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)

Histologic, immunohistologic and electron microscopic findings in three children with primary immunodeficiencies are reported. Classical X-linked infantile agammaglobulinemia Bruton was present in case 1 (male, aged 16 years), selective cellular immunodeficiency with thrombopenia in case 2 (male, aged 2 1/2 years) and non-lymphopenic severe combined immunodeficiency in case 3 (male, aged 1 3/4 years). At autopsy, all three cases exhibited unusual types of pneumonia. In case 2 a generalized cytomegalovirus infection was present. Case 3 disclosed panmyelopathia and chronic liver lesions due to severe GvH-reaction subsequent to bone marrow transplantation. A detailed morphologic study of the immune system revealed distinct alterations in the thymus, spleen, and lymph nodes and the lymphatic tissues of the gastrointestinal tract characteristic of an immunodeficiency state, either humoral (case 1), cellular (case 2) or combined (case 3).
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PMID:Pathomorphology of humoral, cellular and combined primary immunodeficiencies. 19 90

The introduction of preparations of immune serum globulin that are safe for intravenous use (IVIG) has made possible safe and effective prophylactic treatment for patients with a variety of humoral immunodeficiencies. These include not only primary agammaglobulinemia and common variable hypogammaglobulinemia but also the antibody deficiencies that accompany chronic lymphocytic leukemia (CLL) and multiple myeloma, as well as the hypogamma-globulinemia found in very low birth weight newborns who have not received adequate transplacental IgG from their mothers. In contrast, trials to date have not shown efficacy of IVIG in preventing sepsis in burn patients. The ease of administration and efficacy of IVIG in preventing respiratory symptoms in hypogammaglobulinemic patients has suggested that many other patients presenting with sinusitis and asthma, recurrent bronchitis, and other chronic chest symptoms might also benefit from IVIG and that they should be worked up for IgG subclass or specific antibody deficiencies. Side effects of IVIG administration are generally minor and may be prevented by slow administration and/or pretreatment with aspirin or Benadryl. The only contraindication to IVIG treatment is anaphylactic sensitivity to IgA, which is extremely rare. IVIG is thus an effective and safe form of prophylaxis that can reduce the incidence of pneumonia and other respiratory infections in patients with antibody deficiency as a predisposing factor.
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PMID:Role of gamma globulin. 251 39

A kindred with five affected maternally related male members showed variable phenotypic expression of the X-linked lymphoproliferative syndrome. In one of the children, agammaglobulinemia developed following infectious mononucleosis. His brother had aplastic anemia in infancy and 21/2 years later died of overwhelming pneumonia resulting from ornithosis. A third brother died of reticulum cell sarcoma. Two maternally related male cousins died of reticulum cell sarcoma of the neck.
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PMID:X-linked lymphoproliferative syndrome. A new kindred with variable phenotypic expression. 388 47

This review has concentrated on clinical syndromes for which a congenital basis of polymorphonuclear neutrophil dysfunction has been identified. The first clinical syndrome found to be associated with dysfunctional polymorphs was chronic granulomatous disease of childhood. Identification of a cellular defect in oxidative metabolism and microbicidal activity of polymorphonuclear neutrophils from patients with CGD stimulated intense investigation of the function of phagocytes in several clinical entities characterized by increased susceptibility to infection. Other diseases with a probable congenital basis for polymorph dysfunction include Chediak-Higashi syndrome, myeloperoxidase deficiency, severe glucose-6-phosphate dehydrogenase deficiency, and Down's syndrome. Functional defects have also been identified in neutrophils with morphologic abnormalities, such as the Pelger-Huet anomaly and the May-Hegglin anomaly, and in neutrophils without alkaline phosphatase or with a disorder of the glutathione system. The evidence for a relation between these cellular disorders and susceptibility to infection is tentative. Patients with congenital disorders of polymorphonuclear neutrophil microbicidal function frequently suffer prolonged infections in spite of appropriate antimicrobial therapy, and severe lesions recur with discouraging frequency. These lesions are usually soft tissue or bone abscesses, and the etiologic agents are typically staphylococci, gram-negative enteric species, or fungi. The infectious disease problems of patients with phagocytic cell disorders are usually quite distinct from the problems of patients without immunoglobulins or with complement deficiency. Patients with agammaglobulinemia, for example, suffer recurrent septicemia or meningitis due to Streptococcus pneumonia or H. influenzae. Septicemia, especially with the pyogenic bacterial species, is unusual in patients with polymorphoinuclear dysfunction. A major contribution of the currently intense investigation of cells from patients with congenital disorders of phagocyte function has been the greatly increased understanding of the molecular events necessary for the normal function of these cells. The role of the oxidative metabolic burst during phagocytosis has been clearly identified as essential to the microbicidal function of polymorphs and monocytes, and the glutathione system has been identified as essential to the regulation of these oxidative reactions. It is anticipated that these studies may lead to practical methods for "stimulating the phagocytes" in patients with increased susceptibility to infection.
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PMID:Congenital disorders of the function of polymorphonuclear neutrophils. 625 30

