title: Clinicopathological and biochemical findings of thyroid amyloid in hereditary transthyretin amyloidosis with and without liver transplantation.
abstract: Hereditary transthyretin (TTR) amyloidosis is a fatal disease causing systemic organ dysfunctions. Histopathological studies revealed that thyroid glands are major target tissues. However, details about thyroid functions remain to be fully elucidated in this disease. For patient treatment, liver transplantation (LT) reportedly prolongs patient survival, but thyroid gland function after LT still remains poorly understood. In this study, we investigated the thyroid functions in 101 patients with hereditary TTR amyloidosis and the effects of LT on thyroid functions in those patients. In addition, we investigated histopathological and biochemical findings of thyroid specimens obtained at autopsy. Disease duration and age at examination inversely correlated with serum levels of free triiodothyronine (fT3) in hereditary TTR amyloidosis. On the contrary, in patients who underwent transplantation, time from disease onset to transplantation and age at transplantation clearly correlated with serum fT3and thyroid stimulating hormone (TSH) levels. In autopsy studies, amounts of thyroid amyloid deposits in patients with transplantation were significantly lower than those in patients without transplantation. Mass spectrometric analyzes also revealed that proportions of wild-type (WT) TTR in thyroid amyloid deposits in patients with hereditary TTR amyloidosis who underwent transplantations were higher than those in patients without transplantation. Thyroid hormone functions may diminish according to the disease progression. LT could prevent thyroid dysfunction in hereditary TTR amyloidosis.
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Guannan
authors.initials: G
authors.lastname: Huang
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Mitsuharu
authors.initials: M
authors.lastname: Ueda
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.|b Department of Morphological and Physiological Sciences , Graduate School of Health Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan.
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Tasaki
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Taro
authors.initials: T
authors.lastname: Yamashita
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Misumi
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Teruaki
authors.initials: T
authors.lastname: Masuda
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Genki
authors.initials: G
authors.lastname: Suenaga
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Yasuteru
authors.initials: Y
authors.lastname: Inoue
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Kinoshita
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Matsumoto
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Mayumi
authors.initials: M
authors.lastname: Mizukami
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Yukimoto
authors.initials: Y
authors.lastname: Tsuda
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Toshiya
authors.initials: T
authors.lastname: Nomura
authors.affiliation: b Department of Morphological and Physiological Sciences , Graduate School of Health Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan.
authors.forename: Konen
authors.initials: K
authors.lastname: Obayashi
authors.affiliation: a Department of Neurology , Graduate School of Medical Sciences, Kumamoto University , Chuo-ku, Kumamoto , Japan and.
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ando
mesh_terms: D000682:Amyloid / Q000378:metabolism*; D028226:Amyloidosis, Familial / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D016031:Liver Transplantation; D009154:Mutation; D011228:Prealbumin / Q000378:metabolism*; D013961:Thyroid Gland / Q000378:metabolism* / Q000473:pathology*; D014284:Triiodothyronine / Q000378:metabolism
chemical_list: D000682:Amyloid; D011228:Prealbumin; D014284:Triiodothyronine
keywords: ATTR; thyroid gland; thyroxine; transthyretin V30M; triiodothyronine