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Leg mij uitgebreid het volgende onderwerp uit: The accurate assessment and regular monitoring of growth and
nutritional status is a cornerstone of the management of pwCF to

improve outcomes. In infants and children, assessment of nutri-
tional status must be comprehensive, using multiple data points

over time and preferably multiple different parameters. For infants
who are diagnosed by newborn screening, attention to nutrition is
key to maintain normal growthdeven before signs of the CF
phenotype become evident [8]. Monitoring of growth should be

more frequent at a younger age or if the patient has a poor nutri-
tional status at any age [8].

Adequate nutrition in infants and children with CF is considered

when growth is similar to that of an age-matched healthy popu-
lation with an additional focus on body composition. The goal of

WFL >50th percentile in children <2 years and BMI 50th
percentile in those over two to 18 years old should continue to be
used [11]. For CF adults over 18 years of age, the target is a BMI at or
above 22 kg/m2 for females and 23 kg/m2 for males [8]. It should be
noted that WFL or BMI might be falsely normal or high in stunted
patients [12].
TH can be included to evaluate if a child is growing within
genetic potential [13,14]. Height evaluation based on HFA alone
may result in underestimation or overestimation of growth,
which may induce inappropriate nutrition interventions. Using
HFA adjusted for TH (HFA/TH) which means HFA-z-score minus
TH for age z-score, can indicate how the current HFA z-score
relates to the TH z-score. Generally, a difference of more than 2
SD z-score is considered abnormal and indicates growth outside
the genetic potential which could be related to nutritional
deficits.
Pediatric growth charts should be chosen according to the
regular practice in the country or center [11,15e17]. CDC, WHO,
and IOTF charts provide comparable associations between BMI
z-scores and pulmonary function [18]. Despite this, some studies
have emphasized that there are differences that should be noted
when interpreting the growth charts [11,15e17]. CDC and WHO
charts show some discrepancies when determining underweight

in infants, toddlers, and school-aged children [18e20]. CDC
charts more frequently classify children aged zero to two years
and those at school age as underweight while WHO charts
would not, while adolescents show comparable estimations of

thinness between the CDC and WHO charts [19,20]. This in-
dicates that WHO charts are adequate to diagnose both under

and overnutrition [21] but may provide a false sense of secu-
rity when applied for CF, especially in younger children [22].

Additionally, children who reached the 50th percentile WFL at
two years of age by both WHO and CDC charts had better
clinical outcomes [11,15]. IOTF has been widely employed in
epidemiology but may not be appropriate in the clinical setting
[23,24].
Since the development of the previous guideline, there is
growing evidence that body composition is a fundamental
component of a comprehensive nutritional status assessment in
pwCF and a target of care as BMI does not represent body
composition and can mask a low FFM and/or high FM
[23,25e36]. There is a stronger association between various
outcome parameters (such as lung function) to FFM than BMI,
and a low FFM is associated with decreased inspiratory muscle
strength [22,23,25e37].
Additionally, adults with CF that have normal weight obesity
(NWO, normal BMI <25 kg/m2

, body fat percentage >30 % in
women and 23 % in men) had even lower fat-free mass index (FFMI)
and forced expiratory volume in 1s (FEV1) in percent when
compared to overweight and obese pwCF. This indicates that

increased adiposity along with low FFM may be even more detri-
mental to pulmonary function in pwCF and that using a BMI >0 SD

as the goal to maximize overall health status including lung func-
tion can actually mask low FFM [12].

PwCF have less FFM, and bone mineral density (BMD) when
compared with controls [37]. This translates into a higher risk of
sarcopenia and osteopenia. Therefore, the assessment of body
composition may allow timely interventions to increase FFM such
as exercise and/or anabolic therapies (i.e., growth hormone after
endocrinology assessment), to reduce the risk of metabolic and
respiratory complications [27,37]. De uitleg moet geschreven zijn op het niveau van de Universiteit.

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