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Hyper IgM syndrome presenting as chronic suppurative lung disease

Overview of attention for article published in Italian Journal of Pediatrics, September 2012
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Title
Hyper IgM syndrome presenting as chronic suppurative lung disease
Published in
Italian Journal of Pediatrics, September 2012
DOI 10.1186/1824-7288-38-45
Pubmed ID
Authors

Silvia Montella, Silvia Montella, Marco Maglione, Giuliana Giardino, Angela Di Giorgio, Loredana Palamaro, Virginia Mirra, Matilde Valeria Ursini, Mariacarolina Salerno, Claudio Pignata, Carlo Caffarelli, Francesca Santamaria

Abstract

The Hyper-immunoglobulin M syndromes (HIGM) are a heterogeneous group of genetic disorders resulting in defects of immunoglobulin class switch recombination. Affected patients show humoral immunodeficiency and high susceptibility to opportunistic infections. Elevated serum IgM levels are the hallmark of the disease, even though in few rare cases they may be in the normal range. Hyper IgM is associated with low to undetectable levels of serum IgG, IgA, and IgE. In some cases, alterations in different genes may be identified. Mutations in five genes have so far been associated to the disease, which can be inherited with an X-linked (CD40 ligand, and nuclear factor-kB essential modulator defects) or an autosomal recessive (CD40, activation-induced cytidine deaminase, and uracil-DNA glycosylase mutation) pattern. The patient herein described presented with recurrent upper and lower respiratory infections and evidence of suppurative lung disease at the conventional chest imaging. The presence of low serum IgG and IgA levels, elevated IgM levels, and a marked reduction of in vivo switched memory B cells led to a clinical and functional diagnosis of HIGM although the genetic cause was not identified.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 18 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 18 100%

Demographic breakdown

Readers by professional status Count As %
Librarian 2 11%
Professor > Associate Professor 2 11%
Researcher 2 11%
Student > Postgraduate 2 11%
Student > Ph. D. Student 2 11%
Other 7 39%
Unknown 1 6%
Readers by discipline Count As %
Medicine and Dentistry 6 33%
Agricultural and Biological Sciences 3 17%
Immunology and Microbiology 3 17%
Biochemistry, Genetics and Molecular Biology 1 6%
Pharmacology, Toxicology and Pharmaceutical Science 1 6%
Other 2 11%
Unknown 2 11%

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 24 September 2012.
All research outputs
#3,532,556
of 5,028,713 outputs
Outputs from Italian Journal of Pediatrics
#170
of 295 outputs
Outputs of similar age
#53,346
of 81,830 outputs
Outputs of similar age from Italian Journal of Pediatrics
#19
of 27 outputs
Altmetric has tracked 5,028,713 research outputs across all sources so far. This one is in the 16th percentile – i.e., 16% of other outputs scored the same or lower than it.
So far Altmetric has tracked 295 research outputs from this source. They receive a mean Attention Score of 2.2. This one is in the 27th percentile – i.e., 27% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 81,830 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 18th percentile – i.e., 18% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 27 others from the same source and published within six weeks on either side of this one. This one is in the 18th percentile – i.e., 18% of its contemporaries scored the same or lower than it.