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)

A 24-year-old Japanese man presented with dyskeratosis congenita (DC, Zinsser-Cole-Engman syndrome) complicated by non-cirrhotic portal hypertension, signet ring carcinoma of the rectum and Pneumocystis carinii pneumonia. At the age of 9 years, he was diagnosed as having DC on the basis of typical clinical manifestations including atrophic lingual papillae, hyperpigmentation of the skin, thrombocytopenia, and ophthalmological abnormalities. A few years later pancytopenia and splenomegaly developed. At 24 years, signet ring carcinoma of the rectum was detected but could not be resected because of the severity of the pancytopenia. Death was due to respiratory failure from P. carinii pneumonia. At autopsy the case illustrated several unique findings for DC, including non-cirrhotic portal hypertension, atrophy of frontal lobe and markedly slender folia of the cerebellum and superimposed infections with herpes zoster virus and P. carinii. Striking lymphocyte depletion and atrophy of lymphoid parenchyma in lymph nodes, tonsils, spleen, gastrointestinal tract, or thymus were seen histologically. The morphological picture supports the suggestion that there is a defect in the cell-mediated immune system in patients with DC, although immunoglobulin levels in the blood are normal. The cell-immune deficiency is a major factor in the poor prognosis.
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PMID:Dyskeratosis congenita (Zinsser-Cole-Engman syndrome). An autopsy case presenting with rectal carcinoma, non-cirrhotic portal hypertension, and Pneumocystis carinii pneumonia. 216 77

An adenovirus (isolate 1452) associated with inclusion body hepatitis of bobwhite quails (Colinus virginianus) was characterized as a group I, serotype 1 avian adenovirus and was indistinguishable from quail bronchitis virus. Bobwhite quails were inoculated via the intratracheal or intraperitoneal route with 10(6) mean tissue-culture infective dose of isolate 1452 at 1, 3, 6, or 9 weeks of age. Lesions produced by either route of inoculation were similar to those of quail bronchitis and included necrotizing tracheitis, proliferative and necrotizing bronchitis and pneumonia, and multifocal necrotizing hepatitis, necrotizing splenitis with or without hyperplasia of splenic macrophages, and lymphoid necrosis and atrophy of the bursa of Fabricius. Basophilic intranuclear viral inclusions were present in respiratory mucosal epithelium, hepatocytes and occasionally bile duct epithelium, and the mucosal epithelium overlying follicles of the bursa. Results indicate that isolate 1452 is a field isolate of quail bronchitis virus and that inclusion body hepatitis of bobwhite quails is a manifestation of quail bronchitis.
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PMID:Further characterization of an avian adenovirus associated with inclusion body hepatitis in bobwhite quails. 217 32

The mechanisms that allow circulating basement membrane antibodies (Ab) to interact with the alveolar basement membrane (ABM) inducing Goodpasture's hemorrhagic pneumonitis are unknown. In laboratory animals the ABM is inaccessible to phlogogenic amounts of ABM Ab unless the permeability of the unfenestrated alveolar endothelium is increased. This study was designed to test the hypothesis that in the mouse polypeptide mediators, generated by activated lymphoid cells or cells infected by viruses, contribute to the pathogenesis of passive Goodpasture's hemorrhagic pneumonitis. In naive mice that received rabbit ABM Ab, these bound to the glomerular basement membrane but not to the ABM and their lungs were normal. In the lungs of mice injected with human recombinant IL-2 and IFN-alpha specific binding of ABM IgG, C3, and fibrinogen to the ABM, diffuse and severe erythrocyte extravasation, and accumulation of mononuclear and polymorphonuclear leukocytes were constantly observed. ABM Ab and IL-2 or ABM Ab and IFN-alpha did not produce comparable effects. Mice injected only with IL-2 and IFN-alpha had enlarged, edematous lungs without pulmonary hemorrhages. The results show that the synergism of IL-2 and IFN-alpha convert the lung into a preferential target for AMB Ab, suggesting that cytokines may have a role in the pathogenesis of human Goodpasture's pneumonitis.
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PMID:Pathogenesis of an experimental model of Goodpasture's hemorrhagic pneumonitis. 218 75

