Liver cyst penetration of antibiotics at the target site of infection: a randomized pharmacokinetic trial.

Background: The EASL cystic liver disease guideline states that drug penetration at the site of infection (liver cyst) is essential for successful treatment, but pharmacokinetic (PK) data on cyst penetration are limited.

Objectives: This study aims to investigate tissue penetration of four antibiotics in non-infected liver cysts and explores influencing factors.

Methods: We performed a prospective, randomized single-dose PK-study.

Clinical Characteristics and Outcomes of Patients With Cutibacterium acnes Endocarditis.

Importance: It is suggested that patients with Cutibacterium acnes endocarditis often present without fever or abnormal inflammatory markers. However, no study has yet confirmed this statement.

Objective: To assess the clinical characteristics and outcomes of patients with C acnes endocarditis.

[Scaling up Outpatient Parenteral Antimicrobial Therapy (OPAT) service in the Netherlands].

Treatment of infections with intravenous antibiotics does not always require hospital care; specialized home care nurses can administer parenteral treatment at home. Dedicated Outpatient Parenteral Antimicrobial Therapy (OPAT) teams are emerging in an increasing number of hospitals in the Netherlands to supervise treatment selection and provide safety monitoring for patients with home treatment. This specialized, nurse driven team facilitates home treatment by collaborating with infectious disease specialists, pharmacy and home care teams, as well as the patient and treating physician.

Protocol for a randomized controlled multicenter trial assessing the efficacy of leuprorelin for severe polycystic liver disease: the AGAINST-PLD study.

Background: In patients with severe polycystic liver disease (PLD), there is a need for new treatments. Estrogens and possibly other female sex hormones stimulate growth in PLD. In some patients, liver volume decreases after menopause.

T cell protein tyrosine phosphatase protects intestinal barrier function by restricting epithelial tight junction remodeling.

Genome-wide association studies revealed that loss-of-function mutations in protein tyrosine phosphatase non-receptor type 2 (PTPN2) increase the risk of developing chronic immune diseases, such as inflammatory bowel disease (IBD) and celiac disease. These conditions are associated with increased intestinal permeability as an early etiological event. The aim of this study was to examine the consequences of deficient activity of the PTPN2 gene product, T cell protein tyrosine phosphatase (TCPTP), on intestinal barrier function and tight junction organization in vivo and in vitro.

Tamoxifen for the treatment of polycystic liver disease: A case report.

Rationale: Polycystic liver disease is a rare disease characterized by the growth of numerous cysts in the liver. The liver function remains well preserved, but liver volumes can grow very large, and some patients ultimately need a liver transplantation. Other treatment options are limited and there is an unmet need for new therapeutic options.

Estrogens in polycystic liver disease: A target for future therapies?

Background And Aims: Patients suffering from polycystic liver disease (PLD) can develop large liver volumes, leading to physical and psychological complaints, reducing quality of life. There is an unmet need for new therapies in these patients. Estrogen seems to be a promising target for new therapies.

Incident Gallstones During Somatostatin Analog Treatment are Associated with Acute Biliary Complications Especially After Discontinuation.

Introduction: Gallstones are a known adverse effect of somatostatin analogs, but the exact incidence and clinical implications are unknown.

Objectives: The aim of this study was to investigate the incidence of gallstones on imaging and related complications in unbiased trial data.

Methods: Data from the DIPAK 1 trial, in which 305 polycystic kidney disease patients were randomized to standard of care (SoC) or lanreotide for 120 weeks, were used.

Efficacy and safety of selective decontamination of the digestive tract (SDD) to prevent recurrent hepatic cyst infections in polycystic liver disease: a retrospective case series.

Background: Hepatic cyst infection is a complication of polycystic liver disease (PLD) that causes substantial morbidity. Repetitive infection is frequent and is increasingly difficult to treat. As translocated gut bacteria are considered the cause, we hypothesize that selective decontamination of the digestive tract (SDD) reduces recurrence of hepatic cyst infection.

Symptom relief and quality of life after combined partial hepatectomy and cyst fenestration in highly symptomatic polycystic liver disease.

Background: Polycystic liver disease can cause severe symptomatic hepatomegaly. Combined partial hepatectomy and cyst fenestration can be performed to reduce liver volume and symptom burden. We aimed to assess change in symptom relief and quality of life 6 months after partial hepatectomy and cyst fenestration in polycystic liver disease patients.

New insights into targeting hepatic cystogenesis in autosomal dominant polycystic liver and kidney disease.

: Polycystic liver disease (PLD) is a rare disease defined by the growth of hepatic cysts and occurs either isolated or as an extrarenal manifestation of polycystic kidney disease. While surgery has been the mainstay in treatment of symptomatic PLD, recently discovered regulatory mechanisms affecting hepatic cystogenesis provide potential new therapies to reduce hepatic cyst burden.: This review summarizes intracellular pathways and therapeutic targets involved in hepatic cystogenesis.

Management of portal hypertension and ascites in polycystic liver disease.

Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication.

Position statement on access to care in rare liver diseases: advancements of the European reference network (ERN) RARE-LIVER.

The European Reference Network for rare liver diseases (ERN RARE-LIVER) is a Europe-wide network of paediatric and adult hepatologists from expert centres in close collaboration with patient advocates from the various disease-areas covered in our ERN. The ERN is focused on providing more equitable care across Europe and creates a network of both medical specialists and patient experts in rare liver disease. This position paper summarizes the achievements of the first year and plots the route for the near future for ERN RARE-LIVER, as discussed during a strategy meeting that took place 27 and 28 February 2018 in Nijmegen, the Netherlands.

Estrogen-Containing Oral Contraceptives Are Associated With Polycystic Liver Disease Severity in Premenopausal Patients.

The association between estrogen-containing oral contraceptives and history of pregnancies with disease severity in women with polycystic liver disease (PLD) is unclear. We performed a cross-sectional cohort study to assess this association by selecting female patients with PLD of which imaging was available prior to any liver volume-reducing therapy. Patients received a questionnaire to collect detailed information on estrogen use and pregnancies.

Clinical response after laparoscopic fenestration of symptomatic hepatic cysts: a systematic review and meta-analysis.

Background: Laparoscopic fenestration is one of the treatment options for symptomatic hepatic cysts, either solitary or in context of polycystic liver disease (PLD), but indications, efficacy and surgical techniques are under debate.

Methods: A systematic literature search (1950-2017) of PubMed, Embase, Web of Science and the Cochrane Library was performed (CRD42017071305). Studies assessing symptomatic relief or symptomatic recurrence after laparoscopic fenestration in patients with symptomatic, non-parasitic, hepatic cysts were included.