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11/26. Increased susceptibility to infection in hypothermic children: possible role of acquired neutrophil dysfunction.

    The addition of hypothermia to regimens to control cerebral edema in children at our institution has been associated with a substantial incidence of infectious complications. Of the 13 children maintained at 30 degrees C to prevent cerebral edema, 3 developed haemophilus influenzae pneumonia and 2 developed streptococcus pneumoniae sepsis (one with pneumonia). The importance of neutrophil (PMN) function for elimination of bacterial pathogens prompted in vitro studies of PMN function at clinically attainable hypothermic temperatures. neutrophils at 30 degrees C had significantly less ability to migrate towards a chemotactic stimulus (45 /- 10% inhibition; P less than 0.02), to ingest staphylococci (22 /- 5% inhibition; P less than 0.01) and to be metabolically activated as measured by superoxide production (35 /- 10% inhibition; P less than 0.01) or by chemiluminescence (18 /- 8% inhibition; P less than 0.05). These in vitro findings support the clinical observation that persons with decreased body temperature may be at an increased risk for bacterial infections secondary to PMN dysfunction.
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ranking = 1
keywords = bacterial infection, infection
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12/26. cryptococcosis of the colon resembling Crohn's disease in a patient with the hyperimmunoglobulinemia E-recurrent infection (Job's) syndrome.

    A 29-yr-old woman presenting with granulomatous colitis and a chronic perirectal abscess was found to have localized cryptococcosis associated with the hyperimmunoglobulinemia E-recurrent infection (Job's) syndrome. Similarity to previous cases of esophageal cryptococcosis and ileocecal histoplasmosis suggests an association between the hyperimmunoglobulinemia E-recurrent infection syndrome and localized fungal infections of the alimentary tract. To our knowledge, this is the first well-documented case of cryptococcosis confined to the colon and perirectal tissues.
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ranking = 0.14316410094394
keywords = infection
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13/26. Evolution of the hyperimmunoglobulin E and recurrent infection (HIE, JOB's) syndrome in a young girl.

    The hyperimmunoglobulin E and recurrent infection syndrome is difficult to diagnose in children with markedly elevated IgE and recurrent superficial staphylococcus aureus infections who have not presented with a severe infection. The patient, the child of a woman with HIE, had elevated cord blood IgE. In early infancy, she had cutaneous colonization with S. aureus followed by frank impetiginous lesions. Anti-S. aureus IgE was easily detected with a highly specific ELISA assay at 2 years of age (2 years before her presentation with a S. aureus subcutaneous abscess). Thus, the measurement of anti-S. aureus IgE by this technique may be a useful laboratory test for the diagnosis of HIE before the appearance of a severe infection.
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ranking = 0.1636161153645
keywords = infection
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14/26. Hyperimmunoglobulinemia E and pregnancy: a case report.

    Hyperimmunoglobulinemia E is characterized by recurrent bacterial sinopulmonary and skin infections from birth or early childhood, with IgE levels at least 10 times greater than the upper limits of normal. The following case describes a young black woman with hyperimmunoglobulinemia E syndrome who had an uneventful pregnancy and delivery. The infant has been diagnosed as suffering from hyperimmunoglobulinemia E syndrome as well.
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ranking = 0.020452014420563
keywords = infection
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15/26. Neutrophil pyruvate kinase deficiency with recurrent staphylococcal infections: first reported case.

    A woman with an intracellular killing defect in the neutrophils had neutrophil pyruvate kinase deficiency. She had had recurrent staphylococcal infections throughout her life. The enzyme present was unstable and its kinetics were abnormal.
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ranking = 0.10226007210281
keywords = infection
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16/26. Neutrophil dysfunction associated with states of chronic and recurrent infection.

    Infants, children, and young adults who suffer chronic and recurrent bacterial or fungal infection despite adequate numbers of circulating granulocytes and normal or elevated levels of immunoglobulins should be suspected of having fundamental defects in granulocyte functioning. This article considers clinical disorders for which there is evidence for associated defects of polymorphonuclear leukocytes.
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ranking = 0.10226007210281
keywords = infection
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17/26. Esophageal cryptococcosis in a patient with the hyperimmunoglobulin E-recurrent infection (Job's) syndrome.

    patients with the hyperimmunoglobulin E-recurrent infection (Job's) syndrome, which is characterized by an elevated immunoglobulin e level, recurrent staphylococcal infections, and an abnormality of neutrophil chemotaxis, have been reported to have visceral Candida infections in addition to their more frequent pyogenic infections. We report a patient with Job's syndrome who presented with massive hematemesis secondary to esophageal cryptococcosis. A thorough evaluation for an occult neoplasm or extraesophageal cryptococcosis was negative. The patient received a 6-wk course of amphotericin b (970 mg) and 5-fluorocytosine with complete radiographic and endoscopic resolution of the lesion. He is doing well 18 mo after therapy. The patient was not anergic, and his response to T-cell mitogens, helper-to-suppressor T-cell ratio, total number of T cells, and immunoglobulin-producing capability were all normal. This case is unusual in that it is the first documentation of a cryptococcoma of the esophagus and underscores the importance of culturing abnormal specimens for unsuspected pathogens in unusual clinical circumstances.
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ranking = 0.1636161153645
keywords = infection
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18/26. Systemic lupus erythematosus in staphylococcus aureus hyperimmunoglobulinaemia E syndrome.

    The prevalence of autoimmune diseases, including systemic lupus erythematosus, is increased in failure of certain host defence mechanisms. Systemic lupus erythematosus, however, has not been recorded as a late complication of the staphylococcus aureus hyperimmunoglobulinaemia E (hyper-IgE) syndrome. Such a case was investigated in a man suffering from a classic example of the syndrome. Antinuclear antibodies were analysed on a molecular basis. The emergence of immunological and clinical features of systemic lupus erythematosus in patients with defective host defence mechanisms against staphylococcal infections is unlikely to be fortuitous and may help elucidate the pathogenesis of systemic lupus erythematosus. The observations will also aid the long term management of patients with S aureus hyper-IgE syndrome.
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ranking = 0.020452014420563
keywords = infection
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19/26. Persistent mandibular infection in three patients with lazy and incompetent phagocyte syndromes.

    Three patients with the lazy phagocyte syndrome suffered from recurrent infections in the orofacial region which persisted despite treatment with antibiotics. All had neutrophil counts at the lower normal limits which did not increase after strenuous exercise or rise during infections. Tests of chemotaxis, random mobility and the skin window were abnormal. Ingestion and intracellular killing, however, were normal in one patient and abnormal in two. The findings are compatible with the lazy leucocyte syndrome but the symptoms were not manifest in early childhood as originally described by Miller et al. (1971), and the neutrophil counts were not as low as in his patients. Two of our patients also differed in showing a defective intracellular killing for candida albicans. The lazy leucocyte syndrome should now be recognized to include different variants, some with late onset, a range in the degree of neutropenia, and some with defective ingestion and killing, though all possess a basic intrinsic defect of movement.
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ranking = 0.12271208652338
keywords = infection
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20/26. A new familial defect in neutrophil bactericidal activity.

    A 4-year-old boy with recurrent infections and his clinically healthy father showed a severe, isolated defect in bactericidal activity of peripheral neutrophil leukocytes (the mother and the only sister were normal). lymph nodes, spleen and liver of the child presented a massive infiltration by macrophages. Such infiltration and the segmentary albinism of the hair resemble traits of the chediak-higashi syndrome, but some of the most relevant traits of this syndrome are absent, since all other neutrophil functions were normal in our patient.
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ranking = 0.020452014420563
keywords = infection
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