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

We describe our experience at Jackson Memorial Hospital in Miami, Florida, with 172 children who were given diagnoses of perinatally acquired infection with human immunodeficiency virus type 1 (HIV-1). The 146 mothers of the children acquired HIV-1 through heterosexual contact (69 percent), intravenous drug use (30 percent), or blood transfusion (1 percent). The children presented with symptomatic disease at a median age of eight months; only 21 percent presented after the age of two years. The most common first manifestations of disease were lymphoid interstitial pneumonia (in 17 percent), encephalopathy (in 12 percent), recurrent bacterial infections (in 10 percent), and candida esophagitis (in 8 percent), for which the median survival times from diagnosis were 72, 11, 50, and 12 months, respectively. Nine percent of the children had Pneumocystis carinii pneumonia at a median age of five months and had a median survival of only one month. The median survival for all 172 children was 38 months from the time of diagnosis. Mortality was highest in the first year of life (17 percent), and by proportional-hazard analysis the probability of long-term survival is low. In multivariate analyses, early age at diagnosis and the first identifiable pattern of clinical disease were found to be independently related to survival. We conclude that children with perinatally acquired HIV-1 infection have a very poor prognosis and that most become symptomatic before one year of age. Early diagnosis is important, since there is only a short interval in which to initiate prophylactic or antiviral treatment before progressive disease begins.
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PMID:Survival in children with perinatally acquired human immunodeficiency virus type 1 infection. 259 38

Damage to the immune system induced by the human immunodeficiency virus (HIV) leads to a spectrum of opportunistic infections of which the lung is the most common site. In Europe and North America, pneumocystis carinii pneumonia is the presenting symptom in 64% of cases of acquired immunodeficiency syndrome (AIDS) and occurs at some point in 80% of AIDS victims. This infection is less common in Africa, where tuberculosis is the predominant opportunistic infection. Other AIDS-related lung infections that are gaining in prevalence include pneumonia due to pyogenic bacteria, pulmonary infection with Mycobacterium tuberculosis, and lymphoid interstitial pneumonitis. In addition, there is evidence that the lung may be extensively involved in Kaposi's sarcoma. Given the importance of the lung as a site for AIDS-related opportunistic infections, respiratory physicians will be required to become more involved in the diagnosis and management of AIDS cases.
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PMID:AIDS and the lung. Introduction. 259 18

Twenty-six lung biopsies were performed on immunocompromised children with interstitial pneumonia over a 4-year period. More than 50% of the patients had either bone marrow transplants or immunodeficiency syndromes. Biopsy diagnosis included viral (9), nonspecific interstitial pneumonitis (9), Pneumocystis carinii (7), and bacterial (1) etiologies. Findings caused a change in treatment in 15 (58%) patients, and nine of these 15 (60%) survived. Survivors included five children with viral infections treated with antiviral agents. Only one of nine patients requiring preoperative intubation survived, while 11 of 17 (65%) not intubated before operation survived. Overall survival was 46% and included 5 of 5 patients with leukemia, 2 of 3 patients with liver transplants, 2 of 6 patients with immunodeficiency syndromes, and 1 of 8 patients with bone marrow transplants. This report shows that (1) an infectious etiology was found in 65% of the cases; (2) there was a high incidence of viral pneumonitis; (3) biopsy indicated a change in treatment for the majority of the patients; (4) the change in treatment was associated with survival in 60%; (5) viral infections may be effectively treated; and (6) the timely use of lung biopsy is an important adjunct in the diagnostic and therapeutic regimen for immunocompromised children with interstitial pneumonia.
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PMID:Emergency lung biopsy in immunocompromised pediatric patients. 278 44

