ICD-9 Code V77.3

Screening for phenylketonuria (PKU)

  1. ICD-9 Index
  2. Chapter: E
  3. Section: V70-V82
  4. Block: V77 Special screening for endocrine, nutritional, metabolic, and immunity disorders
  5. V77.3 - Screen-phenylketonuria

Not Valid for Submission

V77.3 is a legacy non-billable code used to specify a medical diagnosis of screening for phenylketonuria (pku). This code was replaced on September 30, 2015 by its ICD-10 equivalent.

ICD-9: V77.3
Short Description:Screen-phenylketonuria
Long Description:Screening for phenylketonuria (PKU)

Convert V77.3 to ICD-10

The following crosswalk between ICD-9 to ICD-10 is based based on the General Equivalence Mappings (GEMS) information:

  • Z13.228 - Encounter for screening for other metabolic disorders

Code Classification

  • Supplementary classification of factors influencing health status and contact with health services (E)
    • Persons without reported diagnosis encountered during examination and investigation of individuals and populations (V70-V82)
      • V77 Special screening for endocrine, nutritional, metabolic, and immunity disorders

Information for Medical Professionals

Index to Diseases and Injuries

References found for the code V77.3 in the Index of Diseases and Injuries:

    • Screening for V82.9
      • phenylketonuria V77.3

Information for Patients


Phenylketonuria (PKU) Screening

What is a PKU screening test?

A PKU screening test is a blood test given to newborns 24–72 hours after birth. PKU stands for phenylketonuria, a rare disorder that prevents the body from properly breaking down a substance called phenylalanine (Phe). Phe is part of proteins that are found in many foods and in an artificial sweetener called aspartame.

If you have PKU and eat these foods, Phe will build up in the blood. High levels of Phe can permanently damage the nervous system and brain, causing a variety of health problems. These include seizures, psychiatric problems, and severe intellectual disability.

PKU is caused by a genetic mutation, a change in the normal function of a gene. Genes are the basic units of heredity passed down from your mother and father. For a child to get the disorder, both the mother and father must pass down a mutated PKU gene.

Although PKU is rare, all newborns in the United States are required to get a PKU test.

  • The test is easy, with virtually no health risk. But it can save a baby from lifelong brain damage and/or other serious health problems.
  • If PKU is found early, following a special, low-protein/low-Phe diet can prevent complications.
  • There are specially-made formulas for infants with PKU.
  • People with PKU need to stay on the protein/low-Phe diet for the rest of their lives.

Other names: PKU newborn screening, PKU test

What is it used for?

A PKU test is used to see if a newborn has high levels of Phe in the blood. This may mean the baby has PKU, and more tests will be ordered to confirm or rule out a diagnosis.

Why does my baby need a PKU screening test?

Newborns in the United States are required to get a PKU test. A PKU test is usually part of a series of tests called a newborn screening. Some older infants and children may need testing if they were adopted from another country, and/or if they have any symptoms of PKU, which include:

  • Delayed development
  • Intellectual difficulties
  • A musty odor in the breath, skin, and/or urine
  • Abnormally small head (microcephaly)

What happens during a PKU screening test?

A health care provider will clean your baby's heel with alcohol and poke the heel with a small needle. The provider will collect a few drops of blood and put a bandage on the site.

The test should be done no sooner than 24 hours after birth, to ensure the baby has taken in some protein, either from breast milk or formula. This will help ensure the results are accurate. But the test should be done between 24–72 hours after birth to prevent possible PKU complications. If your baby wasn't born in the hospital or if you left the hospital early, be sure to talk to your child's health care provider to schedule a PKU test as soon as possible.

Will I need to do anything to prepare my baby for the test?

There are no special preparations needed for a PKU test.

Are there any risks to the test?

There is very little risk to your baby with a needle stick test. Your baby may feel a little pinch when the heel is poked, and a small bruise may form at the site. This should go away quickly.

What do the results mean?

If your baby's results were not normal, your health care provider will order more tests to confirm or rule out PKU. These tests may include more blood tests and/or urine tests. You and your baby may also get genetic tests, since PKU is an inherited condition.

