Physical Growth and Nutritional Status of Children with Phenylketonuria

Protein status in phenylketonuria: A scoping review

“…There was greater variation in participant age for studies including biochemical measurements of protein status than anthropometric measurements of protein studies. Six studies were conducted in children (age range 13.7 days to 10 years) [33,38,42,84,85,89], ten studies were in children and adolescents (age range 2.4 monthse18 years) [44,56,62,69,74,76,77,83,86,90], 15 in adolescents and adults (11e49 years) [7,39,40,45,46,57,63,64,70,72,75,79,91e93] and two in adults only [41,82]. Seven studies included mixed-age cohorts (age range 7 monthse54 years) [26,36,37,80,81,94,95].…”

“…A range of biochemical measurements were used to assess protein status, and most studies included multiple measurement (Table 3). Serum albumin [36,41,42,56,62,63,69,72,74,78,85,86,95], prealbumin [26,34,37,57,80,81,84,87,89] or both [33,38,40,45,46,76,77,82,83,88,90,91,93,94] were the most common biochemical measurements reported. Total protein [36,40e42,56,63,72,76,78,80,81,85,86,91,93,94] and blood urine nitrogen (BUN) [33,40,45,74,75,78,85,86,93] were also frequently used.…”

“…Serum albumin [36,41,42,56,62,63,69,72,74,78,85,86,95], prealbumin [26,34,37,57,80,81,84,87,89] or both [33,38,40,45,46,76,77,82,83,88,90,91,93,94] were the most common biochemical measurements reported. Total protein [36,40e42,56,63,72,76,78,80,81,85,86,91,93,94] and blood urine nitrogen (BUN) [33,40,45,74,75,78,85,86,93] were also frequently used. Three acute phase studies were included: two studies measured whole-body protein metabolism using valine and leucine stable isotopes [39,41], and one measured nitrogen excretion and leucine kineti...…”

“…Total protein levels were similar between people with PKU and controls [80]. Despite normal levels of total protein, abnormal albumin (high) and BUN (low) were identified in children and adolescents where total protein intake was 62 ± 15% of RDA [86]. Low protein levels in adolescents and adults with low SMM, and normal protein levels in participants with high SMM were also observed [70].…”