Case Report

Korean J Hematol 2005; 40(1):

Published online March 30, 2005

https://doi.org/10.5045/kjh.2005.40.1.54

© The Korean Society of Hematology

의식장애를 동반한 고암모니아혈증과 혈청 아미노산이상을 보인 원발성 유전분증 1예

김유리, 김진석, 박선영, 박인혜, 정준원, 이승태, 민유홍, 한지숙, 기정혜

연세대학교 의과대학 내과학교실,
국민건강보험공단 일산병원 병리과

A Case of Serum Amino Acid Disturbance with Hyperammonemia in Patient with Primary Amyloidosis

Yu ri Kim, Jin Seok Kim, Sun Young Park, In Hae Park, June Won Cheong, Seung Tae Lee, Yoo Hong Min, Jee Sook Hahn, Jeong Hae Kie

Department of Internal Medicine, Yonsei University College of Medicine, Seoul
Department of Pathology, National Health Insurance Cooperation, Ilsan Hospital, Goyang, Korea

Abstract

There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a 71-years-old female patient with amyloidosis, who had shown disturbance of consciousness. At that time, the serum ammonia level was elevated; serum amino acids disturbance was also noted. In particular, the decrease in branched-chain amino acids and increase in aromatic amino acids results in a low Fisher ratio. The Fisher ratio, the ratio of branched-chain to aromatic amino acids, has been suggested as an important indicator of consciousness disturbance. We report, for the first time in Korea, a case of amyloidosis, with mental disturbance due to serum amino acid disturbance.

Keywords Amyloidosis, Amino acid disturbance, Hyperammonemia

Article

Case Report

Korean J Hematol 2005; 40(1): 54-57

Published online March 30, 2005 https://doi.org/10.5045/kjh.2005.40.1.54

Copyright © The Korean Society of Hematology.

의식장애를 동반한 고암모니아혈증과 혈청 아미노산이상을 보인 원발성 유전분증 1예

김유리, 김진석, 박선영, 박인혜, 정준원, 이승태, 민유홍, 한지숙, 기정혜

연세대학교 의과대학 내과학교실,
국민건강보험공단 일산병원 병리과

A Case of Serum Amino Acid Disturbance with Hyperammonemia in Patient with Primary Amyloidosis

Yu ri Kim, Jin Seok Kim, Sun Young Park, In Hae Park, June Won Cheong, Seung Tae Lee, Yoo Hong Min, Jee Sook Hahn, Jeong Hae Kie

Department of Internal Medicine, Yonsei University College of Medicine, Seoul
Department of Pathology, National Health Insurance Cooperation, Ilsan Hospital, Goyang, Korea

Abstract

There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a 71-years-old female patient with amyloidosis, who had shown disturbance of consciousness. At that time, the serum ammonia level was elevated; serum amino acids disturbance was also noted. In particular, the decrease in branched-chain amino acids and increase in aromatic amino acids results in a low Fisher ratio. The Fisher ratio, the ratio of branched-chain to aromatic amino acids, has been suggested as an important indicator of consciousness disturbance. We report, for the first time in Korea, a case of amyloidosis, with mental disturbance due to serum amino acid disturbance.

Keywords: Amyloidosis, Amino acid disturbance, Hyperammonemia

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