XXXX : clinical aspects and outcome in a long-term longitudinal study.

@entity1 @entity3930 @entity28772 @entity944 @entity301 @entity16 @entity4148 @entity299 @entity583 @entity70741 @entity1068 @entity1386
BACKGROUND: @entity28772 ( @entity28772 ) is a rare disease characterized by the growth of uncapsulated masses of adipose tissue. @entity28772 is associated with high @entity1068 intake and complicated by @entity4148 and by the infiltration of the adipose tissue at the mediastinal level. To date, the disease is considered as slowly progressive, but long-term longitudinal data are still lacking. In this study, a long-term follow-up of a large series of @entity28772 @entity1 is presented. METHODS: We studied 31 @entity1 with @entity28772 (30 @entity3930 ) first evaluated at our institution from 1973 to 1992. All @entity1 were followed until 1998-1999 or until death, with a mean follow-up of 14.5+/-5.0 y (range 4-26 y). Both at baseline and during follow-up, the location and the size of the subcutaneous @entity1386 , the presence and the extension of deeply localized lipomatous tissue, and the presence and the severity of both @entity4148 were evaluated. RESULTS: Eight @entity28772 @entity1 died during follow-up (25.8% of @entity1 ). A @entity944 was proved to be the cause of death in three @entity1 . All these three @entity1 had severe @entity4148 and none had @entity301 , acute @entity583 or other @entity299 . No signs or symptoms of @entity301 were present in the whole series. In addition to this high fatality rate, a substantial morbidity related to the occupation of the mediastinal space by the lipomatus tissue and to somatic @entity16 was also observed. CONCLUSIONS: @entity28772 is associated with a significant morbidity and mortality. Therefore, the definition of ' @entity70741 ', still adopted by several authors, cannot be justified.
