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)

A unique case of a Chinese boy with Wiskott-Aldrich syndrome (WAS) associated with Takayasu's arteritis is reported. He had eczema, epistaxis and recurrent infections since early infancy and was found to have thrombocytopenia, negative delayed-type skin hypersensitivity, low T cell number and impaired lymphocyte proliferation to phytohaemagglutinin and concanavalin A. He had high normal serum immunoglobulin (Ig)G and IgA with low IgM and isohaemagglutinin. He presented with hypertensive encephalopathy at 5.5 years of age and an aortogram demonstrated abdominal aortic aneurysm with bilateral stenosis of renal arteries resulting in renovascular hypertension. His hypertension was difficult to control medically and autotransplant of his kidneys to the iliac arteries was performed, but he died in the immediate postoperative period. The relationship between immunodeficiency and collagen-vascular disease was discussed.
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PMID:Takayasu's arteritis associated with Wiskott-Aldrich syndrome. 135 86

We describe clinical and postmortem findings in a 44-year-old man with pulmonary hypertension and infection with the human immunodeficiency virus (HIV-1). Plexogenic angiopathy and veno-occlusive lesions were present, in addition to a mild, patchy pulmonary interstitial lymphoid infiltrate. The clinical data for 14 previously reported cases of HIV-associated primary pulmonary hypertension are summarized. We speculate that these vascular changes may be due to damage from a specific immune response to HIV.
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PMID:Primary pulmonary hypertension and human immunodeficiency virus infection in a non-hemophiliac man. 174 Mar 4

The central nervous system (CNS) of 221 adults and 31 infants or children with the acquired immunodeficiency syndrome (AIDS) was examined with immunocytochemistry for infectious agents and for human immunodeficiency virus-1 (HIV-1) antigen (gp41). Since the major risk factor in this population was intravenous drug abuse, there were more female and pediatric patients than in other neuropathology autopsy series. Although children had a different spectrum of pathologic changes, including less frequent opportunistic infections, women did not differ from men in terms of types or incidence of opportunistic infections, vascular disease, neoplasia, and subacute AIDS encephalitis (SAE). Subacute AIDS encephalitis was detected in 26% of adult and 48% of pediatric brains. Immunocytochemical analysis of 100 adult and 20 pediatric brains revealed gp41 immunoreactivity in 78% and 40%, respectively. Virtually all adult brains with SAE had gp41 immunoreactivity in macrophages and microglia. Even brains with no significant pathology had frequent gp41 immunoreactivity, especially in the basal ganglia. In pediatric brains, including cases with SAE, gp41 immunoreactivity was less abundant, suggesting the possibility of latent infection or viral clearance. Spinal cords with vacuolar myelopathy or corticospinal tract degeneration had only rare gp41-positive cells. Brains from 16 aborted fetuses from HIV-1-seropositive women were all negative for gp41 immunoreactivity, but 12 brains were positive for HIV-1 by the polymerase chain reaction. These results may indicate that HIV-1 infection in fetal brains is below the limits of detection of immunocytochemistry. The differences noted between adults and children suggest that adults more often have productive CNS HIV-1 infection.
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PMID:Human immunodeficiency virus-1 infection of the nervous system: an autopsy study of 268 adult, pediatric, and fetal brains. 207 Nov 14

The experience of surgeons in Africa with patients infected with human immunodeficiency virus (HIV) suggests 5 trends: 1) an increased incidence of surgical sepsis--most commonly in the female genital tract, the pleural cavity, large joints, and the anorectal area--in HIV-infected patients; 2) an increase in surgical tuberculosis of spine, bone joints, lymph nodes, and the peritoneal cavity concomitant with an increased incidence of pulmonary tuberculosis in high-incidence countries; 3) impaired healing of wounds, wound breakdown, and the development of skin lesions and ulcers; 4) tumors whose aggressiveness is accelerated by HIV infection; and 5) new pathologies such as nonspecific cystitis, chronic osteitis, and vascular disease. In many cases, HIV infection has not been identified until after hospital patients have demonstrated a rapid, progressive decline after routine surgery. To date, only 1 study has attempted to determine the extent to which HIV infection influences the outcome of surgery. Mortality in an intensive care unit at the University Teaching Hospital in Lusaka, Zambia, differed significantly between HIV-positive and seronegative patients only in terms of pneumonia; however, seropositive patients also have an elevated, albeit nonsignificant, risk of mortality from severe gynecologic sepsis.
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PMID:Surgical pathology of HIV infection: lessons from Africa. 755 18

