Monitoring - TYR I

To maintain good metabolic control and ensure optimal growth, frequent adjustments of the diet are necessary.

Monitoring dietary management and outcome of Tyrosinemia Type I^1,2
Laboratory monitoring	Each clinic visit
Nutritional monitoring dietary intake; food records	Each clinic visit
Anthropometry: Growth (weight, length/height, BMI) Waist circumfence from the age of 5 years	Each clinic visit
Clinical examination, eg skin, hair	Each clinic visit
Quantitative plasma amino acids (3-4 hours fasting)	Annual
Albumin, prealbumin	Annual
25-dihydroxyvitamin D	Annual
DEXA scan	Baseline DEXA scan at 11 years and repeat very 5 years in the absence of osteopenia or osteoporosis
Essential fatty acids³	Annual

Age	Blood monitoring frequency¹
0-1 years	Weekly in infants or newly diagnosed patients until stable
1-4	Every 1-2 weeks
5-10 years	Every 2 weeks
>10 years	Monthly or more frequent if levels are unstable

Dixon MD, MacDonald A, White F, Stafford J. In: Clinical Paediatric Dietetics. 4^th ed: West Sussex: John Wiley & Sons Limited, 2014:381-525.
Van Spronsen Fj, van Rijn G. Tyrosinemie. Carbasius Weber (ed) 1^st Dieet bij metabole ziekten 1st Ed. WKZ/UMCU Utrecht. 2011:38-58.