Thirteen Cases of Erythrocyte Pyruvate Kinase Deficiency Associated with Hereditary Hemolytic Anemia
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- ISHIDA Yoji
- Third Department of Internal Medicine, Yamaguchi University School of Medicine
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- MIWA Shiro
- Department of Internal Medicine, Institute of Medical Science, University of Tokyo
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- MIYAKE Kazuaki
- Division of Pediatrics, Kagoshima University School of Medicine
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- TORII Shozo
- Division of Pediatrics, The Japan Baptist Hospital
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- SHIGEMATSU Yosuke
- Division of Pediatrics, The Japan Baptist Hospital
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- SASAKI Keiko
- Department of Internal Medicine, Kagawa Prefecture Central Hospital
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- TATSUMI Norisuke
- Department of Central Laboratory, Osaka City University School of Medicine
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- KODAMA Soichi
- Division of Pediatrics, Kobe University School of Medicine
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- NINOMIYA Michito
- Division of Pediatrics, Kobe University School of Medicine
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- KAGEOKA Hiroshi
- Department of Internal Medicine, Niihama Rosai Hospital
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- TAKAHASHI Kiyoshi
- Department of Internal Medicine, Niihama Rosai Hospital
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- YOSHIOKA Keiichiro
- Division of Pediatrics, National Osaka Hospital
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- TSUJINO Giichi
- Department of Internal Medicine, Children's Medical Center of Osaka-City
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- TAKEUCHI Shigeki
- Division of Pediatrics, Hiroshima City Hospital
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- TANAKA Hiroshi
- Division of Pediatrics, Gifu Prefecture Hospital
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- KIMURA Hitoshi
- Division of Pediatrics, Gifu Prefecture Hospital
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- FUJIMURA Kingo
- Department of Internal Medicine, Research Institute for Nuclear Medicine and Biology, Hiroshima University
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- IMANAKA Fumio
- Department of Internal Medicine, Research Institute for Nuclear Medicine and Biology, Hiroshima University
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- KAWANO Hiroshi
- First Department of Internal Medicine, Shinshu University School of Medicine
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- YAMAGUCHI Hisomu
- Department of Hematology, Toranomon Hospital
Bibliographic Information
- Other Title
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- 遺伝性溶血性貧血を伴う赤血球ピルビン酸キナーゼ異常症の13例
- 遺伝性溶血性貧血を伴う赤血球ピルビン酸キナーゼ異常症の13例--臨床的並びに生化学的検討
- イデンセイ ヨウケツセイ ヒンケツ オ トモナウ セッケッキュウ ピルビンサン
- —臨床的並びに生化学的検討—
- —Clinical and Biochemical Studies—
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Description
Thirteen cases of hereditary deficiency of erythrocyte pyruvate kinase (PK) associated with hemolytic anemia, who were considered to be heterozygous for two different alleles, were studied clinically and biochemically. Characterizations of these abnormal PKs were performed according to the methods recommended by the International Committee for Standardization in Haematology (ICSH). Following ICSH recommendation, each patient was named PK “Kagoshima”, PK “Kyoto”, PK “Takamatsu”, PK “Abeno”, PK “Kobe”, PK “Marugame”, PK “Hoenzaka”, PK “Osaka”, PK “Motomachi”, PK “Gifu”, PK “Hiroshima, PK “Matsumoto”, and PK “Tama”.<br>The characteristics of mutant PK enzymes suggest that the causes of chronic hemolysis depend mainly on decreased affinity for phosphoenolpyruvate, thermolability, increased inhibition by adenosine triphosphate and low activation by fructose-1, 6-diphosphate.
Journal
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- Rinsho Ketsueki
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Rinsho Ketsueki 22 (3), 306-316, 1981
The Japanese Society of Hematology
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Details 詳細情報について
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- CRID
- 1390282680008099072
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- NII Article ID
- 130004498312
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- NII Book ID
- AN00252940
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- COI
- 1:STN:280:Bi2D3M7gslA%3D
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- ISSN
- 18820824
- 04851439
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- NDL BIB ID
- 2347768
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- PubMed
- 7277702
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL
- PubMed
- CiNii Articles
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- Abstract License Flag
- Disallowed