The association between body mass index (BMI) and pulmonary function in patients with cystic fibrosis (CF) is well established, yet BMI as the sole indicator of nutrition status fails to assess body composition, specifically fat‐free mass (FFM). Reduced FFM is a characteristic of undernutrition and is associated with decreased pulmonary function. A critical review of the literature was undertaken to explore available evidence on the use of FFM derived from dual‐energy X‐ray absorptiometry (DXA), compared with BMI, to assess pulmonary function and thereby nutrition status in patients with CF. Four cross‐sectional studies that met predefined inclusion and exclusion criteria were selected for review. Based on the evidence reviewed, reduced FFM was associated with reduced pulmonary function in both children and adults with CF. FFM was reduced among patients who may not otherwise be identified as at nutrition risk based on BMI alone. FFM, as compared with BMI, appears to be a better indicator of pulmonary function and nutrition status in patients with CF. Although future research is needed to identify and determine FFM measurements that are associated with improved pulmonary function and nutrition status in patients with CF, these findings highlight the potential value and clinical utility of using FFM measurements derived from DXA among patients with CF. Together, FFM and BMI may provide a more comprehensive picture of nutrition status during the nutrition assessment of the patient with CF.

The objective of this study was to identify the use and impact of oral nutrition supplements (ONSs) and appetite stimulants on weight status among pediatric patients diagnosed with malignancy. We performed a literature search of trials using Medline PubMed, CINAHL, Web of Science Core Collection, Scopus, and Cochrane Database of Systematic Reviews and included all prospective studies except review articles and case-reports/series that assessed ONSs or appetite stimulants among patients (0-20 years old) diagnosed with a pediatric malignancy. Databases were searched through May 17, 2022. There were six trials included with three studies related to ONS and the remaining on appetite stimulants. No studies that compared both ONS and appetite stimulants were found. To assess quality, we used the Risk of Bias in Nonrandomized Studies of Interventions and the Revised Cochrane Risk of Bias Tool for Randomized Trials depending on the study design. The studies all had pediatric patients diagnosed with a variety of malignancy types. All studies demonstrated improvement of weight status in the treatment group across various malignancy types. However, none of the studies addressed nutrition intakes outside of ONS consumption, compliance to ONSs, or frequency of ONS use. Despite the short durations (3-6 months) and significant differences in the timing of intervention initiation (ONS or appetite stimulants), these treatment modalities can improve weight status. Further research is needed to identify the best intervention for improving weight status.

Background: Cystic fibrosis (CF) is a life-shortening genetic disease, yet life expectancy has recently increased, shifting the focus to disease management and health-related quality of life (HRQoL). Identification of clinical factors, such as weight status and CF-related diabetes (CFRD), that are associated with HRQoL can inform clinicians about the patient's health perception. The goal of this systematic review was two prong: identify the association of pediatric weight status and HRQoL and determine how CFRD status impacts HRQoL. Methods: A systematic review of published research was conducted following the methodology in the Cochrane Handbook on Systematic Reviews for Interventional Studies. Results were reported according to the Preferred Reporting Items for Systematic Review and Meta-Analyses. Risk of bias was assessed using the National Heart Lung & Blood Institute tool. A meta-analysis was not performed due to variability of the inclusion/exclusion criteria, differences in outcome reporting, and insufficient primary outcome data to pool. Results: Nine studies met inclusion criteria (n = 6 explored weight status and n = 3 studied CFRD), for a total of 1585 subjects (CFRD cases = 87). Pediatric weight status was positively associated with HRQoL, most commonly the Body Image and Eating Disturbance domains. CFRD was negatively associated with HRQoL, specifically the Treatment Burden and Weight domains.Conclusions: Based on the limited data available, improved pediatric weight status appears to increase HRQoL while a CFRD diagnosis appears to decrease HRQoL.More research is needed to fully understand the role of these clinical factors on HRQoL, especially with life expectancy increasing among those with CF.

Adequate folate intake during the female reproductive years plays a vital role in fetal neurodevelopment. To address this public health concern, the FDA required enriched cereal grains to be fortified with folic acid. A positive outcome of fortification with folic acid was a reduction in the number of pregnancies affected by neural tube defects (NTDs). However, there are individuals unable to consume these enriched grains, such as those with a gluten allergy or intolerance. The need for folic acid fortification across all grains, those with and without gluten, should be considered in an effort to provide equivalent folic acid to all and further promote public health efforts aimed at decreasing the incidence of NTDs.

Sufficient vitamin D levels play an essential role in bone health and, more recently, in pulmonary function. Achieving sufficient vitamin D levels is challenging, especially among individuals with malabsorptive diseases, such as cystic fibrosis (CF). Cystic fibrosis is characterized by a progressive loss of pulmonary function and fat malabsorption, increasing the risk for vitamin D deficiency. After reviewing the literature for pediatric patients with CF, the association between vitamin D status and pulmonary function remains unclear. More studies are needed to evaluate whether adequate vitamin D status can assist in maintaining pulmonary function and slowing disease progression.

Gastroesophageal reflux (GER) is common among infants, but when symptoms become troublesome, that is defined as gastroesophageal reflux disease (GERD). Making a diagnosis of GERD is difficult because there is no gold standard. GERD can be especially problematic for infants with cystic fibrosis (CF). There are signs and symptoms (ie, malnutrition, recurring aspiration pneumonias, refusal to eat, wheezing, coughing, and asthma) in addition to invasive and noninvasive methods used to determine a diagnosis. The treatments for GERD span from nonpharmacological to surgical, with a laparoscopic Nissen fundoplication being the gold standard of surgical intervention. Although surgical interventions have been demonstrated to reduce symptoms associated with GERD, there is little known about the weight/growth‐related outcomes. This case report discusses an infant with CF and GERD requiring multiple interventions and ultimately a laparoscopic Nissen fundoplication and the weight changes from presurgery to postsurgery. The case report is expanded upon with a structured literature review of fundoplication and growth studies. There were 4 available studies that assessed changes in weight/growth before and after fundoplication among children with GERD. Because of the heterogeneity of the nutrition outcomes used, the weight/growth benefits after fundoplication are unclear at this time. Further research in needed to assess the nutrition outcomes among patients with GERD requiring fundoplication.

The increased life expectancy seen in patients with cystic fibrosis (CF) shifts the focus of nutrition interventions from supporting survival to improving quality of life. The aim of this narrative review is to explore the importance of weight status and health-related quality of life in adults with CF. This review also identifies research supporting the differences in body image based on sex, which impacts an individual's weight status and health-related quality of life. Based on the literature, the CF care team should incorporate body image screening into clinical practice to optimize weight status and increase health-related quality of life in the adult with CF.

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