ACT Sheets and Algorithms

The ACMG ACTion (ACT) Sheets and their accompanying algorithms are an essential resource for health care providers to help inform clinical decision making when an initial screen positive newborn screen is received. Previously under the auspices of the HRSA-funded National Coordinating Center for the Regional Genetics Networks and continuously developed by the American College of Medical Genetics and Genomics with national topic experts, they provide concise current information about the disorder as well as initial guidance to help manage a screen positive result. This includes prompt actions a healthcare professional can follow when communicating with the family, determining the appropriate next steps to evaluate the newborn and reach a diagnosis, and provide resource links for further information. The accompanying algorithm presents an overview of the basic laboratory steps involved in determining the final diagnosis in the infant.

ACMG ACT Sheets are freely available on the ACMG website regardless of membership status.

Newborn Screening ACT Sheets and Algorithms

Amino Acidemias
Argininosuccinic aciduria; Citrullinemia I; Citrullinemia II; Pyruvate carboxylase deficiencyTime Critical

Analyte: Citrulline

Decreased CitrullineTime Critical

Analyte: Citrulline

Guanidinoacetate Methyltransferase (GAMT) Deficiency

Analyte: Guanidinoacetate

Homocystinuria; Hypermethioninemia; GNMT; Adenosylhomocysteine hydrolase deficiency

Analyte: Methionine

MSUD; HydroxyprolinuriaTime Critical

Analyte: Leucine

Phenylketonuria (PKU); Biopterin cofactor biosynthesis defect; Biopterin cofactor regeneration defect

Analyte: Phenylalanine

Tyrosinemia I; Tyrosinemia II; Tyrosinemia III

Analyte: Tyrosine SUAC Normal

Tyrosinemia I; Tyrosinemia II; Tyrosinemia III

Analyte: Tyrosine Normal/Elevated and SUAC Elevated

Critical Congenital Heart Disease
Critical Congenital Heart Disease

Analyte: Blood oxygen saturation levels

Endocrine Disorders
Congenital central hypothyroidism

Analyte: Low T4 +/- Abnormal TSH

Congenital adrenal hyperplasia (CAH) (21-hydroxylase deficiency)Time Critical

Analyte: Elevated 17-OHP

Fatty Acid Oxidation Disorders
CPT 1 deficiencyTime Critical

Analyte: C0; C0/C16+C18

CPT 2 or CACTTime Critical

Analyte: C16 and/or C18:1

Glutaric acidemia 2 / Ethylmalonic encephalopathyTime Critical

Analyte: C4; C5

LCHAD or TFPTime Critical

Analyte: C16-OH +/- and C18:1-OH

MCADTime Critical

Analyte: C8; C6, C10

Short Chain Hydroxyacyl-CoA Dehydrogenase (SCHAD) DeficiencyTime Critical

Analyte: C4-OH

Short-chain acyl-CoA deficiency (SCAD); Ethylmalonic encephalopathy; Isobutyryl-CoA dehydrogenase deficiency

Analyte: C4

VLCADTime Critical

Analyte: C14:1 +/-

Galactosemias
Primary or Secondary Hypergalactosemia

Analyte: Increased Total Galactose with normal GALT

Classical GalactosemiaTime Critical

Analyte: Absent/Reduced GALT

Genetic Disorders
Biotinidase deficiency

Analyte: Biotinidase

Cystic Fibrosis, Elevated IRT + 0 Variants

Analyte: Elevated Immunoreactive trypsinogen (IRT) + 0 Variants

Cystic Fibrosis, Elevated IRT + 1 Variant

Analyte: Elevated Immunoreactive trypsinogen (IRT) + 1 Variant

Cystic Fibrosis, Elevated IRT + 2 Variants

Analyte: Elevated Immunoreactive trypsinogen (IRT) + 2 Variants

X-Linked Adrenoleukodystrophy (X-ALD)

Analyte: Elevated lysophosphatidylcholines

Hemoglobin Disorders
Sickle cell anemia (HbSS or HbSbeta0 Thalassemia)

Analyte: Hemoglobin FS

Hemoglobin SC disease (HbSC)

