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Author: Maria R Mascarenhas (21)


Nov
2017

Close monitoring of nutritional status is critical to the overall health of a patient with CF. As part of routine CF care, measurement of weight and height (and calculation of weight/length or BMI as appropriate) should be performed and analyzed at each visit. Early recognition of nutritional risk is imperative and evaluation with a multidisciplinary team should be performed to assess for caloric intake, caloric malabsorption, and other causes of poor weight gain and growth.

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Dec
1969

Cystic fibrosis (CF) is associated with different gastrointestinal motility disturbances and syndromes. We aim to assess gastric emptying in patients with CF compared to healthy controls by a systematic review of existing literature. Medical databases and abstracts from major gastroenterology and CF meetings were reviewed.

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Dec
2016

Pancreatic enzyme therapy does not normalize dietary fat absorption in patients with cystic fibrosis and pancreatic insufficiency. Efficacy of LYM-X-SORB (LXS), an easily absorbable lipid matrix that enhances fat absorption, was evaluated in a 12-month randomized, double-blinded, placebo-controlled trial with plasma fatty acids (FA) and coefficient of fat absorption (CFA) outcomes. A total of 110 subjects (age 10.

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Mar
2016

Nutritional Issues in Cystic Fibrosis.

Clin Chest Med 2016 Mar 24;37(1):97-107. Epub 2015 Dec 24.
Missale Solomon, Molly Bozic, Maria R Mascarenhas
The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition.

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Apr
2016

Choline depletion is seen in cystic fibrosis (CF) and pancreatic insufficiency in spite of enzyme treatment and may result in liver, fatty acid, and muscle abnormalities. This study evaluated the efficacy and safety of an easily absorbed choline-rich structured lipid (LYM-X-SORB™ [LXS]) to improve choline status.
Children with CF and pancreatic insufficiency were randomized to LXS or placebo in a 12-month double blind trial.

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Aug
2015

The malabsorption blood test (MBT), consisting of pentadecanoic acid (PA), a free fatty acid, and triheptadecanoic acid (THA), a triglyceride that requires pancreatic lipase for absorption of the heptadecanoic acid (HA), was developed to assess fat malabsorption in patients with cystic fibrosis (CF) and pancreatic insufficiency (PI). The objective was to construct a population pharmacokinetic (PK) model to describe PA and HA disposition in healthy subjects and CF subjects. A model was simultaneously fit to PA and HA concentrations, consisting of 1-compartment disposition and a transit model to describe absorption.

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Sep
2014

The study aim was to determine the effect of a dietary intervention on growth, body composition and resting energy expenditure (REE) in children with cystic fibrosis (CF) and pancreatic insufficiency (PI) in a randomized, double blind, placebo-controlled trial.
Subjects (5 to 17 yrs) participated in a 12-month trial of the organized lipid matrix LYM-X-SORB™ (LXS) vs. placebo dietary supplements with similar calories, total fat and fatty acids.

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Jun
2014

Unexpectedly high serum B12 concentrations were noted in most study subjects with cystic fibrosis (CF) and pancreatic insufficiency (PI) participating in a nutrition intervention at the baseline evaluation. The objectives of this study were to determine dietary, supplement-based, and enzyme-based B12 intake, serum B12 concentrations, and predictors of vitamin B12 status in children with CF and PI.
Serum B12 status was assessed in subjects (5-18 years) and categorized as elevated (serum B12 above reference range for age and sex [Hi-B12]) or within reference range (serum B12 within reference range for age and sex) for age and sex.

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Jul
2013

Gastrointestinal disturbances are common in people with cystic fibrosis (CF); however, motility studies in this population have yielded inconsistent results. This study examined gastric emptying (GE) and small bowel transit (SBT) time in children with CF and pancreatic insufficiency compared with a healthy adult reference group.
Participants consumed an 8-ounce liquid test meal (approximately 550 calories, 32 g of fat) labeled with 300 μCi 99m technetium (Tc) sulfur colloid.

