Polycystic Liver Disease

Background & Aims Polycystic liver disease (PLD), the most common extra-renal manifestation of autosomal-dominant polycystic kidney disease (ADPKD), has become more prevalent due to increased life expectancy, improved renal survival, reduced cardiovascular mortality, and renal replacement therapy. No studies have fully characterized PLD in large cohorts. We investigated whether liver and cyst volumes associate with volume of the hepatic parenchyma, results from liver laboratory tests, and patient-reported outcomes. Methods We performed a cross-sectional analysis of baseline liver volumes, measured by magnetic resonance imaging, and their association with demographics, results from liver laboratory and other tests, and quality of life. The data were collected from a randomized, placebo-controlled trial underway at 7 tertiary-care medical centers to determine whether the combination of an angiotensin I converting enzyme inhibitor and angiotensin II receptor blocker was superior to the inhibitor alone, and whether low blood pressure (<110/75mmHg) was superior to standard blood pressure (120–130/70–80mmHg), in delaying renal cystic progression in 558 patients with ADPKD, stage 1–2 chronic kidney disease, and hypertension (15–49 years old). Results We found hepatomegaly to be common among patients with ADPKD. Cysts and parenchyma contributed to hepatomegaly. Cysts were more common and liver and cyst volumes were greater in women, increasing with age. Patients with advanced disease had relative loss of liver parenchyma. We observed small abnormalities in results from liver laboratory tests, and that splenomegaly and hypersplenism associated with the severity of PLD. Higher liver volumes were associated with lower quality of life. Conclusions Hepatomegaly is common even in early-stage ADPKD and not accounted for by cysts alone. Parenchymal volumes are larger, compared with liver volumes of patients without ADPKD or with those predicted by standardized equations—even among patients without cysts. The severity of PLD is associated with altered biochemical and hematologic features, as well as quality of life.

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“…25, 40 The SF-36 questionnaire has not been validated in PLD and better patient-reported outcome tools are needed.…”

Section: Discussionmentioning

confidence: 99%

Liver Involvement in Early Autosomal-Dominant Polycystic Kidney Disease

Hogan¹,

Abebe²,

Torres³

et al. 2015

Clinical Gastroenterology and Hepatology

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103

108

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“…Unfortunately, we cannot confirm this hypothesis as liver volumes were not measured in subjects with eGFR <60 mL/min/1.73 m 2 . That the enlarged organs as opposed to reduced kidney function are the cause of symptoms is supported by studies that show dramatic relief of symptoms and improvement in HRQoL after organ reduction therapy or removal [19–22]. Even modest reductions in liver volume (4.95% ± 6.77%) with one-year treatment with octreotide in a blinded study, led to improvements in bodily pain and role emotional scales of the SF-36 [23].…”

Section: Discussionmentioning

confidence: 99%

Health-Related Quality of Life in Patients With Autosomal Dominant Polycystic Kidney Disease and CKD Stages 1-4: A Cross-sectional Study

Miskulin

Abebe

Chapman

et al. 2014

American Journal of Kidney Diseases

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100

117

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Background In people with early autosomal dominant polycystic kidney disease (ADPKD) average total kidney volume (TKV) is three times normal and increases by an average of 5% per year despite seemingly normal glomerular filtration rate (GFR). We hypothesized that increased TKV would be a source of morbidity and diminished quality of life that would be worse in subjects with more advanced disease. Study Design Cross-sectional. Setting & Participants 1043 subjects with ADPKD, hypertension and a baseline estimated glomerular filtration rate (eGFR) >20 mL/min/1.73 m2. Predictors 1) eGFR 2) height-adjusted TKV (htTKV) in subjects with eGFR >60 mL/min/1.73 m2. Outcomes 36-Item Short Form Health Survey (SF-36) and the Wisconsin Brief Pain Survey. Measurements Questionnaires were self- administered. eGFR was estimated from serum creatinine using the CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) equation. The htTKV was measured by MRI. Results Back pain was reported by 50% of subjects and 20% experienced it ‘often, usually, or always’. In subjects with early disease (eGFR >60 mL/min/1.73 m2) there was no association between pain and htTKV, except in patients with large kidneys (htTKV >1000 mL/m). Comparing across eGFR levels and including patients with eGFR <60 mL/min/ 1.73 m2, patients with eGFR 20–44 mL/min/1.73 m2 were significantly more likely to report that pain impacted on their daily lives and had lower SF-36 scores than patients with eGFR 45–60 and ≥60 mL/min/1.73 m2. Symptoms relating to abdominal fullness were reported by 20% of patients, and were significantly related with lower eGFR in women but not men. Limitations TKV and liver volume were not measured in subjects with eGFR <60 mL/min/1.73 m2. The number of patients with eGFR <30 mL/min/1.73 m2 is small. Causal inferences are limited by cross-sectional design. Conclusions Pain is a common early symptom in the course of ADPKD, although it is not related to kidney size in early disease (eGFR >60 mL/min/1.73 m2), except in individuals with large kidneys (htTKV >1000 mL/m). Symptoms relating to abdominal fullness and pain are greater in patients with more advanced (eGFR 20–45 mL/min/ 1.73 m2) disease and may be due to organ enlargement, especially in women. More research about the role of TKV in quality of life and outcomes of ADPKD patients is warranted.

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“…Encouragingly, 75% of long-term survivors report improved or normalized performance status, and bodily pain scores on the SF-36 questionnaire appear similar to general population norms. 66 In another series Que and colleagues 67 reported a ~62% mean reduction in liver volume with combined liver resection and cyst fenestration surgery.…”

Section: Surgical Approachesmentioning

confidence: 98%