The circulating lupus anticoagulant is a spontaneously acquired immunoglobulin that has been associated in vivo with an increased risk of thrombotic episodes. We describe the case of a young woman with central retinal-artery occlusion associated with circulating lupus anticoagulant in the absence of systemic lupus erythematosus or any other autoimmune or collagen vascular disease. In cases of central retinal-artery occlusion without apparent cause, the presence of the lupus anticoagulant should be excluded especially in cases that have been exposed to the human immunodeficiency virus.
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PMID:Central retinal-artery occlusion in a patient with circulating lupus anticoagulant. 250 58

Five patients with classic hemophilia were found to have primary pulmonary hypertension, a disorder not previously recognized in this population. All patients had had their coagulation disorder treated for 10 years or more with self-administered lyophilized concentrates of factor VIII, and all had antibodies to human immunodeficiency virus (HIV). Primary pulmonary hypertension was confirmed by histologic means at autopsy in one patient and by lung biopsy findings in another. In the other three patients, the findings are in agreement with this diagnosis. No patient had underlying cardiac or pulmonary disease, or clinical or pathologic evidence of collagen-vascular disease, vasculitis, parasitic disorders, hemoglobinopathy, or exposure to anorexigenic agents. Whether the primary pulmonary hypertension was related to treatment with lyophilized factor VIII, or to the presence of antibodies to HIV, or both, is unknown.
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PMID:Primary pulmonary hypertension in patients with classic hemophilia. 280 26

Autoantibodies directed against the common red-cell antigen Vel are unusual, with only 2 previous cases reported. This report describes an infant girl with steroid-resistant autoimmune hemolytic anemia whose serum contained an antibody with apparent specificity for Vel antigen. Transfusion with Vel-positive cells resulted in a massive hemolytic transfusion reaction. Survival of labelled Vel-negative cells was normal up to 8 h following transfusion, and no transfusion reaction followed subsequent use of Vel-negative cells. There was no evidence of a collagen vascular disease, an immunodeficiency syndrome, or a malignancy. Although rare, anti-Vel should be considered in unexplained autoimmune hemolytic anemia.
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PMID:An infant girl with severe autoimmune hemolytic anemia: apparent anti-Vel specificity. 377 36

Apparently primary pulmonary hypertension is rarely described in patients infected with the human immunodeficiency virus (HIV). We observed a case in a HIV seropositive patient with no known risk factor for pulmonary vascular disease. The characteristics of this new association are described.
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PMID:[Primary pulmonary hypertension in a HIV positive patient]. 783 52

Rapidly accumulating evidence suggests that a proportion of patients with acquired immunodeficiency syndrome (AIDS) develop hypertensive pulmonary vascular disease reminiscent of primary pulmonary hypertension. As an initial step to explore the link between AIDS and hypertensive pulmonary vascular disease, the present study determined whether pulmonary hypertension is present in a well-characterized murine model of retrovirus-induced immunodeficiency. In agreement with previous reports, mice infected with the LP-BM5 murine leukemia virus developed polyclonal B and T cell activation followed by progressive and severe B and T cell immunodeficiency. At 12 wk postinfection, when persistent immunodeficiency was established, mice were anesthetized, and right ventricular systolic pressure was determined in open-chest, mechanically ventilated animals. Mean right ventricular systolic pressure was 14.7 +/- 1.3 mm Hg in control animals and was increased significantly to 22.5 +/- 3.2 mm Hg in virus-infected mice. Right ventricular hypertrophy was also present in infected mice as evidenced by a 27% increase in the ratio of right to left ventricular weights; there were no group-dependent differences in the left ventricular to total-body weight ratio. Morphometric evaluation indicated that medial thickness in muscularized pulmonary arteries, expressed as a percentage of the external diameter, was 9.6 +/- 0.4% in control lungs and increased to 14.4 +/- 0.5% in lungs from infected animals. Qualitative histopathologic analysis suggested increased perivascular collagen deposition in lungs from infected animals relative to control animals. Unlike AIDS patients with pulmonary hypertension, infected mice did not exhibit plexiform lesions or intimal fibrosis of the pulmonary arteries.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pulmonary hypertension in a murine model of the acquired immunodeficiency syndrome. 802 49

Bacillary angiomatosis is a newly recognized pseudoneoplastic vascular disease seen in the setting of human immunodeficiency virus (HIV) infection. The disease is characterized by a cutaneous infection with reddish papules or nodules that are similar to pyogenic granulomas or Kaposi's sarcoma in clinical appearance. It is caused by the mildly gram-negative bacillus Rochalimaea henselae, which can be identified in tissue sections by means of Warthin-Starry stain. The historical, clinical, histopathological, and microbiological aspects and the differential diagnosis and therapy of bacillary angiomatosis are reviewed. It is important to be aware of these characteristics, because these lesions can easily be treated with antibiotic therapy.
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PMID:[Bacillary angiomatosis. A pseudoneoplastic infection in AIDS patients]. 833 58


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