Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphopenia and restricted T cell repertoires in humans are often associated with severe eosinophilic disease and a T cell Th2 bias. To examine the pathogenesis of this phenomenon, C57BL/6 Rag2-/- mice received limited (3 x 10(4)) or large (2 x 10(6)) numbers of CD4 T cells. Three to 5 months after transfer, mice that had received 3 x 10(4) T cells, but not those that received 2 x 10(6), developed fulminant macrophage pneumonia with eosinophilia, Ym1 deposition, and methacholine-induced airway hyperresponsiveness, as well as eosinophilic gastritis; esophagitis and other organ damage occurred in some cases. Donor cells were enriched for IL-4, IL-5, and IL-13 producers. When 3 x 10(4) cells were transferred into CD3epsilon-/- hosts, the mice developed strikingly elevated serum IgE. Prior transfer of 3 x 10(5) CD25+ CD4 T cells into Rag2-/- recipients prevented disease upon subsequent transfer of CD25- CD4 T cells, whereas 3 x 10(4) regulatory T cells (Tregs) did not, despite the fact that there were equal total numbers of Tregs in the host at the time of transfer of CD25- CD4 T cells. Limited repertoire complexity of Tregs may lead to a failure to control induction of immunopathologic responses, and limitation in repertoire complexity of conventional cells may be responsible for the Th2 phenotype.
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PMID:Lymphopenic mice reconstituted with limited repertoire T cells develop severe, multiorgan, Th2-associated inflammatory disease. 1720 52

Hyperimmunoglobulin E syndrome (HIES) with recurrent infection is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses, cyst-forming pneumonia and an elevated serum immunoglobulin (Ig)E level. We report an 18-year-old man with recurrent chest infections, skin infections and dermatitis. On examination, he had the characteristic facies of HIES: high arched palate, webbing between his thumb and index finger bilaterally, and extensive scarring from multiple staphylococcal skin abscesses. He had an elevated IgE level of 14 300 kU/L. IgA and IgG deficiencies were also identified, which are rare associations of this syndrome and complicated the patient's treatment. The coexistence of HIES, IgA and IgG deficiencies has, to our knowledge, not been reported previously in the literature.
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PMID:A young man with hyperimmunoglobulin-E syndrome and IgA and IgG deficiencies. 1736 34

A 15-year-old girl with abnormal findings detected on a medical check-up chest x-ray film was admitted to our center. High-resolution computed tomography, performed upon hospitalization, demonstrated panlobular nodular darkening in left lung fields, and an expanding, blended, map-like darkening near the pleura. Since a Grocott stain-positive cyst was confirmed histopathologically by transbronchial lung biopsy, the patient was given a diagnosis of Pneumocystis carinii pneumonia. Drug therapy was initiated with sulfamethoxaxole trimethoprim (Baktar), and on the 58th day, chest CT confirmed that the darkening observed at admission had virtually disappeared. Underlying diseases, such as AIDS, malignant lymphoma and secondary immunodeficiency caused by immunosuppressive agents or adrenocorticosteroids, were excluded as the cause of P. carinii pneumonia based on clinical/laboratory findings. Under the suspicion of the possibility of primary immunodeficiency, various immunological competence tests were performed. However, no abnormal findings indicating cell-mediated immunity, humoral immunity, complement immune function, neutrophil phagocytic capacity, or bactericidal capacity were recognized. Since significant increase of serum IgE suggested hyper-IgE syndrome, IgE antibody specific to Staphylococcal enterotoxin A and B, and the exotoxins of Staphylococcus aureus were measured with positive results. Since all three diagnostic criteria for hyper-IgE syndrome (i.e., high serum IgE values, positive IgE antibody specific to Staphylococcal enterotoxin and recurrent infection) were fulfilled, hyper-IgE syndrome was diagnosed. This is a rare case of hyper-IgE syndrome as a result of P. carinii pneumonia.
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PMID:[Pneumocystis carinii pneumonia in a patient with hyper-IgE syndrome]. 1755 82

