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)

An apparently healthy boy was suffering from recurrent Aspergillus infections. No classical conditions of immunodeficiency were found. Studies on the patient's phagocytic system revealed neutrophils and monocytes to function normally except in Aspergillus killing (microbicidal activity for bacteria and Candida was normal). Aspergillus killing mechanisms may be complex and peculiarly selective, possibly involving both oxygen-dependent and independent mechanisms.
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PMID:Defective phagocyte Aspergillus killing associated with recurrent pulmonary Aspergillus infections. 636 99

Chronic granulomatous disease (CGD) represents an innate immunodeficiency: the reduced production of oxygen radicals in phagocytosing cells results in decreased ability to kill pathogenic microorganisms. The patients concerned suffer from severe recurrent infections due to bacteria and fungi. Prophylactic administration of trimethoprim-sulfamethoxazole, as usual in CGD-patients, has markedly reduced the incidence of bacterial infections. Now as before, however, there is a high risk to become affected by invasive fungal infections, mainly due to Aspergillus spp. which often are lethal. Therefore, a well-compatible antimycotic long-term prophylaxis effective against Aspergillus would be attractive. In the present study the compatibility of the oral triazole itraconazole was tested in 8 CGD-patients with high risk of Aspergillus infections. Itraconazole was administered in capsules with a dosage of 5.1 mg/kg body weight per day on an average for a mean range of 23 months. Periodically liver enzymes, renal retention and electrolytes were assessed as well as itraconazole serum levels. Aspergillus serology tests included complement fixation tests, IgG-ELISA, precipitation tests, IgE determination and Aspergillus-RAST. During the prophylactic treatment in all of the 8 patients no gastrointestinal side effects or hypersensitivity reactions were observed. Renal retention and serum electrolytes as well as liver enzyme values were in normal ranges with all patients. Itraconazole serum levels showed a marked intra- and interindividual variability. However, 82% of the peak levels were in ranges regarded as therapeutically effective for itraconazole. Under prophylaxis a clear decrease of Aspergillus IgG-ELISA values was observed in 5 of 7 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Long-term treatment of patients with itraconazole for the prevention of Aspergillus infections in patients with chronic granulomatous disease (CGD)]. 760 46

Multifocal fungal (Aspergillus terreus) discospondylitis was diagnosed in 2 German shepherd dogs. In one dog, the aetiology was established by means of fluoroscopic-guided disc aspiration, cytology and culture of disc material and urine. Disseminated aspergillosis was confirmed at necropsy and A. terreus cultured from numerous organs in this dog. The aetiology in the other dog was not established until therapeutic failure forced surgical curettage of disc material from which the fungus was cultured. Ketoconazole therapy failed to effect an improvement, and at necropsy, disease was localised to the spinal column, with A. terreus cultured from the affected discs and associated vertebrae. Immunodeficiency was suspected in both cases. In the case of disseminated disease a reduced lymphocyte blastogenic response was demonstrated. Reduced IgA was shown in both cases. The German shepherd breed seems to be predisposed to Aspergillus infections and IgA deficiency.
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PMID:Multifocal Aspergillus terreus discospondylitis in two German shepherd dogs. 928 36

This understood without starting paper describes general histopathological features of deep-seated mycoses in patients with acquired immunodeficiency syndrome (AIDS) detailed histological examination on cryptococcal lesions with a consideration of morphological modification caused by treatment with highly active antiretroviral therapy (HAART). In a histopathological review of 164 patients with total human immunodeficiency virus (HIV) infection, the microscopical appearance of esophageal candidiasis which was common in those with single organ involvement revealed necrotic debris containing proliferating hyphae at the site of mucosal erosions without fungal invasion of underlying tissue. The incidence of oral and esophageal candidiasis was followed by that of pulmonary aspergillosis and Candida pneumonia. Nineteen patients including one treated with HAART had generalized cryptococcosis, representing the most common generalized fungal disease. The essential histologic features of the disease were yeast cell proliferation with a histiocytic response, but only minor lymphocytic and neutrophilic components. This was different from those induced by both Candida and Aspergillus infections. Three histologic patterns were recognized in the pulmonary cryptococcal lesions, two of which could be graded with respect to the degree and type of inflammatory reaction. The first was a mild one consisting of small scattered foci of intra-alveolar cryptococcal proliferation with a histiocytic response. The second pattern involved massive cryptococcal infection, which might have been simply more extensive than that in the former. Capillary involvement of alveolar septa was an important common finding in the eighteen patients who had not been treated with HAART. The absence of T cells and decreasing function of antigen-presenting activity in histiocytes were confirmed by immunohistological examination. These findings suggest that the lungs of AIDS patients without HAART offer little resistance to bloodstream dissemination by Cryptococci. The third pattern demonstrated in the patient treated with HAART was characterized by the presence of CD4+ cells, greater response of histiocytes and multinucleated giant cell formation, and lack of massive capillary involvement.
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PMID:Histopathology of deep-seated fungal infections and detailed examination of granulomatous response against cryptococci in patients with acquired immunodeficiency syndrome. 1214 28

Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immunodeficiency that is characterized by recurrent and life-threatening infections resulting from defects in phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase system and granuloma formation due to increased inflammatory response. The most commonly involved organs are the lungs, skin, lymph nodes, and liver due to infection. It may present with recurrent pneumonia, hilar lymphadenopathy, empyema, abscess, reticulonodular patterns, and granulomas due to lung involvement. In recent years, mycobacterial disease susceptibility has been reported in CGD cases. This article presents two male cases, one of whom is aged 18 months and the other is aged 5 years, who were diagnosed with CGD and tuberculosis during examination due to extended pneumonia. This report is presented because CGD should be considered not only in the presence of skin abscesses and Aspergillus infections, but also in the differential diagnosis for cases with BCG-itis and/or tuberculosis. It should be kept in mind that mycobacterial infections can occur during the course of the disease.
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PMID:Tuberculosis masked by immunodeficiency: a review of two cases diagnosed with chronic granulomatous disease. 2862 Dec 49