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 chronic granulomatous disease (CGD) enzyme-deficient neutrophils and mononuclear cells lack the respiratory burst required for biocidal activity. Recurrent infections lead to granulomas in various organs but brain lesions are rare. In the present case, a 23-year-old male with numerous infections since early childhood died of overwhelming pulmonary aspergillosis. He first began to experience neurological deficits at the age of 17. Computerized tomography and magnetic resonance imaging revealed fleeting white matter lesions that were interpreted as multiple sclerosis (MS). At post mortem, three types of brain lesions were found: (1) Pigmented macrophages in perivascular spaces and the leptomeninges similar to those reported previously. They contained fine, golden-brown, lipofuscin-like material whose chemical composition included a sulfur peak by X-ray analysis. (2) Focal, well-demarcated, "burnt out" white matter lesions with loss of both myelin and axons and intense sclerosis. (3) Diffuse areas of mild pallor in the centrum ovale which spared the U fibers. The pigmented macrophages are characteristic of those seen in the periphery in CGD. The origin of the discrete, destructive white matter lesions is unclear. They may have resulted from: (i) earlier activity by CGD macrophages; (ii) previous infections due to sepsis or embolism; or (iii) possibly post-infectious encephalomyelitis. The more diffuse, mild, white matter lesions are attributed to edema. Evidence for MS, progressive multifocal leukoencephalopathy, or human immunodeficiency virus encephalitis was lacking. This case is presented to alert us to look more carefully for brain lesions in CGD, characterize them and to help determine their cause.
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PMID:Brain lesions in chronic granulomatous disease. 202 50

Under normal condition, serum IgE concentrations are low and controlled by complex regulating mechanisms. Increased IgE synthesis may be associated with a response of IgE antibodies to allergens or parasites. It may also betray a wider disorder in the regulation of IgE production, resulting in a rise of total IgEs of unknown specificity. Broadly speaking, high IgE levels are observed in two main circumstances: allergic ans parasitic diseases. In allergic diseases the diagnostic value of high IhE concentrations is modest, since a search for specific IgEs by cutaneous tests ot laboratory techniques (RAST) is more rewarding than total IgE determination. However, total IgE assays are useful in the detection of atopy in neonates at risk and the identification and therapeutic follow-up of allergic pulmonary aspergillosis. High IgE levels are frequently associated with parasitic diseases, especially helminthiasis. They also reflect a disorder of IgE homeostasis in some types of immunodeficiency, including the classical IgE hyperproduction syndrome. Except for this syndrome, high IgE concentrations merely constitute a secondary diagnostic factor in a wide pathology, but they often bear witness to a subjacent dysregulation of T-cell function.
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PMID:[Significance of an increase of total IgE]. 204 13

Since January 1985 more than 100 patients with deep fungal infections have been treated with itraconazole (200 to 400 mg/day) in Northern Italy. Evaluation of the drug efficacy and tolerance was possible in one patient with sporotrichosis, in 34 with aspergillosis, and in 36 with cryptococcosis (mainly patients positive for human immunodeficiency virus). Response to itraconazole alone was obtained in the case of sporotrichosis and in 24 of 34 patients with different forms of aspergillosis (of the 18 patients with invasive pulmonary aspergillosis, 15 were cured). Patients with cryptococcosis received itraconazole for active infection and/or for prevention of relapse. Active infection was treated successfully with itraconazole alone in 9 of 12 patients and with itraconazole plus flucytosine in 8 of 10 patients. Of the 31 patients who received itraconazole maintenance therapy for up to 27 months, 4 (13%) had relapses; 14 (45%) did not have relapses, and decline of serum antigen was detected in 12 of them; and 13 (42%) were completely cured (serum antigen titer dropped to zero). With the exception of hypokalemia in one patient, itraconazole was well tolerated even in patients who received the drug for several months or years.
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PMID:European experience with itraconazole in systemic mycoses. 217 Apr 78

Patients with asthma who have incomplete control of their symptoms or require regular systemic steroidal therapy are said to have recalcitrant asthma. A systematic approach may significantly improve quality of life. Factors that should be evaluated include living with an antigen, occupational exposure, use of beta-adrenoreceptor blockers, use of nonsteroidal anti-inflammatory agents, sensitivity to dietary chemicals, endocrinopathies, gastroesophageal reflux, sinusitis, bronchopulmonary aspergillosis, and noncompliance. Other diseases may mimic asthma or exacerbate nonspecific bronchial hyperreactivity. These include congestive heart failure, chronic infectious bronchitis resulting from cystic fibrosis, ciliary dysfunction syndrome, and immunodeficiency syndromes, upper airway obstruction, pertussis syndrome, psychogenic coughs, bronchiolitis obliterans, chronic eosinophilic pneumonia, and vasculitides. A systematic approach to the evaluation of coexisting factors and potential exacerbating diseases is presented.
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PMID:Recalcitrant asthma: an allergist's approach. 229 75

