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劳-穆-比三氏综合征中的先天性肾脏异常。

Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.

作者信息

Bluett N H, Chantler C, Singer J D, Saxton H M

出版信息

Arch Dis Child. 1977 Dec;52(12):968-70. doi: 10.1136/adc.52.12.968.

Abstract

Two children with Laurence-Moon-Biedl syndrome had radiographic evidence of cysts in the renal medulla, and one had impaired renal function and infection. The frequency of cystic disease in this syndrome implies that intravenous urography should be carried out on all patients with this syndrome. Children with renal abnormalities should be followed to avoid further damage from urinary infection, or from dehydration in those with a concentrating defect. Regular renal function tests should be carried out on other children. and full investigation should follow if abnormalities are found in order that the natural history of the often fatal renal lesion can be clarified.

摘要

两名患有 Laurence-Moon-Biedl 综合征的儿童有肾髓质囊肿的影像学证据,其中一名肾功能受损并伴有感染。该综合征中囊性疾病的发生率表明,应对所有患有该综合征的患者进行静脉尿路造影。对有肾脏异常的儿童应进行随访,以避免因尿路感染或浓缩功能缺陷者的脱水而造成进一步损害。应对其他儿童定期进行肾功能检查。如果发现异常,则应进行全面检查,以便阐明这种往往致命的肾脏病变的自然病程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1564/1545009/73f242074fa0/archdisch00810-0069-a.jpg

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