China
Africa
Explore chapters and articles related to this topic
Glutaric Acidemia/Glutathione Synthetase Deficiency
Published in Charles Theisler, Adjuvant Medical Care, 2023
Diet: High-protein foods such as meat, fish, cheese, eggs, and nuts need to be eliminated.3 Strict dietary control may help limit progression of the neurological damage. Restriction of lysine, hydroxylysine, and tryptophan is essential. Supplementation with amino acid based formulas provide energy, nitrogen, vitamins, and minerals which can promote anabolism and growth.4
Malnutrition
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Laura Gearman, Catherine Larson-Nath
When providing enteral nutrition via a feeding tube, consideration should be given to type of formula, route of delivery, and feeding regimen to provide the most appropriate intervention to treat the etiology of malnutrition. For children whose main cause of malnutrition is inadequate oral intake, a standard formula is likely appropriate. A partially hydrolyzed or amino acid-based formula may be appropriate for children whose cause of malnutrition is related to altered gut function or intolerance to standard formula or foods. Enteral nutrition may be used to supplement oral intake or provide 100% of the patient’s nutrition needs for treatment of malnutrition. In the acute setting, the use of a nasogastric or nasojejunal tube is recommended to provide tube feeding formula. If the duration of malnutrition is chronic and/or not improving with previous interventions and if tube feeding is required for a longer period of time, a gastrostomy or gastrojejunostomy tube may be more appropriate. Feeding regimen should be determined for each patient individually based upon clinical condition and history. Bolus tube feeding schedules are the most physiologic; however, patients with malabsorption or intolerance of gastric feeding as a cause of malnutrition may require continuous feedings. See Chapter 8 for more information on enteral nutrition.
What GPs need to know about breastmilk substitutes
Published in Amy Brown, Wendy Jones, A Guide to Supporting Breastfeeding for the Medical Profession, 2019
The majority of infants diagnosed with cows’ milk protein allergy will require an extensively hydrolysed formula (which is prescribable and costs about £20/800 g). Very few will need an amino acid-based formula but these are heavily marketed as first-line treatment (although they often cost £60/800 g). For details of all specialist milks on the UK market suitable for infants see Specialised Infant Milks in the UK at www.firststepsnutrition.org/composition-claims-and-cost.
Real food in enteral nutrition for chronically ill children: overview and practical clinical cases
Published in Current Medical Research and Opinion, 2022
Valeria Dipasquale, Antonella Diamanti, Chiara Maria Trovato, Domenica Elia, Claudio Romano
A 22-month-old girl was diagnosed with nemaline myopathy, which is caused by a homozygous missense truncating mutation in TNNT1. Her older sister had the same mutation and disease, and both parents had a single mutation of the gene. Progressive and severe muscle weakness and wasting, respiratory insufficiency, pectus carinatum deformities, and failure to thrive manifested by the age of four months. For these reasons, she was admitted to the hospital at the age of 19 months and given a tracheostomy and a percutaneous endoscopic gastrostomy (PEG). She began to be ventilated mechanically 24 h a day. She was also started on a special diet consisting of blended natural food via PEG and nocturnal enteral nutrition consisting of 240 ml of a semi-elemental formula delivered at a rate of 30 ml/h for 8 h. No complications (i.e. gastrostomy tube obstruction, microbial contamination episodes) were observed with the use of blended natural food. The semi-elemental formula was not tolerated; thus, an amino acid-based formula was chosen instead.
Pathophysiology of eosinophilic esophagitis: recent advances and their clinical implications
Published in Expert Review of Clinical Immunology, 2019
Melanie A Ruffner, Katie Kennedy, Antonella Cianferoni
Food allergy has been shown in numerous studies to be a common trigger of EoE, because dietary restriction therapies have been successfully used globally as effective treatment options [23,24,72–78]. Elemental diets have been shown in several reports to induce histological remission in both children and adults with EoE [24,76,79]. This strategy employs an amino acid-based formula and is difficult to adhere to, especially for older children and adult patients. As such, multi-food elimination diets have come into play as more realistic options for patients that have also been shown to improve symptoms [23,24,72–78]. Studies have shown clinical improvement with both 6-food (milk, wheat, egg, soy, peanut/treenut, fish/shellfish) and comparatively 4-food (milk, wheat, egg, soy) elimination diets with a recent prospective study showing benefit to 2-food elimination (milk, wheat) with step-wise additional restriction if clinically necessary [23,24,72–78]. Sequential single food reintroduction once remission is achieved in order to identify the trigger is necessary. In EoE like in food allergic disease, immunological response to food is very reproducible [23,24,72–78] and food allergens have been demonstrated to have a causative role in EoE following Koch’s postulate as demonstrated by clinical and endoscopic resolution of EoE once the food is removed and exacerbation when the same food is reintroduced [11,23,73,74].
Cow milk protein allergy and other common food allergies and intolerances
Published in Paediatrics and International Child Health, 2019
Wiparat Manuyakorn, Pornthep Tanpowpong
Allergen avoidance is the first treatment for food allergy. Parents and caregivers need to be instructed to read food labels and to avoid food cross-contamination when preparing food. Children who experience FDEIA should be instructed to avoid exercise 2 hours after consuming the causative food [10]. Proper nutritional support is needed. Infants with cow milk allergy can be breastfed unless the mother is ingesting cow milk and dairy produce as cow milk protein has been shown to be detected in breast milk up to 7 days after a single dose of cow milk [8]. Alternatively, an appropriate infant formula such as an extensively hydrolysed formula or amino acid-based formula can be used. Soy formula may also be considered after 6 months of age [20]. Goat milk or other mammalian milk such as buffalo is not recommended because of high homology and cross-reactivity to cow milk [21]. However, only the causative food should be avoided. Avoidance of previously tolerated food in children with atopic dermatitis has been shown to increase the risk of an immediate reaction to an avoided food after reintroduction [22]. There is a high degree of cross-reactivity between peanuts and tree nuts – 25 to 50% of patients with peanut allergy are co-allergic to tree nuts (e.g. walnuts and pecans or pistachio and cashew nuts). However, patients with peanut allergy can generally tolerate other legumes, including soy. As a result, the empirical avoidance of all legumes is not recommended in peanut allergy patients [17].