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Table 5 Diet prescription, assessment, and monitoring

From: Nutritional management of phenylalanine hydroxylase (PAH) deficiency in pediatric patients in Canada: a survey of dietitians’ current practices

 

n (%)

Factors considered “very important” in deciding which formula to prescribea

 Nutrient composition of formula

18 (95)

 Patient’s age

17 (89)

 Preferences of the patient or family

17 (89)

 Availability of the product

15 (79)

 Price of the product

4 (21)

 Severity of PAH deficiency

4 (21)

Approaches to assessing patient adherence to formula and diet prescriptiona

 By patient’s/caregiver’s verbal report

17 (89)

 Monitoring weight and height

15 (79)

 By laboratory tests:

 

  Phenylalanine

16 (84)

  Pre-albumin

13 (68)

  Tyrosine

13 (68)

  Iron

12 (63)

  B12

12 (63)

  Albumin

5 (26)

 Checking how much formula was released by the dispensing authority

12 (63)

 By analyzing written dietary questionnaires

10 (53)

Technique that is most successful in improving adherence to diet

 Individualized PAH deficiency nutrition counseling

11 (58%)

 Motivational interview techniques

4 (21%)

 Reporting results of blood dots to patients

4 (21%)

 Regular reminders for Phe blood dots

0

Nutrients that are routinely monitored for “most” patients, based on diet recordsa

 Dietary Phe intake

19 (100%)

 Protein intake

18 (95%)

 Calorie intake

14 (74%)

 Mineral intake (any)

12b (67%)

 Vitamin intake (any)

12 (63%)

 Fat intake

5c (29%)

 Other

0

Components that are always/often included in routine clinical visit assessmentsa

 Anthropometric measurements

19 (100)

 Diet education

17 (90)

 Dietary analysis

17 (90)

  1. aMultiple choice questions, the percentages are expected to add up to more than 100%
  2. bOne missing response (n = 18)
  3. cTwo missing responses (n = 17)