title: Impact of liver transplantation on the natural history of oculopathy in Portuguese patients with transthyretin (V30M) amyloidosis.
abstract: OBJECTIVE
  Evaluation of the impact of liver transplantation in the natural history of ocular disorders in familial amyloidotic polyneuropathy (FAP) amyloidosis TTR V30M related (ATTR V30M) patients.
  
  
  METHODS
  A clinical, retrospective and cross-sectional study of 64 Portuguese FAP ATTR V30M patients was carried out between January 2005 and December 2011.
  
  
  METHODS
  Thirty-two liver transplanted patients (both eyes) aged 39.6-53.8 years old, 32/32 male/female, were paired with an equal number of non-transplanted patients, matching for age, gender, age at onset, disease duration and gender of transmitting parent. Intervention or observation procedure: Routine ophthalmological observation.
  
  
  METHODS
  Slit-lamp observation for abnormal conjunctival vessels (ACV), tears break-up time, iris, lens; fundus observation for vitreous, retina and optic disc; Schirmer test.
  
  
  RESULTS
  Liver transplantation had no influence on tears break-up time, deposition of amyloid on the iris and retinal amyloid angiopathy. Slight, non-statistically significant protective effects of liver transplantation were noted in the first years for some ocular manifestations (ACV and scalloped iris), except for the abnormal Schirmer test, which was significantly more prevalent in non-transplanted patients' eyes (81% versus 56%, p = 0.002). On the other hand, deposition of amyloid on the lens, vitreous amyloidosis and glaucoma were apparently more common in transplanted patients. Those differences tended to disappear with time.
  
  
  CONCLUSIONS
  Ocular manifestations of FAP were not influenced by liver transplantation in a meaningful way. Both transplanted and non-transplanted FAP patients need similar regular follow-up due to long-term risk of serious ocular disease.
authors.affiliation: Opthalmology Service, Hospital de Santo António , Porto , Portugal .
authors.forename: João Melo
authors.initials: JM
authors.lastname: Beirão
authors.forename: Jorge
authors.initials: J
authors.lastname: Malheiro
authors.forename: Carolina
authors.initials: C
authors.lastname: Lemos
authors.forename: Eduarda
authors.initials: E
authors.lastname: Matos
authors.forename: Idalina
authors.initials: I
authors.lastname: Beirão
authors.forename: Paulo
authors.initials: P
authors.lastname: Pinho-Costa
authors.forename: Paulo
authors.initials: P
authors.lastname: Torres
mesh_terms: D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005128:Eye Diseases / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D011174:Portugal; D011228:Prealbumin / Q000235:genetics*; D015995:Prevalence; D012189:Retrospective Studies
chemical_list: D011228:Prealbumin
keywords: ATTR V30M; Amyloid; amyloidosis; familial amyloidotic polyneuropathy; liver transplantation; oculopathy; transthyretin