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)

Infections caused by fungi of the genus Aspergillus have become a major and dangerous problem in the modern treatments of blood diseases using cytotoxic drugs. Invasive pulmonary aspergillosis, generally caused by A. fumigans and A. flavius, is the usual clinical manifestation of the disease, but subacute forms have been described in patients with little immunodeficiency. The clinical signs, not very suggestive, are those of an infective pneumonia, and localized radiological forms are initially more frequent in most cases. Diagnosis rests on pathological data, but in many cases the only possible examinations are broncho-alveolar lavage and bronchial brushing which give both sensitive and specific results. Treatment is basically medical and consists of intravenous infusions of amphotericin B. The earlier the treatment the better the outcome which seems to be more favourable in patients with localized infection.
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PMID:[Invasive pulmonary aspergillosis]. 281 48

The characteristics of chest x-ray films in 19 patients with saprophytic pulmonary aspergillosis secondary to acid-fast bacilli infection were reported. The saprophytic form, exemplified by the classic fungus ball or mycetoma, results from the growth of fungal mycelia within a pre-existing area of destroyed lung, typically a pre-existing cavity such as that resulting from tuberculosis. Aspergillus fumigatus was detected in 52.6% of sputum cultures from 19 patients. Aspergillus precipitin test was positive in 68.4% of patients. Thickening of cavitary walls was first found in 85% of chest x-ray films. Eighty percent of patients had pulmonary aspergillosis within 3 years after cavitary lesions were stable. Invasive pulmonary aspergillosis (IPA) is characterized by hyphal invasion and destruction of pulmonary tissue. The risk of acquiring IPA correlates with the duration and degree of immunosuppression or neutropenia. IPA recently has been encountered in patients with human immunodeficiency virus (HIV) infection. The number of case reports on chronic necrotizing pulmonary aspergillosis is increasing, but it is supposed the clinical entity of this disease still has not been established.
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PMID:[Saprophytic and invasive pulmonary aspergillosis]. 907 Oct 93

Invasive pulmonary aspergillosis is difficult to diagnose and a critical ill with high mortality. In this paper, the diagnosis and treatment of invasive pulmonary aspergillosis complicated in 3 cases of hematological malignancy (2 acute leukemias and 1 MDS-RA) were retrospectively analysed. All patients had histories of hypoimmunity and were received prophylactic antifungal treatment. Pulmonary aspergillosis infection still occurred and confirmedly diagnosed by sputum examination. After 7 to 14 days of combination treatment of liposomal amphotericin B, itraconazole and flucytosine, 2 cases were cured and another showed effective. In conclusion, early diagnosis and treatment of invasive pulmonary aspergillosis are very critical and the therapeutic effectiveness of combined scheme with liposomal amphotericin B, itraconazole and flucytosine is very effective for pulmonary aspergillosis.
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PMID:[Diagnosis and treatment of invasive pulmonary aspergillosis in malignant hematological diseases-3 cases reported]. 1274 49

Aspergillus is a mould which may lead to a variety of infectious, allergic diseases depending on the host's immune status or pulmonary structure. Invasive pulmonary aspergillosis occurs primarily in patients with severe immunodeficiency. The significance of this infection has dramatically increased with growing numbers of patients with impaired immune state associated with the management of malignancy, organ transplantation, autoimmune and inflammatory conditions; critically ill patients and those with chronic obstructive pulmonary disease appear to be at an increased risk. The introduction of new noninvasive tests, combined with more effective and better-tolerated antifungal agents, has resulted in lower mortality rates associated with this infection. Chronic necrotising aspergillosis is a locally invasive disease described in patients with chronic lung disease or mild immunodeficiency. Aspergilloma is usually found in patients with previously formed cavities in the lung, whereas allergic bronchopulmonary aspergillosis, a hypersensitivity reaction to Aspergillus antigens, is generally seen in patients with atopy, asthma or cystic fibrosis. This review provides an update on the evolving epidemiology and risk factors of the major manifestations of Aspergillus lung disease and the clinical manifestations that should prompt the clinician to consider these conditions. Current approaches for the diagnosis and management of these syndromes are discussed.
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PMID:Pulmonary aspergillosis: a clinical review. 2188 Nov 44

