deficiency syndrome (AIDS), and is caused mainly by a number of opportunistic microorganisms, most commonly Pneumocystis carinii. Despite the extensive involvement of the lung in the pathogenesis of AIDS, until recently little was known about the role of human immunodeficiency (HIV). In this review we will discuss the cellular tropism and phenotypic characterisation of HIV strains isolated from the lung. The available literature on HIV infection of the lung is reviewed, and the mechanisms of HIV-induced pathogenesis in the lung is discussed.
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PMID:Evidence for human immunodeficiency virus infection of the lung. 825 79


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