E71.542
ICD-10-CMThis code identifies a rare, inherited metabolic disorder affecting peroxisomes, specifically those classified as "group 3." These disorders involve defects in the synthesis of plasmalogens and other ether phospholipids, leading to a range of neurological, skeletal, and developmental abnormalities. The severity and specific manifestations can vary widely among affected individuals.
Assign this code for documented diagnoses of a group 3 peroxisomal disorder, such as rhizomelic chondrodysplasia punctata type 1 (RCDP1) or other related conditions where the specific type is not otherwise specified but falls within group 3. Documentation should clearly indicate a diagnosis of a peroxisomal disorder with specific mention of its classification as "group 3" or a condition known to be a group 3 peroxisomal disorder.
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