Oral megadose methylprednisolone for Diamond-Blackfan anemia [letter; comment]
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چکیده
منابع مشابه
Oral megadose methylprednisolone for Diamond-Blackfan anemia.
I have read the report of Olivieri et al’ in the May 1, 1994 issue of BLOOD. Although the investigators stated that “in standard practice, treatment options are limited to two: steroid therapy or RBC transfusions,” they ignore our very good results obtained with megadose methylprednisolone (MDMP; initial dose 30 to 100 mgkg/d for 3 days followed progressive halving of the dose every 4 to 7 days...
متن کاملDiamond Blackfan anemia.
Mutations affecting genes encoding ribosomal proteins cause Diamond Blackfan anemia (DBA), a rare congenital syndrome associated with physical anomalies, short stature, red cell aplasia, and an increased risk of malignancy. p53 activation has been identified as a key component in the pathophysiology of DBA after cellular and molecular studies of knockdown cellular and animal models of DBA and o...
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Diamond-Blackfan anemia (DBA) is a rare, congenital, hypoplastic anemia that usually presents in early infancy. Congenital anomalies, particularly of the head and upper limbs, are present in about a quarter of reported patients. The disease is characterized by a moderate-to-severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia,...
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Diamond-Blackfan Anemia (DBA) is a red cell aplasia characterized with physical abnormalities. The incidence of the disease is reported to be five to seven (5-7) cases per million births in Europe and 4-5 per million live births in the UK and Netherlands with equal sex ratio. It was first recognized in 1938 but an exact pathophysiology of the disease has not been described yet. These abnormalit...
متن کاملIncidence of neoplasia in Diamond Blackfan anemia: a report from the Diamond Blackfan Anemia Registry.
Diamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome characterized by red cell aplasia and congenital anomalies. A predisposition to cancer has been suggested but not quantified by case reports. The DBA Registry of North America (DBAR) is the largest established DBA patient cohort, with prospective follow-up since 1991. This report presents the first quantitative assessmen...
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ژورنال
عنوان ژورنال: Blood
سال: 1994
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v84.9.3245.3245