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Author: Maria Mascarenhas (45)


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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Nov
2017

Practitioners often avoid administering iron dextran in parenteral nutrition (PN) for hospitalised children because of the concern for anaphylaxis. The primary aim of the present study was to determine the risk of anaphylaxis associated with exposure to PN containing iron dextran in the inpatient setting.
Charts were reviewed for all children admitted to The Children's Hospital of Philadelphia from January 1, 2011 to December 30, 2013 who received PN containing low molecular weight (LMW) iron dextran.

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Nov
2017

Rhabdomyolysis is a process of muscle destruction that can present with varying clinical manifestations. In pediatric patients, its main etiology is infectious diseases. We present a previously healthy adolescent who was admitted to our emergency department with a four-day history of myalgia, muscle weakness and dark urine.

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Oct
2017

Autoimmune pancreatitis (AIP) is an increasingly recognized disease entity, but data in children are limited. AIP presentation and outcome in children might differ from the adult experience. We aim to determine the characteristic features of AIP in children.

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Mar
2017

Parenteral nutrition (PN) represents one of the most notable achievements of modern medicine, serving as a therapeutic modality for all age groups across the healthcare continuum. PN offers a life-sustaining option when intestinal failure prevents adequate oral or enteral nutrition. However, providing nutrients by vein is an expensive form of nutrition support, and serious adverse events can occur.

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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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May
2016

The present study determined the plasma amino acid status in children with cystic fibrosis (CF) and pancreatic insufficiency (PI) in the modern medical and nutritional care setting and investigated the effect of choline supplementation on amino acid status. A total of 110 children aged 5 to 18 years with CF and PI were randomized to receive choline-enriched structured lipid (LYM-X-SORB) or placebo with similar energy and fat content. Plasma amino acids were measured at baseline and 3 and 12 months.

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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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Dec
1969

The regulation of hematopoietic stem cell (HSC) emergence during development provides important information about the basic mechanisms of blood stem cell generation, expansion, and migration. We set out to investigate the role that cytokine signaling pathways play in these early processes and show here that the 2 cytokines interleukin 3 and thrombopoietin have the ability to expand hematopoietic stem and progenitor numbers by regulating their survival and proliferation. For this, they differentially use the Janus kinase (Jak2) and phosphatidylinositol 3-kinase (Pi3k) signaling pathways, with Jak2 mainly relaying the proproliferation signaling, whereas Pi3k mediates the survival signal.

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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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Jun
2016

Integrative Nutrition for Pediatrics.

Curr Probl Pediatr Adolesc Health Care 2016 Jun 28;46(6):165-71. Epub 2016 Jan 28.
Jessi Erlichman, Amanda Hall, Amy Dean, Bridget Godwin, Maria Mascarenhas
Food is essential for life. Yet, poor food choices may cause poor health. Dietary manipulation is frequently integrated into the management of common chronic pediatric conditions.

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Nov
2015

Anthropometric measurements are vital for safe care in pediatric intensive care units.
To identify barriers to anthropometric measurements and determine if perceptions of barriers differ between ordering providers and nurses.
A 21-item survey to elicit perceptions of barriers to obtaining anthropometric measurements was distributed via e-mail to societies with members who provide care in pediatric intensive care units.

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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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Apr
2016

To study the effect of two intravenous maintenance fluids on plasma sodium (Na), and acid-base balance in pediatric intensive care patients during the first 24h of hospitalization.
A prospective randomized controlled study was performed, which allocated 233 patients to groups: (A) NaCl 0.9% or (B) NaCl 0.

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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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Nov
2014

Nutrition deficiency is common among hospitalized children. Although oral nutrition supplements (ONS) may improve malnutrition in this population, the benefits and healthcare costs associated with their use have not yet been fully explored. The objective of this study was to assess the effect of ONS use on inpatient length of stay (LOS) and episode cost in hospitalized children.

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Apr
2015

Children and adolescents with sickle cell disease (SCD) have a higher incidence of sleep pathology and obstructive sleep apnea syndrome (OSAS). The nocturnal hypoxemia is a risk to vaso-occlusive crisis among other SCD morbidities. Our aim was to compare polysomnography (PSG) results in a sample of children with SCD with a sample of children with suspected OSAS without SCD.

