Primary hyperoxaluria, unspecified

This code identifies a rare, inherited metabolic disorder characterized by the overproduction of oxalate, leading to its accumulation in the kidneys and other organs. This accumulation primarily results in recurrent kidney stones, nephrocalcinosis, and progressive kidney failure. The "unspecified" nature indicates that the specific genetic subtype of primary hyperoxaluria has not been determined.

Use this code when the patient's medical record clearly documents a diagnosis of primary hyperoxaluria, but the physician has not specified the exact type (e.g., Type 1, Type 2, Type 3). Documentation should include evidence of elevated oxalate levels, kidney stone formation, or other clinical manifestations consistent with the condition, without further genetic or enzymatic testing results.

• Do not use this code if the specific type of primary hyperoxaluria (e.g., Type 1, Type 2) is documented; instead, use the more specific codes.
• Always sequence this code as a primary diagnosis when it is the reason for the encounter.
• Ensure documentation supports the "unspecified" nature; if further investigation is planned, consider querying the provider for a more specific diagnosis once available.