Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0348321 (Haemophilus)
 15,372 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary B-cell immunodeficiency is relatively frequent and may result in recurrent bacterial infections involving notably the respiratory tract, and in chronic severe enteroviral infections in patients with agammaglobulinaemia. Selective IgG2 isotype deficiency results in pneumococcal, Haemophilus influenzae and pseudomonal infections, since it is associated with defective production of antibodies that are specifically directed against bacterial capsular polysaccharides. Progress has recently been achieved in the determination of genetic and molecular bases of some of these immunodeficiencies. In X-linked agammaglobulinaemia, the abnormal gene has been located on the long arm of the X chromosome (Xq22-23); the intrinsic B-cell abnormality blocks differentiation at the pre-B stage, before the genes coding for light chain immunoglobulins are rearranged. There is now a strong suspicion that IgA deficiency, hypoglobulinaemia with variable expression and some selective IgG isotype deficiencies are three ways of expressing one single abnormality a genetic factor of which is located in the class III region of the HLA complex and perhaps also associated with HLA class II DQ. Treatment of deficient IgG production with intravenous immunoglobulin has thoroughly altered the prognosis of these diseases. Complete IgA deficiency carries a risk of accident by production of anti-IgA antibodies, which means that patients with isolated IgA deficiency should not be treated, that these antibodies should systematically be looked for in patients with IgA deficiency associated with partial deficiency of other immunoglobulins, and that these patients should be treated with IgA-free immunoglobulin preparations.
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PMID:[Primary immune deficiencies of B-lymphocytes]. 204 14

With the exception of a few consistent pathogens--Pasteurella multocida strains of bovine hemorrhagic septicemia and fowl cholera, Actinobacillus (Haemophilus) pleuropneumoniae, Haemophilus aegyptius and Haemophilus paragallinarum--members of the family Pasteurellaceae are commensal parasites on mucous membranes of vertebrate animals. Many have pathogenic potential, which becomes manifest under conditions of immunodeficiency and stress. Pathogenesis (except in porcine atrophic rhinitis) depends on mobilization of inflammatory responses probably in large part by endotoxin with contributions from protein toxins, which interfere with leukocyte activity and, by their cytotoxicity, cause exacerbation of the inflammatory reaction. Disease patterns include pneumonic/septicemic, upper respiratory and local/traumatic. Acquired resistance is chiefly antibody-dependent, and, with current and emerging biotechnical resources, stands a good chance of being artificially achievable for many important diseases attributed to Pasteurellaceae.
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PMID:Our understanding of the Pasteurellaceae. 219 10

Twenty patients with chronic purulent rhinosinusitis were treated with TP-1 (Serono; 1 mg/kg body weight), in a double-blind cross-over trial. TP-1 was administered by daily i.m. injections for the first 14 days followed by two injections/week for 6 further weeks. The patients were immunologically special in that they had defects in their cell-mediated immune system. Fourteen showed a decreased chemotactic responsiveness of their peripheral blood monocytes as measured in the polarization assay. This defective function can probably be ascribed to the presence in serum of low molecular weight factors (LMWFs; less than 25 kD). As reported earlier, this factor shows a structural homology to the envelope protein of murine and feline leukaemia virus (P15E). Thirteen patients showed a defective delayed-type hypersensitivity (DTH) skin test reactivity towards candidin and/or streptokinase-streptodornase (Sk/Sd) antigen, 14 had a defective MIF production from their peripheral blood lymphocytes towards candidin, Sk/Sd and/or Haemophilus influenzae antigen. Eighteen patients completed the TP-1 trial and showed clinical improvements: 12 out of 15 were feeling better during TP-1 therapy and the nasal mucosa showed on inspection absent mucopurulent secretion in 13 patients. Positive bacterial culture rates for the nose decreased from 14 out of 16 to five out of 15. Placebo treatment had no significant effects. The clinical improvements were accompanied by a better performance of the cell-mediated immune system; the most significant effects were recorded in the monocyte polarization assay. The suppressive P15E-like LMWFs in serum clearly decreased during TP-1 treatment. In vitro TP-1 neutralized the immunosuppressive effect of the LMWFs. The restoring effects of TP-1 on monocyte polarization and its neutralizing activity of P15E-like LMWFs could explain the beneficial effects of thymic hormone treatment reported in adults with clinical signs of immunodeficiency in the presence of a full T cell repertoire.
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PMID:Beneficial effects of the thymic hormone preparation thymostimulin in patients with defects in cell-mediated immunity and chronic purulent rhinosinusitis. A double-blind cross-over trial on improvements in monocyte polarization and clinical effects. 219 46

