Diarrhea |
Lactase deficiency Mitochondrial disorders* Abetalipoproteinemia Enteropeptidase deficiency Lysinuric protein intolerance Sucrase/isomaltase deficiency |
Intolerance to exercises |
Fatty acids oxidation disorders Glycogenolysis disorders Mitochondrial disorders* Myoadenylate deaminase deficiency |
Myocardial infarction |
5,10-methylenetetrahydrofolate reductase deficiency Familial hypercholesterolemia Fabry disease Homocystinuria |
Muscle spasms |
Multiple deficiency of carboxylase HHH syndrome |
Peripheral neuropathy |
Mitochondrial disorders* Peroxisomal disorders Metachromatic leukodystrophy Genetic disorders of glycosylation |
Recurrent emesis |
Galactosemia 3-oxothiolase deficiency d-2-hydroxyglutaric aciduria |
Pancreatitis symptoms |
Mitochondrial disorders* Type I glycogenosis Types I and IV hyperlipoproteinemia Lipoprotein lipase deficiency Lysinuric protein intolerance |
Eye muscle paralysis |
Mitochondrial disorders* Type C Niemann-Pick disease |
| *Disorders of mitochondrial etiology may be related to respiratory chain components, oxidation of lipids and various other components of the organelle. Adapted from [16] Table 1: Symptoms associated to inborn errors of metabolism |
|
Nutrient |
Estimated needs |
Calorie (kcal/kg) |
80–90 |
Carbohydrates (g/kg) |
16 |
Proteins (g/kg) |
3–4* |
Lipids (g/kg) |
3–4 |
Sodium (mEq/kg) |
3–4* |
Potassium (mEq/kg) |
2–4 |
Calcium (mg/kg) |
80–120 |
Phosphorus (mg/kg) |
60–90 |
Magnesium (mg/kg) |
9–10 |
Copper (g/kg) |
65 |
Zinc (g/kg) |
350–450* |
Vitamin A (IU/kg) |
500 |
Vitamin D (IU/kg) |
160 |
Vitamin E (IU/kg) |
2.8 |
Vitamin K (IU/kg) |
80 |
Vitamin B1 (mg/kg) |
350 |
Vitamin B2 (mg/kg) |
150 |
Niacin (NE/kg) |
6.8 |
Vitamin B12 (g/kg) |
0.4 |
Vitamin C (mg/kg) |
32 |
| *Ileostomy, surgical drain and other procedures may result in increase in demand. Adapted from [64] Table 2: Nutritional recommendations for parenteral nutrition in high-risk newborns |
|
0-24h |
Day 2 |
Days 3-7 |
- Glucose 6-8 mg/Kg/min - Amino acids 3 g/Kg - Calcium - Fat 0.5-1 g/Kg/day |
- Glucose 6-8 mg/Kg/min - Amino acids 3-3.5 g/Kg - Calcium - Electrolytes - Fat 0.5-1 g/Kg/day |
- Glucose increase to goal of 11-13 mg/Kg/min - Amino acids 3 g/Kg - Calcium - Electrolytes - Phosphorus - Vitamins - Trace elements - Fat increase to goal of 3-4 g/Kg/day |
Day 7 |
Week 2 |
Variable requirements |
- Glucose goal of 11-13 mg/Kg/min - Amino acids 3 g/Kg - Calcium - Electrolytes - Phosphorus - Vitamins - Trace elements - Fat goal of 3-4 g/Kg/day |
- Glucose goal of 11-13 mg/Kg/min - Amino acids 3 g/Kg - Calcium - Electrolytes - Phosphorus - Vitamins - Trace elements - Fat goal of 3-4 g/Kg/day - Carnitine 5 mg/Kg |
- Extremely low birth weight: increase goal fat and calories - Growth restriction: polyunsaturated fatty acids, iron and total calories - IEM* and CNS** malformations: increase long-chain polyunsaturated fatty acids - Dehydration: Fluid and electrolyte replacement - IEM*: remove/increase amino acids (i.e. tryptophan) and carnitine - Anemia and low oxygen perfusion: Iron supplementation - Pseudoaldosteronism: Sodium replacement - Retinopathy of prematurity: increase goal fat and calories |
| *Inborn errors of metabolism. **Central nervous system. Adapted from [30], [46], [47], [63] and [64] Table 3: Variable requirements in neonate progression through parenteral nutrition (PN) |
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| *(1º) after raising a hypothesis, search parameters were determined and scientific documents searched within indexed databases. (2º) Obtained documents (3º) were read in full and non-related/low quality works were excluded. (4º) Final group of documents were obtained (5º) and the process repeated during the period for data analysis to ensure the inclusion of newly published works. Adapted from [7] Figure 1: Bibliographic search and inclusion steps |