1.Tafamidis for a Transplant Patient with Transthyretin Amyloid Polyneuropathy.
Jesús ROMERO-IMBRODA ; Teresa SAGRARIO-FUSTERO ; Clementina DEL CANTO-PÉREZ
Journal of Clinical Neurology 2017;13(4):444-446
No abstract available.
Amyloid Neuropathies*
;
Amyloid*
;
Humans
;
Prealbumin*
3.Plasma transthyretin is a nutritional biomarker in human morbidities.
Frontiers of Medicine 2022;16(4):540-550
Transthyretin (TTR) is a small liver-secreted plasma protein that shows close correlations with changes in lean body mass (LBM) during the entire human lifespan and agglomerates the bulk of nitrogen (N)-containing substrates, hence constituting the cornerstone of body building. Amino acids (AAs) dietary restriction causes inhibition of TTR production and impairs the accretion of LBM reserves. Inflammatory disorders result in cytokine-induced abrogation of TTR synthesis and urinary leakage of nitrogenous catabolites. Taken together, the data indicate that malnutrition and inflammation may similarly suppress the production of TTR through distinct and unrelated pathophysiological mechanisms while operating in concert to downsize LBM stores. The hepatic synthesis of TTR integrates both machineries, acting as a marker of reduced LBM resources still available for defense and repair processes. TTR operates as a universal surrogate analyte that allows for the grading of residual LBM capacity to reflect disease burden. Measurement of TTR is a simple, rapid, and inexpensive micro-method that may be reproduced on a daily basis, hence ideally suited for the follow-up of the most intricated clinical situations and as a reliable predictor of any morbidity outcome.
Biomarkers
;
Humans
;
Inflammation/metabolism*
;
Liver/metabolism*
;
Prealbumin
4.A case of wild-type transthyretin cardiac amyloidosis.
Ying QIN ; Xiao Lu SUN ; Dong WANG ; Wen JIANG ; Hong Yue WANG ; Xiao Xin SUN ; Wei FANG ; Jian LI ; Zhuang TIAN ; Lei SONG ; Lian Ming KANG
Chinese Journal of Cardiology 2021;49(10):1023-1026
9.A Case of Hypothyroxinemia with Thyroxine-Binding-Globulin Deficiency.
Soon Mi HUR ; Shin Hee KIM ; Min Jin KIM ; Dong Won BYUN ; Kyo Il SUH ; Myung Hi YOO ; Hyeong Kyu PARK
Soonchunhyang Medical Science 2011;17(2):161-163
The transport proteins such as thyroxine-binding-globulin (TBG), albumin and transthyretin carry over 99% of circulating thyroid hormones. TBG is a major thyroid hormone transport protein in serum. Although TBG deficiency does not have metabolic consequences, it has diagnostic implications as it can lead to an incorrect interpretation of thyroid function tests. We experienced a case that a man who had an abnormal thyroid function showed unexpectedly low concentrations of serum total thyroxine. We detected the low TBG in his serum and he was diagnosed the TBG deficiency. We report this case along with a review of the related literature.
Carrier Proteins
;
Prealbumin
;
Thyroid Function Tests
;
Thyroid Gland
;
Thyroid Hormones
;
Thyroxine
10.Familial Amyloidotic Polyneuropathy With Transthyretin Gene Mutation.
Young Jin KIM ; Joonhwa LEE ; Jongkeun PARK ; Sungjoo KIM ; Ileok JUNG ; Hee Jin LIM ; Tae Sook HWANG ; Jeeyoung OH
Journal of the Korean Neurological Association 2011;29(3):220-223
Familial amyloidotic polyneuropathy (FAP) is a rare hereditary amyloidosis that is characterized by slowly progressive peripheral polyneuropathy with other systemic involvement. More than 100 amyloidogenic transthyretin gene mutations have been reported, mainly in endemic areas of Portugal, Japan, and Sweden. We describe two brothers who exhibited progressive painful sensorimotor polyneuropathy with autonomic dysfunction. Gene analysis revealed a heterozygous Asp38Ala substitution in the transthyretin gene; this represents the first reported case of FAP in Korea.
Amyloidosis
;
Amyloidosis, Familial
;
Humans
;
Japan
;
Korea
;
Polyneuropathies
;
Portugal
;
Prealbumin
;
Siblings
;
Sweden