THE FIRST JAPANESE CASE OF HB-SANTA-ANA, AN UNSTABLE ABNORMAL HEMOGLOBIN, IDENTIFIED RAPIDLY BY ELECTROSPRAY-IONIZATION MASS-SPECTROMETRY

Authors
MIYAZAKI A NAKANISHI T KISHIKAWA M SHIMIZU A JOZAKI K YONEZAWA T
Citation
A. Miyazaki et al., THE FIRST JAPANESE CASE OF HB-SANTA-ANA, AN UNSTABLE ABNORMAL HEMOGLOBIN, IDENTIFIED RAPIDLY BY ELECTROSPRAY-IONIZATION MASS-SPECTROMETRY, Internal medicine, 36(5), 1997, pp. 365-370
Citations number
14
Categorie Soggetti
Medicine, General & Internal
Journal title
Internal medicine → ACNP
ISSN journal
09182918
Volume
36
Issue
5
Year of publication
1997
Pages
365 - 370
Database
ISI
SICI code
0918-2918(1997)36:5<365:TFJCOH>2.0.ZU;2-C
Abstract
Two members of a family had chronic hemolytic anemia due to unstable h emoglobin, The abnormal beta-chain with a molecular weight of 16 u sma ller than the normal beta-chain was found within 5 minutes by analysis using electrospray ionization mass spectrometry, The substitution of amino acid was also determined rapidly by this new strategy, The leuci ne at the 88th position of the normal beta-chain was substituted by pr oline in the hemoglobin as in Hb Santa Ana, This is the first report o f a Japanese case of Hb Santa Ana; the clinical course was similar to that in the previously reported cases.