Hereditary hemolytic-uremic syndrome

This code represents a rare, inherited disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. It results from genetic mutations affecting the complement system, leading to uncontrolled complement activation and endothelial damage.

Use this code for patients diagnosed with hereditary hemolytic-uremic syndrome, often presenting with symptoms such as pallor, fatigue, petechiae, and decreased urine output. Documentation should clearly indicate a genetic basis or a family history consistent with the hereditary form of HUS, distinguishing it from acquired forms.

• Differentiate from acquired forms of HUS (e.g., typical HUS caused by Shiga toxin-producing E. coli) which are coded elsewhere.
• Ensure documentation specifies "hereditary" or "genetic" to support this specific code.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, followed by codes for associated manifestations like acute kidney failure.