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Incontinentia pigmenti (Bloch-Sulzberger syndrome).

Overview of attention for chapter in “Neurocutaneous Syndromes”
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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.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 48 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 1 2%
Unknown 47 98%

Demographic breakdown

Readers by professional status Count As %
Researcher 10 21%
Student > Master 7 15%
Other 6 13%
Student > Doctoral Student 5 10%
Student > Bachelor 4 8%
Other 8 17%
Unknown 8 17%
Readers by discipline Count As %
Medicine and Dentistry 25 52%
Biochemistry, Genetics and Molecular Biology 4 8%
Nursing and Health Professions 3 6%
Agricultural and Biological Sciences 3 6%
Neuroscience 2 4%
Other 2 4%
Unknown 9 19%

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
#11,435,453
of 12,863,956 outputs
Outputs from Handbook of clinical neurology
#482
of 603 outputs
Outputs of similar age
#229,468
of 277,328 outputs
Outputs of similar age from Handbook of clinical neurology
#16
of 18 outputs
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We're also able to compare this research output to 18 others from the same source and published within six weeks on either side of this one. This one is in the 1st percentile – i.e., 1% of its contemporaries scored the same or lower than it.