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Assessment and management of pulmonary alveolar proteinosis in a reference center

Overview of attention for article published in Orphanet Journal of Rare Diseases, March 2013
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Title
Assessment and management of pulmonary alveolar proteinosis in a reference center
Published in
Orphanet Journal of Rare Diseases, March 2013
DOI 10.1186/1750-1172-8-40
Pubmed ID
Authors

Ilaria Campo, Francesca Mariani, Giuseppe Rodi, Elena Paracchini, Eric Tsana, Davide Piloni, Isabella Nobili, Zamir Kadija, Angelo Corsico, Isa Cerveri, Claudia Chalk, Bruce C Trapnell, Antonio Braschi, Carmine Tinelli, Maurizio Luisetti

Abstract

Pulmonary alveolar proteinosis (PAP) is a term defining an ultra-rare group of disorders characterised by a perturbation in surfactant homeostasis, resulting in its accumulation within airspaces and impaired gas transfer. In this report we provide data from a cohort of PAP patients (n=81) followed for more than two decades at the San Matteo University Hospital of Pavia, Italy. In agreement with other large series in PAP individuals, 90% of the study subjects were affected by autoimmune/idiopathic PAP, while the remaining subjects were divided as follow: congenital 1%, secondary 4% and PAP-like 5%. The disease affected males and females with a ratio of 2:1 and approximately one third of PAP patients were lifelong nonsmokers. Occupational exposure was reported in 35% of subjects in this series. With reference to the PAP clinical course, in 29 patients (7% with spontaneous remission) disease severity did not necessitate whole lung lavage (WLL) in the long-term follow up. On the other hand, 44 PAP patients underwent therapeutic WLL: in 31 subjects a single WLL was sufficient to provide long term, durable benefit, whereas 13 patients required multiple WLLs. The intra-patient mean interval between two consecutive WLLs was 15.7±13.6 months. When baseline data among never lavaged and PAP patients lavaged at least once were compared, the need for lavage was significantly associated with serum biomarkers (CEA, Cyfra, LDH), lung function parameters forced vital capacity (FVC), and lung diffusing capacity (Dlco). We conclude that patient cohorts with an ultra-rare disease, such as PAP, referred to a single reference center, can provide useful information on the natural history and clinical course of the disease.

Twitter Demographics

The data shown below were collected from the profile of 1 tweeter who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 27 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Argentina 1 4%
Unknown 26 96%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 4 15%
Lecturer 3 11%
Student > Bachelor 3 11%
Researcher 3 11%
Student > Master 3 11%
Other 5 19%
Unknown 6 22%
Readers by discipline Count As %
Medicine and Dentistry 18 67%
Biochemistry, Genetics and Molecular Biology 2 7%
Unknown 7 26%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 14 March 2013.
All research outputs
#17,250,712
of 21,346,377 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,955
of 2,383 outputs
Outputs of similar age
#131,729
of 174,786 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#7
of 10 outputs
Altmetric has tracked 21,346,377 research outputs across all sources so far. This one is in the 11th percentile – i.e., 11% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,383 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one is in the 7th percentile – i.e., 7% of its peers scored the same or lower than it.
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