重症先天性好中球減少症
WordNet
- very bad in degree or extent; "a severe worldwide depression"; "the house suffered severe damage"
- unsparing and uncompromising in discipline or judgment; "a parent severe to the pitch of hostility"- H.G.Wells; "a hefty six-footer with a rather severe mien"; "a strict disciplinarian"; "a Spartan upbringing" (同)spartan
- intensely or extremely bad or unpleasant in degree or quality; "severe pain"; "a severe case of flu"; "a terrible cough"; "under wicked fire from the enemys guns"; "a wicked cough" (同)terrible, wicked
- set or keep apart; "sever a relationship" (同)break_up
- present at birth but not necessarily hereditary; acquired during fetal development (同)inborn, innate
- detached by cutting; "cut flowers"; "a severed head"; "an old tale of Anne Bolyn walking the castle walls with her poor cut-off head under her arm" (同)cut_off
- leukopenia in which the decrease is primarily in number of neutrophils (the chief phagocytic leukocyte)
PrepTutorEJDIC
- (人・法律などが)『厳しい』,厳格な / (態度・表情が)厳粛な,いかめしい / (病気などが)『重い』,危険な / (様式・趣味などが)簡素な,じみな,飾りのない / (人の体に)厳しくこたえる,激しい / (試験などが)難しい,厳しい
- (全体から)…‘を'切断する《+『名』+『from』+『名』》 / (いくつかの部分に)…‘を'分ける《+『名』+『in』(『into』)+『名』》 / 〈関係など〉‘を'断つ / 切れる,分かれる,分裂する
- (病気・身体的欠陥など)生まれつきの,先天的な
UpToDate Contents
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English Journal
- [Severe congenital neutropenia: analysis of clinical features, diagnostic methods, treatment and long-term outcome].
- Milá M, Rufach A, Dapena JL, Arostegui JI, Elorza I, Llort A, Sánchez de Toledo J, Díaz de Heredia C.SourceServicio de Oncología y Hematología Pediátricas, Hospital Materno-Infantil Vall d'Hebron, Barcelona, España.
- Anales de pediatría (Barcelona, Spain : 2003).An Pediatr (Barc).2011 Dec;75(6):396-400. Epub 2011 Jul 14.
- INTRODUCTION: Severe congenital neutropenia (SCN), a heterogeneous condition with onset at early ages, is characterised by primary myelopoiesis failure with an absolute neutrophil count (ANC) < 0.5 x10(9)/L, severe infections and risk of leukaemic transformation.OBJECTIVE: The aim of the study wa
- PMID 21757412
- A truncation mutant of Csf3r cooperates with PML-RARα to induce acute myeloid leukemia in mice.
- Kunter G, Woloszynek JR, Link DC.SourceDivision of Oncology, Department of Medicine, Washington University School of Medicine, St Louis, Mo., USA.
- Experimental hematology.Exp Hematol.2011 Dec;39(12):1136-43. Epub 2011 Sep 10.
- Severe congenital neutropenia is associated with a marked propensity to develop myelodysplasia or acute myeloid leukemia (AML). Truncation mutations of CSF3R, encoding the granulocyte colony-stimulating factor receptor (G-CSFR), are associated with development of myelodysplasia/AML in severe congeni
- PMID 21911095
Japanese Journal
- 先天性好中球減少症発症機構解明の進展 (特集 血液疾患における病態解析研究の進歩)
- HAX1遺伝子変異を有する重症先天性好中球減少症に合併する中枢神経症状の検討
- 中村 和洋,小林 正夫
- 日本産婦人科・新生児血液学会誌 = The Japanese journal of obstetrical, gynecological & neonatal hematology 18(2), 149-153, 2009-03-01
- NAID 10026710054
Related Links
- Severe congenital neutropenia is a condition that causes affected individuals to be prone to recurrent infections. People with this condition have a shortage ( deficiency) of neutrophils, a type of white blood cell that plays a role in inflammation ...
Related Pictures
![child with severe congenital neutropenia](https://1mg.info/0/e/8/1595.jpg)
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![severe congenital neutropenia scn is elane](https://1mg.info/0/e/8/1599.jpg)
★リンクテーブル★
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- 英
- severe congenital neutropenia
- 同
- Kostmann症候群 コストマン症候群、コストマン型先天性好中球減少症 Kostmann type congenital neutropenia、コストマン型好中球減少症 Kostmann neutropenia、コストマン病、Kostmann病、Kostmann disease
- 遺伝性好中球減少症 hereditary neutropenia 、遺伝性無顆粒球症 hereditary agranulocytosis
- 関
- 好中球減少症
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- 厳しい、厳格な。厳密な(⇔mild)。容赦しない、血も涙もない、痛烈な
- ひどい、猛烈な。骨の折れる。過酷な
- 関
- critically ill、grave、heavily、intense、life-threatening、rampant、serious、seriously、severe disease、severely、strenuous、severeness
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- 関
- ablate、ablation、amputate、amputation、break、breakage、cleavage、cleave、scission、transection、truncate、truncation
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- 関
- congenital、congenitally
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先天性好中球減少症