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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Note:  The following article synopsis was NOT provided by the AHA. It was created by Find-A-Code/innoviHealth.

Article Overview

This article covers a diagnosis-related coding update for a rare inherited disorder affecting the liver, along with a plain-language overview of the condition’s typical effects on other body systems and how it may be recognized clinically. It is relevant to coders, billing and coding professionals, and healthcare staff who need to understand the scope of the coding change and the associated medical context without relying on the full premium text.

Why This Topic Matters

It helps users identify whether the article is relevant to liver-related congenital conditions, pediatric presentations, and diagnosis coding updates tied to this disorder.

What You Will Learn

  • The general clinical context of a rare inherited disorder affecting the liver and other organ systems.
  • How the article frames a coding update associated with congenital liver malformations.
  • The broad signs and diagnostic context discussed for this condition.
  • The body systems commonly described as potentially involved.

Who Should Read This

  • Medical coders
  • Coding auditors
  • Billing staff
  • Clinical documentation staff
  • Healthcare administrators

Codes Discussed

  • ICD-10-CM: Q44.7
  • ICD-10-CM: Q44.70
  • ICD-10-CM: Q44.71
  • ICD-10-CM: Q44.79

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