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Hereditary Tyrosinemia

Overview of attention for book
Cover of 'Hereditary Tyrosinemia'

Table of Contents

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    Book Overview
  2. Altmetric Badge
    Chapter 1 Discovery of Hereditary Tyrosinemia in Saguenay- Lac St-Jean
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    Chapter 2 Biochemical and Clinical Aspects of Hereditary Tyrosinemia Type 1
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    Chapter 3 Molecular Aspects of the FAH Mutations Involved in HT1 Disease
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    Chapter 4 Molecular Pathogenesis of Liver Injury in Hereditary Tyrosinemia 1
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    Chapter 5 Tyrosinemia and Liver Transplantation: Experience at CHU Sainte-Justine
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    Chapter 6 The Liver in Tyrosinemia Type I: Clinical Management and Course in Quebec
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    Chapter 7 Liver Transplantation for Hereditary Tyrosinaemia Type 1 in the United Kingdom
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    Chapter 8 NTBC and Correction of Renal Dysfunction
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    Chapter 9 Liver Cancer in Tyrosinemia Type 1
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    Chapter 10 Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients
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    Chapter 11 Diagnosing Hepatorenal Tyrosinaemia in Europe: Newborn Mass Screening Versus Selective Screening
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    Chapter 12 Tyrosinemia Type I in Japan: A Report of Five Cases
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    Chapter 13 Newborn Screening for Hereditary Tyrosinemia Type I in Québec: Update
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    Chapter 14 Hepatorenal Tyrosinemia in Mexico: A Call to Action
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    Chapter 15 Hereditary Tyrosinemia Type 1 in Turkey
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    Chapter 16 From Weed Killer to Wonder Drug
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    Chapter 17 The Québec NTBC Study
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    Chapter 18 Dietary Considerations in Tyrosinemia Type I
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    Chapter 19 Remaining Challenges in the Treatment of Tyrosinemia from the Clinician’s Viewpoint
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    Chapter 20 Fah Knockout Animals as Models for Therapeutic Liver Repopulation
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    Chapter 21 Gene Therapy in Tyrosinemia: Potential and Pitfalls
Attention for Chapter 7: Liver Transplantation for Hereditary Tyrosinaemia Type 1 in the United Kingdom
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2 tweeters
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Chapter title
Liver Transplantation for Hereditary Tyrosinaemia Type 1 in the United Kingdom
Chapter number 7
Book title
Hereditary Tyrosinemia
Published in
Advances in experimental medicine and biology, January 2017
DOI 10.1007/978-3-319-55780-9_7
Pubmed ID
Book ISBNs
978-3-31-955779-3, 978-3-31-955780-9
Authors

Patrick McKiernan, McKiernan, Patrick

Abstract

Fourteen children have undergone liver transplantation for hereditary tyrosinaemia type 1 (HT1) at Birmingham Children's hospital (BCH) since 1989; six were treated prior to the availability of Nitisinone in 1993 and eight in the post Nitisinone era. Prior to 1993 essentially all children with HT1 were referred for transplantation. In the Nitisinone era only those with unresponsive liver failure or suspected malignancy were considered for transplantation. Those who were treated pre-emptively following newborn screening have no evidence of liver disease and none have required transplantation.Absolute patient survival is 86% for the whole group and 100% in the Nitisinone era. There has been a functional correction of the metabolic defect in all cases allowing a normal diet. Persistent renal succinylacetone production was universal but did not appear to have any clinical consequence. Renal function appeared better, and hypertension less common in those treated in the Nitisinone era.Outcome was poorer for those four children with established malignancy; one was unfit for transplantation and another developed a pulmonary metastasis, which was successfully resected.

Twitter Demographics

The data shown below were collected from the profiles of 2 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 4 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 4 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 2 50%
Professor 1 25%
Student > Master 1 25%
Readers by discipline Count As %
Nursing and Health Professions 1 25%
Agricultural and Biological Sciences 1 25%
Medicine and Dentistry 1 25%
Unknown 1 25%

Attention Score in Context

This research output has an Altmetric Attention Score of 2. 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 04 March 2019.
All research outputs
#8,711,149
of 14,437,320 outputs
Outputs from Advances in experimental medicine and biology
#1,334
of 3,352 outputs
Outputs of similar age
#145,768
of 268,851 outputs
Outputs of similar age from Advances in experimental medicine and biology
#2
of 3 outputs
Altmetric has tracked 14,437,320 research outputs across all sources so far. This one is in the 37th percentile – i.e., 37% of other outputs scored the same or lower than it.
So far Altmetric has tracked 3,352 research outputs from this source. They receive a mean Attention Score of 3.6. This one has gotten more attention than average, scoring higher than 56% 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 268,851 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 41st percentile – i.e., 41% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 3 others from the same source and published within six weeks on either side of this one.