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)

X-linked chronic granulomatous disease (X-CGD) is a primary immunodeficiency with complete absence or malfunction of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells. Life-threatening infections especially with aspergillus are common despite optimal antimicrobial therapy. Bone marrow transplantation (BMT) is contraindicated during invasive aspergillosis in any disease setting. We report an 8-year-old patient with CGD who underwent HLA-genoidentical BMT during invasive multifocal aspergillus nidulans infection, nonresponsive to treatment with amphotericin-B and gamma-interferon. During the first 10 days post-BMT, the patient received granulocyte colony-stimulating factor (G-CSF)-mobilized, 25 Gy irradiated granulocytes from healthy volunteers plus G-CSF beginning on day 3 to prolong the viability of the transfused granulocytes. This was confirmed in vitro by apoptosis assays and in vivo by finding nitroblue tetrazolium (NBT)-positive granulocytes in peripheral blood 12 and 36 hours after the transfusions. Clinical and biological signs of infection began to disappear on day 7 post-BMT. Positron emission tomography with F18-fluorodeoxyglucose (FDG-PET) and computed tomography (CT) scans at 3 months post-BMT showed complete disappearance of infectious foci. At 2 years post-BMT, the patient is well with full immune reconstitution and no sign of aspergillus infection. Our results show that HLA-identical BMT may be successful during invasive, noncontrollable aspergillus infection, provided that supportive therapy is optimal.
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PMID:Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin-B. 976 55

Patients infected with the human immunodeficiency virus are predisposed to develop a variety of common and uncommon infectious and neoplastic pulmonary diseases. Clinical information that can stratify the risk of occurrence of these pulmonary conditions includes: 1) CD4 cell count-the most important determinant; 2) concurrent antimicrobial therapy; 3) prior travel history; 4) known latent infections that may reactivate: and 5) underlying respiratory disease. Specific pulmonary diseases are discussed including: bacterial pneumonia, bronchitis, mycobacterial and fungal infections, pneumocystis carinii pneumonia, toxoplasmosis, cytomegalovirus, Kaposi sarcoma, lymphoma, and lung cancer. A differential diagnosis can be generated based on the chest radiographic pattern. Focal or multifocal areas of consolidation usually represent conventional bacterial pneumonia or, less commonly, tuberculosis. In severely immunocompromised patients, unusual diseases causing consolidation should be considered including: Rhodococcus infection, nocardiosis, cryptococcosis, aspergillosis, and lymphoma. Nodules can be present in tuberculosis, histoplasmosis, cryptococcosis, and Kaposi sarcoma. Interstitial opacities are common in pneumocystis carinii pneumonia, histoplasmosis, and cytomegalovirus pneumonia. Cavitation and cysts are features of pneumocystis carinii pneumonia, tuberculosis, aspergillosis, and lung cancer. Disease of the airways is increasingly recognized in those with acquired immunodeficiency syndrome. Lymphadenopathy is most common in mycobacterial infection, but can be a feature of fungal infection, lymphoma, Kaposi sarcoma, and lung cancer. The combined use of clinical information, knowledge of typical conditions associated with the human immunodeficiency syndrome, and radiographic patterns offers a useful approach to the diagnosis of pulmonary disease in the patient with the human immunodeficiency virus.
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PMID:Approach to the diagnosis of pulmonary disease in patients infected with the human immunodeficiency virus. 979 33

We report the first case (to our knowledge) of vocal cord paralysis as a primary manifestation of invasive pulmonary aspergillosis, which occurred in a 69-year-old woman without immunodeficiency. Her chest radiograph showed left upper lobe infiltration with pleural thickening, and a computed tomogram of her chest showed a thick pleural reaction and fibrosis around the arch of the aorta. A transbronchial biopsy specimen revealed Aspergillus infection. The patient was treated with oral itraconazole. However, since vocal cord paralysis persisted, the patient underwent type I thyroplasty to improve vocal function. A review of the literature showed that the incidence of invasive pulmonary aspergillosis has increased, even in nonimmunocompromised subjects, and that the disease has a potential for recurrent laryngeal nerve palsy. Therefore, invasive pulmonary aspergillosis should be considered in patients with vocal cord paralysis.
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PMID:Left vocal cord paralysis as a primary manifestation of invasive pulmonary aspergillosis in a nonimmunocompromised host. 1036 29

