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)

In a previously healthy 13-year-old girl with disseminated blastomycosis, immunodeficiency was considered because of lymphopenia and the slow response of her lung disease to therapy with amphotericin B. Cellular immunity was found to be profoundly impaired, with absent delayed cutaneous hypersensitivity to several common antigens, a decreased count of thymus-dependent lymphocytes in the peripheral blood and a greatly diminished in-vitro proliferative response of lymphocytes to phytohemagglutinin (PHA). Humoral immunity was intact. Two additional types of therapy were assessed: subcutaneous injection of transfer factor was associated with an unsustained increase in lymphocyte counts and a positive cutaneous response to PHA but no clinical change; parenteral alimentation to ensure an adequate energy intake was associated with rapid clinical improvement, the development of delayed hypersensitivity to four additional antigens, and the return of lymphocyte counts and proliferative response to normal. These findings suggest that increased energy intake rather than transfer factor therapy was responsible for the child's recovery, and they emphasize the importance of adequate nutrition in the maintenance of intact cellular immunity.
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PMID:Cellular immunity and nutrition in refractory disseminated blastomycosis. 9 21

Among 40 hospitalized infants and children with cytomegalovirus infection, 14 (35%) had interstitial pneumonitis, 4 (10%) had wheezing or tachypnoea but without x-ray evidence of classical interstitial pneumonia, the remaining 22 (55%) were free of pulmonary involvement. Most patients had tachypnoea and nonproductive cough of varying durations: those with underlying pulmonary pathology tended to have persistent and prolonged respiratory symptoms. Mortality and severity of the lung disease were related to the underlying immunodeficiency or concomitant pulmonary process.
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PMID:Pulmonary involvement with cytomegalovirus infections in children. 19 40

Endobronchial scraping was used in 53 immunodeficient children, aged 4 months to 15 years, and divided into three categories (37 receiving immunosuppression treatment, 8 with marasmus, and 8 with immunodeficiency), in order to determine the etiology of their interstitial pneumopathy. The examination was made under blind conditions in 21 cases using an intubation tube (under assisted ventilation), and with bronchoscopy under general anesthesia in the other 32 cases. Three scrapings were required for cytological, bacteriological, and virological and mycological examinations. In 32 cases (60%), the etiology of the interstitial pneumopathy was discovered; in 18 patients it was due to pneumocystis carinii, in 10 cases to bacterial infection, in 7 cases a viral infection, and in 3 others a fungal infection. An association of infective agents was reported in 6 cases. The major incident observed was a pneumothorax in 17% of the cases, more especially in 45% of the children under 20 months of age. Bronchial scraping is a valid examination the results and complications of which compare well with other non-vascular methods of diagnostic evaluation of such lesions.
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PMID:[Results of bronchial scrapings in interstitial pulmonary diseases in immunodeficient children (author's transl)]. 54 12

Twelve patients suffering from ataxia telangiectasia and relatives of three of them were studied. A late diagnosis prevailed and the neurological symptoms in some turned worse after suffering recognizable viral infections. The cellular and humoral immunodeficiencies were evaluated in each case as well as the simultaneous presence of hyperimmunoglobulinemia and/or auto-antibodies. Among the relatives, there were frequent cases of immunological and neoplastic alterations; cellular immunodeficiency was not detected. Five cases showed lung disease; four with absence of IgA, and histologic interstitial lesion detected in three of them. The hypothesis that the defect of cell immunity predisposes mulisysthemic disease and that an early diagnosis with immune reconstructive treatment can modify the evolution of the disease was considered.
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PMID:[Ataxia telangectasia. Probability of damaging immune mechanisms]. 83 20

We evaluated nine patients with humoral immunodeficiency (6 immunodeficiency with hyper-IgM, 2 X-linked agammaglobulinemia, 1 common variable immunodeficiency) who were being treated with intravenous immunoglobulins (IVIG). After the use of the IVIG regimen in a dose of 250-300 mg/kg/4 weeks for one year, the severity and frequency of infections, even in patients with chronic lung disease, decreased significantly. An improvement in pulmonary function tests was observed in four patients who had airway obstruction prior to IVIG therapy. Side effects such as chills and fever were observed in 21 of 91 infusions, particularly in the early months of therapy. Preinfusion administration of aspirin and diphenhydramine prevented these side effects. The inversion of the CD4+/CD8+ ratio was detected in most patients during both intramuscular gammaglobulins (IMIG) and IVIG therapy.
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PMID:Effects of intravenous immunoglobulin on clinical and immunological findings of patients with humoral immunodeficiency diseases. 130 39

Over the last decades, the rate of isolation of tubercle bacilli has declined in the developed countries, while the incidence of infection with nontuberculous mycobacteria (NTM) has increased. In a retrospective study, we analyzed all cases of patients negative for human immunodeficiency virus (HIV) and from whom NTM were isolated in the Zurich area of Switzerland from 1983 to 1988. During the 6-year study period, 513 patients infected with NTM were identified, 34 of whom had clinically significant disease. The presentation of mycobacteriosis was found to be lung disease in 23 cases, soft-tissue disease in 10 cases, and disseminated disease in one case. The highest attack rate of pulmonary mycobacteriosis was 0.49% and was found in the group of patients 41-50 years old. During the 6-year period, the incidence of tuberculosis declined from 16.2 to 13.2 per 100,000 population, while the incidence of mycobacteriosis increased from 0.4 to 0.9 per 100,000 population. Clinically nonsignificant NTM isolates were found more frequently in patients with chronic lung diseases (P less than .01) and especially in patients with a history of tuberculosis (P less than .001).
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PMID:Epidemiology and clinical significance of nontuberculous mycobacteria in patients negative for human immunodeficiency virus in Switzerland. 152 Jul 69