If the results were normal, but the test was done sooner than 24 hours after birth, your baby may need to be tested again at 1 to 2 weeks of age.

Learn more about laboratory tests, reference ranges, and understanding results.

Is there anything else I need to know about a PKU screening test?

If your baby was diagnosed with PKU, he or she can drink formula that does not contain Phe. If you would like to breastfeed, talk your health care provider. Breast milk does contain Phe, but your baby may be able to have a limited amount, supplemented by the Phe-free formula. Regardless, your child will need to stay on a special low-protein diet for life. A PKU diet usually means avoiding high-protein foods such as meat, fish, eggs, dairy, nuts, and beans. Instead, the diet will probably include cereals, starches, fruits, a milk substitute, and other items with low or no Phe.

Your child's health care provider may recommend one or more specialists and other resources to help you manage your baby's diet and keep your child healthy. There are also a variety of resources available to teens and adults with PKU. If you have PKU, talk to your health care provider about the best ways to take care of your dietary and health needs.

References

  1. American Pregnancy Association [Internet]. Irving (TX): American Pregnancy Association; c2018. Phenylketonuria (PKU); [updated 2017 Aug 5; cited 2018 Jul 18]; [about 3 screens]. Available from: http://americanpregnancy.org/birth-defects/phenylketonuria-pku
  2. Children's PKU Network [Internet]. Encinitas (CA): Children's PKU Network; The PKU Story; [cited 2018 Jul 18]; [about 2 screens]. Available from: http://www.pkunetwork.org/Childrens_PKU_Network/What_is_PKU.html
  3. March of Dimes [Internet]. White Plains (NY): March of Dimes; c2018. PKU (Phenylketonuria) in Your Baby; [cited 2018 Jul 18]; [about 3 screens]. Available from: https://www.marchofdimes.org/complications/phenylketonuria-in-your-baby.aspx
  4. Mayo Clinic [Internet]. Mayo Foundation for Medical Education and Research; c1998–2018. Phenylketonuria (PKU): Diagnosis and treatment; 2018 Jan 27 [cited 2018 Jul 18]; [about 4 screens]. Available from: https://www.mayoclinic.org/diseases-conditions/phenylketonuria/diagnosis-treatment/drc-20376308
  5. Mayo Clinic [Internet]. Mayo Foundation for Medical Education and Research; c1998–2018. Phenylketonuria (PKU): Symptoms and causes; 2018 Jan 27 [cited 2018 Jul 18]; [about 3 screens]. Available from: https://www.mayoclinic.org/diseases-conditions/phenylketonuria/symptoms-causes/syc-20376302
  6. Merck Manual Consumer Version [Internet]. Kenilworth (NJ): Merck & Co. Inc.; c2018. Phenylketonuria (PKU); [cited 2018 Jul 18]; [about 3 screens]. Available from: https://www.merckmanuals.com/home/children-s-health-issues/hereditary-metabolic-disorders/phenylketonuria-pku
  7. National Cancer Institute [Internet]. Bethesda (MD): U.S. Department of Health and Human Services; NCI Dictionary of Cancer Terms: gene; [cited 2018 Jul 18]; [about 3 screens]. Available from: https://www.cancer.gov/publications/dictionaries/cancer-terms/search?contains=false&q=gene
  8. National PKU Alliance [Internet]. Eau Claire (WI): National PKU Alliance. c2017. About PKU; [cited 2018 Jul 18]; [about 2 screens]. Available from: https://npkua.org/Education/About-PKU
  9. NIH U.S. National Library of Medicine: Genetics Home Reference [Internet]. Bethesda (MD): U.S. Department of Health and Human Services; Phenylketonuria; 2018 Jul 17 [cited 2018 Jul 18]; [about 3 screens]. Available from: https://ghr.nlm.nih.gov/condition/phenylketonuria
  10. NIH U.S. National Library of Medicine: Genetics Home Reference [Internet]. Bethesda (MD): U.S. Department of Health and Human Services; What is a gene mutation and how do mutations occur?; 2018 Jul 17 [cited 2018 Jul 18]; [about 3 screens]. Available from: https://ghr.nlm.nih.gov/primer/mutationsanddisorders/genemutation
  11. NORD: National Organization for Rare Disorders [Internet]. Danbury (CT): NORD: National Organization for Rare Disorders; c2018. Phenylketonuria; [cited 2018 Jul 18]; [about 3 screens]. Available from: https://rarediseases.org/rare-diseases/phenylketonuria
  12. University of Rochester Medical Center [Internet]. Rochester (NY): University of Rochester Medical Center; c2018. Health Encyclopedia: Phenylketonuria (PKU); [cited 2018 Jul 18]; [about 2 screens]. Available from: https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=167&contentid=pku
  13. UW Health [Internet]. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2018. Health Information: Phenylketonuria (PKU) Test: How It Feels; [updated 2017 May 4; cited 2018 Jul 18]; [about 6 screens]. Available from: https://www.uwhealth.org/health/topic/medicaltest/phenylketonuria-pku-test/hw41965.html#hw41978
  14. UW Health [Internet]. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2018. Health Information: Phenylketonuria (PKU) Test: How It is Done; [updated 2017 May 4; cited 2018 Jul 18]; [about 5 screens]. Available from: https://www.uwhealth.org/health/topic/medicaltest/phenylketonuria-pku-test/hw41965.html#hw41977
  15. UW Health [Internet]. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2018. Health Information: Phenylketonuria (PKU) Test: Test Overview; [updated 2017 May 4; cited 2018 Jul 18]; [about 2 screens]. Available from: https://www.uwhealth.org/health/topic/medicaltest/phenylketonuria-pku-test/hw41965.html#hw41968
  16. UW Health [Internet]. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2018. Health Information: Phenylketonuria (PKU) Test: What To Think About; [updated 2017 May 4; cited 2018 Jul 18]; [about 10 screens]. Available from: https://www.uwhealth.org/health/topic/medicaltest/phenylketonuria-pku-test/hw41965.html#hw41983
  17. UW Health [Internet]. Madison (WI): University of Wisconsin Hospitals and Clinics Authority; c2018. Health Information: Phenylketonuria (PKU) Test: Why It Is Done; [updated 2017 May 4; cited 2018 Jul 18]; [about 3 screens]. Available from: https://www.uwhealth.org/health/topic/medicaltest/phenylketonuria-pku-test/hw41965.html#hw41973

