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AVED is caused by an autosomal recessive mutation in the TTPA gene that encodes -TTP

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The input variables considered, obtained from a systematic review of the literature and data of the 1997 Nationwide Inpatient Sample, were: the estimated mortality rate after elective laparoscopic (1.4%, range 0%-5.2%) and emergency (5.4%) PEH repair, the annual probability of developing symptoms progression (13.8% range 8.1%-21.7%), the annual probability of acute presentation requiring emergency surgery of untreated patients (1.1% range 0.7%-1.9%), and the annual probability of HH recurrence after surgical repair (1.9% range 0.3%-5.4%)