不安定ヘモグロビン症
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 blood disease characterized by the presence of abnormal hemoglobins in the blood (同)haemoglobinopathy
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
UpToDate Contents
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English Journal
- Thalassemia-like phenotype in a novel complex hemoglobinopathy With α, β, δ globin chain abnormalities.
- Sabio H, Dixon N, Patel N, Obiaga C, Zhuang L, Meiler SE, Kutlar A, Kutlar F.SourceDepartment of Pediatrics, Wake Forest University School of Medicine, Winston-Salem, NC, USA.
- Journal of pediatric hematology/oncology.J Pediatr Hematol Oncol.2011 Dec;33(8):589-91.
- The occurrence of multiple abnormalities of α, β, δ, and γ globin genes may lead to unusual and complex phenotypes when they arise simultaneously in the same individual. Here, we report the findings of an African American boy who coinherited 3 heterozygous globin gene abnormalities: the unstable
- PMID 22042275
- The second mini-transplant for unstable mixed chimerism within the first 100 days after hematopoietic stem cell transplant in severe thalassemia.
- Choeyprasert W, Pakakasama S, Anurathapan U, Songdej D, Sirachainun N, Sirireung S, Panthangkool W, Hongeng S.SourceDepartment of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
- Pediatric transplantation.Pediatr Transplant.2011 Sep 5. doi: 10.1111/j.1399-3046.2011.01577.x. [Epub ahead of print]
- Choeyprasert W, Pakakasama S, Anurathapan U, Songdej D, Sirachainun N, Sirireung S, Panthangkool W, Hongeng S. The second mini-transplant for unstable mixed chimerism within the first 100 days after hematopoietic stem cell transplant in severe thalassemia. Pediatr Transplantation 2011. © 2011 Joh
- PMID 21895905
- Hb Extremadura [β64(E8)Gly→Ser;β133(H11)Val→Leu]: a new molecular analysis.
- Ropero P, Martín-Nuñez G, González FA, Villegas A, de la Fuente-Gonzalo F, Martínez-Nieto J, Vinuesa L, Díaz-Mediavilla J.SourceHematology Service, Hospital Clínico San Carlos, Madrid, Spain. propero.hcsc@salud.madrid.org
- Hemoglobin.Hemoglobin.2011;35(4):423-7.
- Unstable hemoglobin (Hb) variants account for 9.5% of structural hemoglobinopathies. The majority of these unstable variants are the result of gene point mutations resulting in the substitution of a single amino acid by another. The presence of two mutations in the same allele is infrequent: of the
- PMID 21797709
Japanese Journal
- Brief Notes on Selected Hemoglobinopathies Experienced in Yamaguchi University
- Ohba Yuzo
- The bulletin of the Yamaguchi Medical School 46(3-4), 61-69, 1999-12
- … Hb Ms as a couse of herediatary nigremia marked the first stage of hemoglobinopahthy research in Japan, and a variety of unstable hemoglobins causing hemolytic disease the second stage. … Virtually all types of abnormal Hbs were found among Japanese: hereditary nigremia, unstable Hb hemolytic disease, hyperunatable Hb disease mimicking a relatively severe form of thalassemia sydrom sydrome, hereditary erythremia due to high oxygen affinity Hbs, and so on. …
- NAID 120000863911
- 伊東 重義,久保 章,高橋 利通,鈴木 良人,松井 孝輔
- 日本消化器外科学会雑誌 24(1), 119-123, 1991-01-01
- 球状赤血球症をはじめとする溶血性疾患に胆石症が合併することは多く報告されており, その外科的治療についても検討されている. しかし不安定ヘモグロビン症に合併する胆石症についての報告はほとんど見られない. 今回不安定ヘモグロビン症で脾臓摘出後出後に胆嚢胆管結石を合併した症例を経験したので報告する. 症例は17歳, 男性で, 不安定ヘモグロビン症のため4歳時に脾臓摘出をうけている. 上腹部痛を主訴に受 …
- NAID 110001323076
Related Links
- Some mutations of globin genes decrease the solubility of the molecule in the red cell. Substitutions in the primary sequence of globin in these unstable hemoglobins can alter the tertiary or quaternary structure of the molecule and ...
- Preferred test for molecular confirmation of β thalassemia or a hemoglobinopathy involving the β-globin gene Clinical sensitivity – 99% (~97% by sequencing and ~2% by deletion analysis) for β thalassemia and hemoglobinopathies ...
★リンクテーブル★
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- 英
- unstable hemoglobinopathy, unstable hemoglobin disease
- 同
- 不安定血色素症、不安定ヘモグロビン溶血性貧血 unstable hemoglobin hemolytic anemia、先天性ハインツ小体溶血性貧血 congenital Heinz body hemolytic anemia
- 関
- 異常ヘモグロビン症 hemoglobinopathy
- 異常ヘモグロビンが変性して沈殿し、溶血を促進させる疾患。
- 溶血貧血を来す → 正球性貧血
- 赤血球中にハインツ小体が見られる
日本で見られる不安定ヘモグロビン症をきたすグロビンの変異 IMD.960
- Hb Geneva , β鎖
- Hb Hammersmith , β鎖
- Hb Tochigi , β鎖
- Hb Bristol , β鎖
- Hb Mizuno , β鎖
- Hb Koln , β鎖
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- 英
- congenital Heinz body hemolyticanemia
- 同
- 不安定ヘモグロビン症 unstable hemoglobinopathy
[★]
- 関
- labile、unsettled、unsteadiness
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異常ヘモグロビン症