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ICD-9 Footnotes

General Equivalence Map Definitions
The ICD-9 and ICD-10 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent.
  • No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system.
  • Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system.

Index of Diseases and Injuries Definitions

  • And - The word "and" should be interpreted to mean either "and" or "or" when it appears in a title.
  • Code also note - A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.
  • Code first - Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions, the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists, there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation.
  • Type 1 Excludes Notes - A type 1 Excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • Type 2 Excludes Notes - A type 2 Excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.
  • Includes Notes - This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
  • Inclusion terms - List of terms is included under some codes. 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.
  • NEC "Not elsewhere classifiable" - This abbreviation in the Alphabetic Index represents "other specified". When a specific code is not available for a condition, the Alphabetic Index directs the coder to the "other specified” code in the Tabular List.
  • NOS "Not otherwise specified" - This abbreviation is the equivalent of unspecified.
  • See - The "see" instruction following a main term in the Alphabetic Index indicates that another term should be referenced. It is necessary to go to the main term referenced with the "see" note to locate the correct code.
  • See Also - A "see also" instruction following a main term in the Alphabetic Index instructs that there is another main term that may also be referenced that may provide additional Alphabetic Index entries that may be useful. It is not necessary to follow the "see also" note when the original main term provides the necessary code.
  • 7th Characters - Certain ICD-10-CM categories have applicable 7th characters. The applicable 7th character is required for all codes within the category, or as the notes in the Tabular List instruct. The 7th character must always be the 7th character in the data field. If a code that requires a 7th character is not 6 characters, a placeholder X must be used to fill in the empty characters.
  • With - The word "with" should be interpreted to mean "associated with" or "due to" when it appears in a code title, the Alphabetic Index, or an instructional note in the Tabular List. The word "with" in the Alphabetic Index is sequenced immediately following the main term, not in alphabetical order.

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