Mortality in XXXX . A changing pattern of the causes of @entity204 .

@entity1 @entity66 @entity55 @entity177 @entity364 @entity358 @entity299 @entity370 @entity204
Forty-one @entity204 occurred among 1,090 @entity1 with the clinical diagnosis of @entity370 between 1960 and 1977. Thirteen @entity1 died of causes unrelated to @entity370 , whereas @entity204 was related to @entity370 in 28 @entity1 (68%). The most prevalent lesion leading to @entity204 was advanced @entity358 (22/28 @entity1 or 78%) with various late complications: @entity177 , gram-negative @entity55 , @entity364 . The average clinical course among these 22 @entity1 was 10 years from the onset of the disease. The average age at @entity204 was 39 years. @entity1 who died of central nervous system and @entity299 were younger, and their clinical course was shorter. Subclinical @entity370 does not seem to affect life span. Autopsy in 25 @entity1 showed @entity66 in all, including asymptomatic @entity1 and those presenting with clinical manifestations limited to the CNS and the heart. The discrepancy implies difficulty in making the diagnosis, and suggests the need of intensive diagnostic work-up. Improved medical therapy, particularly the control of infections and management of @entity177 , has resulted in a protracted clinical course and a changing pattern of the causes of @entity204 in @entity370 .
