• Michele Barone, Patrizia Suppressa, Maria Teresa Viggiani, Gennaro Mariano Lenato, Arnaldo Scardapane, Luigi Chiumarulo, Nicola Ambrosini, LeoAlfredo DiADGastroenterology Unit, Department of Emergency and Organ Transplantation, (D.E.T.O.), University of Bari Aldo Moro, Policlinico University Hospital, Piazza G. Cesare 11, 70124 Bari, Italy., and Carlo Sabbà.
• Gastroenterology Unit, Department of Emergency and Organ Transplantation, (D.E.T.O.), University of Bari Aldo Moro, Policlinico University Hospital, Piazza G. Cesare 11, 70124 Bari, Italy. Electronic address: michele.barone@uniba.it.
• Eur. J. Intern. Med. 2021 Aug 1; 90: 43-48.

BackgroundPortosystemic shunts in Hereditary Haemorrhagic Telangiectasia (HHT) are often overlooked by conventional imaging although they could reduce hepatic clearance of gut-derived toxins.AimsTo evaluate, the presence of subclinical neurological alterations (SNAs), that we named "minimal portosystemic encephalopathy" (mPSE) in HHT patients without advanced liver disease (ALD).MethodsIn this cross sectional study, consecutive HHT outpatients were firstly screened by critical flicker frequency (CFF) test (abnormal ≤39Hz), and the simplified animal naming test (S-ANT1) (abnormal <15) was used to confirm the diagnosis of mPSE. Furthermore, we evaluated the effect of lactulose administration on mPSE. Multi-slice CT, cerebral dynamic magnetic resonance, laboratory analyses and transient elastography were also used.ResultsNone of the 37 enrolled patients showed portosystemic shunts at imaging techniques. However, 33 patients had normal CFF values (CFF-) and 4 displayed CFF alterations (37.0±0.7Hz, CFF+). The S-ANT1 confirmed an impaired neurological performance (10.2±2.8) in CFF+ patients thus confirming the presence of mPSE. Noteworthy, lactulose administration determined a CFF increase (39.1±0.4Hz) and S-ANT1 normalization in these patients. Neither mPSE- nor mPSE+ patients had ALD and showed similar demographic, clinical and laboratory parameters. Finally, no mPSE+ patient showed radiologically-detectable brain vascular malformations or other brain abnormalities at imaging.ConclusionsHHT patients represent a human model of mPSE secondary to portosystemic shunts escaping radiological detection. mPSE evaluation should be incorporated in HHT surveillance protocols since it can affect both health-related/social aspects and pharmacokinetics of orally administered drugs with a narrow therapeutic index and high hepatic first-pass uptake.Copyright © 2021. Published by Elsevier B.V.

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