CPT 0383U: Tyrosinemia FollowUp Panel, SelfCollect Blood Spot

CPT code 0383U is a Proprietary Laboratory Analyses (PLA) code that describes the Tyrosinemia FollowUp Panel, SelfCollect, Blood Spot, performed by Mayo Clinic. The panel measures tyrosine, phenylalanine, methionine, succinylacetone, and nitisinone in a patient-collected dried blood spot using LC–MS/MS. As a PLA code, 0383U applies to a single manufacturer or laboratory test and signals proprietary assay reporting and billing.

Nationally, this code matters for follow-up metabolic monitoring of patients with tyrosinemia or those on nitisinone therapy, and for payers and providers managing home-collected laboratory workflows. Key payers included in the analysis are Aetna, Blue Cross Blue Shield, Cigna, UnitedHealthcare, and Medicare. Information on coverage policies and reimbursement benchmarks for PLA codes is important for laboratory billing, prior authorization processes, and patient access to at-home monitoring.

Readers will learn the clinical purpose of the test, the typical service and site of service, payer coverage scope, and where to find policy and billing considerations specific to PLA-coded assays. Data not available in the input: associated taxonomies, ICD-10 diagnoses, related codes, and detailed payer policy specifics.

Billing Code Overview

CPT code 0383U is a Proprietary Laboratory Analyses (PLA) code for the Tyrosinemia FollowUp Panel, SelfCollect, Blood Spot, performed by Mayo Clinic. The test evaluates a patient-collected blood spot specimen for levels of tyrosine, phenylalanine, methionine, succinylacetone, and nitisinone using liquid chromatography with tandem mass spectrometry (LC–MS/MS).

Service Type: Laboratory — specialty biochemical genetic/metabolic monitoring

Typical Site of Service: Patient-collected at home with specimen returned to the laboratory; testing performed in a clinical laboratory (Mayo Clinic)

Clinical Context

A patient with hereditary tyrosinemia or a person receiving nitisinone therapy collects a blood spot specimen at home using the manufacturer-supplied self-collection kit and returns the sample to Mayo Clinic for analysis. The test, billed with 0383U, measures tyrosine, phenylalanine, methionine, succinylacetone, and nitisinone concentrations using LC–MS/MS to monitor biochemical control and drug levels. Typical workflow: the clinician orders the Tyrosinemia FollowUp Panel for outpatient monitoring or post-discharge follow-up, the laboratory provides the self-collection kit and instructions, the patient collects a dried blood spot (DBS) and ships it to Mayo Clinic, the laboratory performs LC–MS/MS analysis, generates a report, and the ordering clinician reviews results to guide metabolic management and nitisinone dosing adjustments. Typical site of service is outpatient/home collection with laboratory analysis at a reference diagnostic laboratory. Typical patient scenarios include routine follow-up of diagnosed hereditary tyrosinemia type I on nitisinone therapy, assessment of suspected drug nonadherence or subtherapeutic nitisinone levels, and monitoring after a change in therapy or clinical status.

Coding Specifications

Modifier	Description	When to Use
`00`	No modifier — standard billing	Use when no special billing modifiers apply to this PLA test.

Taxonomy Code	Specialty	Notes
`207Q00000X`	Clinical Pathology	Clinical pathologists oversee laboratory testing and interpretation.
`208000000X`	Pediatric Medicine	Pediatricians order and interpret follow-up testing for inherited metabolic disorders.
`207L00000X`	Clinical Molecular Genetics	Specialists in biochemical genetic testing and result interpretation.
`207L00000`	Biochemical Genetics (approximate)	Providers in metabolic/genetics clinics managing tyrosinemia patients.
`363A00000X`	Nurse Practitioner	NPs in metabolic clinics coordinate testing and patient education.

Related Diagnoses

ICD-10 Code	Description	Clinical Relevance
`E75.241`	Tyrosinemia type I	Primary diagnosis for which serial monitoring of succinylacetone, tyrosine, and nitisinone levels is required.
`E74.39`	Other disorders of aromatic amino-acid metabolism	Covers related metabolic conditions where tyrosine/phenylalanine profiling may be relevant.
`E70.0`	Phenylketonuria	Amino acid metabolism disorder where amino acid panels are relevant for differential diagnosis and monitoring.
`E88.09`	Metabolic disorder, unspecified	Used when a specific metabolic diagnosis is pending and biochemical monitoring is performed.
`Z79.899`	Other long term (current) drug therapy	Used to indicate ongoing nitisinone therapy requiring therapeutic drug monitoring.

Related CPT Codes

CPT Code	Description	Relationship to This Procedure
`36415`	Collection of venous blood by venipuncture	May be used if a venous sample is collected instead of or in addition to the patient-collected blood spot for confirmatory testing.
`81025`	Urine pregnancy test, by dipstick or tablet reagent for pregnancy, presumptive	May be ordered in reproductive-age patients before treatment changes that affect teratogenic risk; not specific to this panel.
`80053`	Comprehensive metabolic panel	May be ordered alongside to assess liver and renal function relevant to metabolic disease management.
`82310`	Vitamin D; 25-hydroxy	Occasionally ordered in metabolic follow-up panels when nutritional status is assessed.
`G0452`	Drug testing, presumptive; definitive or confirmatory testing	Definitional relationship: separate drug level testing or confirmation may be ordered if additional therapeutic drug monitoring is required beyond this panel.

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Summary & Overview