منابع مشابه
The pathology of honeycomb lung.
A form of cystic lung, characterized by numerous, widespread and usually small cavities, is a wellrecognized though apparently uncommon condition, which, according to earlier accounts, occurred especially in children (Sharkey, 1894; Tooth, 1897; Fletcher, 1901; Bernstein, 1905). Such lungs were described by Fowler and Godlee (1898) as having a worm-eaten or honeycomb appearance. Sharkey and Fle...
متن کاملHoneycomb lung: history and current concepts.
OBJECTIVE Originally used to describe the macroscopic appearance of various pathologic processes with multiple cysts, honeycomb lung is now the term used to describe endstage pulmonary fibrosis. The purpose of this article is to discuss the history and imaging of honeycomb lung. CONCLUSION Honeycomb lung is considered one of the important CT findings in usual interstitial pneumonia. An additi...
متن کاملSpinal xanthomatosis: a variant of cerebrotendinous xanthomatosis.
We describe seven Dutch patients from six families with a slowly progressive, mainly spinal cord syndrome that remained for many years the sole expression of cerebrotendinous xanthomatosis (CTX). MRI demonstrated white matter abnormalities in the lateral and dorsal columns of the spinal cord. Post-mortem examination of one of the patients showed extensive myelin loss in these columns. An array ...
متن کاملCerebrotendinous xanthomatosis
Cerebrotendinous Xanthomatosis (CTX; OMIM #213700) is a rare lipid storage metabolic disease with autosomal recessive inheritance. It is characterized by deficiency of mitochondrial sterol 27-hydroxylase (CYP27) which is a key enzyme in bile acid biosynthesis. The enzyme converts cholesterol into bile acids hence deficiency results in abnormal deposition of cholesterol and cholestenol in multip...
متن کاملCerebrotendinous xanthomatosis.
Cerebrotendinous xanthomatosis is a rare genetic metabolic disorder of cholesterol and bile acid metabolism that results in systemic and neurologic abnormalities. Typically, the disease begins in infancy with chronic diarrhoea. Cataracts become evident in childhood or adolescence, and xanthomata develop in the second and third decades of life. Significant neurologic impairment also occurs; this...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 1954
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.29.144.127