Severity in polycystic liver disease is associated with aetiology and female gender: Results of the International PLD Registry
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064165%3A_____%2F19%3A10393572" target="_blank" >RIV/00064165:_____/19:10393572 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11110/19:10393572
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=answDq5zFu" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=answDq5zFu</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/liv.13965" target="_blank" >10.1111/liv.13965</a>
Alternative languages
Result language
angličtina
Original language name
Severity in polycystic liver disease is associated with aetiology and female gender: Results of the International PLD Registry
Original language description
Background & Aims: Polycystic liver disease (PLD) occurs in two genetic disorders, autosomal-dominant polycystic kidney disease (ADPKD) and autosomal-dominant polycystic liver disease (ADPLD). The aim of this study is to compare disease severity between ADPKD and ADPLD by determining the association between diagnosis and height-adjusted total liver volume (hTLV). Methods: We performed a cross-sectional analysis with hTLV as endpoint. Patients were identified from the International PLD Registry (>10 liver cysts) and included in our analysis when PLD diagnosis was made prior to September 2017, hTLV was available before volume-reducing therapy (measured on computed tomography or magnetic resonance imaging) and when patients were tertiary referred. Data from the registry were retrieved for age, diagnosis (ADPKD or ADPLD), gender, height and hTLV. Results: A total of 360 patients (ADPKD n = 241; ADPLD n = 119) met our inclusion criteria. Female ADPKD patients had larger hTLV compared with ADPLD (P = 0.008). In a multivariate regression analysis, ADPKD and lower age at index CT were independently associated with larger hTLV in females, whereas in males a higher age was associated with larger hTLV. Young females (<=51 years) had larger liver volumes compared with older females (>51 years) in ADPKD. Conclusion: Aetiology is presented as a new risk factor associated with PLD severity. Young females with ADPKD represent a subgroup of PLD patients with the most severe phenotype expressed in hTLV.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30219 - Gastroenterology and hepatology
Result continuities
Project
—
Continuities
V - Vyzkumna aktivita podporovana z jinych verejnych zdroju
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Liver International
ISSN
1478-3223
e-ISSN
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Volume of the periodical
39
Issue of the periodical within the volume
3
Country of publishing house
DK - DENMARK
Number of pages
8
Pages from-to
575-582
UT code for WoS article
000459453900018
EID of the result in the Scopus database
2-s2.0-85054597922