2026 ICD-10-CM Diagnosis Code E72.538Other specified primary hyperoxaluria
E72.538 is a billable ICD-10-CM diagnosis code for other specified primary hyperoxaluria. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as primary hyperoxaluria.
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Primary hyperoxaluria
- Primary hyperoxaluria type III
- Primary hyperoxaluria, type II
Tabular List NotesGuidance
Coding notes and annotation back-references applicable to this code.
Inclusion Terms
- Primary hyperoxaluria, type 2
- Primary hyperoxaluria, type 3
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
- Disorder (of) - See Also: Disease;
- hyperoxaluria - R82.992
- specified type NEC - E72.538
- type 2 - E72.538
- type 3 - E72.538
External Cause of Injuries IndexGuidance
References for this code in the External Cause of Injuries Index.
- Disorder(of)
- glycine metabolism
- hyperoxaluria
- primary
- specified type NEC
- Disorder(of)
- glycine metabolism
- hyperoxaluria
- primary
- type 2
- Disorder(of)
- glycine metabolism
- hyperoxaluria
- primary
- type 3
Clinical InformationClinical
AGXT wt Allele|AGT|AGT1|AGXT1|Alanine--Glyoxylate Aminotransferase Gene|Alanine--Glyoxylate and Serine--Pyruvate Aminotransferase Gene|Alanine-Glyoxylate Aminotransferase wt Allele|Glycolicaciduria Gene|Hepatic Peroxisomal Alanine:Glyoxylate Aminotransferase Gene|L-Alanine: Glyoxylate Aminotransferase 1 Gene|Oxalosis I Gene|PH1|Primary Hyperoxaluria Type 1 Gene|SPAT|SPT|Ser-PyrAT|Serine:Pyruvate Aminotransferase Gene|TLH6
human agxt wild-type allele is located in the vicinity of 2q37.3 and is approximately 12 kb in length. this allele, which encodes alanine-glyoxylate aminotransferase protein, is involved in gluconeogenesis, l-serine metabolism and glyoxylate detoxification. mutations in the gene are associated with primary hyperoxaluria 1.GRHPR wt Allele|GLXR|GLYD|Glycerate-2-Dehydrogenase Gene|Glyoxylate and Hydroxypyruvate Reductase wt Allele|MSTP035|PH2|Primary Hyperoxaluria Type 2 Gene
human grhpr wild-type allele is located in the vicinity of 9p13.2 and is approximately 14 kb in length. this allele, which encodes glyoxylate reductase/hydroxypyruvate reductase protein, plays a role in hydroxypyruvate and glyoxylate metabolism. mutation of the gene is associated with type ii primary hyperoxaluria.Primary Hyperoxaluria
a hereditary disorder characterized by excessive oxalate production, leading to hyperoxaluria.Primary Hyperoxaluria Type I
recessively inherited primary hyperoxaluria due to alanine-glyoxylate aminotransferase (agxt) deficiency.Primary Hyperoxaluria Type II
recessively inherited primary hyperoxaluria due to glyoxylate reductase/hydroxypyruvate reductase (grhpr) deficiency.Primary Hyperoxaluria Type III
recessively inherited primary hyperoxaluria due to mitochondrial 4-hydroxy-2-oxoglutarate aldolase (hoga1) gene mutations.
Code History & ChangesHistory
New Code E72.538 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.
Replacement E72.538 replaces the following previously assigned code(s):
- E72.53 - Hyperoxaluria
- E72.53 - Primary hyperoxaluria
Questions About E72.538Overview
Is E72.538 (Primary hyperoxaluria) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report other specified primary hyperoxaluria on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