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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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Apr
2014

The aim of the study was to assess the impact of LYM-X-SORB (LXS), an organized lipid matrix that has been shown to be absorbable without pancreatic enzyme therapy on fat-soluble vitamin status in children with cystic fibrosis (CF) and pancreatic insufficiency (PI).
Children with CF and PI were randomized to daily LXS or an isocaloric placebo comparison supplement for 12 months. Serum vitamins A (retinol), D (25-hydroxyvitamin D[25D]), E (α-tocopherol, α-tocopherol:cholesterol ratio), and K (percentage of undercarboxylated osteocalcin [%ucOC] and plasma proteins induced by vitamin K absence factor II [PIVKA II]) were assessed at baseline and 12 months.

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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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Apr
2013

In Cavernous transformation of portal vein (CTPV), therapeutic options are limited; however the restoration of circulation by mesenteric-portal bypass (MPB) is an option in selected cases. CASE REPORT 1: 13-year-old female with polymalformative syndrome. Admission at 4 months of age to Intensive Care Unit due to severe pneumonia with hemodynamic instability.

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Sep
2012

A tuberculoma is a rare form of presentation of tuberculosis (TB) in children. We describe the case of a 13-year-old girl, with 3 weeks of progressive tiredness and asthenia and a 48 h fever and cough. Physical examination revealed diminished pulmonary sounds in the left lower hemithorax.

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Jul
2012

High-output cardiac failure is rare in newborns. Emergent diagnosis and management of this pathology is crucial. We report the case of a child, currently 12-months old; obstetric background is non-contributory.

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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
2012

Local anesthetics (LA) are frequently used in medical and surgical and dental procedures. Adverse reactions to LA are rare, and hypersensitivity reactions are very rare. Nevertheless, they are the third more frequent cause of referral to Allergy Clinics due to drug allergy, after betalactamic antibiotics and non-steroidal anti-inflammatory agents.

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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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Oct
2009

Since the identification of cystic fibrosis (CF) in the 1940s, nutrition care of patients who have CF has been a challenge. Through optimal caloric intake and careful management of malabsorption, patients are expected to meet genetic potential for growth. Yet factors beyond malabsorption, including nutrient activity at the cellular level, may influence growth and health.

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Nov
2009

The first adult-repopulating hematopoietic stem cells (HSCs) are detected starting at day 10.5 of gestation in the aorta-gonads-mesonephros (AGM) region of the mouse embryo. Despite the importance of the AGM in initiating HSC production, very little is currently known about the regulators that control HSC emergence in this region.

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Nov
2009

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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Nov
2009

To determine the prevalence of exclusive breastfeeding during the first 3 months of life and its determinant factors in a city in the South region of Brazil.
Prospective study of a cohort of babies born between September 2002 and May 2003 in the city of Pelotas, RS. Data were obtained in interviews, at maternity units and during home visits, with mothers of babies aged between 1 and 3 months.

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Dec
2005

Eosinophilic esophagitis (EoE) is a disorder characterized by a severe, isolated eosinophilic infiltration of the esophagus unresponsive to aggressive acid blockade but responsive to the removal of dietary antigens. We present information relating to our 10-year experience in children diagnosed with EoE.
We conducted a retrospective study between January 1, 1994, and January 1, 2004, to evaluate all patients diagnosed with EoE.

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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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Oct
2002

Understanding cytokine production patterns in early mucosal lesions of pediatric patients newly diagnosed with inflammatory bowel disease (IBD) may be critical to understanding IBD pathogenesis. Interleukin-6 (IL-6) has a central role in a multitude of immune system reactions; however, inconsistent lamina propria and serum IL-6 has been reported in IBD patients. Newly diagnosed pediatric IBD patients have not previously been evaluated for lamina propria or serum IL-6.

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Feb
2002

Eosinophilic esophagitis is a disease entity in which patients have (1) elevated eosinophils on esophageal biopsy and (2) symptoms of gastroesophageal reflux. The symptoms do not improve on aggressive acid blockade but do improve on elimination diet or corticosteroid treatment, which tentatively links food allergies to this disorder.
The purpose of this study was to identify potential food antigens in eosinophilic esophagitis.

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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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