Invasive pulmonary aspergillosis (IPA) has emerged as a severe infection in patients with immunocompromised hosts. However, recently, several IPA cases, without an apparent predisposition to immunodeficiency, has been reported. A 72-year-old woman was admitted for evaluation of general weakness and poor oral intake. She reported no medical history, except for intraarticular injection of a corticosteroid for joint pain for the duration of two months. A chest radiography revealed multiple cavitary nodules in both lungs. Examination of specimens, obtained by percutaneous needle biopsy, led to a diagnosis of invasive aspergillosis. Brain magnetic resonance imagining revealed numerous peripheral thin enhancing cystic nodules in both cerebral hemispheres. We initiated intravenous administration of amphotercin B. However, the patient died after nine days. Here, we report an invasive aspergillosis case, which involves the lungs and brain after a short period of steroid injection.
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PMID:Invasive aspergillosis involving the lungs and brain after short period of steroid injection: a case report. 2310 Oct 10

Fungal infections of the lung are uncommon and mainly affect people with immune deficiency. There are crucial problems in the diagnosis and treatment of this condition. Invasive pulmonary aspergillosis and candidiasis are the most common opportunistic fungal infections. Aspergillus species (spp.) are saprophytes molds that exist in nature as spores and rarely cause disease in immunocompetent individuals. In patients with immune deficiency or chronic lung disease, such as cavitary lung disease or bronchiectasis, Aspergillus may cause a variety of aspergillosis infections. Here we present a case of a 57-year-old patient without immunodeficiency or chronic lung disease who was diagnosed with endotracheal fungus ball and chronic fungal infection, possibly due to Aspergillus. Bronchoscopic examination showed a paralyzed right vocal cord and vegetating mass that was yellow in color, at the posterior wall of tracheal lumen. After 3 months, both the parenchymal and tracheal lesions were completely resolved.
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PMID:Simultaneous Chronic Invasive Fungal Infection and Tracheal Fungus Ball Mimicking Cancer in an Immunocompetent Patient. 2741 30

Invasive pulmonary aspergillosis is a life-threatening mycosis that only affects patients with immunosuppression, chemotherapy-induced neutropenia, transplantation, or congenital immunodeficiency. We studied the clinical, genetic, histological, and immunological features of 2 unrelated patients without known immunodeficiency who developed extrapulmonary invasive aspergillosis at the ages of 8 and 18. One patient died at age 12 with progressive intra-abdominal aspergillosis. The other patient had presented with intra-abdominal candidiasis at age 9, and developed central nervous system aspergillosis at age 18 and intra-abdominal aspergillosis at age 25. Neither patient developed Aspergillus infection of the lungs. One patient had homozygous M1I CARD9 (caspase recruitment domain family member 9) mutation, while the other had homozygous Q295X CARD9 mutation; both patients lacked CARD9 protein expression. The patients had normal monocyte and Th17 cell numbers in peripheral blood, but their mononuclear cells exhibited impaired production of proinflammatory cytokines upon fungus-specific stimulation. Neutrophil phagocytosis, killing, and oxidative burst against Aspergillus fumigatus were intact, but neither patient accumulated neutrophils in infected tissue despite normal neutrophil numbers in peripheral blood. The neutrophil tissue accumulation defect was not caused by defective neutrophil-intrinsic chemotaxis, indicating that production of neutrophil chemoattractants in extrapulmonary tissue is impaired in CARD9 deficiency. Taken together, our results show that CARD9 deficiency is the first known inherited or acquired condition that predisposes to extrapulmonary Aspergillus infection with sparing of the lungs, associated with impaired neutrophil recruitment to the site of infection.
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PMID:Extrapulmonary Aspergillus infection in patients with CARD9 deficiency. 2777 81

