Questionnaire PLD-complaint-specific assessment identifies need for therapy in polycystic liver disease: A multi-centric prospective study.

BACKGROUND AND AIMS: Polycystic liver disease (PLD) can lead to extensive hepatomegaly. Symptom relief is the primary goal of the treatment. The role of the recently developed disease-specific questionnaires for identification of the thresholds and the assessment of therapy needs further investigation. METHODS: A five-year prospective multi-centric observational study in 21 hospitals in Belgium gathered a study population of 198 symptomatic PLD-patients of whom the disease-specific symptom questionnaire PLD-complaint-specific assessment (POLCA) scores were calculated. The thresholds of the POLCA score for the need for volume reduction therapy were analyzed. RESULTS: The study group consisted of mostly (82.8%) women with baseline mean age of 54.4 years ±11.2, median liver volume expressed as height-adjusted total liver volume(htLV) of 1994 mL (interquartile range [IQR] 1275; 3150) and median growth of the liver of +74 mL/year (IQR +3; +230). Volume reduction therapy was needed in 71 patients (35.9%). A POLCA severity score (SPI) ≥ 14 predicted the need for therapy both in the derivation (n = 63) and the validation cohort (n = 126). The thresholds to start somatostatin analogues (n = 55) or to consider liver transplantation (n = 18) were SPI scores of ≥14 and ≥ 18 and the corresponding mean htLVs were 2902 mL (IQR 1908; 3964) and 3607 mL (IQR 2901; 4337), respectively. Somatostatin analogues treatment resulted in a decrease in the SPI score -6.0 versus + 4.5 in patients without somatostatin analogues (p < 0.01). Changes in the SPI score were significantly different between the liver transplantation group and no liver transplantation group, +4.3 ± 7.1 versus -1.6 ± 4.9, respectively, (p < 0.01). CONCLUSION: A polycystic liver disease-specific questionnaire can be used as a guide on when to start a volume reduction therapy and to assess the effect of treatment.

Cholestasis-induced pruritus treated with ultraviolet B phototherapy: an observational case series study.

BACKGROUND & AIMS: Pruritus is a disabling complication of cholestatic liver disorders. Its management remains challenging. Ultraviolet B (UVB) phototherapy has been successfully used to treat pruritus in other indications. METHODS: This is an observational case series. The study population consists of 13 patients (10 females, mean age 52 years) with pruritus due to different cholestatic liver disorders: PBC (n=4), PSC (n=2), drug-induced (n=3) and persistent cholestasis after liver transplantation (LT) (n=4). Serum alkaline phosphatase levels were: 686 ± 363 µ/L and serum bile acids levels: 147 ± 15 µmol/L. In all patients, conventional medical treatment had failed to control pruritus. Perception of pruritus was recorded by the visual analogue scale (VAS). RESULTS: The mean follow-up was 3 years. Ten patients (77%) had more than 60% reduction in perceived pruritus of which 4 had more than an 80% reduction. Median [25-75% percentiles] VAS score before and after treatment decreased from 8.0 [8.0-10] to 2.0 [1.5-2.1] (p<0.001). The mean number of irradiations required to obtain this effect was 26 ± 17 (average duration of phototherapy: 8 weeks). No significant changes in cholestatic serum markers were observed. Four patients (30%) needed an additional phototherapy course because of recurrent pruritus and in all of them again a marked improvement of pruritus was observed. The therapy was well tolerated, except in two patients who developed, during retreatment, pronounced erythema in one case and paresthesia in the other case. CONCLUSIONS: UVB phototherapy appears to be a promising and well tolerated treatment also for cholestasis-associated pruritus.

Endoscopic trimming of metallic stents with the use of argon plasma.

BACKGROUND: The endoscopic placement of metallic stents for palliation of malignant obstruction of the GI or biliary tract is an established practice and as such is often applied. Use of these stents, however, has its problems. Stent migration may cause obstruction of the bowel lumen. Migration of a biliary stent into the contralateral duodenal wall may cause difficulty in gaining access to the biliary tract, as will the placement of a duodenal stent across the ampulla. OBJECTIVE: We report on 6 patients in whom trimming of the metallic nitinol stent was performed. DESIGN: Single-center, retrospective case series. SETTING: Secondary referral center. PATIENTS: Of 6 patients included, 2 patients each had an uncovered duodenal stent, 2 had an uncovered biliary stent each, 1 had an uncovered colorectal stent, and 1 had a covered gastroduodenal stent. INTERVENTIONS: Under direct endoscopic vision, an argon plasma beam was used to cut self-expandable metallic stents, as appropriate. MAIN OUTCOME MEASUREMENT: The main objective was relief of the obstruction to the bowel lumen or bile duct, facilitating successful passage of an endoscope or biliary canulation, respectively. RESULTS: In all 5 patients with uncovered metallic stents, we were able to re-establish access to the obstructed bowel lumen or the biliary tree, as indicated. An attempt to tailor the length of a covered metallic gastroduodenal stent failed. No complications were observed and no hemorrhage or perforation occurred. LIMITATIONS: The study was limited by retrospective design and small sample size. CONCLUSIONS: The endoscopic cutting and tailoring of an uncovered metallic prosthesis, by means of an argon plasma beam, is feasible, effective, and safe. Trimming of covered stents is not advocated.