In a prospective, randomized, double-blind trial, 149 patients with advanced human immunodeficiency virus (HIV) infection were randomized to receive itraconazole capsules (200 mg daily) and 146 to receive a matched placebo. Both groups were monitored for evidence of fungal infections. Baseline characteristics of the two groups were similar. Failure of prophylaxis occurred in 29 (19%) of the itraconazole recipients and 42 (29%) of the placebo recipients (P = .004; log-rank test). There were 6 invasive fungal infections in the itraconazole group (4, histoplasmosis; 1, cryptococcosis; 1, aspergillosis) and 19 in the placebo group (10, histoplasmosis; 8, cryptococcosis; 1, aspergillosis) (P = .0007; log-rank test). Itraconazole significantly delayed time to onset of histoplasmosis (P = .03; log-rank test) and cryptococcosis (P = .0005; log-rank test). Prophylaxis failure due to recurrent or refractory mucosal candidiasis occurred with similar frequency in the two groups (itraconazole, 15%; placebo, 16%). A survival benefit was not demonstrated. Itraconazole generally was well tolerated. Primary prophylaxis with itraconazole capsules prevents histoplasmosis and cryptococcosis in patients with HIV infection.
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PMID:Itraconazole prophylaxis for fungal infections in patients with advanced human immunodeficiency virus infection: randomized, placebo-controlled, double-blind study. National Institute of Allergy and Infectious Diseases Mycoses Study Group. 1045 33

An association exists between human immunodeficiency virus (HIV) and an increased incidence of lung cancer. Superior vena cava syndrome (SVCS) is an oncological emergency seen in the presence of chest tumours. We report on an otherwise well HIV-positive male who presented with SVCS due to lung cancer. He was commenced on dexamethasone and radiotherapy with curative intent. Treatment was complicated by accelerated steroid- and radiation-induced morbidity. The patient died of disseminated aspergillosis after receiving 27 of 35 planned radiotherapy fractions. The management of SVCS in those with HIV is challenging and requires the judicious use of steroids, antifungal prophylaxis and palliative radiotherapy doses.
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PMID:HIV complicates the management of oncological emergencies: a case involving the superior vena cava syndrome. 1059 26

The thoracic surgeon is often called on to diagnose or treat a variety of disorders associated with human immunodeficiency virus (HIV) infection. Surgical mediastinal exploration through cervical and anterior approaches is a safe and valuable modality in appropriately selected patients with unexplained mediastinal lymphadenopathy. Open lung biopsy is used in a small subset of HIV-infected patients with undiagnosed diffuse or multifocal pulmonary disease, with an anticipated diagnostic yield of more than 70%. The biopsy can be performed either thoracoscopically or via thoracotomy, based on the expertise and discretion of the surgeon. Open lung biopsy should be used very selectively and in patients with bronchoscopically confirmed diagnoses who are failing optimal medical therapy, because the impact on outcome is minuscule and because open lung biopsy is best avoided altogether in patients with established respiratory failure. Patients with acquired immune deficiency syndrome (AIDS) have an increased incidence of pneumothorax, often associated with Pneumocystis carinii pneumonia. Depending on the clinical scenario, tube thoracostomy, pleurodesis, or pleurectomy may be used. Thoracic empyema in AIDS patients requires urgent intercostal drainage and close clinical surveillance to discern the need for decortication or rib resection and open drainage. A surgical approach to pyogenic lung abscess or invasive aspergillosis is occasionally useful. Although it is controversial whether the incidence of lung cancer is increased in patients with HIV infection, HIV-positive patients with early stage nonsmall-cell lung cancer who are otherwise surgical candidates should undergo resection, especially in the era of highly active antiretroviral therapy.
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PMID:Thoracic surgical spectrum of HIV infection. 1063 16

