A Rare Association: Hereditary Hemorrhagic Telangiectasia with Liver Cirrhosis Causing Portal Hypertension.

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a vascular disorder of autosomal dominant etiology. The hallmark clinical feature is the presence of recurrent episodes of epistaxis in patients with vascular malformations and a tendency to bleed. We present the case of a 71-year-old woman who presented to the emergency department with upper gastrointestinal bleeding caused by esophageal varices, in conjunction with gastric angiodysplasias. The presence of oronasopharyngeal telangiectasias and hepatomegaly raised suspicion of HHT. The diagnostic workup confirmed the presence of angiodysplasia in the gastric region, portal arteriovenous malformation, and a pulmonary shunt.

Effect of a monitored exercise protocol in cerebral and hepatic hemodynamics in patients with cirrhosis and portal hypertension.

BACKGROUND: Physical exercise (PE) has been proven to be beneficial in patients with cirrhosis; effects in cognitive function and cerebral hemodynamics, are yet to be explored. AIM: To evaluate the effects of a PE program (LFN-exercise protocol) in hepatic/cerebral hemodynamics. METHODS: Randomized open clinical trial in patients with cirrhosis; Control: Diet(n = 13),Intervention: Diet + exercise(n = 14) for 12 weeks. Patients received an educational session, mental exercises (printed book and sudoku), and high-protein diet. Exercise intervention consisted of walking 4 times/week with an intensity rated between 12 and 14 on the Borg scale, monitored through bracelet accelerometers. Patients received weekly text messages to encourage adherence and had monthly in-person visits. RESULTS: Patients were mainly Child-Pugh A(88.9 %), median MELD 8(8-10), mean age 53±8 years. In the exercise group the number of steps increased from 9667±3008 to 11,931±4463 (p = 0.002), vs 8004±3224 to 8903±3504 (p = 0.053) in controls. Exercise decreased HVPG from 11(8-14) to 8(6-11)mmHg (p = 0.032) vs no change in the control group from 14(12-16) to 15(11-17)mmHg (p = 0.959). Intervention group showed better cerebral hemodynamics, cognitive function, nutritional status and quality of life after the intervention. Adherence was >90 %, with no adverse events. CONCLUSION: The LFN-exercise protocol improves portal hypertension, cerebral hemodynamics and cognitive function, as well as nutritional status and quality of life. GOV NUMBER: NCT03932552.