Analyte: Hemoglobin FSC

Hemoglobin S/beta Thalassemia (HbSbeta+)

Analyte: Hemoglobin FSA

Sickle cell carrier (trait) (HbAS)

Analyte: Hemoglobin FAS

Hemoglobin V (variant)

Analyte: Hemoglobin FAV

Hemoglobin FE or Hemoglobin E/Beta Zero Thalassemia (Hb EE or Hb E/beta0 Disease)

Analyte: Hemoglobin FE

Beta Thalassemia Major and Intermedia

Analyte: Hemoglobin F

Alpha (α) Thalassemia

Analyte: Hemoglobin FA + Unquantified Barts Hb

Alpha (α) Thalassemia: Silent Carrier and Alpha Thalassemia Trait

Analyte: Hemoglobin FA + Low/Moderate Barts Hb

Alpha (α) Thalassemia: Hb H Disease

Analyte: Hemoglobin FA + High Barts Hb

Hemoglobin CC Disease or Hemoglobin C/Beta Zero Thalassemia (HbC/beta0 Disease)

Analyte: Hemoglobin FC

Hemoglobin C/Beta Plus Thalassemia (HbC/beta+ Disease)

Analyte: Hemoglobin FCA

Hemoglobin E/Beta Plus Thalassemia (HbE/B+ Disease)

Analyte: Hemoglobin FEA

Lysosomal Storage Diseases
Musculoskeletal Disease
DMD Pathogenic Variant

Analyte: Pathogenic Variants in DMD gene

DMD Elevated Creatine Kinase Muscle Isoform

Analyte: Elevated Creatine Kinase Muscle Isoform

No Pathogenic Variant DMD Gene

Analyte: No Pathogenic Variant in DMD gene

Spinal Muscular Atrophy (SMA)Time Critical

Analyte: Exon 7 Deletion (Pathogenic Variant) in SMN1 gene

Organic Acidemias
Beta-ketothiolase deficiency; Biotinidase deficiency; Holocarboxylase deficiency; HMG-CoA lyase deficiency; 2M3HBA; 3MGA; 3MCCTime Critical

Analyte: C5-OH

Glutaric acidemia 1Time Critical

Analyte: C5-DC

Isovaleric acidemia; Short/branched chain acyl-CoA dehydrogenase deficiencyTime Critical

Analyte: C5

Malonic acidemiaTime Critical

Analyte: C3-DC

Methylmalonic acidemias; Propionic acidemiaTime Critical

Analyte: C3

Prenatal Cell-Free DNA Screening ACT Sheets

Secondary Findings ACT Sheets

Familial Adenomatous Polyposis

Biomarker: APC Pathogenic Variants

Familial Hypercholesterolemia

Biomarker: APOB, LDLR, PCSK9 Pathogenic Variants

Hereditary Breast and Ovarian Cancer

Biomarker: BRCA1 and BRCA2 Pathogenic Variants

Lynch Syndrome

Biomarker: MLH1, MSH2, MSH6, PMS2, EPCAM Pathogenic Variants

Malignant Hyperthermia

Biomarker: RYR1 and CACNA1S Pathogenic Variants

*”Time Critical” is a condition in which acute symptoms or potentially irreversible damage could develop in the first week of life, and for which early recognition and treatment can reduce risk of morbidity and mortality.

Disclaimer: This ACT sheet and algorithm is designed primarily as an educational resource for medical geneticists and other clinicians to help them provide quality medical services. Adherence to this ACT sheet and algorithm is completely voluntary and does not necessarily assure a successful medical outcome. This ACT sheet and algorithm should not be considered inclusive of all proper procedures and tests or exclusive of other procedures and tests that are reasonably directed to obtaining the same results. In determining the propriety of any specific procedure or test, the clinician should apply their own professional judgment to the specific clinical circumstances presented by the individual patient or specimen. Clinicians are encouraged to document the reasons for the use of a particular procedure or test, whether or not it is in conformance with this ACT sheet and algorithm. Clinicians also are advised to take notice of the date this ACT sheet and algorithm was adopted, and to consider other medical and scientific information that becomes available after that date. It also would be prudent to consider whether intellectual property interests may restrict the performance of certain tests and other procedures. Where individual authors are listed, the views expressed may not reflect those of the authors’ employers or affiliated institutions.

