Updates in Nutrition Management of Cystic Fibrosis in the Highly Effective Modulator Era.

Attainment and maintenance of good nutrition has been an important aspect of management in cystic fibrosis (CF) for decades. In the era of highly effective modulator therapy for CF, the quality of the nutrients we recommend is increasingly important. Our therapy must support our patients' health for many years beyond what we previously thought. Preventing cardiovascular disease, reducing hyperlipidemia, and optimizing lean body mass for active, longer lives now join the long-standing goal of promoting lung function through nutrition. This chapter summarizes recent developments in nutrition in people with CF, with an eye to the evolution of our practice.

Update on the management of vitamins and minerals in cystic fibrosis.

Advancements in respiratory and nutrition management have significantly improved the survival of patients with cystic fibrosis (CF). With the availability of several nutrition interventions such as oral/enteral nutrition supplements, enteric-coated pancreatic enzymes, and water-miscible CF-specific vitamin supplements, frank vitamin deficiencies-with the exception of vitamin D-are rarely encountered in current clinical practice. Whereas they were previously considered as micronutrients, our current understanding of fat-soluble vitamins and minerals as antioxidants, immunomodulators, and disease biomarkers has been evolving. The impact of highly effective modulators on the micronutrient status of patients with CF remains elusive. This narrative review focuses on the updates on the management of fat-soluble vitamins and other micronutrients in CF in the current era and identifies the gaps in our knowledge.

Eosinophilic esophagitis in cystic fibrosis: A case series with long-term follow-up.

Challenging mealtime behaviors in young children and difficulties in meeting their dietary intake recommendations are sources of parenting stress and associated with negative quality of life. The gastrointestinal (GI) manifestations of cystic fibrosis (CF) can often present similarly to a GI pathology unrelated to CF. Specifically, this case series focuses on three toddlers with CF who presented with oral aversion and challenging mealtime behaviors and later were diagnosed with eosinophilic esophagitis (EoE). Though EoE often presents with dysphagia, younger patients commonly present with nonspecific GI symptoms such as regurgitation, emesis, abdominal pain, failure to thrive, food intolerance, and oral aversion. Given the overlap of GI manifestations in CF and EoE, it can be challenging for clinicians to diagnose the coexistent EoE in patients with CF. We describe the presenting symptoms, treatment, and successful outcomes of three pediatric patients with CF and EoE. To our knowledge, this is the second case series with a detailed description of EoE in CF.

Dysprosium chloride as a nonabsorbable gastrointestinal marker for studies of stable isotope-labeled triglyceride excretion in man.

OBJECTIVE: The aim of this work was to determine if dysprosium chloride (DyCl(3)) is a suitable nonabsorbable marker for studies of labeled-triglyceride excretion in cystic fibrosis patients allowing excretion to be determined accurately after analysis of one or two stools. METHODS: A series of 66 absorption studies were conducted in 36 young cystic fibrosis patients over a five year period. All tests consisted of ingesting a single test meal containing both (13)C-labeled triglyceride (TG*) and DyCl(3); in most studies the food colorant brilliant blue (FD&C blue #1) was administered along with the DyCl(3). Ingestion of the test meal was followed by collection of individual stools for 72 to 96 hours. Stools were analyzed for (13)C-Excess ((13)C*) and Dy. RESULTS: Excretion of Dy in cystic fibrosis patients who exhibited a wide-range of steatorrhea was quantitative. Fractional excretion of Dy and (13)C* in individual stools showed a high linear correlation (r(2) = 0.969) with a slope and y-intercept close to unity and zero, respectively. As a result, estimates of TG* excretion based on analysis of only two stools (partial pool method, PPM) were not different from those based on the analysis of all stools or stool composites. This was true both when Dy content and when stool color due to ingested brilliant blue was used to determine which stools to analyze for the PPM. CONCLUSIONS: Combining the use of Dy and brilliant blue permits reasonably accurate estimates of fecal TG* excretion after analysis of samples from two easily identified stools. This practical method can be used to address many important clinical and experimental questions regarding triglyceride digestion and absorption that may otherwise go unanswered.

Effect of triglyceride structure on fecal excretion of 13C-labeled triglycerides.

OBJECTIVE: The aim of this work was to determine the effects of specific changes in the structure of (13)C-labeled triglyceride (TG*) on its fecal excretion relative to total stool fat excretion determined simultaneously in patients with reduced exocrine pancreatic function. METHODS: A series of 47 studies were conducted in 26 young cystic fibrosis (CF) patients and 11 adult patients with chronic pancreatitis over a five year period. Each test consisted of ingesting a single high fat test meal containing both (13)C-labeled triglyceride (TG*) and dysprosium chloride (DyCl(3)) a nonabsorbable marker of intestinal transit; in most studies the food colorant brilliant blue (FD&C blue #1) was administered along with the DyCl(3). The TG*s tested were: P*P*P* = TRIPALMITIN-1,1,1-(13)C(3); SO*S = 2-OCTANOYL-1,3-DISTEARIN-2-octanoyl-1,2-(13)C(2); and P*LP* = 2-LAURYL-1,3-DIPALMITIN-dipalmitoyl-1,1,2,2-(13)C(4). Ingestion of the test meal was followed by collection of individual stools for at least 72 hours. Stools were analyzed for (13)C-Excess ((13)C*), total fat, and Dy. RESULTS: Excretion of P*LP* showed a high degree of linear correlation with stool fat (r(2) = 0.924) over a wide-range of fecal fat values. Excretion of SO*S was also significantly correlated with stool fat, but its excretion was less than 10% at all levels of steatorrhea and the slope of the regression line relating TG* excretion to stool fat was some four to five times smaller than observed for P*LP*. Fecal excretion of P*P*P* was highly correlated with stool fat (r(2) = 0.941) in patients with moderate steatorrhea (<25 g fat/24 hours) and the slope of the regression line (3.20) was considerably greater than for P*LP*. Only results from those studies in which stool collections were complete (Dy excretion >90%) were utilized in the statistical comparisons (36 of 47 studies). CONCLUSIONS: The observed highly significant linear correlation between P*LP* and stool fat over the entire range of steatorrhea suggests that P*LP* excretion may be a suitable surrogate for fecal fat in patients with reduced exocrine pancreatic function. Because fecal excretion of TG* administered as described can be accurately determined by sampling only two visually marked stools, development of a noninvasive test to replace the current 72-hour stool fat test using this approach is possible. Use of other engineered TG*s and/or labeled fatty acids, may provide a method for non-invasive in vivo assessment of the specific defect(s) leading to steatorrhea in other patient groups.