High-resolution CT (HRCT) and chest radiographs were compared in nine patients with miliary lung disease. In all cases, miliary disease was documented to be infectious in etiology; six of these patients proved to be human immunodeficiency virus (HIV) positive. A mixture of both sharply and poorly defined 1-3 mm nodules was seen in all cases, many of the latter having an appearance indistinguishable from airspace nodules. Other features attributable to the presence of nodules included nodular interlobular septae, nodular irregularity of vessels, subpleural dots, and studded fissures. Diffuse intra- and interlobular septal thickening also proved common, seen in all but one case (91%). Based on limited HRCT-pathologic correlation, CT findings appear primarily to be due to granulomatous foci developing in a seemingly random distribution involving both pulmonary airspaces as well as the interstitium. It is concluded that in the appropriate clinical setting this constellation of findings is characteristic of miliary disease; the role of HRCT especially in the early diagnosis of miliary disease in HIV positive patients remains to be determined prospectively.
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PMID:High resolution CT findings in miliary lung disease. 159 20

Asthma is a multifactorial, reversible, obstructive lung disease that manifests airway inflammation as well as airway hyperreactivity. In addition to IgE-mediated respiratory reactions, the pathophysiology of asthma can be triggered by both viral respiratory and bacterial sinopulmonary infections. Even though most asthma patients do not manifest undue susceptibility to infection, a subset of asthma patients with recurrent sinopulmonary as well as upper-respiratory infections may have an associated immune deficiency syndrome. In a subset of these patients, deficiencies of serum IgG subclasses have also been described in the presence of low-normal or normal serum IgG and also deficient serum IgA. In addition to the usual asthma therapy with beta 2 agonist and theophylline bronchodilators as well as cromolyn and steroids, many of these immunodeficiency patients will benefit from iv gamma-globulin therapy. However, we suggest that an inability to synthesize specific serum antibody to injected vaccines or immunogens be a prerequisite before initiating iv gamma-globulin therapy. The clinician should not rely on serum IgG subclass levels alone as a criterion for initiation of passive immune globulin therapy. There may be another cohort of asthma patients who could benefit from iv gamma-globulin therapy. In a small open-label pilot study severe steroid-dependent asthma patients who were not immunodeficient and did not have undue susceptibility to infection were treated with iv gamma-globulin with a very large dosage protocol of 2000 mg/kg monthly.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Asthma. A role for IVIG therapy? 160 21

We retrospectively compared the results of 67Ga chest scans and 99mTc-DTPA aerosol clearance measurements with those of fiberoptic bronchoscopy in 88 patients infected with the human immunodeficiency virus. Of 100 investigations, a pulmonary infection was diagnosed in 39, mainly Pneumocystis carinii pneumonia and a noninfectious disorder was found in 42, mainly Kaposi's sarcoma and lymphocytic alveolitis. Gallium scans and DTPA clearance were abnormal respectively in 74% and 92% of infectious complications, and in 12% and 60% of noninfectious disorders. In 10 cases, DTPA clearance was accelerated, while chest x-ray, arterial blood gases and even gallium scanning were normal. A value of DTPA clearance greater than 4.5%.min-1 was both sensitive and specific for the diagnosis of Pneumocystis carinii pneumonia. The gallium scan was always normal in bronchopulmonary Kaposi's sarcoma. We conclude that in symptomatic patients: (1) DTPA clearance measurements are useful for detecting lung disease when chest x-ray and/or PaO2 are normal and (2) a gallium scan is indicated to distinguish progressive Kaposi's sarcoma from a superimposed second process when radiological abnormalities of pulmonary Kaposi's sarcoma are present.
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PMID:Technetium-99m-DTPA aerosol and gallium-67 scanning in pulmonary complications of human immunodeficiency virus infection. 173 Oct 2

Tumor necrosis factor-alpha (TNF) is a cytokine involved in the pathogenesis of shock and in granuloma formation, tissue necrosis, and fibrosis, in many organ systems, including the lung. It has been suggested that cells from patients infected by the human immunodeficiency virus (HIV + ve) are primed for TNF release. We postulated that TNF release from the alveolar macrophages (AM) of such patients with lung disease might lead to their observed pulmonary dysfunction. We present data confirming that peripheral blood monocytes (PBM) and demonstrating that AM from HIV + ve patients with pulmonary manifestations show significantly greater TNF production than those from HIV-negative (HIV - ve) subjects. In addition, we found sequentially significant increases in TNF production from AM and PBM of HIV + ve patients with no pathogens detected at bronchoscopy (NB), bacterial pneumonia (BP), and those with Pneumocystis carinii pneumonia (PCP). The overall TNF levels were greater from AM than PBM in all groups other than spontaneous production from HIV - ve subjects. Adherent populations of PBM and AM were incubated for 4 h with lipopolysaccharide (10 micrograms/ml) or control medium alone. Cell-free supernatants were examined for the presence of TNF using an immunoassay. The TNF levels (mean +/- SD) in IU/ml from stimulated PBM of the PCP, BP, NB, and control groups, respectively, were 186 +/- 36, 140 +/- 30, 95 +/- 18, and 55 +/- 10 and the spontaneous levels were 123 +/- 25, 100 +/- 22, 75 +/- 24, and 11 +/- 5.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Production of tumor necrosis factor-alpha by blood and lung mononuclear phagocytes from patients with human immunodeficiency virus-related lung disease. 189 44


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