Remission rate of leukemia has been improved by development of recent antileukemic chemotherapy. But opportunistic infection in secondary immunodeficiency has increased. Especially pulmonary mycoses often occurs and its prognosis is poor. But if patient with leukemia complicates pulmonary mycoses, it should be cured as soon as possible and antileukemic chemotherapy should be continued. We successfully performed surgical treatment for 2 pulmonary aspergillosis, which were complicated in stage of remission induction of acute leukemia.
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PMID:[Two resected cases of pulmonary aspergilloma in acute leukemia]. 237 11

A 24-year-old man with an 11-year history of i.v. drug use rapidly developed parkinsonism clinically indistinguishable from MPTP toxicity and Parkinson's disease. Although tests were negative for the human immunodeficiency virus, radiologic evaluation revealed bilateral striatal lesions. Stereotactic biopsy demonstrated septate hyphae consistent with either aspergillosis or mucormycosis. Gradual improvement followed systemic therapy with amphotericin B.
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PMID:Parkinsonism secondary to bilateral striatal fungal abscesses. 281 92

Pulmonary infection with Aspergillus can present a variety of clinical manifestations. Essentially, allergic bronchopulmonary aspergillosis, aspergilloma, and invasive aspergillosis are the three main forms. The coexistence of more than one of these entities is not unusual. Predisposing factors for such mycotic infections include previous lung-structure damage, immunodeficiency, and disturbances in the equilibrium of the resident flora of the respiratory tract. The radiological changes in pulmonary aspergillosis are described in association with clinical presentations and laboratory findings and are demonstrated by actual examples.
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PMID:[Clinical pictures of pulmonary aspergillosis]. 331 91

This report describes the first two Japanese cases of AIDS developed in patients with hemophilia. One was a 48-year-old man with hemophilia B and the other was a 62-year-old patient with hemophilia A. They had nonspecific prodromal syndromes of AIDS initially, and later developed fatal opportunistic infections with Candida or atypical mycobacteriosis and Aspergillosis. They had marked cellular immunodeficiency, and antibodies to HTLV-III. Subsequently, three more cases of AIDS in hemophiliacs have been reported in Japan. Because most of the clotting factor concentrates for treatment of hemophilia are imported from the United States and European countries, hemophiliac patients in Japan are also at high risk for AIDS.
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PMID:Occurrence of AIDS in hemophiliacs in Japan. 407 Oct 60

Mice with either chemically suppressed immune response or with specifically induced T cell or B cell immunodeficiency were challenged with Aspergillus fumigatus spores. Chemical immunosuppressants used were cortisone, cyclophosphamide or silica. T cell deficiency was produced by thymectomy and ALS administration, whereas, B cell deficiency was produced by administration of anti-mouse IgM antibodies in newborn mice. The susceptibility of such animals to A. fumigatus challenge was monitored by mortality pattern and by demonstration of dissemination of fungus in different organs. The administration of cortisone or silica made mice highly susceptible to A. fumigatus infection, whereas, cyclophosphamide initially lowered host's resistance but later had little effect. Mice with B cell deficiency also did not differ significantly from normal animals. Similarly passive transfer of specific antibodies to A. fumigatus did not enhance the protection of the host to experimental aspergillosis. However, the deficiency of T cells and macrophages either alone or in combination made the animals highly susceptible to experimental aspergillosis. The results provide evidence that T cells and macrophages play essential role in immunity to murine aspergillosis. Whereas, B cell deficiency or even passive transfer of specific antibodies to A. fumigatus does not alter much the host's susceptibility to experimental aspergillosis.
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PMID:Studies on experimental aspergillosis in immunodeficient mice. 632 25

A case of invasive aspergillosis complicated by the formation of an aspergilloma is described. The patient, a 48-year-old man, was apparently healthy except for mild alcoholic steatosis of the liver. A review of the literature revealed that 5 of the 14 previously reported cases of invasive aspergillosis in seemingly immunocompetent hosts were associated with liver disease. Immunologic investigation in this case revealed transient cutaneous anergy during the acute illness and normal lymphocyte function. Assessment of polymorphonuclear leukocyte function, however, showed abnormalities of phagocytosis as well as impairment of intracellular bactericidal activity. These abnormalities may have contributed to a relative immunodeficiency. Impairment of immune function may play a role in the pathogenesis of invasive aspergillosis in some apparently healthy patients.
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PMID:Invasive aspergillosis in a "healthy" patient. 637 50


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