同: HPFH

WordNet

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
of or relating to a fetus; "fetal development" (同)foetal

PrepTutorEJDIC

《所有・所属》…『の』,…のものである,…に属する・《材料・要素》…『でできた』,から成る・《部分》…『の』[『中の』] ・《数量・単位・種類を表す名詞に付いて》…の・《原因・動機》…『で』,のために(because of) ・《主格関係》…『の』,による,によって・《目的格関係》…『を』,の・《同格関係》…『という』・《関係・関連》…『についての』[『の』],の点で・《抽象名詞などと共に》…の[性質をもつ] ・《『It is』+『形』+『of』+『名』+『to』 doの形で,ofの後の名詞を意味上の主語として》・《分離》…『から』・《起原・出所》…『から』[『の』](out of) ・《『名』+『of』+『a』(『an』)+『名』の形で》…のような・《『名』+『of』+『mine』(『yours, his』など独立所有格)の形で》…の…・《時》(1)《副詞句を作って》…に《形容詞句を作って》…の・《時刻》《米》…前(to,《米》before)
(…を)あくまでもやり抜くこと,(…における)ねばり強さ,がん張り;執ようさ《『+in+名(doing』)》 / (…が)永く続くこと,(…の)持続《+『of』+『名』)
ヘモグロビン,血色素
世襲の,親譲りの / 遺伝的な,遺伝性の

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/10/21 10:06:43」(JST)

wiki en

Hereditary persistence of fetal hemoglobin (HPFH, BrE: Hereditary persistence of foetal haemoglobin) is a benign condition in which significant fetal hemoglobin (hemoglobin F) production continues well into adulthood, disregarding the normal shutoff point after which only adult-type hemoglobin should be produced.^[1]

Causes

This is usually caused by mutations in the β-globin gene cluster.^{[citation needed]} The percentage of incorrect expression might be as low as 10-15% or as high as 100% of the total hemoglobin, usually higher in homozygotes than in heterozygotes.^[2]

Epidemiology

HPFH may alleviate the severity of certain hemoglobinopathies and thalassemias, and is selected for in populations with a high prevalence of these conditions (which in turn are often selected for in areas where malaria is endemic). Thus, it has been found to affect Americans of African and Greek descent.^[3]

Presentation

The condition is usually asymptomatic, and is only noticed when screening for other hemoglobin disorders.

Benefit to persons with sickle cell disease

In persons with sickle cell disease, high levels of fetal hemoglobin as found in a newborn or as found abnormally in persons with hereditary persistence of fetal hemoglobin, the HbF causes the sickle cell disease to be less severe. In essence the HbF inhibits polymerization of HbS. A similar mechanism occurs with persons who have sickle cell trait. Approximately 40% of the hemoglobin is in the HbS form while the rest is in normal HbA form. The HbA form interferes with HbS polymerization.^[4]

References

^ http://cancerweb.ncl.ac.uk/cgi-bin/omd?hereditary+persistence+of+foetal+haemoglobin^{[dead link]}
^ http://www.enerca.org/PublicPages/Anaemiascovered/HereditarypersistanceoffoetalhaemoglobinHPFH/tabid/177/Default.aspx^{[dead link]}
^ Friedman S, Schwartz E (January 1976). "Hereditary persistence of foetal haemoglobin with beta-chain synthesis in cis position (Ggamma-beta+-HPFH) in a negro family". Nature 259 (5539): 138–40. doi:10.1038/259138a0. PMID 1246351.
^ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009-05-28). Robbins and Cotran Pathologic Basis of Disease, Professional Edition: Expert Consult - Online (Robbins Pathology) (Kindle Locations 33411-33412). Elsevier Health. Kindle Edition.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 正常ヒトヘモグロビンの構造および機能 structure and function of normal human hemoglobins
2. 異常ヘモグロビン症の検査室診断 laboratory diagnosis of the hemoglobinopathies
3. 異型鎌状赤血球症候群 variant sickle cell syndromes
4. サラセミア症候群の分子病理 molecular pathology of the thalassemic syndromes
5. 鎌状細胞貧血における臨床的ばらつき clinical variability in sickle cell anemia

English Journal

Biochemical markers of glucose metabolism may be used to estimate the degree and progression of iron overload in the liver and pancreas of patients with β-thalassemia major.

Bas M1, Gumruk F, Gonc N, Cetin M, Tuncer M, Hazırolan T, Yildirim G, Karabulut E, Unal S.
Annals of hematology.Ann Hematol.2015 Jul;94(7):1099-104. doi: 10.1007/s00277-015-2342-y. Epub 2015 Mar 6.
The use of cardiac and hepatic T2* MRI measurements to predict the amount of iron accumulation in these organs has been studied extensively and was suggested to be used reliably. However, it may not be practical to screen other organs with MRI related to economical issues and also the prolonged imag
PMID 25740381
PMID 25816754

Development of a capillary zone electrophoresis method for rapid determination of human globin chains in α and β-thalassemia subjects.

Lin L1, Chen DN1, Guo J2, Zhou WJ1, Xu XM3.
Blood cells, molecules & diseases.Blood Cells Mol Dis.2015 Jun;55(1):62-7. doi: 10.1016/j.bcmd.2015.03.003. Epub 2015 Mar 26.
Thalassemia is an inherited autosomal recessive blood disorder characterized by the underproduction of globin chains as a consequence of globin gene defects, resulting in malfunctioning red blood cells and oxygen transport. Analysis of globin chains is an important aspect of thalassemia research. In
PMID 25976469

Japanese Journal

Molecular Mechanism of High Hemoglobin F Production in Southeast Asian-Type Hereditary Persistence of Fetal Hemoglobin

CHANGSRI Khaimuk,AKKARAPATHUMWONG Varaporn,JAMSAI Duangporn,WINICHAGOON Pranee,FUCHAROEN Suthat
International journal of
NAID 10017422015

Molecular characterization of hereditary persistence of fetal hemoglobin in the Karen people of Thailand

TRACHOO O
Hemoglobin
NAID 30017360959

Molecular basis of hereditary persistence of fetal hemoglobin

FORGET BG.
Ann N Y Acad Sci. 850, 38-44, 1998
NAID 30010188677

Related Pictures

★リンクテーブル★

リンク元	「HPFH」
関連記事	「persistence」「fetal」「hereditary」「fetal hemoglobin」「hemoglobin」

「HPFH」

Hereditary persistence of fetal hemoglobin
	Classification and external resources
ICD-10	D56.4
ICD-9	282.7
OMIM	141749

　　[★]

同: hereditary persistence of fetal hemoglobin

「persistence」

　　[★]

n.

頑固、固執、しつこさ、根深さ。永続、持続性、しっかりした忍耐。(刺激が無くなった後の感覚の)残留/残留性。(生)(有害遺伝子の)残存/残存率

this altered immune state allows the persistence of tachyzoites and gives rise to progressive focal destruction that results in organ failure(HIM.1306)