Pediatric Nutrition in Practice

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placement. Calorically dense formulas (1.5–2.0
kcal/ml) are well tolerated, and, initially, nocturnal
infusion is encouraged to promote normal eating
behavior during the day. Our experience is that
once families see a success after 6–8 weeks of naso-
gastric feeding, gastrostomy placement is wel-
comed. Patients with excessive nausea, bloating or
vomiting may benefit from prokinetic drugs or
semielemental or elemental formula.


Growth Hormone and Appetite Stimulants


The efficacy of growth hormone therapy in CF
has recently been reviewed [19]. While growth
parameters and pulmonary function seem to im-
prove in treated patients, the overall benefits to
health cannot be determined from the moderate
evidence available. One recent multicenter trial in
which growth hormone was administered for 12
months to patients with reduced growth and
bone age indicated its effectiveness in improving
growth and lung volumes [20]. Larger trials with
appropriate patient selection are needed in order
to establish its safety and effectiveness.
Appetite stimulants (AS) are often requested
by individuals with CF, or by parents of a child
with CF, in anticipation of an enhanced appetite


and increased energy intake to promote weight
gain [21]. While megestrol acetate is one of the
most studied AS in CF, results are not conclusive
on the use of AS in patients with CF at this time,
and larger, controlled studies are needed.

Conclusions


  • The overall goal is that every patient with CF
    should achieve normal growth. This requires
    regular surveillance including age-specific in-
    dividualized expert advice with nutritional
    care plans to suit each patient. Nutritional in-
    tervention should be appropriately timed to
    influence the evolution of the disease

  • Nutritional support is an integral part of the
    care of patients with CF

  • At diagnosis, all patients require pancreatic
    and nutritional assessment

  • Patients must be carefully monitored and di-
    etary counseling provided

  • Nutritional evaluation and support is age re-
    lated

  • Patients who fail to respond require enteral
    supplementation

  • Nutritional status impacts on the progression
    of CF


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References

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screening for cystic fibrosis. Wisconsin
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3 Sims E, Clark A, McCormick J, et al:
Cystic fibrosis diagnosed after 2 months
of age leads to worse outcomes and re-
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Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249
DOI: 10.1159/000367876
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