Hyper-immunoglobulin E syndrome (HIES) is a compound primary immunodeficiency characterized by a highly elevated serum IgE, recurrent staphylococcal skin abscesses and cyst-forming pneumonia, with disproportionately milder inflammatory responses, referred to as cold abscesses, and skeletal abnormalities. Although some cases of familial HIES with autosomal dominant or recessive inheritance have been reported, most cases of HIES are sporadic, and their pathogenesis has remained mysterious for a long time. Here we show that dominant-negative mutations in the human signal transducer and activator of transcription 3 (STAT3) gene result in the classical multisystem HIES. We found that eight out of fifteen unrelated non-familial HIES patients had heterozygous STAT3 mutations, but their parents and siblings did not have the mutant STAT3 alleles, suggesting that these were de novo mutations. Five different mutations were found, all of which were located in the STAT3 DNA-binding domain. The patients' peripheral blood cells showed defective responses to cytokines, including interleukin (IL)-6 and IL-10, and the DNA-binding ability of STAT3 in these cells was greatly diminished. All five mutants were non-functional by themselves and showed dominant-negative effects when co-expressed with wild-type STAT3. These results highlight the multiple roles played by STAT3 in humans, and underline the critical involvement of multiple cytokine pathways in the pathogenesis of HIES.
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PMID:Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome. 1767 33

In humans, limited T-cell receptor repertoire and lymphopenia are associated with severe eosinophilic inflammatory disease. A model of lymphopenia and reduced T-cell repertoire was created; C57BL/6 Rag2-/- mice received limited (30,000) or large (2 million) numbers of CD4 T-cells. Three to five months post-transfer, mice that had received 30,000 T-cells, but not those that received 2 million, developed fulminant macrophage pneumonia with eosinophilia, Ym1 deposition. methacholine-induced airway hyperresponsiveness, eosinophilic gastritis and esophagitis. These mice had strikingly elevated serum IgE (in CD3epsilon-/- hosts) and donor-cells were enriched for IL-4, IL-5 and IL-13 producers. Th2 pathology and serum IgE were enhanced when transferred populations were depleted of CD25+ CD4 Tregs, but was more severe when the effector population was derived from limited as compared to the large effector population. Pretreatment of Rag2-/- mice with 300,000 CD25+ CD4 Tregs prior to effector cell transfer prevented disease while pretreatment with 30,000 did not, despite the fact that there were equal numbers of Tregs in the hosts at the time of transfer of effector cells. Limited repertoire complexity of Tregs may lead to a failure to control immunopathologic responses and limited repertoire complexity of conventional cells may be responsible for the Th2 phenotype.
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PMID:Repertoire-dependent immunopathology. 1788 7

Atopy, immunodeficiency and autoimmunity are manifestations of immune system dysfunction. Classically atopy and autoimmunity are referred as distinct immunological reactions. Recent studies suggest the existence of common pathogenic mechanisms. We report the case of a teenager with familial history of asthma and miasthenia gravis in her mother (HLA- B8+) and personal history of recurrent upper respiratory infections from two to four years old, and pneumonia since five years old (3 or 4 episodes/ year, in three consecutive years), with associated dyspnoea and hypoxemia, requiring frequently hospital admission. Investigation was initially negative for atopy markers, and excluded other hypothesis as tuberculosis, cystic fibrosis, -1 antitrypsin deficiency, congenital heart disease, bronchopulmonary malformations or foreign body aspiration. Latter, further exams finally confirmed atopy with a raised IgE, positive RAST and cutaneous sensitivity tests (for house dust mites and pollen) and revealed circulating immune complexes and IgG 2, 3 e 4 deficit. Most frequent autoantibodies and precipitins study were negative, and histocompatibility antigens study revealed HLA- B8 (as her mother). Ventilation-perfusion scintigraphy and respiratory function tests were normal. Antihistamines, topical corticoids and bronchodilators were done with an excellent clinical response. At 16 years- old she is admitted again with the diagnosis of erythema nodosum and the clinical suspicion of Sweet's syndrome, having a good evolution. The relation between atopy and autoimmunity is enfatized by the authors. This simultaneous occurrence does not correspond merely to a statistical association, but may represent a global immune system impairment, with the involvement of different types of hypersensibility.
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PMID:[Atopy and autoimmunity -- a case report]. 1796 91

