Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The new fluoroquinolones have not been tested in children despite their wide spectrum of in vitro activity and efficacy, because of an observed damage to cartilage in young animals. However, in some cases they may be life-saving. We present three pediatric patients with life threatening infections in whom the fluoroquinolones were used when other antibiotics failed: A seven-year-old boy with meningitis due to multiresistant Acinetobacter calcoaceticus, a three-year-old boy with Job's syndrome with line sepsis due to Staphylococcus epidermidis and a four month-old boy with agammaglobulinemia with mixed infection due to Escherichia coli, Pseudomonas aeruginosa, and Acinetobacter spp. All three children were cured of their infections.
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PMID:Parenteral fluoroquinolones in children with life-threatening infections. 221 Aug 57

Since the age of nine weeks a red haired girl suffered from purulent dermatitis and recurrent, systemic E. coli infections. She had an excessive hyperimmunoglobulinemia E, as well as impaired granulocyte adherence and chemotaxis. Though a sepsis was evident, the granulocytes exhibited a random FITC-Concanavalin A fluorescence. In spite of intensive treatment with various antibiotics and several granulocyte transfusions the child died at the age of 2 years and 11 months. As shown by the FITC-Concanavalin A distribution, the hyperimmunoglobulinemia E may have caused a decreased membrane fluidity causing the impaired adherence and chemotaxis. This could explain the pathophysiology of the Job's Syndrome.
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PMID:[Membranes properties of granulocytes in Job's-Syndrome with E. coli-septicemia (author's transl)]. 611 94

We report the case of a 34 year old woman with acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC) due to methicillin-resistant Staphylococcus aureus (MRSA) sepsis with hyperimmunoglobulin E syndrome (HIES). Although chemotactic activity of neutrophils was impaired in this patient, neutrophils accumulated in the lungs as assessed by bronchoalveolar lavage fluid (BALF) counts. In addition to antibiotics and oxygen therapy, the administration of recombinant human granulocyte colony-stimulating factor (rhG-CSF) resulted in a remarkable recovery.
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PMID:Acute respiratory distress syndrome due to methicillin-resistant Staphylococcus aureus sepsis in hyper-IgE syndrome. 877 83

Hyperimmunoglobulin E syndrome (HIE) is a disorder characterized by extremely elevated serum levels of IgE and recurrent infections. Patients are particularly predisposed to have staphylococcal abscesses, usually involving skin, lungs, and joints; but they are also at risk for infections with other bacteria and fungi. We report the case of a 46-month-old boy with HIE who had Candida endocarditis and sepsis with a large fungal mass extending through the tricuspid valve and into the surrounding heart tissue, requiring surgical excision and replacement with a prosthetic valve. He had an indwelling central line for previous antibiotic therapy and had oral thrush for a month before presentation, which had been treated with oral nystatin. He was first seen with very dark urine, a new murmur, petechial rash, in shock, and disseminated intravascular coagulation. The white blood cell count was 38,700 with 70% segmented neutrophils, 9% banded neutrophils, 15% lymphocytes, 4% monocytes, and 2% eosinophils. Hemoglobin was 7.1, and platelet count was 14,000. Prothrombin time was 15.5, and partial thromboplastin time was 31; fibrinogen level was 110 mg/ml, and fibrin degradation products were greater than 40 mg/ml. Serum IgE was 38,664 and 44,510 on repeat measurement. He has had recurrent staphylococcal pneumonias with pneumatoceles, twice requiring segmental lung resection. Blood and tricuspid valve cultures grew Candida albicans. He was treated with amphotericin and flucytosine, and later switched to fluconazole, with good response to therapy. A literature search revealed no other reported case of Candida endocarditis in patients with HIE. Fungai endocarditis is a rare complication, which may occur in patients with HIE and indwelling central catheters.
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PMID:Candida endocarditis in a child with hyperimmunoglobulinemia E syndrome. 921 44

The hyperimmunoglobulin E (HIE) (Job's) syndrome often has it onset in childhood and is characterized by markedly elevated serum IgE levels, chronic dermatitis and recurrent pyogenic infections. Lymphoid malignancies have most commonly been associated with this syndrome while the first case in the literature of carcinoma associated with HIE syndrome was a squamous cell carcinoma of the vulva, described by Clark et al. in 1998. We observed a male patient with Job's syndrome diagnosed at age three who presented with bone pain and a metastatic epithelial tumor of the bone revealed by biopsy. Diagnostic procedures aimed at detecting the primary site showed multiple mediastinal lymph nodes with lung and liver metastases on computed tomography scans and an extradural spinal metastasis at the upper thoracic level on magnetic resonance imaging. Although the patient refused a bronchoscopic procedure, a diagnosis of pulmonary adenocarcinoma was established on the basis of sputum cytology and the clinical aspects of tumor extent. Intravenous corticosteroids and palliative radiotherapy were given for the spinal metastasis. Palliative chemotherapy could not be started because of the patient's poor performance status as well as nosocomial fungal pneumonia and pseudomonal urogenital infection with bacteremia. Despite the antifungal and broad-spectrum antimicrobial treatments, the patient died of pseudomonal sepsis.
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PMID:The development of pulmonary adenocarcinoma in a patient with Job's syndrome, a rare immunodeficiency condition. 1514 86

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

Loss-of-function mutations in the signal transducer and activator of transcription 3 gene (stat3) result in autosomal dominant hyper-IgE syndrome (AD-HIES), a condition in which patients have recurrent debilitating infections, including frequent pneumococcal and staphylococcal pneumonias. stat3 mutations cause defective adaptive TH17 cellular responses, an immune mechanism believed to be critical for clearance of pneumococcal colonization and diminished antibody responses. Here we wished to evaluate the role of stat3 in the clearance of pneumococcal carriage and immunity using mice with a stat3 mutation recapitulating AD-HIES. We show here that naive AD-HIES mice have prolonged nasal carriage of pneumococcus compared to WT mice. Mutant and wild-type mice were then immunized with a pneumococcal whole-cell vaccine (WCV) that provides TH17-mediated protection against pneumococcal colonization and antibody-mediated protection against pneumonia and sepsis. WCV-immunized AD-HIES mice made significantly less pneumococcus-specific interleukin-17A (IL-17A) and antibody than WT mice. The WCV-elicited protection against colonization was abrogated in AD-HIES mice, but immunization with WCV still protected AD-HIES mice against aspiration pneumonia/sepsis. Taken together, our results suggest that impaired clearance of nasopharyngeal carriage due to poor adaptive IL-17A responses may contribute to the increased rates of pneumococcal respiratory infection in AD-HIES patients.
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PMID:Evaluation of the Role of stat3 in Antibody and TH17-Mediated Responses to Pneumococcal Immunization and Infection by Use of a Mouse Model of Autosomal Dominant Hyper-IgE Syndrome. 2946 18