نتایج جستجو برای: fanconi anemia patients
تعداد نتایج: 2119640 فیلتر نتایج به سال:
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 ...
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:...
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:...
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...
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:...
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...
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...
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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