Hyper IgE syndrome (Job's syndrome) is a rare multiorgan disease characterized by the triad: elevated serum IgE level, recurrent sinopulmonary infections, most often staphylococcal, and cutaneous cold abscesses starting in infancy. We report 21 years old patient with hyper IgE syndrome, diagnosed at age of 6 years on the basis of hyperimmunoglobulinaemia E and recurrent pulmonary and cutaneous infections. Now he was admitted because of pneumonia complicating with pneumatocele, which could not be resolved despite intravenous antibiotics. Surgical intervention was necessary. The postoperative period was complicated by Staphyloccocus aureus sepsis.
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PMID:[Pneumatocele during long-lasting observation of hyper IgE patient]. 1797 30

Hyperimmunoglobulin-E syndrome (HIES) is a rare immunodeficiency disorder that is characterized by elevated serum concentration of IgE, eosinophilia and severe, recurrent bacterial and fungal infections. Poor regulation of immune system is evident, with decreased production of cytokines, especially interferon. Production of specific antibodies to capsular polysaccharide antigens is decreased Skeletal malformations have been reported in these patients. They can be caused by excessive production of interleukin-4, which may lead to pathologic bony tissue resorption. Due to immune system deficiency and malformations of skeletal and connective tissue, HIES is a multisystem disorder. We present a patient with recurrent bacterial infections since the early age. At the age of two years he presented with severe destructive staphylococcal pneumonia with pleural effusion, pneumatocela formation and pneumothorax. The patient also had a dysmorphic face and skeletal malformations that were most evident at the head. The diagnosis of HIES was made on the basis of elevated serum concentrations of IgE, hypereosinophily, and decreased leukocyte function in vivo and in vitro. Family history of our patient showed an autosomal-dominant inheritance pattern of HIES.
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PMID:Destructive staphylococcal pleuropneumonia in a two-year-old boy with hyperimmunoglobulin-E syndrome. 1825 11

Hyper IgE syndromes (HIES) are primary immunodeficiency disorders of unknown pathogenesis. Patients are typically affected with 'cold' abscesses of the skin, recurrent cyst-forming pneumonia, chronic mucocutaneous candidiasis and other less frequent features such as progressive skeletal abnormalities. Defective signaling in the Toll-like receptor (TLR) pathways has been suggested as a responsible pathologic mechanism, however, in previous reports, 10 patients revealed no defect in inflammatory cytokine responses to different TLR ligands. Here, we report the increase in pro-inflammatory cytokines TNF-alpha and IL-8, following TLR2 and TLR4 stimulation in a larger cohort of 25 additional patients with HIES, and provide a meta-analysis of the TLR data in HIES.
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PMID:Toll-like receptor stimulation induces higher TNF-alpha secretion in peripheral blood mononuclear cells from patients with hyper IgE syndrome. 1826 86

Clinically there are several different types of eosinophilic pneumonia (EP), but other than for tropical pulmonary eosinophilia, the humoral immune response between different types of EP, such as acute eosinophilic pneumonia (AEP), chronic EP, drug-induced EP, allergic bronchopulmonary aspergillosis, and Churg-Strauss syndrome, has not been examined. Immunoglobulin G (IgG) and E (IgE) serum concentrations were analyzed in patients with EP, or bacterial pneumonia, and in age-matched controls. Patients with AEP had lower IgG levels than the age-matched controls. Serum IgG levels in patients with AEP were significantly lower than in patients with other types EP or bacterial pneumonia. IgG2 and IgG4 were also significantly decreased in AEP, compared to age-matched controls. In AEP, the serum IgG levels were significantly decreased during active disease and increased during remission, but the serum IgE levels did not change significantly, indicating a decrease in serum IgG is a common feature of AEP. Low IgG levels were significantly correlated with serum surfactant protein D and absolute eosinophil counts in the bronchoalveolar lavage fluid of patients with AEP. This is the first reported study of immunoglobulin levels in AEP. The pathogenesis of AEP might negatively affect serum IgG levels, but not IgE levels. The present findings might indicate that serum IgG reflects the inflammatory response in AEP.
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PMID:Reduced IgG levels found during acute eosinophilic pneumonia, which normalize during recovery from disease. 1831 20


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