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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients infected with human immunodeficiency virus have a variety of presentations including fevers, lymphadenopathy, rash, renal dysfunction, and neurologic and hematologic disorders. Many of these features are also seen in patients with systemic lupus erythematosus (SLE). Herein are described five patients ultimately diagnosed as having acquired immunodeficiency syndrome (AIDS) or AIDS-related complex (ARC) in whom the differential diagnosis included SLE because of multi-system disease and autoimmune phenomena, especially positive antinuclear antibodies. Serum samples from 151 consecutive patients with AIDS or ARC were examined and 19 with low titer-positive antinuclear antibodies were found (17 at 1:20 and two at 1:160). These observations suggest that SLE and human immunodeficiency virus infection may share clinical and serologic features.
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PMID:Association of human immunodeficiency virus infection and autoimmune phenomena. 325 53

Early diagnosis of acute human immunodeficiency virus (HIV) infection is difficult because patients may be seronegative for HIV at the time of presentation. We have used a serum HIV antigen (HIV-Ag) enzyme immunoassay (EIA) to diagnose acute HIV infection in four high-risk patients. The clinical syndrome in these four patients was characterized by fever (four), rash (three), myalgias-arthralgias (three), and pharyngitis (two). All patients had spontaneous resolution of their symptoms within eight to 12 days. Serum HIV antibody, as measured by a commercially available screening EIA and by Western blot analysis, was negative in all patients at time of presentation and all seroconverted on subsequent testing. Human immunodeficiency virus was isolated from two of two patients during the acute illness. Initial serum samples from all four patients were positive for HIV-Ag. Serum samples of three of four patients became negative for HIV-Ag and positive for HIV antibody. These data suggest that serum HIV-Ag detection by EIA may be useful in the diagnosis of the acute syndrome caused by HIV infection.
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PMID:Diagnosis of human immunodeficiency virus infection in seronegative homosexuals presenting with an acute viral syndrome. 330 9

Three cases of febrile pharyngitis were recorded retrospectively in a cluster of 5 men and 1 woman linked by sexual contact to a human immunodeficiency virus (HIV) carrier. In all 3 patients, a progression into clinical HIV disease was noted during an observation period of 20-25 months. The febrile pharyngitis developed similarly in each patient after an incubation time of 3-5 weeks. High fever of sudden onset and a sore bright red throat were accompanied by extreme lethargy and, in 2/3 patients, a morbilliform rash. The acute illness lasted 4-7 days and was followed by mild lymphadenopathy. All 3 patients were HIV seropositive 17-19 months later, when they first entered the study. By contrast, those 2 cases who did not fall ill, continued to be seronegative for 19-39 months after the exposure. Seroconversion of HIV could retrospectively be demonstrated in 1 of the 3 patients 2 weeks after the onset of the febrile illness. A simultaneous lack of rise in the EBV and CMV titres suggests HIV as the causative agent for this febrile mononucleosis-like pharyngitis.
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PMID:Febrile pharyngitis as the primary sign of HIV infection in a cluster of cases linked by sexual contact. 356 21

In an ongoing prospective study of homosexual men conducted in Vancouver since November 1982, 87 cases of human immunodeficiency virus (HIV) seroconversion have been documented to date. Comparison of laboratory results obtained a mean of 4.9 months before and 5.4 months after the estimated date of seroconversion revealed that a significant increase in the serum IgG level (from 1149 to 1335 mg/dl on average) and in C1q binding (from 8.8% to 14.2% on average) was associated with early HIV infection (p less than 0.001). A marginally significant decrease in the ratio of helper to suppressor (CD4 to CD8) cells (from 1.55 to 1.29 on average) was also noted (p = 0.025). A marked decrease in absolute number of CD4 cells was not seen with seroconversion, which suggests that profound loss of these cells may be a long-term effect of HIV infection. The occurrence of symptoms (including fatigue, fever, night sweats, unintentional weight loss, diarrhea, joint pains, cough unrelated to smoking, shortness of breath, oral thrush, herpes zoster and rash) did not increase with seroconversion. This finding suggests that most cases of HIV seroconversion may be asymptomatic or associated with relatively minor symptoms. On the other hand, generalized lymphadenopathy was found to develop after HIV seroconversion in about 50% of cases.
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PMID:The Vancouver Lymphadenopathy-AIDS Study: 7. Clinical and laboratory features of 87 cases of primary HIV infection. 364 8

