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Predictors of mortality in patients with hereditary hemorrhagic telangiectasia.

K P ThompsonJ NelsonH KimL PawlikowskaD A MarchukM T LawtonMarie E Faughnannull null
Published in: Orphanet journal of rare diseases (2021)
Chronic GI bleeding, anemia and symptomatic liver VMs are associated with increased mortality in HHT patients, independent of age, and in keeping with the limited treatment options for these aspects of HHT. Conversely, mortality does not appear to be associated with pulmonary AVMs or brain VMs, for which patients are routinely screened and treated preventatively at HHT Centres. This demonstrates the need for development of new therapies to treat chronic anemia, GI bleeding, and symptomatic liver VMs in order to reduce mortality among HHT patients.
Keyphrases
  • end stage renal disease
  • chronic kidney disease
  • newly diagnosed
  • ejection fraction
  • peritoneal dialysis
  • type diabetes
  • risk factors
  • atrial fibrillation
  • functional connectivity