Gestational alloimmune liver disease

This diagnosis code identifies a severe and rare form of neonatal liver failure caused by maternal antibodies attacking the fetal liver. It results from an alloimmune response where the mother's immune system mistakenly targets and damages the developing liver cells of the fetus. The condition often presents with acute liver failure in the newborn, characterized by coagulopathy, hypoglycemia, and elevated liver enzymes.

Assign this code for newborns diagnosed with gestational alloimmune liver disease, typically confirmed through liver biopsy showing iron deposition and complement staining, or by identifying maternal anti-fetal hepatocyte antibodies. Documentation should clearly state the diagnosis of gestational alloimmune liver disease, distinguishing it from other causes of neonatal liver dysfunction. This code is appropriate when the condition is the primary reason for the newborn's liver failure.

• Ensure documentation explicitly states "gestational alloimmune liver disease" to support this specific code.
• Do not code with hemochromatosis (E83.11-) as per the Excludes1 note, as these are distinct conditions.
• Consider sequencing this code as the principal diagnosis if it is the primary reason for admission or encounter for the newborn.