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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary nocardiosis (PN) is an infrequent and severe infection due to Nocardia spp., microorganisms that may behave both as opportunists and as primary pathogens. The aim of this study and review was to evaluate the clinical features, evolution and prognostic factors of PN. The study group comprised 10 consecutive patients with pulmonary nocardiosis acquired in a community setting, diagnosed and followed in a tertiary teaching hospital. Chronic obstructive pulmonary disease (COPD), neoplastic disease and human immunodeficiency virus (HIV) infection were the most frequent predisposing factors. Four patients were receiving corticosteroid treatment. Clinical course was chronic and diagnosis was delayed 3 weeks or more in seven of the patients. Lobar or multilobar condensation was the most frequent radiographic pattern. Antimicrobial susceptibility testing showed: 100% sensitivity for amikacin; 83% for imipenem; 71% for cefotaxime; and 71% for trimethoprim-sulphamethoxazole. The disease remained localized in the lung in five cases, with a trend toward chronicity in one with bronchiectasis. In the other five, the disease disseminated, affecting subcutaneous tissue, the central nervous system and the kidney. Three patients died, one with disseminated disease and two who were receiving corticosteroid therapy. The following conclusions were reached: 1) pulmonary nocardiosis is difficult to diagnose, diagnosis is frequently delayed and a high level of suspicion is, thus, required in patients with underlying diseases or chronic corticosteroid therapy; 2) there is frequent dissemination and high mortality; and 3) antimicrobial combinations with proven synergy, such as imipenem and amikacin, are recommended for initial therapy.
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PMID:Pulmonary infection with Nocardia species: a report of 10 cases and review. 923 Feb 44

Infection with HIV was first recognized through a clustering of unusual respiratory infections. The lung has been a major target manifesting many of the infectious complications of the immunodeficiency. Noninfectious pulmonary complications in HIV-infected individuals are also common and have been recognized since the advent of the AIDS epidemic. Malignancies involving the respiratory system, specifically Kaposi's sarcoma and non-Hodgkin's lymphoma, are epidemiologically linked to infection with HIV. Although other cancers have been identified in patients with HIV, these malignancies have a relationship to HIV infection that is unknown. Nonetheless, all cancers in the HIV-infected individual appear to follow a very deadly course. Interstitial pneumonitis and an alveolitis are also seen in individuals infected with HIV. Their relationship to the virus is unknown but may involve the lung's immune response to HIV. Pneumothorax and bullous lung disease are the sequela of pulmonary infections in the HIV-infected host. Pulmonary hypertension has been reported in HIV-infected patients, and like the other noninfectious respiratory complications, the link between the disease process and HIV is unknown. Bronchiectasis is now commonly recognized in AIDS patients who have survived prolonged immunosuppression and infection. Bronchoscopists have accumulated a collection of endobronchial lesions uncommonly seen in non-HIV-related pulmonary consultation. In the following review, we discuss the epidemiology, pathology, pathogenesis, clinical features, diagnostic findings, prognosis, and therapeutic options available for each noninfectious pulmonary complication. As the life expectancy for HIV-infected patients increases, the incidence of noninfectious pulmonary complications will rise.
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PMID:The noninfectious respiratory complications of infection with HIV. 936 57

Human immunodeficiency virus (HIV) infection has been associated with a wide spectrum of pulmonary disease. We report three HIV-seropositive patients with rapidly worsening airway obstruction associated with bronchiectasis. All subjects (age range 33-39 yrs) were cigarette smokers. Two had previously used intravenous drugs. The CD4 lymphocyte count ranged 40-250 cells x mm(-3). All individuals had complained of increasing dyspnoea for 3-6 months. Within 1 yr, they all developed severe airway obstruction with a decrease in both forced expiratory volume in one second (FEV1) and ratio of FEV1 to forced vital capacity (FEV1/FVC) to less than 60% of predicted value, and a decrease in mean forced expiratory flow at 25-75% of the forced vital capacity (FEF25-75) to less than 35% of predicted value. Computed tomography of the chest disclosed bilateral dilated and thickened bronchi. No classical causes of genetic or acquired bronchiectasis were identified in our patients. Recurrent bacterial bronchitis occurred in the follow-up period of the three patients. In conclusion, unusually rapid airway obstruction associated with bronchiectasis should be added to the wide spectrum of respiratory complications of human immunodeficiency virus infection.
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PMID:Accelerated obstructive pulmonary disease in HIV infected patients with bronchiectasis. 959 35

