| What Is A1AD? - Page 3 |
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Liver DiseaseFor some people the accumulation of misfolded AAT protein causes cirrhosis of the liver which can be life threatening. It is not understood why only some PiZZ individuals develop severe liver disease but it is suspected that either another gene is involved or perhaps some other liver injury like an unknown hepatitis virus. This form of liver disease is usually seen in older people with A1AD. Around 20 babies a year are born showing symptoms of neo-natal liver disease caused by A1AD. Most of these will improve and many show only mild liver abnormalities by the time the reach puberty. Perhaps two a year will require a liver transplant whilst still an infant. This should not be confused with the "jaundice" often seen in neo-nates as this is not a problem and soon fades. These few babies are very sick indeed and will die if not treated. In the next section we shall look at how this disorder is inherited, who is at risk, and how people can be tested. |
