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Minnesota currently screens for both CPT IA since 2003 and SMA since 2018.
Summary of factual data and analytical methodologies
The Department’s Advisory Committee on Newborn Screening (Committee) recommended to the Department, and the Department concurred with the recommendation to add CPT IA and SMA to the list of congenital disorders for which newborns must be screened. Carntine palmitoyltransferase IA (CPT IA) deficiency is a fatty acid oxidation disorder associated with hypoketotic hypoglycemia and liver failure. Children with CPTIA present with hypoglycemia, liver dysfunction and encephalopathy, cholestatic jaundice and hepatomegaly, as well as renal dysfunction manifesting as renal tubular acidosis, when viral illness or prolonged fasting occurs. Prior to the episodes of metabolic crisis, they typically have normal growth and development. Studies have also found evidence for an association between infant mortality associated with infectious disease and homozygosity for CPT IA mutations. SMA is a neurodegenerative autosomal recessive genetic disease with an estimated incidence of 1 in 10,000 births. It affects the motor neurons in the spinal cord, and involves multiple organs. Cause of death is usually respiratory failure. There are five types of SMA, based on severity. SMA type 0 and I has the earliest onset, and is most severe. Type II usually presents by 18 months of age; children with type II can usually sit but cannot stand or walk. Type III manifests between 18 months and 10 years; children can learn to stand and walk, but usually lose these skills over time. Finally, Type IV is the mildest form, and may not show symptoms until adulthood. It was determined that CPT IA and SMA met the criteria under s. DHS 115.06 for being added to the list of congenital disorders for which WSLH must test the blood samples of newborns.
Analysis and supporting documents used to determine effect on small business
None, no effect on small business was found.
Effect on small business
Agency contact person
Gary Kirk, MD, MPH Chief Medical Officer, Bureau of Community Health Promotion, Division of Public Health, Wisconsin Department of Health Services Ph.: 608-266-5818
Statement on quality of agency data
The Department relied on the following information for the rules and analysis: The Centers for Disease Control and Prevention, US Secretary of Health and Human Services, Department’s Advisory Committee on Heritable Disorders in Newborns and Children, and Wisconsin Newborn Screening Program – Condition Nomination Form (the form is found here:
Place where comments are to be submitted and deadline for submission
Comments may be submitted to the agency contact person that is listed above until the deadline given in the upcoming notice of public hearing. The notice of public hearing and deadline for submitting comments will be published in the Wisconsin Administrative Register and to the department’s website, at Comments may also be submitted through the Wisconsin Administrative Rules Website, at:
SECTION 1. Chapter DHS 115 (title) is amended to read:
SECTION 2. DHS 115.01 is amended to read:
DHS 115.01 Authority and purpose. This chapter is promulgated under the authority of ss. 253.13 (1) and 227.11 (2), Stats., to specify the congenital and metabolic disorders for which each newborn infants are to be is screened and tested.
SECTION 3. DHS 115.04 (title) and (intro.) are amended to read:
DHS 115.04 Congenital and metabolic disorders. Pursuant to s. 253.13 (1), Stats., blood samples taken from each newborn shall be tested for all of the following conditions:
SECTION 4. DHS 115.04 (8) (cm) is created to read:
DHS 115.04 (8) (cm) Carnitine palmitoyltransferase IA deficiency, ICD-10-CM-E71.318.
SECTION 5. DHS 115.04 (15m) is created to read:
DHS 115.04 (15m) Spinal muscular atrophy, ICD-10-CM-G12.9.
SECTION 6. EFFECTIVE DATE: This rule shall take effect on the first day of the month following publication in the Wisconsin administrative register, as provided in § 227.22 (2) (intro), Stats.
Links to Admin. Code and Statutes in this Register are to current versions, which may not be the version that was referred to in the original published document.