Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Sep 15;18(9):e0291716.
doi: 10.1371/journal.pone.0291716. eCollection 2023.

Ocular findings in patients with acquired ATTRv amyloidosis following domino liver transplantation

Junya Kitahara  1 , Tsuneaki Yoshinaga  2   3 , Shinji Kakihara  1 , Takao Hirano  1 , Akira Imai  1 , Teruyoshi Miyahara  1 , Masahide Yazaki  3   4 , Yoshiki Sekijima  2   3 , Toshinori Murata  1
Affiliations

Ocular findings in patients with acquired ATTRv amyloidosis following domino liver transplantation

Junya Kitahara et al. PLoS One. .

Abstract

Purpose: To investigate the presence of amyloidosis-related ocular findings in patients who received domino liver transplantation from ATTRv amyloidosis donors.

Methods: We reviewed the ocular findings in patients who had previously undergone domino liver transplantation and received ophthalmologic examinations between January 2009 and March 2023. The presence of amyloidosis-related ocular findings was retrospectively assessed by two ophthalmologists.

Results: During the study period, a total of 7 patients with 14 eyes were examined. All patients were considered as acquired ATTRv amyloidosis. The mean age at the final visit was 64.6±8.4 years (52-75 years), and the mean time since domino liver transplantation was 167.6±76.2 months (69-257 months). The two evaluators' assessments for amyloidosis-related ocular findings were completely identical. No amyloid fibril deposition was observed in the pupil, lens, or vitreous. Five patients (10 eyes) had a Schirmer test result of 5mm or less than 5 mm, and four patients with a total of 8 eyes underwent fluorescein angiography and indocyanine green angiography, and no evidence of retinal amyloid angiopathy was found on fluorescein angiography. However, three patients with 6 eyes showed choroidal amyloid angiopathy on indocyanine green angiography.

Conclusion: While cases of choroidal amyloid angiopathy were observed, serious amyloidosis-related ocular complications such as vitreous opacity or secondary glaucoma did not occur even in the long term after domino liver transplantation.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1
Fig 1. A representative case exhibiting typical findings of choroidal amyloid angiopathy.
(A) Slit-lamp microscopy shows that there are no abnormalities in the pupillary shape and amyloid deposition in the lens. (B) Fluorescein angiography shows that there is no retinal amyloid angiopathy. (C) Indocyanine green angiography in the later phase shows that there are several hot spots, indicating choroidal amyloid angiopathy (CAA).
See this image and copyright information in PMC

References

    1. Sekijima Y. Hereditary Transthyretin Amyloidosis. In: Adam MP, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, et al., editors. GeneReviews(®). Seattle (WA): University of Washington, Seattle. Copyright © 1993–2023, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.; 1993.
    1. Buxbaum JN, Dispenzieri A, Eisenberg DS, Fändrich M, Merlini G, Saraiva MJM, et al. Amyloid nomenclature 2022: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee. Amyloid. 2022;29(4):213–9. Epub 20221124. doi: 10.1080/13506129.2022.2147636 . - DOI - PubMed
    1. Ueda M. Transthyretin: Its function and amyloid formation. Neurochem Int. 2022;155:105313. Epub 20220223. doi: 10.1016/j.neuint.2022.105313 . - DOI - PubMed
    1. Yamashita T, Ando Y, Okamoto S, Misumi Y, Hirahara T, Ueda M, et al. Long-term survival after liver transplantation in patients with familial amyloid polyneuropathy. Neurology. 2012;78(9):637–43. Epub 20120215. doi: 10.1212/WNL.0b013e318248df18 . - DOI - PubMed
    1. Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M, et al. Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy. N Engl J Med. 2018;379(11):1007–16. Epub 20180827. doi: 10.1056/NEJMoa1805689 . - DOI - PubMed

Substances

Supplementary concepts