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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)