Common variable XXXX : an uncommon disease with high mortality.

@entity1 @entity12089 @entity704 @entity6078 @entity465 @entity1729 @entity7893 @entity204
OBJECTIVE: The objective of this report is to present a case of @entity6078 ( @entity6078 ), to review the literature on the subject, and to perform a mortality analysis on a case from the review. BACKGROUND: Subjects in the original study, having suffered multiple @entity1729 in the recent past, were referred to and followed at the Mt. Sinai Medical Center, New York, N Y, for diagnosis and management of @entity465 during the years 1973-1998. Demonstrating that two of the major immunoglobulin subgroups were more than two standard deviations below the mean for the @entity1 's age made the diagnosis of @entity6078 . In addition, poor antibody response to polysaccharide and protein antigens was demonstrated as a diagnostic criterion. Certain groups of @entity1 were excluded from the cohort. They were those with @entity12089 and any cases proven to be secondary to another pathologic process. @entity1 with @entity7893 and @entity704 were also excluded. METHODS: Standard mortality methodology was used to obtain observed mortality, expected mortality, mortality ratios, excess @entity204 rates, and confidence intervals for the mortality ratios. RESULTS: Ages at entry ranged from 3 to 79 with mean age at diagnosis of 29 for males and 33 for females. Follow-up was from 1 year to 25 years with a mean follow-up of 7 years. Survival in the studied cohort was 64% for males and 67% for females. These data were taken from the Kaplan-Meier curve spanning 20 tears of follow-up and were used throughout the remainder of the calculations. The corresponding mortality ratios were 526% and 639%, respectively. CONCLUSIONS: Markedly increased mortality ratios and excess @entity204 rates were found. These values (MR) were significant at the 95% confidence level, thus underscoring the point that in spite of young age and high functionality, @entity1 with this syndrome have markedly elevated mortality.
