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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Hyper IgE syndrome is a rare disease consisting of recurrent sinusitis and pneumonia, pneumatoceles, chronic dermatitis, and elevated serum levels of IgE. The primary radiographic abnormalities are recurrent alveolar lung disease and pneumatoceles. Pneumothorax may occasionally occur as in one of our cases. Other causes of pneumatoceles are usually easily excluded by the history and other clinical data. Pulmonary scintigraphy and computed tomography may add information valuable to the management of these patients.
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PMID:Hyperimmunoglobulinemia E syndrome: pulmonary imaging considerations. 372 44

A Taiwanese boy was diagnosed as having hyperimmunoglobulin E syndrome at the age of 4 years. At age 18 he was admitted to the hospital because of pneumonia in the left lower lobe. Abdominal pain developed 9 days later, and his condition progressed to peritonitis. Colon perforation, 10 cm distal to the ileocecal valve, was found. Double-barrel ileostomy was performed, and reanastomosis was done 1 1/2 months later. Afterward, he was fine, and he had no significant gastrointestinal problems after being discharged. To the author's knowledge, this is the first reported case of hyperimmunoglobulin E syndrome complicated by colon perforation.
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PMID:Colon perforation in a patient with hyperimmunoglobulin E (Job's) syndrome. 878 94

Three cases of Hyper-IgE syndrome are herein described, corresponding to patients aged six, twelve and 4 years, who from infancy, had suffered a variety of recurrent pulmonary infections, staphylococcal cold abscesses and chronic dermatitis. IgE counts were superior to 2.000 u/ml and eosinophilia surpassed 0.6 x 10(9) cells/l in all three cases. Deficits in the mytogen and chemotaxis responses were also seen. These patients were treated with I.V. gammaglobulin, anti-biotherapy and ascorbic acid. The possible physiopathology of this syndrome and its association with different alterations to pulmonary immunity are discussed in this paper. Hyper-IgE Syndrome is characterized by recurrent dermal and pulmonary infections (pneumonia and abscesses), dominated by elevated IgE levels, and in 1974, this Syndrome was included within the "immunodeficiencies" group, and Hill and Quie described this as a process involving recurrent bacterial infections and eczemas accompanied by elevated IgE levels, together with a defect in the mobility of neutrophils. Previously, in 1966, Job described this Syndrome in fair-skinned, red-headed young girls, suffering from eczemas and recurrent cold abscesses of staphylococcal ethiology, found on the skin, in subcutaneous tissue cells and lymph nodes. Later, Dr. Buckley defined this Syndrome by an increase in serum IgE levels, chronic dermatitis and severe repetitive infections. This Syndrome today, is still an immunological mystery, defined by two clinically compatible criteria and an increase in IgE. Other analytical abnormalities, including neutrophilic mobility, the appearance of IgE antibodies anti-Candida or staphylococcus, are non-constant findings.
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PMID:Hyper-IgE syndrome. Presentation of three cases. 911 74

Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic "cold" abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after IVIG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.
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PMID:Moderate-dose intravenous immunoglobulin treatment of Job's syndrome. Case report. 1108 45

Allergic Bronchopulmonary Aspergillosis (ABPA) is characterized by recurrent pulmonary infiltrates that can result in central bronchiectasis and bronchiolitis obliterans especially if there is a lack of recognition and treatment. The incidence of ABPA is 1-2% in patients with persistent asthma and approximately 7% (range 2-15) in patients with cystic fibrosis. The diagnostic criteria are useful in that there is no single test (with the exception of central bronchiectasis in patients with asthma) that identifies ABPA. The differential diagnosis of ABPA includes many conditions including chronic eosinophilic pneumonia, Churg Strauss Syndrome, Hyper-IgE Syndrome, persistent asthma with lobar collapse, and cases of parasitism. The most useful laboratory assays in patients who have immediate cutaneous reactivity. to Aspergillus mixes or A. fumigatus are total serum IgE concentration, elevated serum IgE-A.fumigatus and serum IgG-A.fumigatus. Prednisone remains the drug of choice yet need not be administered indefinitely.
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PMID:Clinical aspects of allergic bronchopulmonary aspergillosis. 1245 38

