The cardiovascular system in gargoylism.
نویسنده
چکیده
Of interest to cardiologists is a large group of rare systemic diseases in which involvement of the cardiovascular system may produce clinical evidence of cardiovascular disease, or may lead to cardiac failure (Weiss, 1939). An attempt has been made to outline the basic clinical, laboratory and pathological features of a few of these processes (Saphir, 1942;* Weiss et al., 1943; Lindsay, 1946). The accompanying cardiac manifestations may then be correctly interpreted as a part of a generalized disease, and thus be differentiated from the more common forms of cardiac disease. The importance of an accurate etiologic classification of these rare diseases has been emphasized previously (Weiss et al., 1943) since factors of specific therapy must be considered. Gargoylism, a rare disease of infancy and childhood, has been shown to belong to this group of obscure, miscellaneous systemic processes. In recent pathological studies of gargoylism, it was found that the heart and vascular apparatus are often significantly involved in this diffuse disease (Lindsay et al., 1948), and furthermore that a high percentage of children with gargoylism have clinical evidence of cardiovascular disease or die from cardiac failure. It is the main purpose of this report (1) to review the clinical manifestations of gargoylism, stressing those ofcardiovascular origin, (2) to summarize the available pathological data, particularly in regard to the heart and vascular system and (3) to record an additional case ofgargoylism occurring in infancy, in which involvement of the heart by the storage process was responsible for acute fatal myocardial failure.
منابع مشابه
Gargoylism with cardiovascular involvement in two brothers.
The term " Gargoyle" was introduced by Ellis, Sheldon, and Capon in 1936. The condition was first recognized by Thomson in 1900, but his original cases were not reported until later (Henderson and Ellis, 1940). Although about 200 cases have been reported, there are few detailed studies of the cardiovascular system. Necropsy material is rare, and has been reviewed by Strauss (1948) and Lindsay (...
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عنوان ژورنال:
- British heart journal
دوره 12 1 شماره
صفحات -
تاریخ انتشار 1950