Attention for Chapter 458:
Liver Fibrosis Associated with Iron Accumulation Due to Long-Term Heme-Arginate Treatment in Acute Intermittent Porphyria: A Case Series
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About this Attention Score
In the top 25% of all research outputs scored by Altmetric
Among the highest-scoring outputs from this source (#47 of 543)
High Attention Score compared to outputs of the same age (82nd percentile)
Good Attention Score compared to outputs of the same age and source (71st percentile)
Barbara Willandt, Janneke G. Langendonk, Katharina Biermann, Wouter Meersseman, François D’Heygere, Christophe George, Chris Verslype, Diethard Monbaliu, David Cassiman, Willandt, Barbara, Langendonk, Janneke G., Biermann, Katharina, Meersseman, Wouter, D’Heygere, François, George, Christophe, Verslype, Chris, Monbaliu, Diethard, Cassiman, David
Abstract
Acute intermittent porphyria (AIP) is an autosomal dominant disorder of heme biosynthesis due to a mutation in the porphobilinogen deaminase gene. The mutation causes a deficiency in the porphobilinogen deaminase enzyme, thereby causing an accumulation of heme precursors (δ-aminolevulinic acid and porphobilinogen). These neurotoxic heme precursors elicit acute neurovisceral attacks, which can be treated with heme-arginate infusions. Some patients require heme-arginate infusions on a regular basis for many years, which ultimately leads to an iron accumulation (increased serum ferritin and iron accumulation in the liver, spleen, and bone marrow on MRI). We report three AIP patients, who developed iron accumulation (with serum ferritin up to 7,850 microgram/liter) due to multiple heme-arginate infusions. We report for the first time that the iron accumulation in these patients was associated with fibrosis on liver histology.
Regular heme-arginate treatment in AIP does not only lead to increased serum ferritin but may also induce liver fibrosis. This should be taken into account, when weighing the risks and benefits of repeated heme-arginate treatment against the risk and benefits of treating refractory AIP by liver transplantation.
Pharmacology, Toxicology and Pharmaceutical Science
1
4%
Agricultural and Biological Sciences
1
4%
Engineering
1
4%
Other
0
0%
Unknown
10
38%
Attention Score in Context
Attention Score in Context
This research output has an Altmetric Attention Score of 7. 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 29 December 2021.
All research outputs
#4,169,535
of 22,780,165 outputs
Outputs from JIMD Reports
#47
of 543 outputs
Outputs of similar age
#59,304
of 352,944 outputs
Outputs of similar age from JIMD Reports
#4
of 14 outputs
Altmetric has tracked 22,780,165 research outputs across all sources so far. Compared to these this one has done well and is in the 80th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 543 research outputs from this source. They receive a mean Attention Score of 2.8. This one has done particularly well, scoring higher than 90% 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 352,944 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 82% of its contemporaries.
We're also able to compare this research output to 14 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 71% of its contemporaries.
