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Inborn Errors of Metabolism
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Surekha Pendyal, Areeg Hassan El-Gharbawy
The goal of nutrition therapy in UCDs is to maintain stable metabolic control and promote normal growth and development through a nutritionally complete, low-protein diet. Adequate protein and energy supply can be based on the FAO/WHO/UNU 2007 Safe Levels of Protein Intake (Table 23.3). Over-restriction of protein may compromise growth and well-being and can cause metabolic instability. Medical foods used in UCDs contain only essential amino acids (EAA) and do not contain the non-EAA to reduce nitrogen burden. Medical foods are an important source of energy, vitamins, and minerals in patients with UCDs. About 40%–50% of protein allowance is provided by the medical food and the remaining protein is provided as vegetables, fruits, and grains. However, optimal ratio and protein intake must be determined by individual titration. The daily protein is divided equally between three to four meals, and a late-night snack shortens the overnight fast. Medical foods are given with meals for maximum utilization.
Cases
Published in Ira Bedzow, Giving Voice to Values as a Professional Physician, 2018
To avoid any of the rationalizations that Roberta may give, Dr. Delgado should be prepared with the information needed to show Roberta why giving Thomas formula or banked breast milk is in his best interests. She should also have information about which formulas to use and how to purchase them. For example, if Roberta qualifies for it, Medicaid covers the cost of infant formula, though each state’s Medicaid program has its own criteria regarding coverage of infant formula. Also, some states have laws requiring insurance providers to cover certain types of medical food based on a child’s diagnosis. The Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) may also be a resource for Roberta to help her with supplemental foods, health care referrals, and nutrition education.3 The Human Milk Banking Association of North America (HMBANA) may also be able to help Roberta receive breastmilk for Thomas, even if she cannot afford to pay for it.4 Dr. Delgado may not have all of the information needed to advise Roberta properly, but she can ask other members of the healthcare team, such as a lactation consultant, social worker, or patient manager to help provide Roberta with the information that she would need.
Nutritional and Alternative Medicine
Published in Aruna Bakhru, Nutrition and Integrative Medicine, 2018
The FDA should broaden its restrictive policies that favor pharmaceuticals over natural products. It should expand the category of “medical foods” to encompass a wider array of nutritional products, and eliminate antiquated aspects of medical food regulation. Medical foods provide patients with specially formulated nutritional products designed to assist in the management of disease conditions. Medical foods therefore represent the rare instance where a nutritional product can be associated with a “disease” condition without also becoming a “drug.” Medical foods do not treat disease; rather, they help manage those disease states. They are intended to meet distinctive nutritional requirements of a disease or condition, and used under medical supervision.
Preclinical developments of enzyme-loaded red blood cells
Published in Expert Opinion on Drug Delivery, 2021
Luigia Rossi, Francesca Pierigè, Alessandro Bregalda, Mauro Magnani
Met depletion induces many modifications in tumor cells, including cell arrest in the S and G2 phases of the cell cycle, apoptosis, decrease of glutathione (GSH) content, and reduced activity of the O6-methylguanine-DNA methyltransferase (MGMT) [32,33]. In addition to inhibiting cancer cell growth, an increasing efficacy of chemo- and radiotherapy has been observed in animal models following MR [34]. Moreover, the avidity of aggressive brain tumor cells for Met led to use 11C-Met positron emission tomography (PET) in the clinical management of glioblastoma. Some phase I–II clinical trials of dietary Met restriction in association with chemotherapeutic drugs were carried out in patients with solid tumors and demonstrated feasibility, good safety, and good compliance of the Met-free diet on short period (1–3 days every 2 weeks) [35–37]. Differently, patients found it difficult to maintain sufficient energy needs and experienced weight loss, early satiety, and lack of appetite in a long-term study of Met restriction [38]. A phase 2 clinical study (ClinicalTrials.gov Identifier: NCT03186937) is recruiting patients with breast cancer to confirm that MR enhances cell surface expression of TRAIL receptor-2 in triple-negative breast cancer. The overall aim of this study is to establish the feasibility and acceptability of the dietary intervention, which is based on methionine-free amino acid-modified medical food (Hominex-2, Abbott Nutrition) up to 3 weeks.
How oral probiotics affect the severity of an experimental model of progressive multiple sclerosis? Bringing commensal bacteria into the neurodegenerative process
Published in Gut Microbes, 2020
Leyre Mestre, Francisco J. Carrillo-Salinas, Ana Feliú, Miriam Mecha, Graciela Alonso, Carmen Espejo, Laura Calvo-Barreiro, José L. Luque-García, Héctor Estevez, Luisa María Villar, Carmen Guaza
In the last decade, evidence has emerged of a relationship between gut microbiota and MS, both in patients and in animal models.4,5,18,19 In addition, there is significant support for the gut microbiota playing a critical role in shaping the immune system.20–22 Several reports described, in EAE models, a therapeutic effect of multi-strain probiotics15 and specific bacterial strains like Bacteroides,9,10Prevotella11 or Lactobacillus. However, the involved mechanisms for their benefits seem to be different. For example, while L. paracasei DSM13434 and L. plantarum DSM 15312 increased TGF-β1 in the blood, L. plantarum DSM 15313 enhanced blood levels of IL-27.12 Until date the only probiotic considered a kind of medical food is Vivomixx23.Here, we proved the efficacy of Vivomixx treatment in a model of progressive MS whereby oral administration of this probiotic, during the chronic phase improved the motor disability likely associated to changes in gut microbiota.
Health benefits of Lactobacillus rhamnosus GG and Bifidobacterium animalis subspecies lactis BB-12 in children
Published in Postgraduate Medicine, 2020
Probiotics are frequently marketed as dietary supplements and thus are not generally subject to pharmaceutical regulations; however, probiotics can also be included in foods, medical foods, and drugs [22]. Children’s status as a vulnerable population has prompted the development of guidelines for probiotic use, with many recommending their use to reduce the incidence or severity of necrotizing enterocolitis, acute gastroenteritis (AGE), and AAD [23–29]. We conducted a narrative review that summarizes data of the efficacy of two frequently used probiotic strains – Lactobacillus rhamnosus GG (LGG) and Bifidobacterium animalis subspecies lactis BB-12 (BB-12) [30] – when administered to children. This overview focuses on the prevention or treatment of gastrointestinal (GI) disorders, respiratory infections, otitis media, and growth parameters in infants. In addition, we assess data related to the safety of administering LGG and BB-12 as probiotics to children.