Suramin sodium is a reverse transcriptase inhibitor with in vitro activity against the human immunodeficiency virus (HIV), the causative agent of acquired immunodeficiency syndrome (AIDS). Ninety-eight patients with AIDS manifest as opportunistic infections (n = 38), AIDS with Kaposi's sarcoma (n = 38), AIDS-related complex (n = 20), or AIDS-associated non-Hodgkin's lymphoma (NHL) (n = 2) were treated with suramin sodium at 0.5, 1.0, or 1.5 g/wk for six weeks followed by maintenance therapy with 0.5 or 1.0 g/wk. Of 72 patients who were HIV culture positive before therapy and were assessable for subsequent HIV culture 40% became culture negative during treatment, with no apparent correlation between virus recovery and serum suramin concentration. No immunologic improvement was noted. One complete clinical remission was noted in a patient with Kaposi's sarcoma and stage IV NHL. Seven minor clinical responses were also noted. Toxic reactions were generally reversible, and included fever (78%), rash (48%), malaise (43%), nausea (34%), neurologic symptoms (33%), and vomiting (20%). Suramin-induced neutropenia was noted in 26%, thrombocytopenia in 12%, a serum creatinine level of 180 mumol/L or higher (greater than or equal to 2.1 mg/dL) in 12%, liver dysfunction in 14%, and clinical and/or laboratory evidence of adrenal insufficiency in 23%. Sixteen patients died while receiving suramin or within three weeks of discontinuation of drug therapy due to infection (n = 6), hepatic failure (n = 3), pulmonary Kaposi's sarcoma (n = 2), AIDS encephalitis (n = 2), AIDS-associated NHL (n = 1), iatrogenic hemo-pneumothorax (n = 1), or pulmonary disease of uncertain etiology. Suramin as currently administered cannot be recommended as effective therapy for AIDS.
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PMID:Suramin therapy in AIDS and related disorders. Report of the US Suramin Working Group. 365 Mar 39

Clinical descriptions of acute or primary infection with the human immunodeficiency virus (HIV) are rare. Among cases previously reported, most describe an acute illness resembling infectious mononucleosis. We describe the case of a 32-year-old homosexual man with an acute illness associated with strong serologic evidence of a primary infection with HIV. This case illustrates two new clinical features: an acute, bilateral brachial neuritis, and a vesicular, pustular exanthematous and enanthematous rash. Studies of HIV-related serologic results show differential sensitivities for enzyme-linked immunosorbent assay, Western blot, immunofluorescence, and viral antigen techniques in the acute phase of HIV infection. There appears to be significant clinical heterogeneity of the acute phase of HIV infection.
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PMID:Acute infection with the human immunodeficiency virus (HIV) associated with acute brachial neuritis and exanthematous rash. 368 79

A 50-year-old severely immunodeficient woman with malignant non-Hodgkin lymphoma died from graft-versus-host disease due to transfusion of a single unit of packed red cells. Three days after this transfusion a maculo-papular rash appeared, followed by generalized erythroderma refractory to therapy and eventually progressing into generalized ulcero-squamous dermatitis. This case, and a review of other similar cases published elsewhere, prompt the authors to recommend prophylactic irradiation of blood products prior to their administration to patients with cellular immunodeficiency, particularly in cases of acute leukaemia or malignant lymphoma where patients receive intensive radio- and/or chemotherapy regimens. To appreciate the degree of cellular immunodeficiency in such risk patients, simple criteria should be developed to assess the efficiency of the cellular immune system.
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PMID:[Acute graft-versus-host disease following a single transfusion of erythrocytes]. 396 40

Four infants with skin rash, hepatosplenomegaly, lymphocytosis, eosinophilis, and histiocytic infiltration of the lymph nodes and skin are described; in each of these infants an initial diagnosis of the Letterer-Siwe syndrome was made. Postmortem findings of thymic dysplasia and poorly differentiated, lymphopenic peripheral lympoid tissue in each of the four infants, as well as antimortem clinical findings in one, established a diagnosis of severe combined immunodeficiency. From these and similar cases in the literature, we postulate that the Letterer-Siwe syndrome may not be an unusual presentation of combined immunodeficiency. Appropriate immunologic studies will help to differentiate the two disorders.
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PMID:Combined immunodeficiency presenting as the Letterer-Siwe syndrome. 444 54

Cytomegalovirus (CMV) was repeatedly isolated from urine and saliva of a 20-month-old male child with recurrent episodes of pneumonia, high fever, rash, lymphadenopathy, oral ulceration, and neutropenia. Immunologic evaluation revealed decreased serum IgG and IgA, increased IgM, depressed T- and B-lymphocyte functions, and decreased natural killer (NK) activity for herpes simplex-type I virus-infected targets. NK activity was augmented following exposure of the patient's lymphocytes to interferon (IF) in vitro. The child was treated with interferon (four courses, dosage varying from 2 million U/day to 1 million U three times/week for periods of 10, 28, 80, and 67 days, respectively, interspersed over 9 months) and hyperimmune plasma infusions every 3 weeks. Toward the end of interferon therapy oral Levamisole was started and a feeding gastrostomy was inserted to provide nutritional support. Clinical recovery was associated with reversal of immunologic abnormalities except for the hypogammaglobulinemia. Aggressive antiviral therapy (e.g., with IF) followed by immunostimulation (e.g., with Levamisole) may prove effective in controlling certain viral infections in immunodeficiency disorders.
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PMID:Persistent cytomegalovirus infection: association with profound immunodeficiency and treatment with interferon. 630 74

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
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PMID:New insight into the causes of immunodeficiency disorders. 638 1


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