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Table 3 Recommended Duration of Treatment for PAH Deficiency

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

 

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