No abstract

Significance Polycystic liver disease (PCLD) is an autosomal dominantly inherited disorder characterized by multiple fluid-filled hepatic cysts that may cause an extremely enlarged liver. PCLD is genetically heterogeneous, and mutations in PRKCSH and SEC63 are present in ∼25% of PCLD patients. This research identifies four unique LRP5 mutations in four independent families that were all located at highly conserved protein domains. Functional activity analyses suggest that mutant LRP5 reduces wingless (Wnt) signal activation. This study suggests that imbalanced Wnt signaling is related to hepatic cyst formation.

No abstract

SUMMARY BackgroundPolycystic liver disease (PLD) is a phenotypical expression of autosomal dominant polycystic kidney disease and isolated polycystic liver disease. Somatostatin analogues, such as lanreotide, reduce polycystic liver volume.

Liver cysts are common, affecting 5%-10% of the population. Most are asymptomatic, however 5% of patients develop symptoms, sometimes due to complications and will require intervention. There is no consensus on their management because complications are so uncommon. The aim of this study was to perform a collected review of how a series of complications were managed at our institutions. Six different patients presenting with rare complications of liver cysts were obtained from Hepatobiliary Units in the United Kingdom and The Netherlands. History and radiological imaging were obtained from case notes and computerised radiology. As a result, 1 patient admitted with inferior vena cava obstruction was managed by cyst aspiration and lanreotide; 1 patient with common bile duct obstruction was first managed by endoscopic retrograde cholangiopancreatography and stenting, followed by open fenestration; 1 patient with ruptured cysts and significant medical co-morbidities was managed by percutaneous drainage; 1 patient with portal vein occlusion and varices was managed by open liver resection; 1 patient with infected cysts was treated with intravenous antibiotics and is awaiting liver transplantation. The final patient with a simple liver cyst mimicking a hydatid was managed by open liver resection. In conclusion, complications of cystic liver disease are rare, and we have demonstrated in this series that both operative and non-operative strategies have defined roles in management. The mainstays of treatment are either aspiration/sclerotherapy or, alternatively laparoscopic fenestration. Medical management with somatostatin analogues is a potentially new and exciting treatment option but requires further study.

No abstract

Objectives To evaluate the psychometric properties of the Responses to Positive Affect (RPA) questionnaire in a sample of persons with bipolar disorder (BD). Method Cross‐sectional survey study with 107 persons with BD. The original 3‐factor model of the RPA was compared with a 2‐factor model. Construct validity was determined with measures of well‐being, personal recovery, social role participation, and psychopathology and incremental validity was evaluated. Results The fit of the 3‐factor model was acceptable for most fit indices. Subscores of the RPA revealed a significant relationship with aspects of well‐being, personal recovery, and psychopathology. Dampening and self‐focused positive rumination explained additional variance in personal recovery above and beyond well‐being. Conclusions The RPA is an internally consistent and valid tool to assess positive emotion regulation processes in persons with BD. Specifically, the processes of dampening and emotion‐focused positive rumination seem to play an important role in BD.

The relevance of personal recovery receives increasing attention in mental health care and is also important for people with bipolar disorder (BD). There is a need for reliable and valid instruments measuring personal recovery. Therefore, the current study evaluated the psychometric properties of a Dutch translation of the Questionnaire about the Process of Recovery (QPR) in a sample of people with BD and explored the relationship with constructs of well-being, social role participation, and psychopathology. A cross-sectional survey study was conducted in which 102 people diagnosed with BD completed the QPR. Factor structure of the QPR was evaluated by conducting confirmatory factor analyses (CFA), and internal consistency was assessed by calculating reliability coefficients. Convergent validation measures assessed well-being, social role participation, and symptomatology. Incremental validity was determined by evaluating the ability of the QPR to explain variance in symptomatology above and beyond well-being. Findings of the CFA supported a unidimensional factor structure, and internal consistency estimates were excellent. Scores of the QPR showed strong correlations with convergent measures, but were only weakly associated with manic symptomatology.Moreover, personal recovery explained additional variance in symptoms of depression and anxiety above and beyond well-being, indicating incremental validity. The QPR appears to be a reliable and valid tool to assess personal recovery in people with BD. Our findings underline the importance of personal recovery in the context of treatment of BD. Personal recovery demonstrates a substantial overlap with well-being.This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.