Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview
Abstract
:1. Introduction
2. Causes of Malnutrition in Cholestatic Children
2.1. Metabolic Changes
2.2. Poor Nutrient Intake
2.3. Increased Requirements or Malabsorption/Maldigestion of Multiple Nutrients
2.3.1. Increased Energy Needs
2.3.2. Water and Electrolytes
2.3.3. Carbohydrates
2.3.4. Proteins
2.3.5. Lipids and Bile–Acid-Dependent Absorption of Fats and Fat-Soluble Nutrients
2.3.6. Medium Chain Triglycerides and Long-Chain Triglycerides
2.3.7. Essential Fatty Acids
2.3.8. Fat-Soluble Vitamins
2.3.9. Water-Soluble Vitamins and Minerals
2.4. Endocrine Dysfunction
3. Issues in the Nutritional Management of Children with Cholestasis
3.1. Anthropometric Measurements
3.2. Biochemical Markers
3.3. Other Investigations
4. Special Diets in Some Common or Special IEM Causing Cholestasis
4.1. Tyrosinemia Type 1
4.2. Galactosemia
4.3. Hereditary Fructose Intolerance
4.4. Citrin Deficiency
5. Pre- and Post-Transplant Nutritional Status of Children with End-Stage Cholestatic Liver Disease
6. Post-Transplant Obesity with Fatty Liver and MetS Risk: The Malnutrition in Excess paradox
Sarcopenia
7. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Conflicts of Interest
References
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Age | Males | Females |
---|---|---|
3–10 years | 22.7 (Weight) + 495 | 22.5 (Weight) + 499 |
10–18 years | 17.5 (Weight) + 651 | 12.2 (Weight) + 746 |
Vit | Signs and Symptoms of Deficiency | How to Monitor | Supplementation | Toxicity |
---|---|---|---|---|
A | Dry skin Xerophthalmia Night blindness. | Plasma retinol/retinol binding protein molar ratio > 0.8 relative dose response When serum retinol levels < 20 µg/dl, RDR test is indicative of Vit A deficiency when the plasma retinol concentration increases after exogenous administration of Vit A dose. | 3000–10,000 IU/day <10 kg: 5000 UI/day, Oral >10 kg: 10,000 UI/day, Oral 50,000 UI/1–3 monthly, IM | Hepatic and neurologic toxicity Development of long bone fractures |
D | Hypocalcemia/hypophosphatemia/tetany Osteomalacia and rickets History of reduced intake, decreased cutaneous synthesis, altered absorption/impaired metabolism in the liver (i.e., where Vit D2 and D3 undergo 25-hydroxylation). Phenobarbital treatment. Breastfeeding | Serum 25-OH-D (Vit D deficiency < 20 ng/mL; insufficiency < 30 ng/mL) Ca, P, AP, PTH, Bone radiography/Bone densitometry used to identify osteomalacia, osteopenia or rickets | Cholecalciferol: 800–5000 IU/day, Oral 1.25-OH cholecalciferol: 0.05–0.2 µg/kg/day, Oral | Hypercalcemia leading to depression of the central nervous system and ectopic calcification. Hypercalciuria leading to nephrocalcinosis |
E | Hypo- or a-reflexia Ataxia Impaired vibratory sensation Proximal muscle weakness Ophthalmoplegia Degenerative lesions of the retina Irreversible neurological lesions if Vit E deficiency remains untreated | Vit E/total lipids ratio (increased lipoprotein levels in cholestasis may falsely elevate serum Vit E levels in a patient with Vit E deficiency) Vit E deficiency: <0.6 mg/g (age <1 year) <0.8 mg/g (age >1 year) | Alpha-tocopherol acetate: 15–25 to 25–200 UI/kg/day, Oral TPGS (tocopheryl polyethylene glycol-1000 succinate): 15–25 UI/Kg, Oral | Potentiation of Vit K deficiency coagulopathy Diarrhea Hyperosmolality (TPGS) |
K | Hemorrhagic disease (other risks of bleeding: portal hypertension gastrointestinal bleeds, thrombocytopenia, platelet dysfunction, reduced hepatic synthesis of other coagulation factors | Prothrombin time International normalized ratio Protein induced in Vit K absence II (PIVKA II) <3 ng/mL Deficiency can be diagnosed if these values improve after a dose of parenteral vitamin K | 2.5–5.0 mg/day from twice a week to every day 5–10 kg: 5 mg, oral >10 kg: 10 mg, oral 5–10 mg/day every two weeks, IM | Hemolytic anemia in glucose 6-phosphate dehydrogenase-deficient infants |
Before Liver Transplant | After Liver Transplant |
---|---|
Energy intake 130–150% EAR | Energy intake 120% EAR |
Carbohydrates (40% to 60% of total energy) 15–20 g/kg/day as monomers, polymers, and starch Balancing the hypoglycemia from end-stage liver disease and hyperglycemia from insulin resistance | Carbohydrates 6–8 g/kg/day as monomers, polymers, and starch Warning: Consider the diabetogenic potential of tacrolimus when it is used for immunosuppression |
Proteins (9% of total energy) 3–4 g/kg/day BCAA-enriched formula can be used (10% of total amino acid) Low protein-diet is needed only when severe encephalopathy is present. Once encephalopathy is resolved, the patient should resume a diet with appropriate protein supply because long-term restriction <2 g/kg/day can induce endogenous muscle protein consumption | Proteins 2.5–3 g/kg/day |
Fats (40% of total energy; 10% of which as LCPUFA) 8 g/kg/day with 30–50% as MCTs. Warning: MCT contents >80% without adequate supplementation of PUFA can lead to a deficiency of essential fatty acids | Fats 5–6 g/kg/day After liver transplantation, when bile flow is established and malabsorption is resolved, children fed with high MCT-containing supplementation pre-transplant can transition to standard formula |
Fluids and electrolytes Fluid requirement is normal for actual weight, unless restriction is needed because of ascites or edema. Sodium intake is 1 mmol/kg/day and potassium about 2 mmol/kg/day | Fluids and electrolytes A “no added salt” diet (3 g sodium/day) is recommended to prevent water retention associated with steroid therapy |
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Tessitore, M.; Sorrentino, E.; Schiano Di Cola, G.; Colucci, A.; Vajro, P.; Mandato, C. Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview. Nutrients 2021, 13, 2785. https://doi.org/10.3390/nu13082785
Tessitore M, Sorrentino E, Schiano Di Cola G, Colucci A, Vajro P, Mandato C. Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview. Nutrients. 2021; 13(8):2785. https://doi.org/10.3390/nu13082785
Chicago/Turabian StyleTessitore, Maria, Eduardo Sorrentino, Giuseppe Schiano Di Cola, Angelo Colucci, Pietro Vajro, and Claudia Mandato. 2021. "Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview" Nutrients 13, no. 8: 2785. https://doi.org/10.3390/nu13082785