Similarities and differences in key diagnosis, treatment, and management approaches for PAH deficiency in the United States and Europe

Table 5 Recommendations for Type/Timing of Routine* Nutritional Monitoring across the Lifespan

ACMG/Nutritional Management Guidelines* [3]	European Guidelines [4, 5]
Infants (to age 1)	All age groups
Plasma amino acids- Monthly to every 3 months Complete blood count- Once Albumin - Once Prealbumin - Once Ferritin -Once Vitamin D 25-OH- Once	Annual measurements of plasma amino acids, homocysteine and or methylmalonic acid (B12 markers), hemoglobin, mean corpuscular volume, and ferritin.
Children, Adolescents, and Adults (age 1 to Adulthood)
Plasma amino acids- Each clinic visit Complete blood count- Yearly Albumin- Six to 12 months Prealbumin- Six to 12 months Ferritin- Yearly Vitamin D 25-OH- Yearly

*Micronutrient or other nutritional testing can be performed due to growth concerns, poor nutritional status, or inadequate adherence to diet: zinc, copper, B12, B6, erythrocyte folate, Vitamin A, selenium, essential fatty acids, and comprehensive metabolic panel [x]
*Vitamin and mineral micronutrient testing can be performed if clinically indicated, to include calcium, zinc, selenium, and parathyroid hormones

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