Severity in polycystic liver disease is associated with aetiology and female gender: Results of the International PLD Registry

Liver International : Official Journal of the International Association for the Study of the Liver
René M M van AertsJoost P H Drenth

Abstract

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). 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. 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 fe...Continue Reading

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Citations

Jun 18, 2019·Clinical Pharmacology and Therapeutics·René M M van AertsJoost P H Drenth
Jan 12, 2021·KI Reports·Xiaorui YinMartin R Prince
Jun 22, 2021·Liver International : Official Journal of the International Association for the Study of the Liver·Sophie E AapkesJoost P H Drenth
Aug 17, 2021·Medicine·Sophie E AapkesJoost P H Drenth

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