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Apr
2013

A Malabsorption Blood Test (MBT) is proposed as an alternative method to the 72-hour stool and dietary collection for assessing the degree of fat malabsorption in people with pancreatic insufficiency. The MBT consists of a simultaneous oral dose of pentadecanoic acid (PA), a free fatty acid, and triheptadecanoic acid (THA), a triglyceride with three heptadecanoic (HA) saturated fatty acids requiring hydrolysis by pancreatic lipase before HA can be intestinally absorbed. The aim of this study is to demonstrate the ability of MBT to detect fat malabsorption in healthy adult subjects using the pancreatic lipase (PL) inhibitor Orlistat (Xenical®), and in subjects with CF and PI while on and off routine pancreatic enzyme doses.

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Feb
2013

Pediatric gastroenterology is the only pediatric subspecialty with nutrition as part of its official curriculum and objective; however, pediatric gastroenterology fellows believe that their baseline knowledge in nutrition is suboptimal. The purpose of the present study was to assess the perceived effectiveness of nutrition training among pediatric gastroenterologists, identify areas of need for additional education, and determine the perceived role of the gastroenterologist in obesity management.
A survey was sent to members and fellows of the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition to assess general nutrition education as well as obesity management and educational needs.

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Aug
2012

The aim of the study was to assess the methodology and content of nutrition education during gastroenterology fellowship training and the variability among the different programs.
A survey questionnaire was completed by 43 fellowship training directors of 62 active programs affiliated to the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition, including sites in the United States, Canada, and Mexico. The data were examined for patterns in teaching methodology and coverage of specific nutrition topics based on level 1 training in nutrition, which is the minimum requirement according to the published North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition fellowship training guidelines.

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Aug
2010

Childhood and adolescence are important periods for bone development. Any disease that affects bone health has the potential to affect the bones not only in the short term but also later in life. Bone health abnormalities in patients with inflammatory bowel disease are being increasingly recognized.

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Aug
2010

The nutrition care of children who are neurologically impaired is a challenge for the nutrition care team. Many factors should be considered in the assessment and development of a nutrition plan. That these children can have significant abnormalities in nutrition status, growth, and body composition should be kept in mind.

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Aug
2003

Refeeding syndrome has been well documented over the years, primarily through case reports and literature reviews. Awareness of refeeding syndrome is crucial in preventing the occurrence of, and the metabolic and physiologic complications associated with, aggressive nutrition support in malnourished populations. Once compromised patients have been identified to be at risk of refeeding syndrome, nutrition rehabilitation should be cautiously initiated.

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Oct
2005

Poor nutrition and growth status are common in people with cystic fibrosis (CF), and females often have a worse clinical course. Relationships between sexual maturity, nutrition, resting energy expenditure (REE), and pulmonary status in females with CF and pancreatic insufficiency (PI) were evaluated.
Pre- and post-menarcheal females with CF and PI (8-29 yr) were compared to healthy females.

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Oct
2003

Treatment of Gastrointestinal Problems in Cystic Fibrosis.

Curr Treat Options Gastroenterol 2003 Oct;6(5):427-441
Maria R. Mascarenhas
The gastrointestinal (GI) manifestations of cystic fibrosis (CF) are varied and include pancreatic insufficiency, meconium ileus, distal intestinal obstruction syndrome (DIOS), liver disease, and other less common manifestations. Treatment of pancreatic insufficiency consists of providing appropriate pancreatic enzyme replacement therapy and may include raising duodenal pH to allow for optimal action of these enzymes. Despite a number of pancreatic enzyme replacement products, malabsorption cannot be normalized.

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Feb
2002

The past decade has seen a paradigm shift in the goals of nutritional care from basic delivery of macronutrient calories to the provision of specific nutrients, which, in addition to supporting growth, have health and disease modulating effects. The appreciation of the central role of nutrition in heath and disease may lead to increased use of a new array of diets that are more specific for underlying health and disease pathophysiologic processes. An improved understanding of disease-specific pathophysiology, and limited but promising data from ongoing clinical trials, are fueling this positive change.

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Feb
2002

During the past decade, a tremendous wealth of information regarding the pathogenesis, genetics, and therapy of IBD has been discovered. Judging by the number of new publications published every month in scientific journals and the great enthusiasm at scientific meetings, this outstanding pace surely will continue. In the near future, clinicians may be able to classify IBD into several subtypes depending on patients' cytokine and gene profiles.

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