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Hereditary sensory and autonomic neuropathies: types II, III, and IV

Overview of attention for article published in Orphanet Journal of Rare Diseases, October 2007
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  • Good Attention Score compared to outputs of the same age (68th percentile)
  • Good Attention Score compared to outputs of the same age and source (76th percentile)

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4 Wikipedia pages

Citations

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118 Dimensions

Readers on

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103 Mendeley
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Title
Hereditary sensory and autonomic neuropathies: types II, III, and IV
Published in
Orphanet Journal of Rare Diseases, October 2007
DOI 10.1186/1750-1172-2-39
Pubmed ID
Authors

Felicia B Axelrod, Gabrielle Gold-von Simson

Abstract

The hereditary sensory and autonomic neuropathies (HSAN) encompass a number of inherited disorders that are associated with sensory dysfunction (depressed reflexes, altered pain and temperature perception) and varying degrees of autonomic dysfunction (gastroesophageal reflux, postural hypotention, excessive sweating). Subsequent to the numerical classification of four distinct forms of HSAN that was proposed by Dyck and Ohta, additional entities continue to be described, so that identification and classification are ongoing. As a group, the HSAN are rare diseases that affect both sexes. HSAN III is almost exclusive to individuals of Eastern European Jewish extraction, with incidence of 1 per 3600 live births. Several hundred cases with HSAN IV have been reported. The worldwide prevalence of HSAN type II is very low. This review focuses on the description of three of the disorders, HSAN II through IV, that are characterized by autosomal recessive inheritance and onset at birth. These three forms of HSAN have been the most intensively studied, especially familial dysautonomia (Riley-Day syndrome or HSAN III), which is often used as a prototype for comparison to the other HSAN. Each HSAN disorder is likely caused by different genetic errors that affect specific aspects of small fiber neurodevelopment, which result in variable phenotypic expression. As genetic tests are routinely used for diagnostic confirmation of HSAN III only, other means of differentiating between the disorders is necessary. Diagnosis is based on the clinical features, the degree of both sensory and autonomic dysfunction, and biochemical evaluations, with pathologic examinations serving to further confirm differences. Treatments for all these disorders are supportive.

Mendeley readers

The data shown below were compiled from readership statistics for 103 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United Kingdom 1 <1%
Germany 1 <1%
Switzerland 1 <1%
Brazil 1 <1%
Unknown 99 96%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 17 17%
Student > Ph. D. Student 14 14%
Student > Postgraduate 11 11%
Researcher 10 10%
Other 10 10%
Other 29 28%
Unknown 12 12%
Readers by discipline Count As %
Medicine and Dentistry 39 38%
Agricultural and Biological Sciences 22 21%
Biochemistry, Genetics and Molecular Biology 7 7%
Engineering 5 5%
Neuroscience 4 4%
Other 10 10%
Unknown 16 16%

Attention Score in Context

This research output has an Altmetric Attention Score of 3. 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 15 December 2016.
All research outputs
#2,305,050
of 8,775,174 outputs
Outputs from Orphanet Journal of Rare Diseases
#406
of 1,157 outputs
Outputs of similar age
#71,104
of 244,376 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#10
of 43 outputs
Altmetric has tracked 8,775,174 research outputs across all sources so far. This one has received more attention than most of these and is in the 60th percentile.
So far Altmetric has tracked 1,157 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.2. This one has gotten more attention than average, scoring higher than 60% of its peers.
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 244,376 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 68% of its contemporaries.
We're also able to compare this research output to 43 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 76% of its contemporaries.