[ @entity2916 : typical complications of a XXXX and therapeutic options].

@entity1 @entity1032 @entity8 @entity18871 @entity2916 @entity358 @entity74 @entity302 @entity4607
BACKGROUND: @entity2916 is an @entity74 (incidence and prevalence 1:10,000) which is characterized by @entity4607 in various organs. @entity1 AND METHODS: We demonstrate three @entity1 with different and partly atypical manifestations: All had @entity18871 . No @entity1 was @entity1032 and @entity302 was observed only intermittently. In all cases periventricular calcifications were noted and @entity8 were found. Two @entity1 requested dialysis after nephrectomy. One @entity1 suffered from severe complications of the rare @entity358 . RESULTS: Therapy of this multiorgan disease is only symptomatic. Operations should be considered as second line treatment. In one case, successful renal transplantation is described. CONCLUSIONS: Genetic counselling is warranted in this disease with high penetrance, particularly the broad spectrum of manifestations should be taken into account.
