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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, January 2014
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  • High Attention Score compared to outputs of the same age and source (81st percentile)

Mentioned by

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3 tweeters
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1 patent

Citations

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111 Dimensions

Readers on

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153 Mendeley
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Title
Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management
Published in
Orphanet Journal of Rare Diseases, January 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.

Twitter Demographics

The data shown below were collected from the profiles of 3 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 153 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Libya 1 <1%
Unknown 152 99%

Demographic breakdown

Readers by professional status Count As %
Student > Master 24 16%
Other 19 12%
Researcher 19 12%
Student > Bachelor 16 10%
Student > Ph. D. Student 13 8%
Other 33 22%
Unknown 29 19%
Readers by discipline Count As %
Medicine and Dentistry 79 52%
Biochemistry, Genetics and Molecular Biology 13 8%
Agricultural and Biological Sciences 8 5%
Nursing and Health Professions 4 3%
Pharmacology, Toxicology and Pharmaceutical Science 4 3%
Other 10 7%
Unknown 35 23%

Attention Score in Context

This research output has an Altmetric Attention Score of 5. 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 20 June 2019.
All research outputs
#3,995,200
of 15,283,645 outputs
Outputs from Orphanet Journal of Rare Diseases
#480
of 1,646 outputs
Outputs of similar age
#46,505
of 191,407 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#5
of 37 outputs
Altmetric has tracked 15,283,645 research outputs across all sources so far. This one has received more attention than most of these and is in the 73rd percentile.
So far Altmetric has tracked 1,646 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one has gotten more attention than average, scoring higher than 69% 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 191,407 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 75% of its contemporaries.
We're also able to compare this research output to 37 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 81% of its contemporaries.