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Diamond-Blackfan anemia.
Da Costa L, Willig TN, Fixler J, Mohandas N, Tchernia G. Da Costa L, et al. Curr Opin Pediatr. 2001 Feb;13(1):10-5. doi: 10.1097/00008480-200102000-00002. Curr Opin Pediatr. 2001. PMID: 11176237 Review.
Diamond-Blackfan Anemia (DBA) is a rare, congenital hypoplastic anemia often diagnosed early in infancy. A moderate to severe aregenerative anemia is found in association with erythroblastopenia in an otherwise normocellular bone marrow. ...
Diamond-Blackfan Anemia (DBA) is a rare, congenital hypoplastic anemia often diagnosed early in infancy. A moder …
Molecular pathogenesis in Diamond-Blackfan anemia.
Ito E, Konno Y, Toki T, Terui K. Ito E, et al. Int J Hematol. 2010 Oct;92(3):413-8. doi: 10.1007/s12185-010-0693-7. Epub 2010 Sep 30. Int J Hematol. 2010. PMID: 20882441 Review.
Diamond-Blackfan anemia (DBA) is a congenital anemia and a broad spectrum of developmental abnormalities that presents soon after birth. The anemia is due to a failure of erythropoiesis with normal platelet and myeloid lineages. ...
Diamond-Blackfan anemia (DBA) is a congenital anemia and a broad spectrum of developmental abnormalities that pr …
The metabolic basis of inherited neutropenias.
Oyarbide U, Crane GM, Corey SJ. Oyarbide U, et al. Br J Haematol. 2024 Jan;204(1):45-55. doi: 10.1111/bjh.19192. Epub 2023 Dec 4. Br J Haematol. 2024. PMID: 38049194 Review.
Neutrophils are the shortest-lived blood cells, which requires a prodigious degree of proliferation and differentiation to sustain physiologically sufficient numbers and be poised to respond quickly to infectious emergencies. More than 10(7) neutrophils are produced every …
Neutrophils are the shortest-lived blood cells, which requires a prodigious degree of proliferation and differentiation to sustain physiolog …
Diagnosis, treatment, and surveillance of Diamond-Blackfan anaemia syndrome: international consensus statement.
Wlodarski MW, Vlachos A, Farrar JE, Da Costa LM, Kattamis A, Dianzani I, Belendez C, Unal S, Tamary H, Pasauliene R, Pospisilova D, de la Fuente J, Iskander D, Wolfe L, Liu JM, Shimamura A, Albrecht K, Lausen B, Bechensteen AG, Tedgard U, Puzik A, Quarello P, Ramenghi U, Bartels M, Hengartner H, Farah RA, Al Saleh M, Hamidieh AA, Yang W, Ito E, Kook H, Ovsyannikova G, Kager L, Gleizes PE, Dalle JH, Strahm B, Niemeyer CM, Lipton JM, Leblanc TM; international Diamond-Blackfan anaemia syndrome guideline panel. Wlodarski MW, et al. Lancet Haematol. 2024 May;11(5):e368-e382. doi: 10.1016/S2352-3026(24)00063-2. Lancet Haematol. 2024. PMID: 38697731 Review.
Diamond-Blackfan anaemia (DBA), first described over 80 years ago, is a congenital disorder of erythropoiesis with a predilection for birth defects and cancer. ...These changes include lowering the prednisone maintenance dose to maximum 0.3 mg/kg per day, raising th …
Diamond-Blackfan anaemia (DBA), first described over 80 years ago, is a congenital disorder of erythropoiesis with a predilect …
Non-Diamond Blackfan anemia disorders of ribosome function: Shwachman Diamond syndrome and 5q- syndrome.
Burwick N, Shimamura A, Liu JM. Burwick N, et al. Semin Hematol. 2011 Apr;48(2):136-43. doi: 10.1053/j.seminhematol.2011.01.002. Semin Hematol. 2011. PMID: 21435510 Free PMC article. Review.