An empirical approach to treating Pneumocystis carinii pneumonia was adopted in a prospective study of 73 men with antibodies to human immunodeficiency virus 1 (HIV-1) presenting with respiratory problems. At presentation 49 patients (group 1) were thought to have a history, findings at clinical examination, chest radiograph, and arterial blood gas tensions typical of pneumocystis pneumonia, and empirical treatment was begun immediately. Twenty four patients (group 2) were thought to have features not typical of pneumocystis pneumonia. All patients were subsequently referred for bronchoscopy to determine the diagnosis. In group 1 four patients were excluded from the analysis because bronchoscopy was not possible. Of the remaining 45, 42 had pneumocystis pneumonia, which was diagnosed at bronchoscopy in 40, and on the basis of the clinical response to co-trimoxazole in two who had negative results from investigations. Of the three patients without pneumocystis pneumonia, one patient with lymphoid interstitial pneumonitis and Branhamella catarrhalis infection would have failed to respond to empirical treatment. The other two had multiple bacterial pathogens at bronchoscopy; one already had Kaposi's sarcoma and the other would have been misdiagnosed as having AIDS. In group 2 a specific diagnosis was made at bronchoscopy in 21 cases, including pneumocystis pneumonia in seven (all had atypical chest radiographs). In three cases no diagnosis was made and spontaneous recovery occurred. Adopting an empirical approach to treatment for typical pneumocystis pneumonia (group 1) led to the correct treatment in 43 of 45 cases (95%) and would have saved 44 of the 45 of bronchoscopies in this group. Adopting an empirical approach would have caused one patient to be misdiagnosed as having AIDS. Overall, 44 out of 69 bronchoscopies (64%) would have been saved; the specificity for the diagnosis of pneumocystis pneumonia was 85% and the sensitivity was 85%. Adopting an "empirical" treatment policy for typical pneumocystis pneumonia will cause a large reduction in the number of "high risk" bronchoscopies performed.
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PMID:Empirical treatment without bronchoscopy for Pneumocystis carinii pneumonia in the acquired immunodeficiency syndrome. 278 35

Following ablative treatment with supralethal doses of chemotherapy and total body irradiation, patients demonstrate multiple immunologic deficiencies after bone marrow transplantation. Immune function usually recovers and the risk of infection decreases within six to 12 months. However, patients in whom chronic graft-versus-host disease (GVHD) develops have persisting B and T cell abnormalities, and in vivo and in vitro studies show impaired immunoglobulin regulation and function despite normal levels of serum immunoglobulin G. This review summarizes 12 published clinical trials of immunoglobulin therapy to correct immunodeficiency and prevent infection after marrow grafting. In five controlled studies, cytomegalovirus infection developed in a total of 52 of 172 (30 percent) immunoglobulin recipients and 71 of 165 (43 percent) control patients not given globulin. In four controlled trials, interstitial pneumonia developed in a total of 21 of 127 (17 percent) immunoglobulin recipients and 40 of 94 (43 percent) control patients. Three randomized trials reported a reduced rate of GVHD or post-engraftment septicemia in immunoglobulin recipients. However, methods of immunoglobulin preparation, antibody titer, and dose and schedule of prophylaxis varied widely in these studies, as did other critical patient, transplant regimen, and supportive care factors. Accordingly, data should be interpreted with caution. Ongoing controlled clinical trials will further define the proper role of immunoglobulin therapy in bone marrow transplantation.
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PMID:Immunoglobulin therapy in bone marrow transplantation. 282 2

Conditioning therapy with aggressive chemotherapy and irradiation induces a state of transient combined immunodeficiency in bone-marrow transplant recipients. This promotes the occurrence of severe cytomegalovirus (CMV) infections, the most frequent lethal complication after bone-marrow transplantation (BMT) at present. Forty-four BMT recipients received CMV-IgG-hyperimmunoglobulin for CMV prophylaxis intravenously. The efficacy of this prophylaxis and possible risk factors for the occurrence of CMV-induced interstitial pneumonia (IP) were analyzed. Risk factors for the promotion of a CMV-IP were: additional immunosuppressive therapy after BMT, CMV-positive serostatus of the recipient, CMV-seropositive granulocyte transfusion, CMV infection immediately prior to BMT, and HLA-haploidentical BMT. In this study the incidence of graft-versus-host disease was low and was not associated with the incidence of CMV infections. The use of T-cell-depleted grafts did not result in increased CMV infections or IP and may possibly have improved the immunological reconstitution.
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PMID:Cytomegalovirus (CMV) infections in patients receiving CMV-IgG-hyperimmunoglobulin prophylaxis after bone-marrow transplantation. 282 58

The acquired immunodeficiency syndrome (AIDS) is a devastating new disease caused by the human immunodeficiency virus (HIV). This retrovirus causes profound immunoincompetence in its infected hosts, who are thereafter susceptible to develop myriad severe and relapsing protozoal, fungal, bacterial, viral, and arthropodal opportunistic infections, as well as unusual malignancies. The more than 50,000 patients who have developed AIDS in the United States have produced a sudden unexpected deluge of diagnostic dilemmas that are stressing laboratories of pathology everywhere. This paper describes the gross and microscopic pathology of the numerous complications in patients infected by HIV: (a) the prodromal AIDS-related complex with persistent generalized lymphadenopathy, (b) lymphoid infiltration of salivary gland and lung, including the complex of lymphoid interstitial pneumonitis-pulmonary lymphoid hyperplasia, (c) extranodal non-Hodgkin's lymphomas, (d) multifocal mucocutaneous and visceral Kaposi's sarcoma, (e) small cell undifferentiated (oat cell) carcinomas, (f) protozoal infections caused by Pneumocystis carinii, Toxoplasma gondii, Acanthamoeba, Cryptosporidium species (sp.), and Isospora belli, (g) the causes of chronic enteritis, (h) mycotic infections caused by Candida sp., Cryptococcus neoformans, Histoplasma capsulatum, Coccidioides immitis, and Sporothrix schenckii, (i) bacterial infections caused by Mycobacterium avium-intracellulare, M. tuberculosis, M. kansasii, Nocardia sp., Listeria monocytogenes, Legionella sp., Treponema pallidum, and others, (j) viral infections caused by cytomegalovirus, herpes simplex and zoster, polyomavirus (progressive multifocal leukoencephalopathy), hepatitis B, molluscum contagiosum, and papillomavirus, (k) oral hairy leukoplakia, (l) subacute encephalopathy, and (m) Norwegian scabies.
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PMID:The pathology of AIDS. 283 78

