↓ Skip to main content

Neurocutaneous Syndromes, 1st Edition

Overview of attention for book
Attention for Chapter: Incontinentia pigmenti (Bloch-Sulzberger syndrome).
Altmetric Badge

About this Attention Score

  • Average Attention Score compared to outputs of the same age

Mentioned by

twitter
1 X user

Citations

dimensions_citation
2 Dimensions

Readers on

mendeley
57 Mendeley
You are seeing a free-to-access but limited selection of the activity Altmetric has collected about this research output. Click here to find out more.
Chapter title
Incontinentia pigmenti (Bloch-Sulzberger syndrome).
Book title
Neurocutaneous Syndromes
Published in
Handbook of clinical neurology, January 2015
DOI 10.1016/b978-0-444-62702-5.00020-2
Pubmed ID
Book ISBNs
978-0-444-62702-5
Authors

Narayanan, Mohan J, Rangasamy, Sampathkumar, Narayanan, Vinodh, Mohan J. Narayanan, Sampathkumar Rangasamy, Vinodh Narayanan

Abstract

Incontinentia pigmenti (IP; Bloch-Sulzberger syndrome; OMIM #308300) is an X-linked dominant neurocutaneous disorder with presumed male lethality. It is usually diagnosed in female newborns based on skin features (erythematous, vesicular, or bullous eruption in linear streaks). The skin lesions evolve into a verrucous stage, followed by atrophy and scarring, leaving linear areas of hypopigmentation and hyperpigmented macules in bizarre patterns following Blaschko's lines. Systemic and neurologic complications include focal seizures and hemorrhagic cerebral infarction in infants, and retinal vasculopathy leading to blindness. Hypodontia, conical or pegged teeth, and linear areas of alopecia persist into adulthood. IP is caused by mutation of the IKBKG/NEMO gene on Xq28. Deletion of exons 4 to 10 (NEMOΔ4-10) accounts for about 80% of cases (familial and sporadic). NEMO mutation leads to loss of function of NF-κB, a critical protein that modulates cellular proliferation, apoptosis, and response to proinflammatory factors, leading to the characteristic features of IP. In female carriers, selective loss of cells expressing the mutant X-chromosome results in completely skewed X-inactivation in the majority of cases. Study of mouse models in which various components of the NF-κB pathway (including NEMO) have been knocked out has contributed significantly to our understanding of disease pathogenesis.

X Demographics

X Demographics

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

Mendeley readers

The data shown below were compiled from readership statistics for 57 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 1 2%
Unknown 56 98%

Demographic breakdown

Readers by professional status Count As %
Researcher 9 16%
Student > Postgraduate 6 11%
Other 6 11%
Student > Doctoral Student 6 11%
Student > Master 6 11%
Other 9 16%
Unknown 15 26%
Readers by discipline Count As %
Medicine and Dentistry 28 49%
Biochemistry, Genetics and Molecular Biology 3 5%
Agricultural and Biological Sciences 3 5%
Nursing and Health Professions 2 4%
Neuroscience 2 4%
Other 3 5%
Unknown 16 28%
Attention Score in Context

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 August 2016.
All research outputs
#15,349,796
of 22,832,057 outputs
Outputs from Handbook of clinical neurology
#754
of 1,279 outputs
Outputs of similar age
#209,045
of 353,186 outputs
Outputs of similar age from Handbook of clinical neurology
#56
of 88 outputs
Altmetric has tracked 22,832,057 research outputs across all sources so far. This one is in the 22nd percentile – i.e., 22% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,279 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. 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 353,186 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 31st percentile – i.e., 31% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 88 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.