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: Guannan Huang, Mitsuharu Ueda, Masayoshi Tasaki, Taro Yamashita, Yohei Misumi, Teruaki Masuda, Genki Suenaga, Yasuteru Inoue, Yumiko Kinoshita, Sayaka Matsumoto, Mayumi Mizukami, Yukimoto Tsuda, Toshiya Nomura, Konen Obayashi, Yukio Ando
• Journal: Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis (Amyloid) Vol. 24 Issue 1 Pg. 24-29 (Mar 2017) ISSN: 1744-2818 [Electronic] England
• PMID: 28081656 (Publication Type: Journal Article)

Chemical References

• Amyloid
• Prealbumin
• Triiodothyronine

Topics

• Amyloid (metabolism)
• Amyloidosis, Familial (genetics, metabolism, pathology)
• Humans
• Liver Transplantation
• Mutation
• Prealbumin (metabolism)
• Thyroid Gland (metabolism, pathology)
• Triiodothyronine (metabolism)