| Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. | |
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MedLine Citation:
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PMID: 21471097 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Adrienne A Nassar; Dawn E Jaroszewski; Richard A Helmers; Thomas V Colby; Bhavesh M Patel; Farouk Mookadam |
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Publication Detail:
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Type: Case Reports; Journal Article; Review Date: 2011-03-25 |
Journal Detail:
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Title: American journal of respiratory and critical care medicine Volume: 184 ISSN: 1535-4970 ISO Abbreviation: Am. J. Respir. Crit. Care Med. Publication Date: 2011 Jul |
Date Detail:
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Created Date: 2011-07-08 Completed Date: 2011-09-13 Revised Date: 2012-03-07 |
Medline Journal Info:
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Nlm Unique ID: 9421642 Medline TA: Am J Respir Crit Care Med Country: United States |
Other Details:
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Languages: eng Pagination: 8-16 Citation Subset: AIM; IM |
Affiliation:
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Division of Internal Medicine, Department of Medicine, Mayo Clinic, Scottsdale, AZ 85259-5499, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cough
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etiology Dyspnea / etiology Female Humans Hyperplasia Lung / pathology Lung Neoplasms / diagnosis*, pathology, physiopathology Middle Aged Multiple Pulmonary Nodules / diagnosis, pathology Neuroendocrine Cells / pathology* Precancerous Conditions / diagnosis*, pathology, physiopathology Respiratory Function Tests |
| Comments/Corrections | |
Comment In:
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Am J Respir Crit Care Med. 2012 Feb 1;185(3):341; author reply 341-2
[PMID:
22298367
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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