Protist organisms (protozoa and fungi) have become increasingly prominent as opportunistic pathogens among persons infected with human immunodeficiency virus (HIV) and among organ transplant recipients--two immunocompromised populations that have increased dramatically in the past two decades. Pneumocystis carinii pneumonia continues to be the most common serious opportunistic infection (OI) among HIV-infected persons in the United States, occurring frequently among persons not previously receiving medical care. Toxoplasmosis, cryptococcosis, cryptosporidiosis, and isosporiasis occur frequently in HIV-infected persons in the developing world. Candidiasis and aspergillosis are common OIs in organ transplant recipients. As these populations of immunosuppressed patients continue to expand worldwide new OIs caused by protist pathogens are likely to emerge.
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PMID:Protists as opportunistic pathogens: public health impact in the 1990s and beyond. 1065 Dec 90

A 31-year-old Caucasian man with AIDS developed a crusted violaceous plaque under adhesive tape near a central venous catheter insertion site. Histological examination demonstrated a ruptured hair follicle containing collections of fungal hyphae typical of Aspergillus spp. A culture of the biopsy material grew Aspergillus fumigatus. The patient responded to removal of the catheter and the occlusive dressing, in addition to itraconazole therapy. Aspergillosis must be considered in the differential diagnosis of cutaneous lesions in human immunodeficiency virus-infected patients, in particular when the lesion occurs under adhesive tape or an occlusive dressing.
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PMID:Human immunodeficiency virus-related primary cutaneous aspergillosis. 1081 7

Naso-sinusal aspergillosis is an infrequent ailment which true incidence is not correctly assumed and furthermore the cases are increasing because of the progression of immunodeficiency problems. Suspicious become accentuated when repeated antibiotic treatments fail or in patients with previous dental pathology or radiologically show inside of sinus images of forcing bodies. Despite of which the diagnosis should be histological thanks to the examination of the surgical piece gained following the classic approaches through the canine fossa or endoscopic surgery. The only real treatment is surgery. We report one clinical case of an acute naso-sinusal aspergillosis with ineffectiveness of all used treatments and whose diagnose was achieved only through the anatomopathological study of the piece supplied by the Caldwell-Luc procedure.
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PMID:[Maxillary sinusitis caused by Aspergillus]. 1082 86

Immunorestitution disease (IRD) is defined as an acute symptomatic or paradoxical deterioration of a (presumably) preexisting infection that is temporally related to the recovery of the immune system. We report the temporal sequence of events that led to IRD caused by Pneumocystis carinii and Aspergillus terreus in 2 human immunodeficiency virus (HIV)-negative patients soon after the recovery of adaptive and innate immunity, respectively, and we review episodes noted in the English-language literature that fit the definition of IRD (109 episodes in 107 patients). The median time from the recovery of neutrophil counts or termination of steroid therapy to the development of IRD was 8 days in cases of pulmonary aspergillosis (23 episodes) and hepatosplenic candidiasis (8) and 21 days for viral diseases such as hepatitis B (24) and viral pneumonitis (6). For IRD due to mycobacteriosis (27 episodes) and cryptococcosis (4) in HIV-positive patients, the median interval between the initiation of highly active antiretroviral therapy (HAART) and the onset of IRD was 11 days; for viral infections, including those due to cytomegalovirus (14), hepatitis B virus (1), and hepatitis C virus (2), the median interval was 42 days. As an emerging clinical entity, IRD merits further study to optimize treatment of immunosuppressed patients.
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PMID:Immunorestitution disease involving the innate and adaptive response. 1122 60


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