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)

Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2--8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.
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PMID:Hyperimmunoglobulinemia E syndrome: radiographic observations. 45 Dec 23

Recurrent pneumonia is defined as two or (usually) more separate episodes of lower respiratory tract infection that generally are accompanied by fever, leucocytosis, and purulent sputum production. These episodes are separated by an asymptomatic interval of at least 1 month or clearing of the chest visible by radiograph. Clinical improvement and radiological clearing should result after appropriate antimicrobial therapy. Chronic pneumonia is an illness that lasts at least 6 weeks and is caused by a microorganism. The chest radiograph usually shows diffuse or focal shadows. The incidence of either chronic or recurrent chest infections is unknown. Neither condition is common, but when present, they frequently present a difficult diagnostic challenge. Chronic pneumonias are usually caused by slow-growing organisms, such as fungi or mycobacteria. Occasionally, chronic pneumonias cannot be diagnosed, even when lung biopsy specimens are cultured or studied histopathologically. When a patient presents with recurrent pulmonary parenchymal infections, the clinician needs to identify the likely etiologies. Possible etiologies are structural abnormalities, underlying medical conditions, and immunological abnormalities, including infection by the human immunodeficiency virus (HIV).
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PMID:Chronic and recurrent pneumonia. 130 31

A 22-year-old male was diagnosed as having immunodeficiency with hyper-IgM based upon recurrent pneumonia, marked elevation of serum IgM and markedly decreased level of IgG. IgG-or IgA-bearing B cells were not detected in peripheral blood while a number and a proportion of peripheral blood T lymphocytes were normal. Peripheral blood lymphocytes from this patient proliferated normally in response to T-independent and T-dependent B cell mitogens, and to T cell mitogens. Furthermore, the same type of dysgammaglobulinemia with increased IgM was found in the patient's father and brother. From these observations, it is suggested that it is a rare case of autosomal dominant or polygenal inheritance of hyper-IgM immunodeficiency.
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PMID:A familial case of hyper-IgM immunodeficiency. 141 63

Phenytoin-induced panhypogammaglobulinemia mimicking the common variable immunodeficiency syndrome is rare. We describe a patient who, while being treated with phenytoin and corticosteroids, developed panhypogammaglobulinemia, recurrent pneumonia, eosinophilia, and a transient rash. Immunoglobulin levels, which had been normal prior to phenytoin therapy, returned to normal over a period of several months after the drug therapy was stopped. Levels of IgG subclasses and numbers of B cells, T cells, and T-cell subsets were determined during the recovery period. In a review of the reported cases, eosinophilia and rashes were frequently noted. These findings, along with recurrent infections in a patient receiving phenytoin therapy, should prompt a careful evaluation of the patient's immunologic status.
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PMID:Reversible common variable immunodeficiency syndrome induced by phenytoin. 273 Feb 60

A nine-month-old female Jamaican infant presented with a history of severe failure to thrive, recurrent pneumonia and developmental delay. She was found to have hepatosplenomegaly, generalised lymphadenopathy and hypotonia. Investigations revealed polyclonal hypergammaglobulinaemia, cytomegalovirus in her urine, and patchy lung infiltrates on her chest radiographs. Three separate tests were positive for human immunodeficiency virus in both the infant and her mother, suggesting vertical transmission, and confirming AIDS as the cause of the severe failure to thrive.
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PMID:AIDS in an infant causing severe failure to thrive. 366 67

Thirty-two isolates of clinically significant Streptococcus pneumoniae from 11 human immunodeficiency virus (HIV)-seropositive patients with single or multiple episodes of pneumonia were characterized by antibiotic susceptibility testing, serotyping, ribotyping, and repetitive extragenic palindromic polymerase chain reaction (REP-PCR). The isolates comprised 10 serotypes, 12 ribotyping patterns, and 12 REP-PCR patterns. There was close but not absolute correlation between techniques. By combining these characterization methods, 14 strains were identified. Five strains were found in > 1 patient, suggesting their frequent occurrence in this population. Two isolates of different serotype from 1 patient were highly related by ribotyping and REP-PCR, suggesting possible in vivo serotype change. Acute infection was associated with single strains or coinfection by distinct strains. Recurrent pneumonia was identified as relapse with the same strain or reinfection with new strains. The molecular characterization of pneumonococci from HIV-seropositive persons refines our understanding of pneumonococcal infection in these patients.
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PMID:Characterization of Streptococcus pneumoniae from human immunodeficiency virus--seropositive patients with acute and recurrent pneumonia. 756 Dec 19

