Lipoprotein deficiency

This code represents a group of rare genetic disorders characterized by abnormally low levels of lipoproteins in the blood. These deficiencies can impact the transport of lipids, leading to various systemic manifestations, including neurological, ophthalmological, and gastrointestinal issues.

Assign this code when documentation indicates a diagnosis of a specific lipoprotein deficiency, such as abetalipoproteinemia, hypobetalipoproteinemia, or Tangier disease. Supporting documentation should include laboratory findings confirming low lipoprotein levels and a definitive diagnosis from a physician.

• Do not confuse this with hyperlipidemia (E78.0-E78.5), which represents elevated lipid levels.
• Always review the medical record for specific types of lipoprotein deficiencies to ensure the most precise coding if a more specific code is available.
• Consider sequencing this code as a primary diagnosis when it is the reason for the encounter or as a secondary diagnosis when it is a comorbidity affecting patient care.