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Argininosuccinic acidemia (ASA)

Borderline elevation of Citrulline

1. What does this mean? This infant may have an inborn error of metabolism.

2. What to do?

a. Contact parent/guardian to check on the health of the infant.

Clinical considerations:

  • Poor feeding
  • Vomiting
  • Lethargy
  • Hypotonia
  • Tachypnea
  • Seizures
  • Measure ammonia if infant is symptomatic

    b. No dietary intervention is needed at this time. Continue to feed the baby if well.

    c. Draw a repeat newborn screening specimen and submit to the State PH Laboratory

    3. Referral indicated: If the infant has any signs or symptoms, you may wish to consult with a pediatric metabolic specialist or refer the patient to the Newborn Screening Clinic at Arkansas Children's Hospital. Call 501-364-4050 to speak with the Newborn Screening Coordinator or page at 501-364-1100.

    For more information, refer to the health department (ADH) letter that was faxed to you and information at the ADH website.


    Critical elevation of Citrulline

    1.What does this mean? This infant may have a urea cycle disorder. Further testing is required.

    2. What to do:

    a. Contact parent/guardian TODAY to check on the health of the infant.

    Clinical considerations:

    • Poor feeding, vomiting, lethargy, hypotonia
    • Tachypnea, Coma, Seizures
    • Hyperammonemia
    • Liver disease (later signs)

    Referral: If any sign is present or infant is ill initiate emergency treatment for hyperammonemia in consultation with metabolic specialist. For assistance with clinical evaluation and testing, you may wish to refer the patient to the Newborn Screening Clinic at Arkansas Children's Hospital. Call 501-364-4050 to speak with the Newborn Screening Coordinator.

    b. No dietary intervention is needed at this time. Continue to feed the baby if well.

    3. Do the following tests NOW:

    1. Plasma ammonia
    2. Plasma amino acids
    3. Urine organic acids

    4. Referral indicated: If the infant has any signs or symptoms, or if testing confirms the diagnosis, a referral to a pediatric metabolic specialist is recommended. For assistance with clinical evaluation and testing, you may wish to refer the patient to the Newborn Screening Clinic at Arkansas Children's Hospital. call 501-364-4050 to speak with the Newborn Screening Coordinator or page at 501-364-1100.


    Have more questions?

    For more information, refer to the health department (ADH) letter that was faxed to you and information at the ADH website.

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