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

Job's or hyper immunoglobulin E recurrent infection syndrome (Hyper-IgE syndrome) is a rare, often inherited multisystem disorder, characterized by cutaneous abscesses, pneumonia, elevated IgE levels and skeletal defects. We report a case of a 22-year-old man with Job's syndrome who presented with back pain. He was found to have diffuse large B-cell lymphoma involving his second lumbar vertebrae and spleen. Treatment with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) chemotherapy achieved a complete remission after 4 cycles. A review of reported cases of lymphoma in Job's syndrome indicates an increase in relative risk of 259 (95% confidence interval 102, 416). The cause of the increased risk has yet to be defined but has similarities to a pathogenetic model of AIDS related lymphoma. In previous reports of lymphoma in Job's syndrome, patients presented with extranodal disease and had poor outcomes. With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission.
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PMID:Non-Hodgkin's lymphoma in Job's syndrome: a case report and literature review. 1562 72

The hyper-immunoglobulin E (IgE) syndromes (HIES) are primary immunodeficiencies characterized by the clinical triad of recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia, and an elevated serum IgE level of >2000 IU/ml. Most cases are sporadic; however, multiplex families displaying autosomal dominant (AD) and autosomal recessive (AR) inheritance have been described. In most sporadic and AD cases, the HIES clinical triad is part of a multisystem disorder including abnormalities of the soft tissue, skeletal, and dental systems. In contrast, those with AR-HIES have severe molluscum contagiosum and other viral infections and may develop severe neurological complications. Unlike patients with sporadic HIES and AD-HIES, those with AR-HIES lack skeletal or dental involvement and do not develop lung cysts. Additional variants of HIES are discussed in this review. The etiology of HIES is still unresolved. Recent research points toward a skewed T helper 1 (Th1) cell/Th2 cell ratio and the involvement of chemokines. Therapy for HIES is directed at prevention and management of infections by using sustained systemic antibiotics and antifungals along with topical therapy for eczema and drainage of abscesses. Anti-staphylococcal antibiotic prophylaxis is useful. Interferons, immunoglobulin supplementation, or low-dose cyclosporine A have been reported to benefit selected patients, but they are not generally indicated.
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PMID:Hyper-IgE syndromes. 1566 Oct 34

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency associated with elevated serum IgE levels, eczematous skin, recurrent cutaneous infections, and distinctive musculoskeletal features. We report two cases seen at our institution and review the current literature. Patient 1 was an 18-month-old African American boy with recurrent staphylococcal cold abscesses, pneumonia, and bacteremia. He had severely eczematous skin, ultimately complicated by eczema herpeticum. After treatment of systemic infections with culture-directed antibiotics, a brief course of cyclosporine, 5 mg/kg, improved the dermatitis and allowed transition to long-term therapy with oral trimethoprim-sulfamethoxazole. Patient 2 was a 15-year-old Caucasian boy with long-standing HIES. He has been maintained on a regimen of interferon gamma injections given 3 times weekly and monthly intravenous immunoglobulin since the age of 3 years, prophylactic antibiotics, and low-dose fluconazole. He has occasional episodes of cold abscesses and sinusitis, but has had excellent control since institution of this regimen and has not experienced any adverse effects.
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PMID:Hyperimmunoglobulin E syndrome: two cases and a review of the literature. 1663 66

Hyperimmunoglobulin E syndrome (HIES) with recurrent infection is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses, cyst-forming pneumonia and an elevated serum immunoglobulin (Ig)E level. We report an 18-year-old man with recurrent chest infections, skin infections and dermatitis. On examination, he had the characteristic facies of HIES: high arched palate, webbing between his thumb and index finger bilaterally, and extensive scarring from multiple staphylococcal skin abscesses. He had an elevated IgE level of 14 300 kU/L. IgA and IgG deficiencies were also identified, which are rare associations of this syndrome and complicated the patient's treatment. The coexistence of HIES, IgA and IgG deficiencies has, to our knowledge, not been reported previously in the literature.
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PMID:A young man with hyperimmunoglobulin-E syndrome and IgA and IgG deficiencies. 1736 34


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