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E72.03 ICD-10-CM Code: Lowe's syndrome

E72.03 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC coding software

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E72.03

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Lowe's syndrome

A rare inherited metabolic disorder affecting amino acid transport and kidney function, causing intellectual disability, vision problems, and progressive kidney disease.

Buddy the Bee presenting code insight

Buddy Insight

Lowe syndrome (oculocerebrorenal syndrome) is a rare X-linked recessive disorder affecting the eyes, brain, and kidneys, caused by mutations in the OCRL1 gene encoding inositol polyphosphate-5-phosphatase.

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

HCC 23

RAF 0.194

ACA/HHS

HCC 28

Varies by metal level

ESRD/PACE

HCC 23

RAF 0.036

RXHCC

HCC 43

RAF 0.063

Code Book Path

Official
E72Other disorders of amino-acid metabolism
E72.0Disorders of amino-acid transport
E72.03Lowe's syndrome

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E72.03 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E72.03 in this effective period.

Related Child Codes

Official
E72.00Disorders of amino-acid transport, unspecified
E72.01Cystinuria
E72.02Hartnup's disease
E72.04Cystinosis
E72.09Other disorders of amino-acid transport

Includes

Official

ICD-10-CM does not list Includes notes for E72.03 in this effective period.

Excludes 1

Official
  • disorders of tryptophan metabolism (E70.5)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E72.03 in this effective period.

Use Additional

Official
  • code for associated glaucoma (H42)

Code Also

Official

ICD-10-CM does not list Code Also instructions for E72.03 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Document the triad of ocular, neurological, and renal findings, OCRL1 gene mutation testing, ophthalmologic examination (cataracts), developmental and cognitive assessment, renal function tests showing tubular dysfunction, and genetic counseling for the family.
Record current management including bicarbonate supplementation and developmental support services.

MEAT Support

HCC Buddy guidance
Document the triad of ocular, neurological, and renal findings, OCRL1 gene mutation testing, ophthalmologic examination (cataracts), developmental and cognitive assessment, renal function tests showing tubular dysfunction, and genetic counseling for the family.
Record current management including bicarbonate supplementation and developmental support services.

Audit Caution

HCC Buddy guidance
Lowe syndrome may be misdiagnosed as non-syndromic Fanconi syndrome if the ocular and neurological features are not recognized.
Ensure documentation identifies the syndrome rather than coding individual organ manifestations separately.

Common Mistakes

HCC Buddy guidance
E72.04 (Cystinosis) which also causes renal Fanconi syndrome but through different mechanism
E72.09 (Other disorders of amino-acid transport) which is a broader category
Q87.8 (Other specified congenital malformation syndromes) which might be used if the metabolic basis is not recognized.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E72.03 an HCC code?

Yes. E72.03 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code
E72.03
Description
Lowe's syndrome
HCC (V28)
No CMS-HCC V28 mapping
RAF
Billable
Yes
Payment year
2026

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders
0.194
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
0.036
RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders
0.063

Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work E72.03 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E72.03

For E72.03 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E72.03 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E72.03 is the ICD-10-CM diagnosis code for lowe's syndrome. A rare inherited metabolic disorder affecting amino acid transport and kidney function, causing intellectual disability, vision problems, and progressive kidney disease. E72.03 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E72.03 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Also known as oculocerebrorenal syndrome; documentation may use either name. Because E72.03 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E72.03 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Also known as oculocerebrorenal syndrome; documentation may use either name
  • X-linked recessive inheritance pattern; primarily affects males

Clinical Significance

Lowe syndrome (oculocerebrorenal syndrome) is a rare X-linked recessive disorder affecting the eyes, brain, and kidneys, caused by mutations in the OCRL1 gene encoding inositol polyphosphate-5-phosphatase. The classic triad includes congenital cataracts, intellectual disability with hypotonia, and renal tubular dysfunction (Fanconi syndrome). Progressive chronic kidney disease is a major source of morbidity, and most patients require lifelong supportive care.

Documentation Requirements

  • Document the triad of ocular, neurological, and renal findings, OCRL1 gene mutation testing, ophthalmologic examination (cataracts), developmental and cognitive assessment, renal function tests showing tubular dysfunction, and genetic counseling for the family.
  • Record current management including bicarbonate supplementation and developmental support services.

Use Additional Code

  • code for associated glaucoma (H42)

Commonly Confused Codes

  • E72.04 (Cystinosis) which also causes renal Fanconi syndrome but through different mechanism
  • E72.09 (Other disorders of amino-acid transport) which is a broader category
  • Q87.8 (Other specified congenital malformation syndromes) which might be used if the metabolic basis is not recognized.

Child Codes

Code Hierarchy

Because E72.03 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

Work E72.03 in HCC Buddy

Open E72.03 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 7 days to try everything, no card required.