We tried to characterize the clinical features and findings on chest high resolution computed tomography (HRCT) of patients with Mycobacterium avium-intracellulare (MAI) pulmonary infection without known predisposing lung disease and with no immunodeficiency. We also aimed to clarify the small airway and alveolar inflammation using bronchoalveolar lavage (BAL) from the affected regions. MAI infection was diagnosed in 53 patients from respiratory samples, including sputum and materials obtained using a fiberoptic bronchoscope. None had a predisposing lung disease or immunodeficiency, as assessed by medical history, routine laboratory data, and previously normal chest radiographs and/or CT scans. The mean age of the 53 patients was 60 +/- 11 years, and 48 were nonsmoking females. They had few respiratory symptoms, although 42% had chronic paranasal sinusitis. Chest HRCT findings showed centrilobular small nodules and ectasia of small bronchi and/or bronchioles located mainly in segment (S) 2, 3, 4, and 5. S1, which is usually affected by pulmonary tuberculosis, was completely free of these opacities. The BAL study revealed that the predominant cells were activated T lymphocytes and neutrophils. The CD4+/CD8+ ratio increased significantly. Bacteriology was negative for other bacteria and fungi. Although our patients did not present with distinct respiratory symptoms, the regions affected by MAI showed a chronic inflammation of mainly neutrophils and activated T lymphocytes. The presence of chronic sinusitis may be merely coincidental. However, its high prevalence and the finding of bronchiectasis in chest HRCT raise the question of whether silent bronchiectasis may be a predisposition.
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PMID:Mycobacterium avium-intracellulare pulmonary infection in patients without known predisposing lung disease. 978 Feb 96

The case history is presented of a patient with common variable immunodeficiency in whom heart lung transplantation has been carried out with success. Transplantation was the only long term therapeutic option in this patient due to the progressive respiratory failure resulting from bronchiectasis, emphysema, and granulomatous lung disease.
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PMID:Heart lung transplantation in a patient with end stage lung disease due to common variable immunodeficiency. 979 64

The aim of our study was to evaluate the success, complications, and morbidity following a modified Thal fundoplication in children with reflux-associated respiratory disease (RARD). We used a procedure consisting of retroesophageal hiatal plasty, wrapping the gastric fundus around the gastroesophageal junction 180 degrees, and fixation of the lesser curvature at the abdominal wall. Follow-up by questionnaire of 128 (77 male, 51 females) out of 196 antireflux procedures between 1992 and 1995 was achieved. Surgical therapy was considered justified whenever there was gastroesophageal reflux resulting in severe recurrent respiratory symptoms. Eleven percent of the children suffered from bronchiectasis. The diagnosis of RARD was based on a high index of suspicion, barium swallow with fluoroscopy, 24-hr two-level pH-monitoring, bronchoscopy, bronchoalveolar lavage and detection of lipid-laden alveolar macrophages, esophago-gastroscopy, and esophageal biopsy. Patients with bronchopulmonary diseases such as allergy, immunodeficiency, cystic fibrosis, primary ciliary dyskinesia, and malformation of the bronchial tree or vessels had been excluded. "Evident improvement" as a result of surgery was reported in 88%, "no change" in 10%, and a "change for the worse" in 2% of patients. Persistent mild difficulties in swallowing were observed in 11%. Paraesophageal hernia, gas-bloat syndrome, and dumping syndrome were not observed. Two children needed a second operation because of relapse. The use of emergency steroidal medication for acute respiratory distress decreased impressively (219 single doses/year before surgery vs. 30 single doses/year after surgery). The need for more than 4 times/year of antibiotic therapy before surgery was reduced from 52. 3% before to 14% after surgery. Most (90.6%) of the parents stated they would agree to have surgery done again if medically indicated. In conclusion, Thal fundoplication is sufficient, safe, and effective in the management of RARD. Complications of the procedure were minor and of little consequence to the patient.
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PMID:Antireflux surgery in children suffering from reflux-associated respiratory diseases. 1128 21

