“…On initial laboratory tests, including assessment of liver function, 4 patients (40%) had mild liver dysfunction with slightly elevated levels of serum aspartate transaminase (AST), aspartate aminotransferase (ALT), and gammaglutamyl transpeptidase (γ-GTP). No patient had a hemorrhagic and numbers of hepatic cysts [6][7][8] . Documented complications of nonparasitic hepatic cystsinclude obstructive jaundice [9,10] , rupture [11][12][13] , intracystic hemorrhage [13][14][15][16] , and infection [17][18][19] .…”
AIM:The present study evaluated the safety and effectiveness of repeated injections of minocycline hydrochloride (MINO) for symptomatic polycystic liver disease (PLD).
METHODS:We retrospectively studied patients who received percutaneous MINO injections for symptomatic PLD and were followed up for at least 2 years between 2002 and 2015. Huge hepatic cysts (diameter > 10 cm) were treated by continuous drainage and multiple MINO injections. Small hepatic cysts underwent one-step drainage followed by a single injection of MINO. Only several cysts that caused complications were treated by percutaneous injection of MINO. Patients were discharged 1 day after treatment. RESULTS: Ten patients, (4men and 6 women; mean age, 57.8 years) were studied. All patients had Type 2 PLD according to Gigot's classification. The chief complaints were abdominal distension (n = 10), abdominal pain (n = 6), back pain (n = 4), and appetite loss (n = 8). Four patients had mild liver dysfunction. One patient complained of moderate right subscapular pain immediately after the injection, and another had pain at the site of catheter insertion. Liver dysfunction did not develop in any patient after treatment. The mean follow-up was 63.4 months. The interval from the first to last admission for MINO injection therapy was 14 to 148 months (mean, 40.8 months). The number of admissions for MINO injection therapy ranged from 2 to 12 times (mean, 4.0 times). The average interval for MINO injection therapy was 8.3 to 19 months (mean, 14.8 months).The hepatic cysts shrank in all patients after treatment. Complications improved consistently, and all patients were satisfied with the outcome of treatment. CONCLUSIONS: Symptomatic PLD was controllable by 14.8 months of repeated MINO injections on average. Repeated MINO injections are a safe, definitive treatment for symptomatic PLD.
“…On initial laboratory tests, including assessment of liver function, 4 patients (40%) had mild liver dysfunction with slightly elevated levels of serum aspartate transaminase (AST), aspartate aminotransferase (ALT), and gammaglutamyl transpeptidase (γ-GTP). No patient had a hemorrhagic and numbers of hepatic cysts [6][7][8] . Documented complications of nonparasitic hepatic cystsinclude obstructive jaundice [9,10] , rupture [11][12][13] , intracystic hemorrhage [13][14][15][16] , and infection [17][18][19] .…”
AIM:The present study evaluated the safety and effectiveness of repeated injections of minocycline hydrochloride (MINO) for symptomatic polycystic liver disease (PLD).
METHODS:We retrospectively studied patients who received percutaneous MINO injections for symptomatic PLD and were followed up for at least 2 years between 2002 and 2015. Huge hepatic cysts (diameter > 10 cm) were treated by continuous drainage and multiple MINO injections. Small hepatic cysts underwent one-step drainage followed by a single injection of MINO. Only several cysts that caused complications were treated by percutaneous injection of MINO. Patients were discharged 1 day after treatment. RESULTS: Ten patients, (4men and 6 women; mean age, 57.8 years) were studied. All patients had Type 2 PLD according to Gigot's classification. The chief complaints were abdominal distension (n = 10), abdominal pain (n = 6), back pain (n = 4), and appetite loss (n = 8). Four patients had mild liver dysfunction. One patient complained of moderate right subscapular pain immediately after the injection, and another had pain at the site of catheter insertion. Liver dysfunction did not develop in any patient after treatment. The mean follow-up was 63.4 months. The interval from the first to last admission for MINO injection therapy was 14 to 148 months (mean, 40.8 months). The number of admissions for MINO injection therapy ranged from 2 to 12 times (mean, 4.0 times). The average interval for MINO injection therapy was 8.3 to 19 months (mean, 14.8 months).The hepatic cysts shrank in all patients after treatment. Complications improved consistently, and all patients were satisfied with the outcome of treatment. CONCLUSIONS: Symptomatic PLD was controllable by 14.8 months of repeated MINO injections on average. Repeated MINO injections are a safe, definitive treatment for symptomatic PLD.