Authors
ACMG ACT Sheet Advisory Committee (Current)

Dietrich Matern, MD, PhD, FACMG (Chair)
Biochemical Genetics Laboratory, Mayo Clinic, Rochester, MN

Tina Cowan, PhD, FACMG
Department of Pathology, Stanford University Medical Center, Stanford, CA

Amy Gaviglio, MS, CGC
Connetics Consulting, LLC, Minneapolis, MN

Andrea M. Matthews (Public Member)
Children’s Sickle Cell Foundation, Inc., Pittsburgh, PA

Robert Ostrander, MD, FAAFP
Rural Medical Scholars Program, Department of Family Medicine, SUNY Upstate Medical University, Syracuse, NY

Tracy Trotter, MD, FAAP, FACMG
John Muir Health, San Ramon, CA

Tim Wood, PhD, FACMG
Children’s Hospital of Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO

Jing Xiao, PhD, FACMG
New York State Newborn Screening Program, Albany, NY

Nancy C. Rose, MD, FACMG (Medical Consultant)
University of Utah, Salt Lake City, UT

Sandor Roberts (ACMG Staff)

Metabolic ACT Sheet Document Review Work Group (2025-2026)

Katherine Anderson, MD, FACMG
Larner College of Medicine, University of Vermont, Burlington, VT

Troy Coody, PhD, FACMG
Cycle Pharmaceuticals, Salt Lake City, UT

Debra Freedenberg, MD, PhD, FACMG

Dietrich Matern, MD, PhD, FACMG
Biochemical Genetics Laboratory, Mayo Clinic, Rochester, MN

Wendy Smith, MD, FACMG
Division of Genetics, Maine Medical Center, Portland, ME

Janet Thomas, MD, FACMG
University of Colorado School of Medicine, Aurora, CO

Nancy C. Rose, MD, FACMG (Medical Consultant)
University of Utah, Salt Lake City, UT

Sandor Roberts (ACMG Staff)

Critical Congenital Heart Disease Work Group (2026)

Lisa Hom, RN, ESQ
Children’s National Heart & Lung Center, Children’s National Hospital, Washington, DC

Gerard Martin, MD, MACC, FAHA, FAAP
Children’s National Heart & Lung Center, Children’s National Hospital, Washington, DC

Erin Miller, MS
University of Cincinnati College of Medicine; Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

Ana Morales, MS, CGC
Department of Genomic Health, Geisinger, Danville, PA

Matthew Oster, MD, MPH
Children’s Healthcare of Atlanta Cardiology, Emory University School of Medicine, Atlanta, GA

Bryanna Schwartz, MD, MPH
Division of Cardiovascular Sciences, National Heart, Lung, and Blood Institute, Bethesda, MD; Children’s National Heart & Lung Center, Children’s National Hospital, Washington, DC

Nancy C. Rose, MD, FACMG (Medical Consultant)
University of Utah, Salt Lake City, UT

Sandor Roberts (ACMG Staff)

Metachromatic Leukodystrophy Work Group (2025, work completed)

Laura Adang, MD, PhD, MSTR
Division of Child Neurology, Children’s Hospital of Philadelphia, Philadelphia, PA

Joshua Bonkowsky, MD, PhD
Division of Pediatric Neurology, University of Utah, Salt Lake City, UT

Michele Caggana, ScD, FACMG
Retired, New York State Department of Health, Albany, NY

Natalia Gomez-Ospina, MD, PhD
Department of Pediatrics, Stanford University, Stanford, CA

Hannah Hart, LGC
Division of Pediatric Neurology, University of Utah, Salt Lake City, UT

Dietrich Matern, MD, PhD, FACMG
Biochemical Genetics Laboratory, Mayo Clinic, Rochester, MN

Paul Orchard, MD
Division of Blood & Marrow Transplantation & Cellular Therapy, University of Minnesota, Minneapolis, MN

Nancy C. Rose, MD, FACMG (Medical Consultant)
University of Utah, Salt Lake City, UT

Sandor Roberts (ACMG Staff)