Bronchiectasis may occur with various congenital and acquired immunodeficiency diseases. The association of bronchiectasis and the X-linked lymphoproliferative disease (XLP), also known as Duncan's disease is unknown. We describe the case of a 39-year-old man with XLP, the oldest surviving, who developed chronic bronchiectasis with hemoptysis and required a pneumonectomy to control his symptoms.
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PMID:Immune-deficient bronchiectasis associated with X-linked lymphoproliferative disease. 1047 41

A 32-year-old woman with common variable immunodeficiency (CVID) accompanied by sinopulmonary infection was evaluated for purulent sputum, cough, and nasal obstruction that did not respond to regular intravenous immunoglobin (IVIG) infusion. Chest X-ray films revealed bronchiectasis affecting both lung bases, and a bacteriological examination of sputum was positive for Pseudomonas aeruginosa. Long-term chemotherapy with erythromycin (EM) was started, and the patient's respiratory symptoms gradually subsided. Sinopulmonary infection is the dominant clinical complication in patients with CVID. This case suggested that long-term EM chemotherapy is useful for the treatment of IVIG-refractory sinopulmonary infection associated with CVID.
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PMID:[A case of common variable immunodeficiency that responded to long-term erythromycin chemotherapy]. 1054 Aug 36

We present two male siblings suffering from recurrent manifestations of B-cell non-Hodgkin's lymphoma (NHL) and recurrent infections of the lower respiratory tract associated with bronchiectasis. Immunodeficiency could not be demonstrated by any laboratory investigation. In both patients, lymphomas developed without evidence for Epstein-Barr virus (EBV) infection, i.e. no antibody response to EBV-specific antigens, negative EBV-PCR (polymerase chain reaction) in peripheral blood cells, and absence of latent membrane protein (LMP) and EBV-encoded RNA (EBER) in lymphoma cells. Molecular analysis of the SH2D1A, the gene for X-linked lymphoproliferative disease (XLP) led to the identification of a deletion in the first exon in both patients. Therefore, we postulate that the genetic defect and the following dysregulation of the B-/T-cell interaction rendered these patients susceptible to the early onset of B-cell NHL and that EBV infection is not an obligate prerequisite.
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PMID:Recurrent B-cell non-Hodgkin's lymphoma in two brothers with X-linked lymphoproliferative disease without evidence for Epstein-Barr virus infection. 1069 68

Mycobacterium avium-intracellulare (MAI) pulmonary infection may occur in subjects with no preexisting lung disease and no known immunodeficiency, showing radiologically nodular bronchiectasis. There have remained some unresolved problems in the pathogenesis of the disorder, including the predominance in elderly women and the presence of not deteriorated or deteriorated disease. In the present study, we examined whether immunogenetic susceptibility is present in the disorder. We evaluated 64 cases of MAI disease and analyzed their short-term natural history by assessing symptoms, sputum bacteriology, and chest computed tomographic findings. The frequencies of human leukocyte antigen (HLA) alleles in patients were compared with those in 100 healthy Japanese control subjects. We assayed the HLA-A, -B, -C, -DR, and -DQ antigens serologically. Among 64 patients, 37 (35 females) did not show deterioration, whereas 27 (24 females) showed deterioration after an interval of 30 +/- 15 mo. There was no significant frequency of HLA-B and -C alleles in either group. In 37 not deteriorated patients, DR-6 was positive in 14 (37.8%) patients but in only 16 (16%) control subjects (p = 0.0061, odds ratio [OR] = 3.20). DQ-4 was positive in 10 (27.0%) patients but in only 10 (10%) control subjects (p = 0. 0122, OR = 3.33). In 27 deteriorated patients, HLA-A26 was positive in 14 (51.9%) patients but in only 21 (21.0%) control subjects (p = 0.0015, OR = 4.05). MAI pulmonary infection with nodular bronchiectasis shows two types of outcome, deteriorated and not deteriorated. The subjects with A-26 antigen might indicate the deterioration of MAI infection.
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PMID:Analysis of HLA antigens in Mycobacterium avium-intracellulare pulmonary infection. 1076 35


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