WordNet

lacking stability or fixity or firmness; "unstable political conditions"; "the tower proved to be unstable in the high wind"; "an unstable world economy"
disposed to psychological variability; "his rather unstable religious convictions"
highly or violently reactive; "sensitive and highly unstable compounds"
a hemoprotein composed of globin and heme that gives red blood cells their characteristic color; function primarily to transport oxygen from the lungs to the body tissues; "fish have simpler hemoglobin than mammals" (同)haemoglobin, Hb
relating to or involving or causing hemolysis; "hemolytic anemia" (同)haemolytic
a deficiency of red blood cells (同)anaemia
a lack of vitality (同)anaemia
genus of terrestrial or lithophytic ferns having pinnatifid fronds; chiefly of tropical America (同)genus Anemia

PrepTutorEJDIC

(物が)不安定な,ぐらぐらする / 情緒不安定な,落ち着のない / (気候・経済などが)安定性のない,不安定な / (化合物が)分解しやすい
ヘモグロビン,血色素

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1. 不安定ヘモグロビン変異体 unstable hemoglobin variants
2. 薬物および毒素による外因性非自己免疫性溶血性貧血 extrinsic nonautoimmune hemolytic anemia due to drugs and toxins
3. 成人における溶血性貧血の診断へのアプローチ approach to the diagnosis of hemolytic anemia in the adult
4. 小児における溶血性貧血の概要 overview of hemolytic anemias in children
5. 異常ヘモグロビン症の検査室診断 laboratory diagnosis of the hemoglobinopathies

English Journal

First North American case of Hemoglobin Shepherds Bush (beta 74[E18] Gly [rightwards arrow] Asp) in a central Pennsylvania family.

Paradise SL, Estep L, Olson J, Donaldson K.AbstractBACKGROUND: Hemoglobin Shepherds Bush (Human Genome Variation Society name: HBB:c.224G > A) is an unstable hemoglobin variant resulting from a beta 74 GGC to GAC mutation (Gly to Asp) that manifests clinically as hemolytic anemia or gall bladder disease due to chronic subclinical hemolysis.
BMC clinical pathology.BMC Clin Pathol.2014 Jan 15;14(1):4. [Epub ahead of print]
BACKGROUND: Hemoglobin Shepherds Bush (Human Genome Variation Society name: HBB:c.224G > A) is an unstable hemoglobin variant resulting from a beta 74 GGC to GAC mutation (Gly to Asp) that manifests clinically as hemolytic anemia or gall bladder disease due to chronic subclinical hemolysis.CASE P
PMID 24428873

Hb Grand Junction (HBB: c.348_349delinsG; p.His117IlefsX42): A New Hyperunstable Hemoglobin Variant.

Kent MW, Oliveira JL, Hoyer JD, Swanson KC, Kluge ML, Dawson DB, Liang X, Winkler TJ, Breaux CW Jr, Lacount R, Silliman CC.Author information Department of Research, Bonfils Blood Center , Denver, Colorado , USA.AbstractAbstract Hyperunstable hemoglobinopathy (HUH) [dominantly inherited β-thalassemia (β-thal)] is a relatively rare form of congenital hemolytic anemia in which mutations occur in the genes encoding for α and β chains, or both chains of the hemoglobin (Hb) molecule. We describe two Hispanic adolescents with a new unstable Hb variant (HBB: c.348_349delinsG; p.His117IlefsX42), resulting from a frameshift mutation at codons 115/116 of the β-globin gene. Both patients also have a 3.7 kb deletion on one α gene, leading to a decreased imbalance between α and β chain formation, and subsequently a milder phenotype than that seen in other hyperunstable Hb variants.
Hemoglobin.Hemoglobin.2014;38(1):8-12. doi: 10.3109/03630269.2013.853672.
Abstract Hyperunstable hemoglobinopathy (HUH) [dominantly inherited β-thalassemia (β-thal)] is a relatively rare form of congenital hemolytic anemia in which mutations occur in the genes encoding for α and β chains, or both chains of the hemoglobin (Hb) molecule. We describe two Hispanic adolesc
PMID 24432801

Hemoglobin analyses in the Netherlands reveal more than 80 different variants including six novel ones.

van Zwieten R, Veldthuis M, Delzenne B, Berghuis J, Groen J, Ait Ichou F, Clifford E, Harteveld CL, Stroobants AK.Author information Laboratory of Red Blood Cell Diagnostics, Sanquin Blood Supply Organization , Amsterdam , The Netherlands.AbstractAbstract More than 20 000 blood samples of individuals living in The Netherlands and suspected of hemolytic anemia or diabetes were analyzed by high resolution cation exchange high performance liquid chromatography (HPLC). Besides common disease-related hemoglobins (Hbs), rare variants were also detected. The variant Hbs were retrospectively analyzed by capillary zone electrophoresis (CZE) and by isoelectric focusing (IEF). For unambiguous identification, the globin genes were sequenced. Most of the 80 Hb variants detected by initial screening on HPLC were also separated by capillary electrophoresis (CE), but a few variants were only detectable with one of these methods. Some variants were unstable, had thalassemic properties or increased oxygen affinity, and some interfered with Hb A2 measurement, detection of sickle cell Hb or Hb A1c quantification. Two of the six novel variants, Hb Enschede (HBA2: c.308G > A, p.Ser103Asn) and Hb Weesp (HBA1: c.301C > T, p.Leu101Phe), had no clinical consequences. In contrast, two others appeared clinically significant: Hb Ede (HBB: c.53A > T, p.Lys18Met) caused thalassemia and Hb Waterland (HBB: c.428C > T, pAla143Val) was related to mild polycytemia. Hb A2-Venlo (HBD: c.193G > A, p.Gly65Ser) and Hb A2-Rotterdam (HBD: c.38A > C, p.Asn13Thr) interfered with Hb A2 quantification. This survey shows that HPLC analysis followed by globin gene sequencing of rare variants is an effective method to reveal Hb variants.
Hemoglobin.Hemoglobin.2014;38(1):1-7. doi: 10.3109/03630269.2013.849608. Epub 2013 Nov 7.
Abstract More than 20 000 blood samples of individuals living in The Netherlands and suspected of hemolytic anemia or diabetes were analyzed by high resolution cation exchange high performance liquid chromatography (HPLC). Besides common disease-related hemoglobins (Hbs), rare variants were also d
PMID 24200101

Japanese Journal

Sporadic case of hemoglobin Bushwick detected by chance in aplastic crisis

KITAZAWA JUNICHI,TONO CHIKAKO,TERUI KIMINORI,OTOMO HAYATO,ITO ETSURO,HATTORI YUKIO,OHBA YUZO,YOKOYAMA MASARU
Pediatrics international : official journal of the Japan Pediatric Society 42(2), 160-163, 2000-04-01
NAID 10010091874

The First Japanese Case of Hb Santa Ana, an Unstable Abnormal Hemoglobin, Identified Rapidly by Electrospray Ionization Mass Spectrometry

Miyazaki Ayako,Nakanishi Toyofumi,Kishikawa Masahiko [他],SHIMIZU Akira,JOZAKI Kiyoshi,YONEZAWA Takeshi
Internal medicine 36(5), 365-370, 1997-05
… Two members of a family had chronic hemolytic anemia due to unstable hemoglobin. … The leucine at the 88th position of the normal β-chain was substituted by proline in the hemoglobin as in Hb Santa Ana. …
NAID 10007006539