原发性分流性高胆红素血症：先天性红细胞生成异常性贫血的一种变异型。
Primary shunt hyperbilirubinaemia: a variant of the congenital dyserythropoietic anaemias.
作者信息
Bird A R, Knottenbelt E, Jacobs P, Maigrot J
机构信息
University of Cape Town Leukaemia Centre, South Africa.
出版信息
Postgrad Med J. 1991 Apr;67(786):396-8. doi: 10.1136/pgmj.67.786.396.
Abstract
A 19 year old Mauritian male presented with episodic nausea, abdominal discomfort and jaundice. Unconjugated hyperbilirubinaemia and erythroid hyperplasia without dyserythropoiesis led to the diagnosis of primary shunt hyperbilirubinaemia. The similarity between congenital dyserythropoietic anaemia and this entity suggests that patients with these lesions can be considered within a single spectrum of disorders, characterized as congenital ineffective erythropoiesis.
摘要
一名19岁的毛里求斯男性出现发作性恶心、腹部不适和黄疸。非结合性高胆红素血症和红细胞增生但无红细胞生成异常导致原发性分流性高胆红素血症的诊断。先天性红细胞生成异常性贫血与该病症之间的相似性表明，患有这些病变的患者可被视为单一疾病谱中的一部分，其特征为先天性无效红细胞生成。
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