Disturbances of sulphur-bearing amino-acid metabolism

This code signifies a group of rare inherited metabolic disorders affecting the body's ability to process sulfur-containing amino acids, such as methionine and cysteine. These disturbances can lead to the accumulation of toxic byproducts, impacting various organ systems, including the brain, eyes, and skeletal system.

Use this code for documented diagnoses of conditions like homocystinuria, cystathioninuria, or other specific defects in the metabolic pathways of sulfur-bearing amino acids. Documentation should clearly state the specific metabolic error or the clinical manifestation directly linked to such a disturbance.

• Ensure documentation specifies the exact type of sulfur-bearing amino acid metabolism disturbance when possible, as more specific codes may exist in other code sets.
• Do not use this code for general amino acid metabolism disorders; it is specific to sulfur-bearing amino acids.
• 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 co-morbidity impacting treatment.