Over a three-year period, 54 episodes of pneumonia were diagnosed in 45 adults infected with the human immunodeficiency virus (HIV). These episodes were reviewed in order to assess the distribution of pathogens and their clinical presentation. Thirty-six episodes were due to an opportunistic pathogen (Pneumocystis carinii in 31, Mycobacterium avium complex in 3, Mycobacterium tuberculosis in 2), and 18 were caused by non-opportunistic pathogens (11 Streptococcus pneumoniae, 2 Haemophilus influenzae, 5 unknown pathogens that responded to broad-spectrum antibiotics). Non-opportunistic pneumonias were characterized by an abrupt onset (18/18 had pulmonary symptoms of less than 7 days duration), high fever (13/18), and focal lung infiltrates (17/18). In contrast, opportunistic infections infrequently presented with pulmonary symptoms of less than 7 days duration (3/36) or high fever (7/36), and most of the chest radiograms (34/36) disclosed a diffuse lung infiltrate. In HIV-infected patients presenting with pneumonia, simple clinical and radiological data may point to bacterial pathogens. Such data could be used in selected cases to spare invasive procedures and to start empirical antibiotic therapy.
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PMID:Diagnosis of pulmonary infections in patients infected with the human immunodeficiency virus. 249 92

During the 11 month period up to 30 September 1987, 37 patients (26 male, 11 female, mean age 27 years) with respiratory symptoms who were human immunodeficiency virus (HIV) positive, were studied prospectively on 40 occasions to determine the cause of any pulmonary complications. HIV was heterosexually transmitted. Predominant symptoms were cough (89%), fever (89%), weight loss (83%), and dyspnoea (60%). Transnasal fibre-optic bronchoscopy (with bronchoalveolar lavage, bronchial brushings and transbronchial lung biopsies) was performed on 35 patients, twice on 3 patients. 'Tru-cut' lung biopsies were obtained from 2 patients who died before bronchoscopy. Pulmonary tuberculosis was the commonest disease, being found in one-third of the patients (12 of 37). Mycobacterium tuberculosis was cultured from 4; the remainder of the plates were contaminated. Pneumocystis carinii was present in 8 patients: as the sole pathogen in 3, with Streptococcus pneumoniae in 4, Staphylococcus aureus in 2, and one also had tuberculous lymphadenitis. Endobronchial Kaposi's sarcoma was seen in 6 of 7 patients with skin nodules. Bacterial pathogens isolated included Staph. aureus (5), S. pneumoniae (5), Klebsiella pneumoniae (2), Haemophilus influenzae (2), H. parainfluenzae (1) and Pseudomonas aeruginosa (1). Invading Aspergillus fumigatus was diagnosed by lung biopsy in one. No diagnosis was reached for 8 patients. It is concluded that in Central Africa pulmonary complications in AIDS patients are similar to those in Europe and North America but the incidence of different pathogens depends on the prevalence of pathogens in the community. M. tuberculosis is probably the commonest pathogen. This study has confirmed that P. carinii pneumonia does occur, but occurs less frequently.
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PMID:Pulmonary diseases in patients infected with the human immunodeficiency virus in Zimbabwe, Central Africa. 261 33

Four of the 139 children with hemophilia followed up at our center have developed septic arthritis during the past 6 years (2.9% incidence). Two infections were caused by Streptococcus pneumoniae and one each by Staphylococcus aureus and Haemophilus influenzae type B. Common features at time of presentation included fever and a 2- to 7-day history of joint pain and swelling unresponsive to factor replacement infusions. Since three of the patients were human immunodeficiency virus seropositive, we propose that human immunodeficiency virus infection may be responsible for the disproportionately high number of cases of septic arthritis observed in our patient population.
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PMID:Septic arthritis in children with hemophilia. 267 45

