| Autor: |
Vitoria-Miñana I; Metabolic and Nutrition Unit, Hospital Universitari i Politècnic la Fe, 46026 Valencia, Spain., Couce ML; Department of Pediatrics, University Clinical Hospital of Santiago de Compostela, 15704 Santiago de Compostela, Spain.; IDIS-Health Research Institute of Santiago de Compostela, 15704 Santiago de Compostela, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto Salud Carlos III, 28029 Madrid, Spain.; MetabERN, Via Pozzuolo, 330, 33100 Udine, Italy.; Faculty of Medicine, Santiago de Compostela University, 15704 Santiago de Compostela, Spain., González-Lamuño D; Pediatric Nephrology and Metabolism, Hospital Universitario Marqués de Valdecilla, 39008 Santander, Spain.; Research Institute Valdecilla (IDIVAL), University of Cantabria, 39007 Santander, Spain., García-Peris M; Metabolic and Nutrition Unit, Hospital Universitari i Politècnic la Fe, 46026 Valencia, Spain., Correcher-Medina P; Metabolic and Nutrition Unit, Hospital Universitari i Politècnic la Fe, 46026 Valencia, Spain. |
| Abstrakt: |
Human milk (HM) offers important nutritional benefits. However, except for phenylketonuria (PKU), there are little data on optimal levels of consumption of HM and a special formula free of disease-related amino acids (SF-AA) in infants with inborn errors of metabolism of amino acids and proteins (IEM-AA-P). We designed a spreadsheet to calculate the amounts of SF-AA and HM required to cover amino acid, protein, and energy needs in patients with the nine main IEM-AA-P in infants aged under 6 months. Upon entering the infant's weight and the essential amino acid or intact protein requirements for the specific IEM, the spreadsheet calculates the corresponding required volume of HM based on the amino acid concentration in HM. Next, the theoretical daily fluid intake (typical range, 120-200 mL/kg/day) is entered, and the estimated daily fluid intake is calculated. The required daily volume of SF-AA is calculated as the difference between the total fluid intake value and the calculated volume of HM. The spreadsheet allows for the introduction of a range of requirements based on the patient's metabolic status, and includes the option to calculate the required volume of expressed HM, which may be necessary in certain conditions such as MMA/PA and UCD. In cases in which breastfeeding on demand is feasible, the spreadsheet determines the daily amount of SF-AA divided over 6-8 feeds, assuming that SF-AA is administered first, followed by HM as needed. Intake data calculated by the spreadsheet should be evaluated in conjunction with data from clinical and nutritional analyses, which provide a comprehensive understanding of the patient's nutritional status and help guide individualized dietary management for the specific IEM. |