A number of human disorders, dubbed ribosomopathies, are linked to impaired ribosome biogenesis or function. These include but are not limited to Diamond Blackfan anemia (DBA), Shwachman Diamond syndrome (SDS), and the 5q- myelodysplastic syndrome (MDS). ...
A number of human disorders, dubbed ribosomopathies, are linked to impaired ribosome biogenesis or function. These include but are not limit …
Diamond-Blackfan anemia and malignancy. A case report and a review of the literature.
van Dijken PJ, Verwijs W. van Dijken PJ, et al. Cancer. 1995 Aug 1;76(3):517-20. doi: 10.1002/1097-0142(19950801)76:3<517::aid-cncr2820760324>3.0.co;2-8. Cancer. 1995. PMID: 8625135 Review.
BACKGROUND: Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome involving the erythropoietic lineage. ...Review of the literature revealed 11 other cases of malignancy (10 hematologic) in DBA. RESULTS: This patient, together with …
BACKGROUND: Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome involving the erythropoietic lin …
Diamond-blackfan anemia and cyclosporine therapy revisited.
Alessandri AJ, Rogers PC, Wadsworth LD, Davis JH. Alessandri AJ, et al. J Pediatr Hematol Oncol. 2000 Mar-Apr;22(2):176-9. doi: 10.1097/00043426-200003000-00020. J Pediatr Hematol Oncol. 2000. PMID: 10779036 Review.
A girl with Diamond-Blackfan anemia diagnosed in infancy started cyclosporine A (CSA) therapy at 9 years and 8 months of age after experiencing unacceptable side effects while receiving prednisone. ...She is currently maintained on CSA 100 mg twice daily with …
A girl with Diamond-Blackfan anemia diagnosed in infancy started cyclosporine A (CSA) therapy at 9 years and 8 months o …
[New insights into inherited bone marrow failure syndrome].
Ito E, Toki T, Kamio T, Terui K. Ito E, et al. Rinsho Ketsueki. 2021;62(10):1455-1464. doi: 10.11406/rinketsu.62.1455. Rinsho Ketsueki. 2021. PMID: 34732617 Review. Japanese.
Recently, de novo mutations activating TP53 were detected in patients with BMFS, mimicking Diamond-Blackfan anemia (DBA), using whole exome sequencing, and these patients were recognized as having a novel disorder. ...Furthermore, a novel IBMFS, aldehyde degr …
Recently, de novo mutations activating TP53 were detected in patients with BMFS, mimicking Diamond-Blackfan anemia (DBA …
Diamond-Blackfan Anaemia: an overview.
Dianzani I, Garelli E, Ramenghi U. Dianzani I, et al. Paediatr Drugs. 2000 Sep-Oct;2(5):345-55. doi: 10.2165/00128072-200002050-00002. Paediatr Drugs. 2000. PMID: 11022796 Review.
Diamond Blackfan Anaemia (DBA) is a congenital disease characterised by defective erythroid progenitor maturation. ...However, the link between these malformations and defective erythropoiesis is unclear and a defect in a molecule acting on both early embryonic deve …
Diamond Blackfan Anaemia (DBA) is a congenital disease characterised by defective erythroid progenitor maturation. ...However, …
Recent insights into the pathogenesis of Diamond-Blackfan anaemia.
Gazda HT, Sieff CA. Gazda HT, et al. Br J Haematol. 2006 Oct;135(2):149-57. doi: 10.1111/j.1365-2141.2006.06268.x. Epub 2006 Aug 31. Br J Haematol. 2006. PMID: 16942586 Free article. Review.
Diamond-Blackfan anaemia (DBA) is a congenital anaemia and broad developmental disease that develops soon after birth. ...Normal erythropoiesis after transplantation shows that the defect is intrinsic to an erythroid precursor. DBA is inherited in about 10-20 …
Diamond-Blackfan anaemia (DBA) is a congenital anaemia and broad developmental disease that develops soon after birth. ...Norm …
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