[Imerslund-Najman-Grasbeck贫血。关于一个案例]。

Pediatrie Pub Date : 1993-01-01
C Ben Meriem, H Pousse, M Bourgeay-Causse, F Amri, A Besbes, A Belkhir, M T Sfar, T Jrad
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引用次数: 0

摘要

Imerslund-Najman-Grasbeck病是一种罕见的遗传性巨幼细胞性贫血,继发于回肠肠细胞对维生素B12的选择性吸收不良。作者报告了一名4岁的突尼斯女孩,她因脾肿大和重度贫血而表现为内脏婴儿利什曼病。Imerslund病的诊断是基于骨髓中典型的巨幼细胞、永久性蛋白尿和肠外注射B12的良好结果的关联。无叶酸缺乏,无抗内因子抗体,无内因子缺乏。如果维持静脉注射维生素B12,这种疾病的结局总是有利的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Imerslund-Najman-Grasbeck anemia. Apropos of a case].

Imerslund-Najman-Grasbeck disease is a rare inherited megaloblastic anaemia secondary to a selective malabsorption of vitamin B12 by ileal enterocytes. The authors report on a 4 year-old tunisian girl who presented as visceral infantile leishmaniasis because of huge splenomegaly and major anaemia. The diagnosis of Imerslund disease was performed on the basis of the association of typical megaloblastic cells in the marrow, permanent proteinuria and favourable outcome under parenteral B12 administration. In addition, ther were no folate deficiency, no anti-intrinsic factor antibodies and no intrinsic factor deficiency. The outcome of the disease is always favourable if parenteral administration of vitamin B12 maintained.

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