@entity631 : early diagnosis associated with XXXX responsiveness.

@entity1 @entity644 @entity8 @entity101 @entity229 @entity134 @entity1011 @entity1985 @entity811 @entity631 @entity454
@entity1 with @entity631 usually present with @entity1985 , microscopic @entity811 , @entity8 , and @entity101 ; 50% develop @entity454 within a few years. There is no known effective therapy for @entity631 . We describe three @entity1 with biopsy-proven @entity631 , in whom pathognomonic fibrillar deposits of 20-nm diameter were seen by electron microscopy. All @entity1 had @entity1985 and normal renal function at the time of diagnosis. They were treated initially with @entity229 , 1 mg/kg body weight, then tapered individually according to the clinical course. Additional therapy consisted of an angiotensin-converting enzyme inhibitor and diuretics. @entity644 disappeared in two of three @entity1 after 9 and 12 months of therapy. In one @entity1 , @entity644 was reduced from 7.49 mg/mg @entity1011 to 0.63 mg/mg @entity1011 after 6 months of treatment. The kidney function remained normal in all three cases. Two @entity1 are now free of @entity134 therapy for 9 and 6 months. They show no signs of recurrence of @entity8 and have normal renal function without significant @entity644 . @entity134 therapy in @entity1 with biopsy-proven @entity631 , starting with @entity229 , 1 mg/kg body weight, and tapered individually according to the clinical course, is a promising strategy. Early start of treatment in @entity1 with preserved renal function seems to be crucial for a favorable outcome.
