Korean J Hematol.  2005 Mar;40(1):54-57. 10.5045/kjh.2005.40.1.54.

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

• Kim Y
• Kim JS
• Park SY
• Park IH
• Cheong JW
• Lee ST
• Min YH
• Hahn JS
• Kie JH

• KMID: 2252368
• DOI: http://doi.org/10.5045/kjh.2005.40.1.54

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.

Keyword

Amyloidosis; Amino acid disturbance; Hyperammonemia

MeSH Terms

Amino Acids
Amino Acids, Aromatic
Amino Acids, Branched-Chain
Ammonia
Amyloidosis*
Consciousness
Female
Humans
Hyperammonemia*
Korea
Liver
Multiple Myeloma
Unconsciousness
Amino Acids
Amino Acids, Aromatic
Amino Acids, Branched-Chain
Ammonia

Figure

• 

  Fig. 1. Some arteriolar walls are thickened by amorphous material deposits. There are multifocal chronic inflammatory cell infiltration and fibrosis associated tubular atrophy. The deposits are Congo-red positive (A: H&E ×400, B: Congo-red, ×400).

• 

  Fig. 2. Brain computed tomography shows no abnormal findings.

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