The authors reviewed chest radiographs of 16 patients with biopsy-proved lymphocytic interstitial pneumonia (LIP) who also had acquired immunodeficiency syndrome (AIDS) or AIDS-related complex (ARC). Radiographs revealed fine reticular or reticulonodular infiltrates in the pulmonary interstitium in five patients, coarse reticulonodular infiltrates in two, and reticular or reticulonodular opacities with superimposed patchy alveolar infiltrates in nine. Pathologic examination of biopsy specimens revealed lymphocytes, plasma cells, and reticulum cells aggregated about the small arteries and compressing the distal airways. Throughout the course of AIDS or ARC, the radiographic findings of LIP remained stable in 12 of 16 patients. AIDS/ARC-related LIP appears to be a benign reaction of bronchial-associated lymphatic tissue to the human immunodeficiency virus (HIV). However, this indolent process cannot be correctly diagnosed based on radiographic findings alone; the use of lung biopsy is required to differentiate LIP from other infections. The authors suggest that patients with these radiographic findings, HIV seropositivity, but no other signs of opportunistic infection or neoplasia should be classified as having AIDS.
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PMID:HIV-associated lymphocytic interstitial pneumonia: radiologic manifestations and pathologic correlation. 290 25

To date, the acquired immunodeficiency syndrome (AIDS) has been identified in over 50 children in the US, including those with associated hemophilia, high-risk environmental factors (Haitian background, parental intravenous drug abuse, or prostitution), and blood transfusions. The evaluation of an infant or young child in whom AIDS is suspected requires exclusion of congenital disorders of immune function. A specific test is not currently available, but inclusion criteria for childhood AIDS have been developed. The diseases accepted as indicative of underlying cellular immunodeficiency children are the same as those used in defining AIDS in adults, with the exclusion of congenital infections such as toxoplasmosis or herpes simplex virus infection in the 1st month of life or cytomegalovirus infection in the 1st 6 months of life. Specific conditions that must be excluded in children are primary immunodeficiency diseases (e.g., DiGeorge syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia, neutrophil function abnormality) and secondary immuno-deficiency associated with immunosuppressive therapy, lymphoreticular malignancy, or starvation. Almost all young children with AIDS have hepatosplenomegaly, interstitial pneumonitis, and poor growth. The average age of 36 US child AIDS victims studied in detail was 5 months at presentation with findings suggestive of severe immunodeficiency. Mucocutaneous candidiasis was present in 75% of these 36 children, and Pneumocystis carinii and cytomegalovirus were each isolated from 30% of cases. Normal T4:T8 ratios occur in about 15% of pediatric AIDS cases. Laboratory evidence of polyclonal hypergammaglobulinemia generally supports the AIDS diagnosis. Recurrent infection and malnutrition are major problems in the clinical management of child AIDS patients.
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PMID:Acquired immune deficiency syndrome in childhood. 298 8

After bone marrow transplantation (BMT) transient combined immunodeficiency occurs which promotes the occurrence of severe cytomegalovirus (CMV) infections, the most frequent lethal complication at present. 28 patients received a CMV-IgG-hyperimmunoglobulin (CMV-IG) intravenously as CMV-prophylaxis. The efficacy of this treatment and the risk factors for the occurrence of interstitial pneumonia (IP) caused by CMV were analyzed. Risk factors promoting a CMV-IP were: immunosuppression after BMT, CMV-seropositivity of recipient and donor, granulocyte transfusions and HLA-mismatched BMT. In this study graft versus host disease did not influence the occurrence of CMV-IP.
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PMID:[Risk factors for the occurrence of pneumonia caused by cytomegaloviruses in patients with bone marrow transplants during prevention with cytomegalovirus hyperimmune globulin]. 300 Sep 24


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