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Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management

Overview of attention for article published in Orphanet Journal of Rare Diseases, May 2014
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About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (91st percentile)
  • High Attention Score compared to outputs of the same age and source (95th percentile)

Mentioned by

blogs
1 blog
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12 X users
patent
1 patent

Citations

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146 Dimensions

Readers on

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171 Mendeley
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Title
Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management
Published in
Orphanet Journal of Rare Diseases, May 2014
DOI 10.1186/1750-1172-9-69
Pubmed ID
Authors

Wybrich R Cnossen, Joost PH Drenth

Abstract

Polycystic liver disease (PLD) is the result of embryonic ductal plate malformation of the intrahepatic biliary tree. The phenotype consists of numerous cysts spread throughout the liver parenchyma. Cystic bile duct malformations originating from the peripheral biliary tree are called Von Meyenburg complexes (VMC). In these patients embryonic remnants develop into small hepatic cysts and usually remain silent during life. Symptomatic PLD occurs mainly in the context of isolated polycystic liver disease (PCLD) and autosomal dominant polycystic kidney disease (ADPKD). In advanced stages, PCLD and ADPKD patients have massively enlarged livers which cause a spectrum of clinical features and complications. Major complaints include abdominal pain, abdominal distension and atypical symptoms because of voluminous cysts resulting in compression of adjacent tissue or failure of the affected organ. Renal failure due to polycystic kidneys and non-renal extra-hepatic features are common in ADPKD in contrast to VMC and PCLD. In general, liver function remains prolonged preserved in PLD. Ultrasonography is the first instrument to assess liver phenotype. Indeed, PCLD and ADPKD diagnostic criteria rely on detection of hepatorenal cystogenesis, and secondly a positive family history compatible with an autosomal dominant inheritance pattern. Ambiguous imaging or screening may be assisted by genetic counseling and molecular diagnostics. Screening mutations of the genes causing PCLD (PRKCSH and SEC63) or ADPKD (PKD1 and PKD2) confirm the clinical diagnosis. Genetic studies showed that accumulation of somatic hits in cyst epithelium determine the rate-limiting step for cyst formation. Management of adult PLD is based on liver phenotype, severity of clinical features and quality of life. Conservative treatment is recommended for the majority of PLD patients. The primary aim is to halt cyst growth to allow abdominal decompression and ameliorate symptoms. Invasive procedures are required in a selective patient group with advanced PCLD, ADPKD or liver failure. Pharmacological therapy by somatostatin analogues lead to beneficial outcome of PLD in terms of symptom relief and liver volume reduction.

X Demographics

X Demographics

The data shown below were collected from the profiles of 12 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 171 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Libya 1 <1%
Unknown 170 99%

Demographic breakdown

Readers by professional status Count As %
Student > Master 24 14%
Other 20 12%
Researcher 19 11%
Student > Bachelor 16 9%
Student > Ph. D. Student 14 8%
Other 32 19%
Unknown 46 27%
Readers by discipline Count As %
Medicine and Dentistry 82 48%
Biochemistry, Genetics and Molecular Biology 13 8%
Agricultural and Biological Sciences 8 5%
Nursing and Health Professions 4 2%
Pharmacology, Toxicology and Pharmaceutical Science 4 2%
Other 9 5%
Unknown 51 30%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 17. 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 25 August 2023.
All research outputs
#2,144,970
of 25,505,015 outputs
Outputs from Orphanet Journal of Rare Diseases
#246
of 3,140 outputs
Outputs of similar age
#21,047
of 242,401 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#3
of 46 outputs
Altmetric has tracked 25,505,015 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 91st percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 3,140 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 92% 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 242,401 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 91% of its contemporaries.
We're also able to compare this research output to 46 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.