The survival experience and causes of death of acquired immunodeficiency syndrome (AIDS) patients were studied using a cohort of 3,699 AIDS patients in New York State, excluding New York City, whose illness was diagnosed before January 1990 at age 13 years or older. The median length of survival for all cases was 11.5 months, and survival increased over time from 5.3 months pre-1984 to 9.3 months in 1984-1986 and to 13.2 months in 1987-1989. In a Cox proportional hazards model, risk of dying was higher for persons aged 35 years or more at diagnosis and for persons with a diagnosis other than Pneumocystis carinii pneumonia or Kaposi's sarcoma whose illness was diagnosed before 1986. In this AIDS cohort, 2,834 (77 percent) persons died before 1991; 87 percent of the death certificates listed human immunodeficiency virus (HIV)/AIDS or an AIDS indicator disease as one of the multiple causes of death. The finding that 13 percent of the death certificates did not mention AIDS/HIV suggests that use of death certificates alone to count HIV-related deaths would result in an undercount. The recent expansion of the federal AIDS case definition is expected to add HIV-infected persons who die from conditions, such as recurrent pneumonia, that were not included in the earlier definition.
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PMID:Survival and mortality patterns of an acquired immunodeficiency syndrome (AIDS) cohort in New York State. 835 73

On January 1, 1993, the acquired immunodeficiency syndrome (AIDS) surveillance case definition for adolescents and adults was expanded beyond the surveillance definition published in 1987 (1) to include all human immunodeficiency virus (HIV)-infected persons with severe immunosuppression (< 200 CD4+ T-lymphocytes/microL or a CD4+ T-lymphocyte percentage of total lymphocytes of < 14), pulmonary tuberculosis (TB), recurrent pneumonia, or invasive cervical cancer. This report reviews AIDS surveillance reports CDC received from local, state, and territorial health departments during the first quarter of 1993 and summarizes the impact of the changes in the AIDS surveillance case definition.
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PMID:Impact of the expanded AIDS surveillance case definition on AIDS case reporting--United States, first quarter, 1993. 847 24

A case of a woman having a large invasive vulvar carcinoma is reported. Because of the early age of onset and recurrent pneumonia, immunodeficiency was suspected. There appeared to be a repetitive low CD4+ T-lymphocyte count, without evidence of HIV infection or other diseases or therapies known to be clearly associated with T-cell depletion. This is suspected for the rare disorder known as idiopathic CD4+ T-lymphocytopenia which is often associated with opportunistic infections. A case of suspected idiopathic CD4+ T-lymphocytopenia in a patient having an invasive vulvar carcinoma is described.
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PMID:Suspected idiopathic CD4+ T-lymphocytopenia in a young patient with vulvar carcinoma stage IV. 864 26

On January 1, 1993, the case definition of the acquired immunodeficiency syndrome (AIDS) in adults and adolescents used for monitoring the AIDS epidemic in California was expanded to include persons infected with the human immunodeficiency virus (HIV) with CD4 T-lymphocyte counts of less than 200 x 10(6) per liter (< 200 per mm3), pulmonary tuberculosis, recurrent pneumonia, or invasive cervical cancer. To assess the implications of this revision on AIDS case reporting in California, we compared cases reported through the end of 1994 based on 1 or more of the 4 new AIDS-defining conditions added in 1993 to cases reported based on pre-1993 AIDS-defining opportunistic infections and cancers. The 4 new conditions included in the 1993 expanded AIDS case definition accounted for a 23% increase in cumulative AIDS cases reported in California by the end of 1993, a 170% increase in the number of cases reported during 1993, and an 88% increase in the number of patients with AIDS living at the end of 1993. The number of cases reported in 1993 (19,629) was 124% more than that reported in 1992 (8,780) and 69% more than that reported in 1994 (11,587). The proportion of cases among women, injection-drug users, and African Americans also increased as a result of this change in the case definition. The expansion of the case definition may have resulted in a peak or plateau in the AIDS incidence in California because of reporting earlier in the HIV disease progression. The expanded case definition has enhanced the usefulness of AIDS surveillance data for targeting secondary prevention efforts, but more behavioral and HIV serosurveys are still needed to adequately target primary HIV prevention efforts.
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PMID:Human immunodeficiency virus disease in California. Effects of the 1993 expanded case definition of the acquired immunodeficiency syndrome. 877 25


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