“…We found only two studies that investigated QoL in patients with ADPKD on dialysis ( n = 108 and n = 5 respectively), which prevented a reliable meta-analysis in this subgroup [15, 34]. We contacted eight authors for additional information that was not published in the original articles and received relevant data from six of them [15, 34–36, 38, 39]. Four studies included a mixed population of patients with isolated polycystic liver disease (ADPLD) and polycystic liver disease as extrarenal manifestation of ADPKD [35, 36, 39, 40].…”
Section: Resultsmentioning
confidence: 99%
“…We contacted eight authors for additional information that was not published in the original articles and received relevant data from six of them [15, 34–36, 38, 39]. Four studies included a mixed population of patients with isolated polycystic liver disease (ADPLD) and polycystic liver disease as extrarenal manifestation of ADPKD [35, 36, 39, 40]. For all these studies, we obtained separate ADPKD group data by contacting the authors.Fig.…”
Section: Resultsmentioning
confidence: 99%
“…Table 1 describes the characteristics of the nine studies included in meta-analysis [13, 15, 16, 34–39]. The majority were longitudinal interventional studies ( n = 7), including three randomized controlled trials.…”
Section: Resultsmentioning
confidence: 99%
“…One study ( n = 29) did not provide renal function of the included patients [37], resulting in 1594 available patients to assess the impact of renal function on QoL. Seven studies reported kidney volumes ( n = 1238) of the included patients [13, 15, 16, 35–37, 39]. Five studies reported liver volume ( n = 1057), [13, 15, 35, 36, 39] including four studies with severe polycystic liver disease patients (median liver volume > 3000 mL) [15, 35, 36, 39].…”
BackgroundLittle is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). Recent studies suggest that QoL in ADPKD is determined by more factors than mere renal function. We investigated the effect of ADPKD on QoL and evaluated how Qol is affected by disease severity markers renal function, kidney volume and liver volume.MethodsWe performed a systematic review, meta-analysis and meta-regression analyses of cohort studies and randomized controlled trials investigating patient-reported QoL in adult patients with ADPKD not yet on dialysis. EMBASE, MEDLINE, and Web of Science were searched to August 2015 without language restrictions. Two investigators independently reviewed title, abstracts and full text of potentially relevant citations to determine eligibility. We compared pooled QoL summary scores of ADPKD patients using a random-effects meta-analytic model. These scores were compared with mean and age-corrected reference scores of the general population. In a meta-regression analysis, we investigated the univariate effect of renal function, kidney volume and liver volume on QoL.ResultsWe included nine studies in meta-analysis including 1623 patients who completed the SF-36 questionnaire. Pooled physical (PCS) and mental component scores (MCS) of the SF-36 of individuals with ADPKD were lower than those of the reference population (45.7 vs. 50.0 and 47.8 vs. 50.0 points, both P < 0.001). QoL of ADPKD patients remained lower after comparison with age-corrected reference values (age 35–44 year; PCS 52.2, MCS 49.9 points, both P < 0.05). Larger liver volume negatively impacted PCS (P < 0.001) and MCS (P = 0.001), whereas there was no association with renal function (PCS P = 0.1, MCS P = 0.9) and kidney volume (PCS P = 0.5, MCS P = 0. 5). Total liver and kidney volume had no impact on PCS (P = 0.1), but did have impact on MCS (P = 0.02).ConclusionsQoL reported by non-dialysis patients with ADPKD is impaired compared to the general population. Large liver volume was the most important factor that diminishes QoL.PROSPERO International Registry number CRD42015026428.Electronic supplementary materialThe online version of this article (doi:10.1186/s12882-017-0578-6) contains supplementary material, which is available to authorized users.
Treatment of polycystic liver disease (PLD) focuses on symptom improvement. Generic questionnaires lack sensitivity to capture PLD-related symptoms, a prerequisite to determine effectiveness of therapy. We developed and validated a disease-specific questionnaire that assesses symptoms in PLD (PLD-Q). We identified 16 PLD-related symptoms (total score 0–100 points) by literature review and interviews with patients and clinicians. The developed PLD-Q was validated in Dutch (n=200) and US (n=203) PLD patients. We assessed the correlation of PLD-Q total score with EORTC symptom scale, global health visual analogue scale (VAS) of EQ-5D and liver volume. To test discriminative validity, we compared PLD-Q total scores of patients with different PLD-severity stages (Gigot classification), and PLD-Q total scores of PLD patients with general controls and polycystic kidney disease patients without PLD. Reproducibility was tested by comparing original test scores with two week retest scores. In total, 167 Dutch and 124 US patients returned the questionnaire. Correlation between PLD-Q total score and EORTC symptom scale (NL r=0.788; US r=0.811) and global health VAS (NL r=−0.517; US r=−0.593) was good. There was no correlation of PLD-Q total score with liver volume (NL r=0.138, P=0.236; US r=0.254, P=0.052). Gigot type III individuals scored numerically higher than type II patients (NL 46 vs. 40, P=0.089; US 48 vs. 36, P=0.055). PLD patients scored higher on the PLD-Q total score than general controls (NL 42 vs. 17; US 40 vs. 13 points) and polycystic kidney disease patients without PLD (22 points). Reproducibility of PLD-Q was excellent (NL r=0.94 and US 0.96).
Conclusion
PLD-Q is a valid, reproducible and sensitive disease-specific questionnaire which can be used to assess PLD-related symptoms in clinical care and future research.
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