AbstractBackground and AimsPolycystic 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.MethodsA 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.ResultsThe 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).ConclusionA 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.

Augmented n = 315 p value (multivariable) * Age (years) 50 ± 15 58 ± 10 <0.0001* Women (%) 48.3 43.8 0.29* BMI (kg/m 2 ) 24.4 ± 3.9 24.8 ± 4.5 0.96* FEV1 (%) 78 ± 26 47 ± 18 <0.0001*CAT (points) 12 ± 8 19 ± 7 <0.0001*LSM (kPa) 6.5 ± 5.5 6.1 ± 3.9 0.042 LSM ≥7.1 kPa (%) 22.0 19.4 0.020 ALT (% ULN) 76 ± 45 77 ± 42 0.89 AST (% ULN) 74 ± 28 69 ± 26 <0.0001GGT (% ULN) 92 ± 110 86 ± 74 0.021

We present the case of a 57-year-old woman who was referred for the treatment of an intrapancreatic dislocated common bile duct (CBD) stone ([Video 1]).

We describe the case of a first twin pregnancy in a 27 year old patient, who experienced acute onset epigastric and right upper quadrant pain at a gestational age of 32 weeks and 2 days. She was diagnosed with acute liver and renal failure and possible disseminated intravascular coagulopathy (DIC) syndrome without pre-eclampsia. Early labor induction was mandatory to save both mother and foetuses. In this overview we describe the differential diagnosis of severe pregnancy related liver injury in the third trimester of pregnancy without pre-eclampsia. (Acta gastroenterol. belg., 2017, 80, 53-57).

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 mu/L and serum bile acids levels: 147 +/- 15 mu mol/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. (C) 2012 European Association for the Study of the Liver. Published by Elsevier B. V. All rights reserved.

It was with interest that we read the paper by Terjung et al 1, in a recent issue of Gut , where human myeloid cell-specific β-tubulin isotype 5 was identified as the target antigen of atypical p-ANCA in autoimmune liver disorders. The authors suggest cross-reactivity with the bacterial protein FtsZ, probably reflecting an abnormal immune response to intestinal microorganisms.1 In attempts to identify novel anti-nuclear and anti-neutrophil antibodies in autoimmune hepatitis, we performed 2D gel electrophoresis and western blotting starting from nuclear HeLa cell extracts or enriched nuclear envelope extracts from HL60 cells or neutrophils. The blots were incubated with serum samples from patients with autoimmune hepatitis and controls. Spots to which there was reactivity with serum from patients but not from controls were excised and identified by MALDI-TOF/TOF. In experiments in which we used non-myeloid HeLa cell extracts, a prominent protein spot of 49 670.82 Da (pI 4.78) was identified as β-tubulin isotype 5 (TBB5). The immune reactivity to tubulin was confirmed by western blotting. A cohort (n=468) of patients with autoimmune liver disease, viral hepatitis, inflammatory bowel disease and vasculitis was evaluated for reactivity to tubulin by ELISA using porcine tubulin (figure 1A) and recombinant human TBB5 (figure 1B …