Figure 2.

Outcomes of the Swedish Newborn Screening Program (7,11,12). Figure 2 demonstrates the condensed outcomes of 127 ZZ neonates that were identified after an unbiased newborn screen of over 200,000 Swedish neonates. Of those identified, the majority of the ZZ infants were healthy in the neonatal period and without the unbiased newborn screen, this group would have likely remained undiagnosed. Moreover, only 16% of neonates exhibited neonatal cholestasis. Of those, approximately 3% developed severe disease including severe fibrosis, liver failure, and death. The majority of the cohort at 18 years of age, including most of the neonatal cholestasis group, demonstrated unremarkable laboratory exams and pulmonary function tests. Follow up since that time has been published but has not been as stringent or as complete as the original report. ALT, alanine transaminase.