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Ketogenic diet in a patient with congenital hyperinsulinism: a novel approach to prevent brain damage

Overview of attention for article published in Orphanet Journal of Rare Diseases, September 2015
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  • In the top 5% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (95th percentile)
  • High Attention Score compared to outputs of the same age and source (95th percentile)

Mentioned by

blogs
1 blog
twitter
65 X users
facebook
5 Facebook pages
video
1 YouTube creator

Citations

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19 Dimensions

Readers on

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118 Mendeley
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Title
Ketogenic diet in a patient with congenital hyperinsulinism: a novel approach to prevent brain damage
Published in
Orphanet Journal of Rare Diseases, September 2015
DOI 10.1186/s13023-015-0342-6
Pubmed ID
Authors

Arianna Maiorana, Lucilla Manganozzi, Fabrizio Barbetti, Silvia Bernabei, Giorgia Gallo, Raffaella Cusmai, Stefania Caviglia, Carlo Dionisi-Vici

Abstract

Congenital hyperinsulinism (CHI) is the most frequent cause of hypoglycemia in children. In addition to increased peripheral glucose utilization, dysregulated insulin secretion induces profound hypoglycemia and neuroglycopenia by inhibiting glycogenolysis, gluconeogenesis and lipolysis. This results in the shortage of all cerebral energy substrates (glucose, lactate and ketones), and can lead to severe neurological sequelae. Patients with CHI unresponsive to medical treatment can be subjected to near-total pancreatectomy with increased risk of secondary diabetes. Ketogenic diet (KD), by reproducing a fasting-like condition in which body fuel mainly derives from beta-oxidation, is intended to provide alternative cerebral substrates such ketone bodies. We took advantage of known protective effect of KD on neuronal damage associated with GLUT1 deficiency, a disorder of impaired glucose transport across the blood-brain barrier, and administered KD in a patient with drug-unresponsive CHI, with the aim of providing to neurons an energy source alternative to glucose. A child with drug-resistant, long-standing CHI caused by a spontaneous GCK activating mutation (p.Val455Met) suffered from epilepsy and showed neurodevelopmental abnormalities. After attempting various therapeutic regimes without success, near-total pancreatectomy was suggested to parents, who asked for other options. Therefore, we proposed KD in combination with insulin-suppressing drugs. We administered KD for 2 years. Soon after the first six months, the patient was free of epileptic crises, presented normalization of EEG, and showed a marked recover in psychological development and quality of life. KD could represent an effective treatment to support brain function in selected cases of CHI.

X Demographics

X Demographics

The data shown below were collected from the profiles of 65 X users 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 118 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 118 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 19 16%
Researcher 16 14%
Student > Bachelor 14 12%
Student > Postgraduate 10 8%
Other 9 8%
Other 26 22%
Unknown 24 20%
Readers by discipline Count As %
Medicine and Dentistry 45 38%
Nursing and Health Professions 15 13%
Psychology 9 8%
Biochemistry, Genetics and Molecular Biology 5 4%
Neuroscience 4 3%
Other 12 10%
Unknown 28 24%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 52. 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 11 November 2020.
All research outputs
#807,727
of 25,248,775 outputs
Outputs from Orphanet Journal of Rare Diseases
#70
of 3,046 outputs
Outputs of similar age
#11,369
of 281,314 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#3
of 45 outputs
Altmetric has tracked 25,248,775 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 96th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 3,046 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.2. This one has done particularly well, scoring higher than 97% 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 281,314 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 95% of its contemporaries.
We're also able to compare this research output to 45 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 95% of its contemporaries.