Invasive pulmonary aspergillosis (IPA) is a serious opportunistic infection mainly affecting seriously immunocompromised patients. The major risk factor is prolonged granulocytopenia. Most literature on the epidemiology and clinical impact of Aspergillus spp. infections concern patients with hematological malignancies, cancer, stem cell transplantation and solid organ transplant patients. However, evidence from recent literature indicates that Aspergillus spp. may cause invasive disease in other categories of patients without apparent immunodeficiency, including patients in intensive care units (ICUs). Clinical diagnosis of IPA in non-immunocompromised patients is difficult. Standardized diagnostic definitions, developed by the European Organization for the Research and Treatment of Cancer/Mycosis Study Group for research purposes in patients with cancer and in recipients of haematopoietic stem cell transplants, are not feasible for patient categories with an intermediate to low probability for acquiring IPA. In routine clinical practice, most Aspergillus isolates from non-sterile body sites do not represent disease. Invasive diagnostic procedures are often not feasible in patients with severe respiratory insufficiency and critical illness. The presence of systemic risk factors, or underlying predisposing lung disease or general debilitation, may enhance the clinical relevance of a positive culture. The finding of an Aspergillus spp. positive respiratory specimen in an ICU patient should not be discarded; pre-emptive antifungal treatment should be considered, while attempting to substantiate the diagnosis.
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PMID:Aspergillosis in the ICU - The new 21st century problem? 3040 37

Invasive pulmonary aspergillosis (IPA) remains difficult to diagnose and to treat. Most common risk factors are prolonged neutropenia, hematopoietic stem cell or solid organ transplantation, inherited or acquired immunodeficiency, administration of steroids or other immunosuppressive agents including monoclonal antibodies and new small molecules used for cancer therapy. Critically ill patients are also at high risk of IPA. Clinical signs are unspecific. Early computed tomography (CT)-scan identifies the two main aspects, angioinvasive and airway invasive aspergillosis. Although CT-scan findings are not fully specific they usually allow early initiation of therapy before mycological confirmation of the diagnosis. Role of 18F-fludeoxyglucose positron emission tomography with computed tomography (18F-FDG PET/CT) is discussed. Confirmation is based on microscopy and culture of respiratory samples, histopathology in case of biopsy, and importantly by detection of Aspergillus galactomannan using an immunoassay in serum and bronchoalveolar lavage fluid. Deoxyribonucleic acid detection by polymerase chain reaction is now standardized and increases the diagnosis yield. Two point of care tests detecting an Aspergillus glycoprotein using a lateral flow assay are also available. Mycological results allow classification into proven (irrespective of underlying condition), probable or possible (for cancer and severely immunosuppressed patients) or putative (for critically ill patients) IPA. New antifungal agents have been developed over the last 2 decades: new azoles (voriconazole, posaconazole, isavuconazole), lipid formulations of amphotericin B (liposomal amphotericin B, amphotericin B lipid complex), echinocandins (caspofungin, micafungin, anidulafungin). Results of main trials assessing these agents in monotherapy or in combination are presented as well as the recommendations for their use according to international guidelines. New agents are under development.
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PMID:Invasive Pulmonary Aspergillosis. 3200 Feb 86

Aspergillus is a mold that may lead to different clinical pictures, from allergic to invasive disease, depending on the patient's immune status and structural lung diseases. Chronic pulmonary aspergillosis is an infection with a locally invasive presentation, reported especially in patients with chronic pulmonary disease, while aspergilloma is typically found in patients with previously formed cavities in the lungs. Allergic bronchopulmonary aspergillosis is due to a hypersensitivity reaction to Aspergillus antigens and is more frequently described in patients with moderate-severe asthma or cystic fibrosis. Invasive pulmonary aspergillosis mainly occurs in patients with neutropenia or immunodeficiency, but has increasingly been recognized as an emerging disease of non-neutropenic patients. The significance of this infection has dramatically increased in recent years, considering the high number of patients with an impaired immune state associated with the management and treatment of neoplasm, solid or hematological transplantation, autoimmune diseases, and inflammatory conditions. Moreover, prolonged steroid treatment is recognized as an important risk factor, especially for invasive disease. In this setting, critically ill patients admitted to intensive care units and/or with chronic obstructive pulmonary disease could be at higher risk for invasive infection. This review provides an update on the clinical features and risk factors of pulmonary aspergillosis. Current approaches for the diagnosis, management, and treatment of these different forms of pulmonary aspergillosis are discussed.
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PMID:Pulmonary Aspergillosis: An Evolving Challenge for Diagnosis and Treatment. 3263 27


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