We report a case of Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. This is the third reported case of malignancy in the hyper-IgE syndrome. The other two cases were an 18-year-old man with Hodgkin's disease and a 10-year-old girl with histiocytic lymphoma. The patient developed retroperitoneal Burkitt's lymphoma with probable metastasis to the brain. His short life was characterized by recurrent staphylococcal skin, middle ear, and lung infections associated with extremely elevated serum concentrations of IgE. There was also an associated disturbance of bone metabolism with osteoporosis and pathologic fractures and absence of parathormone, findings that have been observed in other patients with hyper-IgE syndrome and other forms of T cell immunodeficiency. At the age of 5 years, inadequate B cell responses to immunization with antigens derived from diphtheria, tetanus, and Haemophilus influenzae type b organisms and with the OX174 bacteriophage were demonstrated in the patient. In his terminal state his in vitro lymphocyte analysis demonstrated findings of anergy. Although the precise immunologic defect in hyper-IgE syndrome is unknown, these cases of associated malignancy stress the role that a completely normal immune system plays in preventing the premature appearance of cancer.
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PMID:Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. 278 97

Immune thrombocytopenia is a well-recognized part of the clinical spectrum of infection with the human immunodeficiency virus. From November 1985 to February 1988, 15 patients who were human immunodeficiency virus-positive underwent splenectomy for refractory immune thrombocytopenia. Eight patients had thrombocytopenia only, and 7 others were pancytopenic prior to splenectomy. Three of the 15 patients fulfilled criteria for acquired immunodeficiency syndrome before splenectomy, and acquired immunodeficiency syndrome developed in 5 patients during the follow-up period. The median duration of thrombocytopenia prior to surgical therapy was 6 months. A bone marrow biopsy specimen showed hypercellularity with increased megakaryocytes. All patients had a therapeutic response to splenectomy. Long-term remission from thrombocytopenia/pancytopenia was achieved in 14 of the 15 patients during a follow-up period of 2 to 21 months. Splenectomy can be accomplished with an acceptable morbidity. Pneumonia developed postoperatively in 2 patients, but they did not manifest the characteristic picture of overwhelming postsplenectomy sepsis. They had received vaccinations against encapsulated organisms preoperatively. We conclude that splenectomy provides a durable and lasting response for HIV-related thrombocytopenia. Vaccination for Streptococcus pneumonia and Haemophilus influenzae should be given prior to splenectomy although its efficacy is not clear in this group.
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PMID:Splenectomy. The treatment of choice for human immunodeficiency virus-related immune thrombocytopenia? 278 77

Alaskan Eskimos have the highest known prevalence of invasive Haemophilus influenzae type b (Hib) disease, primarily meningitis, affecting 1-5% of all children in the first two years of life. In this population a polymorphic genetic variant of the pyrimidine pathway enzyme, uridine monophosphate kinase-3 (UMPK-3), was found to be positively associated with invasive Hib disease (relative risk 3.3) and a tendency towards a younger age at onset of illness. There was no difference in levels of naturally acquired Hib anticapsular antibody between persons with Hib disease and healthy controls in this population. This suggests that UMPK-3 may have a role in mediating non-humoral immunity to Hib. However, unlike other enzyme variants in the nucleoside synthesis pathways which result in syndromes of severe immunodeficiency, this gene appears to confer a more subtle disease susceptibility.
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PMID:Uridine monophosphate kinase 3: a genetic marker for susceptibility to Haemophilus influenzae type B disease. 286 46

A 10 1/2-year-old girl with Haemophilus-meningitis was found to have hypogammaglobulinemia. Further immunologic evaluation revealed common variable immunodeficiency. Patients suffering from severe or recurrent infections should always be suspected to have immunodeficiency disease.
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PMID:[Variable immunologic deficiency syndrome in a 10 1/2-year-old girl with Haemophilus meningitis]. 295 1


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