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Introduction: Phenylketonuria (PKU) requires regular phenylalanine monitoring to ensure optimal outcome. However, home sampling methods used for monitoring suffer high pre-analytical variability, inter-laboratory variability and turn-around-times, highlighting the need for alternative methods of home sampling or monitoring., Methods: A survey was distributed through email and social media to (parents of) PKU patients and professionals working in inherited metabolic diseases in Denmark, The Netherlands, and United Kingdom regarding satisfaction with current home sampling methods and expectations for future point-of-care testing (POCT)., Results: 210 parents, 156 patients and 95 professionals completed the survey. Countries, and parents and patients were analysed together, in absence of significant group differences for most questions. Important results are: 1) Many patients take less home samples than advised. 2) The majority of (parents of) PKU patients are (somewhat) dissatisfied with their home sampling method, especially with turn-around-times (3-5 days). 3) 37% of professionals are dissatisfied with their home sampling method and 45% with the turn-around-times. 4) All responders are positive towards developments for POCT: 97% (n = 332) of (parents of) patients is willing to use a POC-device and 76% (n = 61) of professionals would recommend their patients to use a POC-device. 5) Concerns from all participants for future POC-devices are costs/reimbursements and accuracy, and to professionals specifically, accessibility to results, over-testing, patient anxiety, and patients adjusting their diet without consultation., Conclusion: The PKU community is (somewhat) dissatisfied with current home sampling methods, highlighting the need for alternatives of Phe monitoring. POCT might be such an alternative and the community is eager for its arrival., Competing Interests: Declaration of competing interest F.J van Spronsen has been/ is a member of scientific advisory boards for defects in amino acid metabolism of, Agios, AlltRNA, Arla Food International, BioMarin, Eurocept Int, Homoly, Illumina, LogicBio, Lucane, Nestle-Codexis allience, Moderna, Nutricia, Oprhan Europe, Origin BioSciences, Travere, Ultragenyx. His institute has received research grants from Alexion, Biomarin, Nutricia, SoBi, and Vitaflo, has received grants from patient organizations ESPKU, Metakids, NPKUA, Stofwisselkracht, Stichting PKU research, ZonMW and Tyrosinemia Foundation, and has received honoraria as consultant and speaker from APR, Alexion, Axcella, LogiBio, Pluvia Biotech, PTC, Orphan Europe, Biomarin and Nutricia. AM has been a member of scientific advisory boards for APR, Arla Food International, Nutricia, Vitaflo, and MetaHealth, Her hospital has received research grants from Biomarin, Nutricia, MetaHealth, APR, Vitaflo, Metax, Cambrookes, and PTC., (Copyright © 2023. Published by Elsevier Inc.)

Background & Aims: Intestinal bacterial translocation is involved in activation of liver macrophages in cirrhotic patients. Macrophages play a key role in liver inflammation and are involved in the pathogenesis of cirrhosis and complications. Bacterial translocation may be determined by presence of bacterial DNA and macrophage activation, by the soluble mannose receptor. We hypothesize that the soluble mannose receptor is released from hepatic macrophages in cirrhosis and associated with bacterial DNA, portal pressure and complications., Methods: We investigated 28 cirrhotic patients set for transjugular intrahepatic portosystemic shunt insertion as a result of refractory ascites (n=17), acute (n=3), or recurrent variceal bleeding (n=8). We analysed plasma from the portal and hepatic veins for bacterial DNA and soluble mannose receptor with qPCR and ELISA., Results: The median soluble mannose receptor level was elevated in the hepatic vein compared with the portal vein (0.57(interquartile range 0.31) vs 0.55(0.40) mg/L, P=.005). The soluble mannose receptor levels were similar in bacterial DNA-positive and -negative patients. The soluble mannose receptor level in the portal and hepatic veins correlated with the portal pressure prior to transjugular intrahepatic portosystemic shunt insertion (r=.52, P<.008, both) and the levels correlated with Child-Pugh score (r=.63 and r=.56, P<.004, both). We observed higher soluble mannose receptor levels in patients with acute variceal bleeding compared to other indications (P<.05)., Conclusion: This study showed hepatic soluble mannose receptor excretion with a higher level in the hepatic than the portal vein, though with no associations to bacterial DNA. We observed associations between soluble mannose receptor levels and portal pressure and higher levels in patients with acute variceal bleeding indicating the soluble mannose receptor as a marker of complications of cirrhosis, but not bacterial translocation., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)