WOLMANS DISEASE - RARE LIPIDOSIS WITH ADRENAL CALCIFICATION

被引：72

作者：

MARSHALL, WC

OCKENDEN, BG

FOSBROOKE, AS

CUMINGS, JN

机构：

[1] Hospital for Sick Children, Departments of Morbid Anatomy and Child Health, National Hospital, London

[2] Institute of Child Health, London

[3] Department of Chemical Pathology, Institute of Neurology, National Hospital, London

[4] Department of Pathology, North Staffordshire Royal Infirmary, Stoke-on-Trent, Staffordshire

来源：

ARCHIVES OF DISEASE IN CHILDHOOD | 1969年 / 44卷 / 235期

关键词：

D O I：

10.1136/adc.44.235.331

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Two cases of Wolman's disease occurring in Great Britain are described. This familial lipidosis is characterized by the storage of esterified cholesterol and triglyceride in liver, spleen, reticuloendothelial system, intestine, and other organs; in particular, the adrenals are extensively and symmetrically involved and often radiologically calcified. Diarrhoea, wasting, and hepato-splenomegaly in early infancy, sometimes accompanied by radiological adrenal calcification, should suggest the diagnosis, which may be confirmed by bone-marrow and liver biopsy.

引用

页码：331 / +

页数：1

共 32 条

[1] GENERALIZED XANTHOMATOSIS WITH CALCIFIED ADRENALS [J].