Occurrence of XXXX in the Acute @entity99 .

@entity1 @entity0 @entity706 @entity5 @entity3 @entity209 @entity157 @entity99
UNASSIGNED: The porphyrias are a group of inherited @entity209 resulting from enzymatic deficiencies of specific haem biosynthetic enzymes. They can be classified as primarily acute and non-acute types. Clinically, the acute @entity99 (AHPs) are characterised by acute neurovisceral attacks. @entity1 with AHP may be at increased risk for development of @entity157 ( @entity157 ). However, systematic studies on the occurrence of other @entity5 in @entity1 with the AHPs have not been performed to date. Here, we studied the development of @entity157 and distinct @entity5 in @entity1 with the AHPs registered in a single European porphyria specialist centre. A questionnaire was designed and sent to all individuals (n = 122) diagnosed between 1970 and 2012 of whom a valid address was available (n = 82), requesting information on their personal and family history of @entity5 . Statistical analysis was performed to calculate incidence, prevalence and relative risk of @entity157 . To calculate confidence intervals, a Poisson distribution was assumed. Forty-nine @entity1 (59.8%) returned a completed questionnaire. Overall, @entity157 was diagnosed in one female (2.1%), and the remaining @entity1 reported on six distinct @entity5 . We were able to confirm that @entity157 is an important complication in AHP. The @entity1 in our cohort had an approximately 35-fold increased risk of developing @entity157 , similar to observations in other European countries. In addition, we detected @entity0 as well as @entity706 and a liver @entity3 in @entity1 with AHP. However, considering the small number of @entity5 and @entity1 studied here, the data should be interpreted with caution, and further studies on @entity5 occurrence in AHP @entity1 will require a multicentre setting.
