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Diet and Nutrition: Metabolic Diseases---2nd Edition

A special issue of Nutrients (ISSN 2072-6643). This special issue belongs to the section "Nutrition and Metabolism".

Deadline for manuscript submissions: 25 June 2025 | Viewed by 2163

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Research Group on Foods, Nutritional Biochemistry and Health, Universidad Europea del Atlántico, 39011 Santander, Spain
Interests: nutrition and metabolism; clinical nutrition; dietetics; diet therapy
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Special Issue Information

Dear Colleagues,

Nutrition (food) is composed of proteins, carbohydrates, and fats, broken down by enzymes in the digestive system. The body uses these to store them in the liver, muscle tissues, or body fat.

Metabolic disorders are caused by deficiencies or excesses of nutrients that alter our state of health, deficiencies of the enzymes necessary for a specific chemical reaction, abnormal chemical reactions that make metabolic processes difficult, and organ diseases in the liver, pancreas, or endocrine glands.

The most common metabolic diseases are the two types of diabetes. The cause of Type 1 is unknown, and Type 2 can be acquired or potentially triggered by genetic factors. Prediabetes, metabolic syndrome, non-alcoholic fatty liver disease, dyslipidemia, and arterial hypertension are the principal pathologies that are related to overweight and obesity, especially with abdominal fat distribution.

Glucose–galactose malabsorption, which creates a defect in transporting glucose and galactose through the stomach lining, causes severe diarrhea, with it being necessary to remove lactose, sucrose, and glucose from the diet. Phenylketonuria (PKU), caused by the inability to produce the enzyme phenylalanine hydroxylase, causes organ damage and mental retardation, and maple syrup urine disease (MSUD) disrupts the metabolism of certain amino acids, causing the degeneration of neurons.

Papers addressing these topics are invited to this Special Issue, especially those combining the relation of a person’s diet and nutrition in the prevention or the treatment of metabolic disorders.

Prof. Dr. Iñaki Elío
Guest Editor

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Keywords

  • obesity
  • overweight
  • metabolic syndrome
  • prediabetes
  • diabetes
  • bariatric surgery
  • dyslipidemia
  • eating behavior
  • non-alcoholic fatty liver
  • phenylketonuria
  • maple syrup urine disease
  • glucose-galactose malabsorption
  • homocystinuria
  • urea cycle disorder

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Published Papers (1 paper)

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21 pages, 1937 KiB  
Article
UK Patient Access to Low-Protein Prescription Foods in Phenylketonuria (PKU): An Uneasy Path
by Sharon Evans, Cameron Arbuckle, Catherine Ashmore, Sarah Bailey, Giana Blaauw, Wahid Chaudhry, Clare Dale, Anne Daly, Breanna Downey, Jane Dundas, Charlotte Ellerton, Suzanne Ford, Lisa Gaff, Joanna Gribben, Anne Grimsley, Melanie Hill, Laura Murphy, Camille Newby, Natalia Oxley, Rachel Pereira, Alex Pinto, Rachel Skeath, Alexa Sparks, Simon Tapley, Allyson Terry, Georgina Wood, Alison Woodall, Katie Yeung and Anita MacDonaldadd Show full author list remove Hide full author list
Nutrients 2025, 17(3), 392; https://doi.org/10.3390/nu17030392 - 22 Jan 2025
Viewed by 1622
Abstract
Background: Special low-protein foods are essential in the dietary treatment of phenylketonuria (PKU). In the UK, these are available on prescription through the General Practitioners (GPs) and distributed via nutritional home delivery companies or pharmacies. Methods: A 58-item online non-validated semi-structured questionnaire was [...] Read more.
Background: Special low-protein foods are essential in the dietary treatment of phenylketonuria (PKU). In the UK, these are available on prescription through the General Practitioners (GPs) and distributed via nutritional home delivery companies or pharmacies. Methods: A 58-item online non-validated semi-structured questionnaire was emailed to British Inherited Metabolic Disease Group (BIMDG) dietitians and dietetic support workers (DSW)/administrators working in PKU to ascertain the main system issues and errors with the supply of low-protein prescription foods (LPPF). Results: 73% (n = 53/73) of dietitians and 72% (n = 18/25) of DSW/administrators responded. A total of 80 questionnaires (representing 44 paediatric and 36 adult PKU centres) were completed. A total of 50% (n = 40/80) of respondents reported patient/caregiver problems accessing LPPF at least weekly. The most common problems were unavailable products (82%), missing LPPF in deliveries (79%), and delayed deliveries (66%). For 64% of respondents, >25% of their patients had recurring problems accessing LPPF, and 69% of respondents spent ≥1 h/week and 11% >5 h/week correcting LPPF patient supply issues. The most common foods patients experienced supply issues with were bread (96%), pasta/rice (41%) and milk replacements (35%). This was associated with GP prescription errors (65%), LPPF prescriptions sent to incorrect dispensers/suppliers (60%), and manufacturer supply issues (54%). Problems with patients/caregivers included not ordering LPPF in a timely way (81%), not responding to messages from home delivery companies (73%) and poor understanding of the ordering process (70%). The majority (93%) of respondents reported that prescription issues impacted their patients’ blood Phe control. Suggestions for improving access to LPPF included centralisation of the system to one supplier (76%) and apps for ordering LPPF (69%). Conclusions: The supply of LPPF for PKU in the UK is problematic; it may adversely affect the ability of patients to adhere to dietary management, and a review investigating patient access to LPPF is urgently required. Full article
(This article belongs to the Special Issue Diet and Nutrition: Metabolic Diseases---2nd Edition)
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