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. 2020 Sep 25;15:266. doi: 10.1186/s13023-020-01541-2

Table 3.

Recommended Duration of Treatment for PAH Deficiency

ACMG (2014) European Guidelines (2017)
Recommended Duration and Treatment ranges
Infants 1 year or younger Maintain Diet for Life Levels 120–360 μmol/L > 360 μmol/L
Children < 12

> 360 μmol/L treated

360–600 μmol/L untreated

Adolescents > 600 μmol/L
Adults > 18 > 600 μmol/L

Maintain Diet for Life

Levels > 600 μmol/L

Women
  Prior to conception Maintain Diet for Life Levels 120–360 μmol/L 120–600 μmol/L (for age > 12)
  During Pregnancy 120–360 μmol/L