Results 131 to 140 of about 13,480 (257)

Consensus Guidelines for Diagnosis and Management of Pyruvate Dehydrogenase Complex Deficiency

open access: yesJournal of Inherited Metabolic Disease, Volume 49, Issue 6, November 2026.
ABSTRACT Primary pyruvate dehydrogenase complex deficiency (PDCD) comprises a group of monogenic disorders caused by pathogenic variants in genes encoding subunits of, or regulatory components affecting, the pyruvate dehydrogenase complex. The clinical phenotype spans a broad continuum, ranging from early onset congenital lactic acidosis to infantile ...
Nandaki Keshavan   +23 more
wiley   +1 more source

Amino Acid Deficiency Secondary to Continuous Venovenous Hemofiltration in Acute Decompensation of Organic Acidemias: An Anabolic Dead End? [PDF]

open access: yesCrit Care Explor
Grosyeux C   +11 more
europepmc   +1 more source

Essential Oral Single Nutritional Therapy Products for Inherited Metabolic Diseases: Evidence and Consensus Assessment Using a Modified Delphi Method

open access: yesJournal of Inherited Metabolic Disease, Volume 49, Issue 6, November 2026.
ABSTRACT Nutritional therapy is critical in managing inherited metabolic diseases (IMDs), and includes specialized diets and single nutritional therapy products (sNTPs) such as vitamins, cofactors, and amino acids. Many sNTPs function as medicines, but are regulated as food (e.g., food supplements), which can limit access, reimbursement, and consistent
Nina N. Stolwijk   +39 more
wiley   +1 more source

A species of <i>Coprococcus</i> is related to BMI in patients who underwent malabsorptive bariatric surgery and its abundance is modified by magnesium and thiamin intake. [PDF]

open access: yesFront Endocrinol (Lausanne)
Suárez-Sánchez F   +10 more
europepmc   +1 more source

Tailored Amino Acid Treatment for Pediatric Patients With Mitochondrial Aminoacyl‐tRNA Synthetase Deficiencies

open access: yesJournal of Inherited Metabolic Disease, Volume 49, Issue 6, November 2026.
ABSTRACT Aminoacyl‐tRNA synthetases (ARSs) facilitate the loading of transfer RNAs with their cognate amino acids in the cytosol (ARS1), mitochondria (ARS2), or both (dual ARS). ARS deficiencies are clinically heterogeneous and can cause severe, occasionally fatal disease.
I. J. J. Muffels   +21 more
wiley   +1 more source

Comprehensive assessment of dietary micronutrient profiles and their effects on hemoglobin levels and anemia: provincial nutrition and health monitoring. [PDF]

open access: yesFront Nutr
Wang Y   +13 more
europepmc   +1 more source

Lysine Requirements in Starter‐Phase Japanese Quail (Coturnix japonica) Through Dose–Response Modelling: Interactions With Dietary Protein Level

open access: yesVeterinary Medicine and Science, Volume 12, Issue 6, November 2026.
In starter Japanese quail, optimal lysine requirements (1.32%–1.42%) depend on dietary crude protein level. Supplementation at 1.4% maximizes body weight gain, protein efficiency, and breast yield, while oversupply (1.55%) impairs performance. Linear broken‐line modelling provides the most reliable requirement estimates for precision feeding.
Hamid Valizadeh   +2 more
wiley   +1 more source

Thiamin deficiency in children with chronic kidney disease on peritoneal dialysis and its association with dialysis duration and transport peritoneal membrane status. [PDF]

open access: yesPediatr Nephrol
Suwanboriboon W   +10 more
europepmc   +1 more source

Growth, Carcass Quality and Gut Microbiota Responses to Extruded Rapeseed Meal With Protease in Broiler Chickens

open access: yesVeterinary Medicine and Science, Volume 12, Issue 6, November 2026.
The 5%–7.5% extruded rapeseed meal improved broiler growth, nutrient digestibility, carcass traits, intestinal morphology and gut microbial balance. ABSTRACT Background Soybean meal (SBM) is a major protein source in broiler diets, but its cost and dependence on a limited number of protein sources have increased interest in alternative feed ingredients.
Rohban Hameed   +5 more
wiley   +1 more source

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