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Takahiro Kawaji, Ryuhei Hara, Daisuke Eiki, Hidenobu Tanihara; Diagnostic Usefulness of Indocyanine Green Fundus Angiography for Transthyretin-related Familial Amyloidotic Polyneuropathy. Invest. Ophthalmol. Vis. Sci. 2012;53(14):2677.
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© ARVO (1962-2015); The Authors (2016-present)
We previously reported that multiple sites of hyperfluorescence with tissue staining along retinal and choroidal vessels were detected at the late phase of the indocyanine green angiography (IAG) examination in 3 patients with transthyretin (TTR)-rerated familial amyloidotic polyneuropathy (FAP) (Ophthalmology, 2005). However, the clinical characteristics of these findings were still unclear. The aim of this study was therefore to evaluate these unique findings in IAG in patients with TTR-related FAP.
We obtained clinical data for 39 eyes of 20 FAP patients (17 V30M and 3 T114C) who received IAG test at Kumamoto University Hospital in Japan between 2005 and 2010. We reviewed medical records and evaluated the characteristics of the patients, systemic and ocular histories, clinical findings, and IAG findings.
The mean age at onset of FAP was 34.7 years (range, 24-64 years). Seventeen of 20 patients performed liver transplantation (LT). The mean periods from onset of FAP to LT and IAG were 2.7 years (range, 0.6-5.0 years) and 8.2 years (range, 0.2-18.4 years), respectively. Hyperfluorescence spots in IAG were detected in all patients. IAG finding was only FAP-related ocular finding in some cases and only FAP-related systemic finding in 1 patient. Similar IAG findings were seen in both eyes of same patient. Multivariate analysis showed that there was no significant relevance between IAG grading and the following factors; gender, mutation type, age at onset of FAP, LT and IAG, period from onset of FAP to LT and IAG, follow-up period, period from LT to IAG, grade of systemic neuropathy, and grade of ocular manifestations. IAG findings progressed before LT only and did not change after LT.
IAG may be useful test for early detection of FAP-related ocular and systemic manifestations.
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