Other specified primary hyperoxaluria

This code signifies a rare, inherited metabolic disorder characterized by the overproduction of oxalate, leading to its accumulation in the kidneys and other organs. This specific code is used when the primary hyperoxaluria is not type 1, 2, or 3, but rather another identified genetic defect causing the condition. The excess oxalate can form kidney stones and cause progressive kidney damage.

Use this code for patients diagnosed with a genetically confirmed primary hyperoxaluria that does not fall into the categories of PH1, PH2, or PH3. Documentation should clearly specify the genetic mutation or the specific type of primary hyperoxaluria identified, ruling out the more common types. This code is appropriate when the physician's diagnosis explicitly states "other specified primary hyperoxaluria."

• Ensure documentation specifies the type of primary hyperoxaluria to differentiate from unspecified or other known types.
• Do not use this code if the primary hyperoxaluria is identified as Type 1 (E72.531), Type 2 (E72.532), or Type 3 (E72.533).
• Always sequence this code as a primary diagnosis when it is the reason for the encounter, followed by codes for any associated manifestations like nephrolithiasis (N20.0) or chronic kidney disease (N18.-).