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AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2023 Issue 4; New/Revised ICD-10-CM Codes

Alagille Syndrome

Code Q44.7, Other congenital malformations of liver, has been expanded to create three new codes to identify other congenital malformation of liver, unspecified (Q44.70), Alagille syndrome (Q44.71), and other congenital malformations of liver (Q44.79). Alagille syndrome is a rare inherited disorder in which there are too few bile ducts to drain bile from the liver. This causes the bile to build up in the liver which can ultimately cause the liver to fail. Although Alagille syndrome typically affects the liver, it may also affect other organs of the body including the cardiovascular system, skeletal...

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