Adenovirus type 11, an organism not previously associated with pneumonia, caused the death of a patient with infantile x-linked agammaglobulinemia who had normal cell-mediated immunity. Despite long-standing, regular therapy with immune globulin, his serum lacked neutralizing antibody to the virus. This case confronts the conventional view that viral infections are primarily resisted by cellular immune reactions and reemphasizes the importance of antibody in the host defense against adenoviruses. It further demonstrates the continued vulnerability of such patients to certain pathogens in the presence of presumably adequate standard-dose passive immunization.
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PMID:Fatal disseminated adenovirus 11 pneumonia in an agammaglobulinemic patient. 627 90

Replacement therapy with intravenous immunoglobulin (IVIG) is currently the therapy of choice in patients with antibody-formation deficiency. Our 30-month experience with IVIG treatment in 8 patients with common variable immunodeficiency and in 4 patients with x-linked agammaglobulinemia previously treated by intramuscular immunoglobulin or low-dose IVIG is presented. Long-term dosage was 400 mg/kg once in 3--4 weeks. Ten patients reported an improvement of their health state, especially the signs of their chronic bronchitis improved. Compared to 2 cases of pneumonia which occurred within 30 months before IVIG therapy had started, no case of pneumonia occurred during IVIG treatment. Infusion rate of 4 mg/kg/min was safe enough in 11 of our patients. Serum trough IgG level is the most important for laboratory monitoring of the patients under IVIG therapy.
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PMID:[Personal experience with replacement therapy with intravenous gamma globulin]. 855 94

Usually, presenting infections in children with agammaglobulinemia include pneumonia and otitis media caused by pyogenic bacteria. We report here two cases of Pseudomonas aeruginosa septicemia with ecthyma gangrenosum, in previously healthy boys, leading to the diagnosis of X-linked agammaglobulinemia.
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PMID:X-linked agammaglobulinemia presenting as pseudomonas aeruginosa septicemia. 889 10

The clinical efficacy and tolerance of cefpirome were estimated in the treatment of patients with bacterial infection of the respiratory tract. The estimate included 15 patients with acute and chronic bronchopulmonary diseases: 6 patients with acute pneumonia, 5 with exacerbated chronic purulent obstructive bronchitis and 4 with primary immune deficiency (agammaglobulinemia and acute pneumonia). Excellent and good total efficacy of the drug was stated in 6 and 8 patients respectively. In 1 patient the treatment was discontinued because of acute urticaria. Therefore, cefpirome is to be recommended as a highly efficient agent for the treatment of bronchopulmonary infection.
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PMID:[Experience using cefpirome in patients with infections of the bronchopulmonary system]. 912 81

A-75-year old woman with agammaglobulinemia developed Moraxella catarrhalis bacteremic pneumonia. M. catarrhalis pneumonia is rarely associated with bacteremia, and neutrophils have been reported as a significant factor in the host defense system against this bacteria. This case suggests that immunoglobulin also plays a key role in the host defense system against M. catarrhalis.
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PMID:Bacteremic Moraxella catarrhalis pneumonia in a patient with immunoglobulin deficiency. 1181 May 36

Agammaglobulinemia is characterized by failure of B-cell differentiation (hypogammaglobulinemia) and increased susceptibility to bacterial infections. The present study was set up in order to evaluate the effectiveness of intravenous immunoglobulin (IVIG) treatment on the incidence of pneumonia in patients with agammaglobulinemia. We carried out chart reviews of 23 patients with agammaglobulinemia (mean age 11.5+/-5.4 years), who had been observed in a 22-year period (July 1981-January 2003) in Iran's referral center for primary immunodeficiency disorders. Nineteen of these 23 (82.5%) had been infected with pneumonia at least once before receiving the immunoglobulin treatment and 11 of them had experienced multiple episodes. During treatment with gamma-globulin - over a mean period of 6.8+/-4.1 years (range: 0.8-15.3 years) - the incidence of pneumonia requiring treatment or hospitalization decreased from 0.82 to 0.12 per patient per year (P=0.006). During IVIG replacement, hospitalization due to pneumonia decreased from 0.58 to 0.05 per patient per year (P=0.08) and the immunoglobulin G level (mean+/-S.D.) changed from 66.2+/-63.9 (range: 0-210 mg dl(-1)) to 552.4+/-199.1 (range: 136-942 mg dl(-1)) (P<0.001). Treatment of agammaglobulinemia with IVIG significantly reduced the incidence of pneumonia and hospital admission. Intensive management and regular monitoring is required in order to fully prevent severe respiratory complications.
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PMID:Efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with agammaglobulinemia. 1498 29


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