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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease fanconi anemia
Comorbidity C0030312|bone marrow failure
Sentences 7
PubMedID- 22683204 bone marrow failure in fanconi anemia is triggered by an exacerbated p53/p21 dna damage response that impairs hematopoietic stem and progenitor cells.
PubMedID- 24019220 Oxandrolone for the treatment of bone marrow failure in fanconi anemia.
PubMedID- 24960628 Hematopoietic cell transplantation (hct) can cure bone marrow failure in patients with fanconi anemia (fa), and it is generally accepted that these patients should receive low-intensity conditioning because of the underlying dna repair defect in their cells.
PubMedID- 23547077 Purpose: allogeneic hematopoietic cell transplantation (hct) can cure bone marrow failure in patients with fanconi anemia (fa).
PubMedID- 25534205 bone marrow failure in fanconi anemia (fa) has been linked in part to overproduction of inflammatory cytokines, to which fa stem and progenitor cells are hypersensitive.
PubMedID- 22178060 Treatment of the bone marrow failure in fanconi anemia patients with danazol.
PubMedID- 23275558 For example, fanconi anemia (fa) patients with an advanced bone marrow failure have almost no hematopoietic stem cells (hscs) in their hematopoietic tissues, precluding a classic hsc-based ex vivo gene therapy.

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