| 释义 |
von Gierke, n. Path.|fɒn ˈgɪəkə|
The name of Edgar O. K. von Gierke (1877–1945), German pathologist, used in the possessive (usu. as von Gierke's disease) and occas. attrib. to designate a type of glycogen storage disease (described by him in Beiträge zur path. Anat. LXXXII (1929) 497), caused by a deficiency of glucose-6-phosphatase and characterized by an enlarged liver, obesity, and stunted growth.
1933Brit. Med. Jrnl. 11 Mar. 405/1 During life the presence of von Gierke's glycogenic-accumulation disease is not likely to be absolutely established except by the microscopical examination of an excised piece of the liver or material obtained by liver puncture. 1933Proc. R. Soc. Med. XXVI. 1006 (heading) Hepatomegaly of unknown origin (probably von Gierke's disease). 1936Brit. Med. Jrnl. 22 Feb. 361/2 Unshelm..showed that if normal glycogen-free liver was minced up with von Gierke liver and allowed to incubate, the glycogen was broken down. 1952Metabolism I. 293 The present report is based on studies carried out on a 20-month-old girl with glycogen storage disease of the classical von Gierke type. 1961R. D. Baker Essent. Path. xiv. 344 In some few cases glycogen infiltration of the myocardium is present and the condition is a part of glycogen storage or Von Gierke's disease. 1982R. A. Lawson et al. Pathologic Mechanisms & Human Dis. ii. 22/1 The glycogen stores are metabolically unavailable, and patients with von Gierke's disease suffer from hypoglycemia. |