Source:http://linkedlifedata.com/resource/pubmed/id/21471097
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
2011-7-8
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pubmed:abstractText |
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is being recognized with increasing frequency. Diagnostic and treatment information is limited. A systematic review is presented, focusing on patient demographics, clinical presentation, diagnosis, treatment options, and outcomes. A systematic electronic literature search was conducted for adult DIPNECH cases reported in the English literature during the past 6 years. Twenty-four DIPNECH cases were identified. Another case from our institution is contributed. Women represent 92% (23 of 25). Mean age at diagnosis was 58 years (range, 36-76 yr). Most were nonsmokers (16 of 24). Symptoms included cough (71%), dyspnea (63%), and wheezing (25%) occurring days to years before diagnosis. Pulmonary function testing showed obstructive ventilatory disease in 54%. Lung nodules were seen in 15 patients (63%), ground-glass attenuation in 7 patients (29%), and bronchiectasis in 5 patients (21%). Histological confirmation required surgical lung biopsy for 88%; however, transbronchial biopsies alone were diagnostic in three patients. Treatments strategies included systemic and inhaled corticosteroids, bronchodilators, and lung resection. Available follow-up data in 17 patients showed 6 clinically improved, 7 who remained stable, and 4 clinically deteriorated. The majority of patients presenting with DIPNECH are middle-aged females with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure does occur. Long-term follow-up and treatment remains incomplete. Establishment of a national multicenter DIPNECH registry would allow formulation of optimal evidence-based guidelines for management of these patients.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
AIM
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
1535-4970
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pubmed:author | |
pubmed:issnType |
Electronic
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pubmed:day |
1
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pubmed:volume |
184
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
8-16
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pubmed:meshHeading |
pubmed-meshheading:21471097-Cough,
pubmed-meshheading:21471097-Dyspnea,
pubmed-meshheading:21471097-Female,
pubmed-meshheading:21471097-Humans,
pubmed-meshheading:21471097-Hyperplasia,
pubmed-meshheading:21471097-Lung,
pubmed-meshheading:21471097-Lung Neoplasms,
pubmed-meshheading:21471097-Middle Aged,
pubmed-meshheading:21471097-Multiple Pulmonary Nodules,
pubmed-meshheading:21471097-Neuroendocrine Cells,
pubmed-meshheading:21471097-Precancerous Conditions,
pubmed-meshheading:21471097-Respiratory Function Tests
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pubmed:year |
2011
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pubmed:articleTitle |
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview.
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pubmed:affiliation |
Division of Internal Medicine, Department of Medicine, Mayo Clinic, Scottsdale, AZ 85259-5499, USA.
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pubmed:publicationType |
Journal Article,
Review,
Case Reports
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