A novel X-linked combined immunodeficiency disease was found in five living males in an extended family in the United States. The age of the affected males ranged from 2.5 to 34 yr. The most prominent clinical abnormalities were a paucity of lymphoid tissue; recurrent sinusitis, otitis media, bronchitis, and pneumonia; severe varicella; and chronic papillomavirus infections. The principal immunologic features of the disorder were normal concentrations of serum immunoglobulins but restricted formation of IgG antibodies to immunogens; normal numbers of B cells and NK cells but decreased numbers of CD4+ and CD8+ T lymphocytes, particularly the CD45RA+ subpopulations; diminished proliferative responses of blood T cells to allogeneic cells, mitogens and antigens; and decreased production of IL-2 by mitogen stimulated blood lymphocytes. Thus, affected males in this family carry an abnormal gene on their X chromosome that results in a combined immunodeficiency that is distinct from previously reported disorders.
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PMID:A novel X-linked combined immunodeficiency disease. 224 35

Respiratory disorders during the course of infections in children who are HIV positive are frequent. These are interstitial pneumonia (IP) and bacterial pneumonia. The acute IP are most often infectious in particular opportunist infection, and are dominated by Pneumocystis carinii (PC, 82 to 86% of the infectious agents isolated). This is often a presenting feature of an HIV infection and the radiological picture is very variable and the diagnosis rests on bronchoalveolar lavage which is readily achievable even in the infant. The immediate outcome is usually favourable and cotrimoxazole is efficacious and well tolerated. However pneumonia to PC points to a poor prognosis because they are always associated with a profound deficit or cellular immunity. All these criteria are against the likelihood of lymphoid PI which is, however, much more frequent in children than in adults. It is the chronic interstitial pneumonia with a "large grain" miliary radiological pattern and preserved cellular immunity and the absence of opportunistic infections in particular PC pneumonia. In addition, beyond the absence of PC, the bronchoalveolar lavage liquid shows a constant hyperlymphocytosis without polymorphonuclear leucocytes which is significantly different from that which is observed during the course of PC pneumonia. The long term prognosis of chronic PI, however, remains guarded with the possibility of seeing a worsening of the immune function. As for bacterial pneumonias they usually present as an acute lobar pneumonia with a banal organism but severe gram negative pneumonias are possible justifying a detailed systematic approach in certain cases.
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PMID:[Respiratory manifestations of HIV infections in children]. 227 Mar 39

The clinical-pathological characteristics of 11 patients with intestinal nodular lymphoid hyperplasia (INLH) are described. Five fulfilled the criteria for Herman's syndrome and presented all or several of the following alterations: dysgammaglobulinemia, recurrent respiratory tract infections, sinusitis, pneumonia and giardiasis; of the remaining six cases, in five gammaglobulin levels were not quantified and in one they were normal. All the patients in this group suffered from recurrent pharyngotonsillitis, and Giardia lamblia was isolated in four. In both groups the INLH occurred in young patients with an average age of 21 years. Eight of the 11 were men. The most frequent symptoms included diarrhea, steatorrhea and weight loss. Radiologically, INLH usually was a finding affecting the jejunum and/or the ileum. Prominent lymph nodes in mucosa and submucosa were documented histologically in all cases, and a large decrease or absence of plasma cells in the lamina propria was seen in 7 of the 11. In spite of the diversity in the treatment schemes instituted, symptoms persisted for months or years after diagnosis. In two cases (one with dysgammaglobulinemia and one without) associated intestinal lymphoma existed. Other associated diseases included non-deforming joint arthritis, erythema nodosum, and intestinal infection by E. coli and Entamoeba histolytica.
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PMID:[Nodular lymphoid hyperplasia of the intestine. Clinico-pathologic characteristics in 11 cases]. 227 Mar 66

