States Guam

Program Overview:

According to a report put together by Sylvia M. Au and Lianna Hasegawa, Guam lacks access to most medical specialties and subspecialties which allow a newborn screening program to flourish like in the U.S. states. In Guam, the organization of health care services is similar to the U.S. in terms of medical licensure requirements, health care systems, and a less than optimal public-private health care system focused on acute care rather than preventive services.

Guam has approximately 4,000 births per year. Over 90% of these occur at Guam Memorial Hospital. The remainder of the births occur at the Naval Hospital, at Sagua Mañagu (Guam’s first private birthing center), or at home with the aid of midwives. Guam Memorial Hospital, the Naval Hospital, and Sagua Mañagu all have contracts with different laboratories to provide newborn screening. The result is that a different panel of tests is offered to newborns, depending on where they are delivered.

Guam Memorial Hospital

Guam Memorial Hospital currently contracts with the Colorado Department of Public Health and Environment. Currently, only newborns with health insurance receive newborn screening. The Guam Department of Public Health and Social Services tracks and follows abnormal results from Guam Memorial Hospital. The Colorado Department of Public Health and Environment screens for the following disorders:

Amino Acid Disorders:

1. Arginase deficiency/Argininemia (ARG)
2. Argininosuccinic aciduria (ASA)
3. Citrullinemia, type I (CIT)
4. Citrullinemia, type II (CIT II)
5. Homocystinuria (HCY)
6. Hypermethioninemia
7. Maple syrup urine disease (MSUD)
8. Phenylketonuria (PKU)

Endocrine Disorders:

1. Congenital adrenal hyperplasia (CAH)
2. Congenital hypothyroidism (CH)

Fatty Acid Disorders:

1. Carnitine acylcarnitine translocase deficiency (CACT)
2. Carnitine palmitoyltransferase II deficiency (CPT II)
3. Carnitine palmitoyltransferase deficiency 1a (CPT IA)
4. Carnitine uptake defect (CUD)
5. Long-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)
6. Medium-chain acyl-CoA dehydrogenase deficiency (MCAD)
7. Short-chain acyl-CoA dehydrogenase deficiency (SCAD)
8. Trifunctional protein deficiency
9. Very long-chain acyl-CoA dehydrogenase deficiency (VCLAD)

Hemoglobinopathies:

1. Sickle cell anemia
2. S, ?- thalassemia
3. S,C disease

Organic Acid Disorders:

1. 3-Hydroxy-3-Methylglutaryl-CoA Lyase deficiency (HMG)
2. 3-Methylcrotonyl-CoA carboxylase deficiency (3MCC)
3. 3-Methylglutaconic aciduria (3-MGA)
4. Beta-ketothiolase deficiency (BKD)
5. Biotinidase deficiency (BIOT)
6. Glutaric acidemia type I (GA1)
7. Glutaric acidemia type II (GA2)
8. Isovaleric academia (IVA)
9. Malonic academia (MAL)
10. Methylmalonic acidemias (MUT, Cbl A,B, Cbl C,D)
11. Multiple carboxylase deficiency (MCD)
12. Propionic acidemia (PA)

Other:

1. Galactosemia (GALT)
2. Cystic fibrosis (CF)

Sagua Mañagu:

Sagua Mañagu began delivering babies in July 2000, and it reports an estimated 450 births per year. Sagua Mañagu sends their samples to the Oregon Public Health Laboratory. The Oregon Public Health Laboratory sends all results to the Medical Director at Sagua Mañagu. The Medical Director then sends copies of both normal and abnormal results to your child's health care provider. The Oregon Public Health Laboratory screens for the following disorders:

Endocrine Disorders:

1. Congenital hypothyroidism (CH)
2. Congenital Adrenal Hyperplasia (CAH)

Hemoglobin Disorders:

1. Sickle cell anemia
2. S, ?- thalassemia
3. S,C disease

Metabolic Disorders:

1. Biotinidase Deficiency (BIOT)
2. Galactosemia (GALT)

Amino Acid Disorders:

1. Phenylketonuria (PKU)
2. Homocystinuria (HCY)
3. Tyrosinemia, Type 1
4. Tyrosinemia, Type 2
5. Tyrosinemia, Type 3
6. Arginase deficiency/Argininemia (ARG)
7. Argininosuccinate lyase deficiency (ASA)
8. Citrullinemia type 1 (CIT)
9. Citrullinemia Type 2 (CIT II)

Organic Acid Disorders:

1. Beta-Ketothiolase deficiency (BKD)
2. Glutaric aciduria, Type I (GA1)
3. HMG-CoA lyase deficiency (3-hydroxy-3-methylglutaryl-CoA lyase deficiency) (HMG)
4. Isobutyryl CoA dehydrogenase deficiency (IBG)
5. Isovaleryl-CoA dehydrogenase deficiency (Isovaleric acidemia) (IVA)
6. Malonic aciduria (MAL)
7. Maple Syrup Urine Disease (MSUD)
8. Methylmalonic aciduria, vitamin B-12 responsive (Cbl A,B)
9. Methylmalonic aciduria, vitamin B-12 nonresponsive (MUT)
10. Vitamin B12 metabolic defect with methylmalonicacidemia and homocystinuria (Cbl C,D)
11. Multiple carboxylase deficiency (MCD)
12. Propionic acidemia (PROP))
13. 2-Methyl-3-hydroxybutyryl CoA dehydrogenase deficiency (2M3HBA)
14. 2-Methylbutyryl CoA dehydrogenase deficiency (2MBG)
15. 3-methylcrotonyl-CoA carboxylase deficiency (3-MCC)
16. 3-methylglutaconyl-CoA hydratase deficiency (3MGA)

Fatty Acid Chain Disorders:

1. Carnitine-acylcarnitine translocase deficiency (CACT)
2. Carnitine transporter defect (CUD)
3. Carnitine palmitoyl transferase I deficiency (CPT IA)
4. Carnitine palmitoyl transferase II deficiency (CPT II)
5. Glutaric aciduria, Type II (GA2)
6. Very long chain acyl-CoA dehydrogenase deficiency (VLCAD)
7. Long chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)
8. Medium chain acyl-CoA dehydrogenase deficiency (MCAD)
9. Short chain acyl-CoA dehydrogenase deficiency (SCAD)

Other:

1. Cystic fibrosis (CF)

Naval Hospital

As of October 1st, 2005, the Naval Hospital sends its newborn screening samples to Pediatrix, a commercial laboratory located in Pennsylvania. For a list of conditions which newborns born at the Naval Hospital will be screened for, ask your doctor. After the screening process is complete, all follow-up of newborn screening results is done by the staff at the Naval Hospital with phone or e-mail consultations when necessary from Tripler Army Medical Center in Hawaii (TAMC) or the Naval Medical Center in San Diego (NMCSD). Patients who require more complex consultations are flown to TAMC or NMCSD.