HPE HPE 85 – Spring 2017 - Page 48

Practical therapeutics an enteral feeding tube. Enteral nutrition has an essential role in promoting intestinal adaptation by stimulating the release of hormones from the gastrointestinal tract, 2,3,6 and so it should be started as soon as possible after surgery. Oral feeding has the advantage of maintaining sucking and swallowing functions. However, many infants with SBS develop oral aversion as a result of the numerous interventions during their prolonged hospital stay. Continuous enteral tube feeding is beneficial as it increases contact time with the intestinal mucosa and so absorption may be maximised. Enteral feed tolerance is measured by evaluating stool output and vomiting. 46 Parenteral nutrition Parenteral nutrition (PN) is the mainstay of treatment in SBS. It enables the provision of adequate fluid, electrolytes, macro- and micronutrients to maintain hydration and electrolyte balance and promote growth and development during the period of intestinal adaptation. As enteral feeds are increased, PN is weaned with careful management from the NST. PN may be required for several years until full enteral autonomy can be achieved, so parents/carers are trained to administer PN at home. Guidelines from the European Society of Paediatric Gastroenterology, Hepatology and Nutrition and the European Society for Clinical Nutrition and Metabolism, published in 2005, detail the current standards for paediatric PN. 8 An update to these guidelines is expected to be published soon. The most important complications of SBS are related to PN. Patients require long-term central venous access for PN administration. Infection is the most common complication associated with central venous catheters (CVCs) and can cause significant morbidity and mortality. Adherence to aseptic procedures when handling CVCs is essential to prevent catheter-related bloodstream infections (CRBSIs). Taurolidine, a derivative of the amino acid taurine, has antimicrobial and antifungal activity and is associated with a decreased incidence of CRBSIs when administered as a line lock to patients at risk of infection. 9 IF-associated liver disease (IFALD) is a frequent and severe complication affecting children with SBS who are on long-term PN. Clinically, it can be defined by hyperbilirubinaemia (>50Β΅mol/l) and hospitalpharmacyeurope.com serum alkaline phosphatase and gamma- glutamyl transferase >1.5-times the upper limit of normal. Hepatic steatosis, cholestasis and hepatic fibrosis can occur, with possible progression to cirrhosis and liver failure. 2 Onset of IFALD may be specifically related to the PN composition and administration. Lipids provide a source of non-protein calories and essential fatty acids for patients receiving PN, and also enable fat-soluble vitamins to be included in the PN formulation. However, use of soybean oil-based intravenous lipid emulsions (ILEs) may contribute to IFALD. They contain a higher proportion of omega-6 fatty acids, leading to the production of pro- inflammatory substances with the potential to cause liver damage. Soybean oil-based ILEs provide long-chain triglycerides which are less easily hydrolysed by lipoprotein lipases than medium-chain triglycerides. 10 Fish oil-based ILEs provide a source of anti-inflammatory omega-3 fatty acids, and there is some evidence that the use of a multicomponent fish oil-containing ILE may contribute to a reduction in total bilirubin levels in children with IF on long-term PN. 11 Provision of excess glucose and lipid in PN may cause hepatic steatosis, so the PN formulation should be adapted to the needs of the individual patient. Generally, glucose intake should not exceed 18g/kg/day in infants, and lipids should provide between 25% and 40% of non-protein calories. 8 It is common practice to give β€˜fat-free days’ by omitting lipid from the PN formulation for a number of days each week for patients on long-term PN. Cyclical infusion of PN may also reduce the risk of liver complications and so the infusion time is usually gradually reduced from 24 hours to 12 hours, as tolerated. This requires close monitoring of blood glucose, as well as a stepwise increase and decrease of PN infusion rates to prevent hyper and hypoglycaemia. Infants need to be on sufficient enteral feeds to enable them to maintain normal blood glucose control during the time off from PN. The reduction in infusion time enables PN to be administered overnight and encourages oral feeding during the day. It also enables the child to go to school and participate in other activities. Other risk factors for IFALD include prematurity, recurrent sepsis and lack of enteral feeding. The immature liver of preterm neonates is more susceptible to damage from hepatotoxic substances. Extrahepatic infections (including CRBSIs) cause a release of pro- inflammatory substances that contribute to liver disease. 4,6 Absence of enteral nutrition leads to a lack of stimulation of the enterohepatic circulation and the accumulation of toxic bile salts, causing cholestasis. 4 Medicines Medication may be required to optimise treatment in SBS. Proton p Υ΅ΐ₯Ή‘₯‰₯Ρ½ΙΜ)…Ή‘₯ΝΡ…΅₯Ή” ȁ…ΉΡ…½Ή₯ΝΡ́…ΈΙ•‘Ս”)…ΝΡΙ₯Œ…₯‘εΑ•ΙΝ•Ι•Ρ₯½ΈΈ1½Α•Ι…΅₯‘”)…Έ‘•±ΐΡΌΙ•‘Ս”ΝΡ½½°½ΥΡΑΥЁ‰δ)₯ΉΙ•…Ν₯Ήœ₯ΉΡ•ΝΡ₯Ή…°ΡΙ…ΉΝ₯ЁΡ₯΅”°…Ή)Ρ‘•Ι•‰δ₯ΉΙ•…Ν₯Ήœ…‰Ν½ΙΑΡ₯½ΈΈ ‘₯±‘Ι•Έ)έ₯Ρ ‘½±•ΝΡ…Ν₯́΅…䁉•Ή•™₯Ё™Ι½΄)ΥΙΝ½‘•½α卑½±₯Œ…₯ΡΌ₯΅ΑΙ½Ω”)‰₯±”™±½άΈ)MΥɝ₯…°΅…Ή…•΅•ΉΠ)%Έ‘₯±‘Ι•Έέ₯Ρ M L°Ρ‘”Ν΅…±°‰½έ•°)΅…䁉•½΅”‘₯±…Ρ•…Ή±½Ν”₯Ρ́Ή½Ι΅…°)Α•Ι₯ΝΡ…±Ρ₯Œ…Ρ₯Ω₯Ρ一́MΥɝ•Ι䁡…䁉”)₯Ή‘₯…Ρ•ΡΌ₯ΉΙ•…Ν”Ρ‘”™ΥΉΡ₯½Ή…°)ΝΥΙ™…”…Ι•„½˜Ρ‘”₯ΉΡ•ΝΡ₯Ή”ΡΌ•Ή‘…Ή”)…‰Ν½ΙΑΡ₯½ΈΈ1½Ή₯ΡΥ‘₯Ή…°₯ΉΡ•ΝΡ₯Ή…°)±•ΉΡ‘•Ή₯Ήœ…ΉΡ…Α•Ι₯Ήœ€‘1%1P€°½™Ρ•Έ)Ι•™•ΙΙ•ΡΌ…́ё”ƒŠa ₯…Ή‘€ΑΙ½•‘ΥΙ—Šd°)₯ΉΩ½±Ω•Μ‘₯Ω₯‘₯ΉœΡ‘”‘₯±…Ρ•‰½έ•°₯Έ‘…±˜