A single dose of culture fluid of Bordetella parapertussis freed from cells (CFCF) given intranasally to four-week-old mice free from intercurrent respiratory disease produced a subacute bronchopneumonia, which was similar to that induced by whole cells of ovine isolates of B parapertussis, except that the lesions were less severe and less extensive. From eight hours to 17 days after inoculation, the mice exhibited marked infiltration of neutrophils and macrophages into the alveolar septa, bronchiolar and alveolar spaces, and hyperplasia of peribronchiolar and perivascular lymphoid tissue. Electron microscopy showed damage to ciliated cells, type 1 pneumocytes and alveolar macrophages. These results suggest that extracellular toxic substance(s) produced by ovine isolates of B parapertussis might be involved in the initiation and development of lesions in ovine chronic non-progressive pneumonia.
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PMID:Pneumonia in mice produced by cell-free extract of cultures of Bordetella parapertussis. 230 Jul 9

Chronic pneumonia was investigated in a litter of young Chinese Shar Pei in which 4 of 6 dogs were affected. Serum immunoglobulin concentrations (IgA, IgG, IgM) determined by radial immunodiffusion varied over time, but were not consistently lower in affected dogs, compared with control dogs. Two dogs that died had hydrocephalus and lymphoid depletion, in addition to severe broncho-pneumonia. Evaluation of ciliary ultrastructure in 2 affected dogs revealed random orientation of adjacent respiratory tract or oviductal cilia and a greater number of microtubular disarrangements, compared with control dogs. In vivo tracheal mucociliary clearance of 99mtechnetium macroaggregated albumin was absent in 1 dog examined. The ciliary abnormalities were suspected to have resulted in an inefficient mucociliary transport system predisposing to the development of pneumonia. Further evaluation of 1 Chinese Shar Pei revealed lymphocyte mitogenesis results that were not consistently less than those of a control dog, normal total hemolytic complement values, and normal blood neutrophil chemotaxis.
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PMID:Suspected ciliary dysfunction in Chinese Shar Pei pups with pneumonia. 231 22

Interstitial lymphoid pneumonia is a rare disease characterized by diffuse infiltration of lung tissue by lymphocytes and plasmocytes. The diagnosis can be only achieved by histopathological examination of the biopsied pulmonary tissue. It should be differentiated from malignant processes of the lymphatic system. Immunosuppression is the main line therapy, with prednisone, azatioprine and cyclophosphamide.
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PMID:[Lymphoid interstitial pneumonia]. 232 29

We treated 17 patients with chronic myeloid leukemia (CML) by bone marrow transplantation using marrow from human leukocyte antigen (HLA)-matched unrelated donors. Patients were conditioned with a combination of in vivo monoclonal antibodies, chemotherapy with daunorubicin (n = 7) or busulfan (n = 10) and cyclophosphamide, and both total body and total lymphoid irradiation. Donor marrow was depleted of T cells by incubation with monoclonal antibodies of the Campath series. Fourteen (88%) of 16 evaluable patients had sustained engraftment. Four (27%) of the 15 evaluable patients developed acute graft-versus-host disease (GVHD) of grade II or greater, and 4 of 12 evaluable patients developed chronic GVHD. Three patients developed hematological and two developed cytogenetic evidence of relapse. Eight patients (47%) survive at a median follow-up of 32 months (range 10-51 months), giving an actuarial survival of 44%. Five patients remain alive without evidence of hematological or cytogenetic relapse, giving an actuarial disease-free survival of 27%. Pneumonitis caused or contributed to death in six of the nine patients who died. We conclude that T-cell depletion can prevent the severest forms of GVHD but also increases the risk of relapse after transplant with unrelated donors, as it does with HLA-identical siblings. Nevertheless the use of matched unrelated donors should be considered for CML patients who lack HLA-identical siblings.
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PMID:Bone marrow transplantation for chronic myeloid leukemia: the use of histocompatible unrelated volunteer donors. 233 31


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