نتایج جستجو برای: fanconi anemia patients

تعداد نتایج: 2119640  

ژورنال: مجله دندانپزشکی 1994
تهیدست اکراد, زهرا ,

Fanconi syndrome was first described in 1927 by G.Fanconi. Fanconi syndrome with congenital aplastic anemia is a hereditary disorder in which peripheral blood Pancytopenia and bone marrow hypoplasiais often associated with multiple somatic congenital malformations such as microcephaly, skeletal anomalies (absence or hypoplasia of radial or thumb or both of them), brown hyperpigmentation of the ...

Journal: :Blood 1999
M Cattaneo R Lombardi A Lecchi M L Zighetti

effects in normal human, Fanconi’s anaemia and xeroderma pigmentosum cells. Br J Cancer 67:1285, 1993 3. Hang B, Yeung AT, Lambert MW: A damage-recognition protein which binds to DNA containing interstrand cross-links is absent or defective in Fanconi anemia, complementation group A cells. Nuclic Acids Res 21:4187, 1993 4. Yamashita T, Kupfer G, Naf D, Suliman A, Joenje H, Asano S, D’Andrea AD:...

1999

effects in normal human, Fanconi’s anaemia and xeroderma pigmentosum cells. Br J Cancer 67:1285, 1993 3. Hang B, Yeung AT, Lambert MW: A damage-recognition protein which binds to DNA containing interstrand cross-links is absent or defective in Fanconi anemia, complementation group A cells. Nuclic Acids Res 21:4187, 1993 4. Yamashita T, Kupfer G, Naf D, Suliman A, Joenje H, Asano S, D’Andrea AD:...

2015
Yihang Shen Yuan-Hao Lee Jayabal Panneerselvam Jun Zhang Lenora W. M. Loo Peiwen Fei

An extremely high cancer incidence and the hypersensitivity to DNA crosslinking agents associated with Fanconi Anemia (FA) have marked it to be a unique genetic model system to study human cancer etiology and treatment, which has emerged an intense area of investigation in cancer research. However, there is limited information about the relationship between the mutated FA pathway and the cancer...

1999
Domenico Girelli

effects in normal human, Fanconi’s anaemia and xeroderma pigmentosum cells. Br J Cancer 67:1285, 1993 3. Hang B, Yeung AT, Lambert MW: A damage-recognition protein which binds to DNA containing interstrand cross-links is absent or defective in Fanconi anemia, complementation group A cells. Nuclic Acids Res 21:4187, 1993 4. Yamashita T, Kupfer G, Naf D, Suliman A, Joenje H, Asano S, D’Andrea AD:...

Journal: :Blood 2003
Xiaxin Li P Artur Plett Yanzhu Yang Ping Hong Brian Freie Edward F Srour Christie M Orschell D Wade Clapp Laura S Haneline

The pathogenesis of bone marrow failure in Fanconi anemia is poorly understood. Suggested mechanisms include enhanced apoptosis secondary to DNA damage and altered inhibitory cytokine signaling. Recent data determined that disrupted cell cycle control of hematopoietic stem and/or progenitor cells disrupts normal hematopoiesis with increased hematopoietic stem cell cycling resulting in diminishe...

Journal: :Seminars in hematology 1984
E Gluckman R Berger J Dutreix

Fanconi anemia is a genetic disorder associated with diverse congenital abnormalities, progressive bone marrow failure, and increased risk of leukemia and other cancers. Affected persons often die before 30 years of age. Bone marrow transplantation is an effective treatment, but there are few data regarding factors associated with transplant outcome. We analyzed outcomes of HLA-identical siblin...

Journal: :Archives of otolaryngology--head & neck surgery 2003
David I Kutler Arleen D Auerbach Jaya Satagopan Philip F Giampietro Sat Dev Batish Andrew G Huvos Andy Goberdhan Jatin P Shah Bhuvanesh Singh

BACKGROUND Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by a high degree of genomic instability and predisposition to cancer development. Recent evidence suggests that the incidence of head and neck squamous cell carcinoma (HNSCC) may be increased in patients with FA. OBJECTIVE To determine the cumulative incidence, tumor distribution, and outcome of HNSCC in patie...

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