DR RONALDA DE LACY PAEDIATRIC GASTROENTEROLOGY RED CROSS WAR MEMORIAL CHILDREN S HOSPITAL SAPA 2016

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1 DR RONALDA DE LACY PAEDIATRIC GASTROENTEROLOGY RED CROSS WAR MEMORIAL CHILDREN S HOSPITAL SAPA 2016

2 CAUSES OF MALNUTRITION Multifactorial Poor intake- 70% consuming less than RDA Nausea Anorexia Mucosal congestion Decreased intestinal motility Early satiety organomegaly and ascites Unpalatable diets 50% energy derived from dietary fat Decreased absorption of long chain fatty acids decreased bile acids Essential fatty acid deficiency

3 NUTRITIONAL CONSEQUENCES OF CHRONIC LIVER DISEASE Fat mass is reduced Eventually lean body mass Resting and total energy expenditure increased by 30% in younger children Poor growth occurs sec to reduced growth hormone receptor expression low insulin-like growth factor 1 Puberty often delayed Hypogonadism common

4 IMPLICATIONS High energy intake Avoid fasting No protein restriction Supplement branch chain amino acids

5 ASSESSMENT OF NUTRITIONAL STATUS Assess at first and all subsequent visits Good dietary history dietary diary Assess food variety as at risk of micronutrient deficiencies Symptoms- vomiting, anorexia, diarrhoea, steatorrhoea and pale stools-risk for poor intake and malabsorption Weight for age and weight for height for age underestimate the degree of malnutrition because of organomegaly, ascites and malnutrition Stunting reflects chronic undernutrition or genetic condition i.e. Alagille s Skinfold thickness and mid-upper-arm circumference more reliable Skinfold thickness assesses fat mass Mid-upper-arm circumference assesses muscle mass.

6 MACRONUTRIENT DEFICIENCY Nutritional factor Energy/calorie intake Assessment tools Deficiency Toxic effects Mid-upper arm circumference Triceps/subscapular skin fold thickness Serial measurements weight and height Indirect calorimetry Fat malabsorption Reduced muscle bulk Reduced subcutaneous fat Alopecia, thin sparse hair Pigmentary skin changes Parotid enlargement Financial burden Essential fatty acid deficiency Recommendations Calorie intake % RDI based on weight or height on 50 th centile MCT oil 1-2ml/kg/day 2-4 divided doses Add glucose polymers (polycose powder or solutions) Supplementary overnight nasogastric tube feeds Steatorrhea MCT formulas (Pregestimil, Alimentum, Prenan) Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607

7 MACRONUTRIENT DEFICIENCY Nutritional factor Assessment tools Deficiency Recommendations Carbohydrate Serum glucose Monitor for hypoglycaemia during fasting, illness or reduced intake Protein Mid-upper-arm circumference Serum albumin Pre-albumin Retinol binding protein Transferrin Reduced muscle bulk Alopecia, thin sparse hair Pigmentary skin changes Parotid enlargement Peripheral oedema Psychomotor changes, irritability Infants: protein intake 3-4 g/kg/day Hepatic encephalopathy: protein intake g/kg/day Branched-chain amino acid supplements Fat Essential fatty acid deficiency Triene:tetraene ratio >0.3 Decreased linoleic acid Skin dryness, peeling Alopecia Oral vegetable/corn oil or intravenous lipid emulsions Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607

8 FAT SOLUBLE VITAMINS VITAMIN A DEFICIENCY Fat soluble vitamin status should be assessed every 6 months or more frequently if deficient. Biochemical vitamin A deficiency occurs before it becomes clinically overt. Serum retinol does not always accurately estimate vitamin A deficiency in children with liver disease.

9 VITAMIN A DEFICIENCY Nutritional factor Vitamin A Assessment tools Deficiency: Retinol: RBP molar ratio <0.8 or Serum retinol <20 μg/dl Deficiency Toxic effects Recommendations Xerosis Bitot spots Night blindness Dry skin Follicular keratosis Possible immune dysfunction Liver fibrosis Hypercalcaemia Pseudotumor cerebri Painful bone lesions Vitamin A U/d po

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11 VITAMIN D DEFICIENCY History bone pain or fractures Palpation of spine Assessment of pubertal stage Investigations 25 OH vitamin D, calcium, phosphate, magnesium, alkaline phosphatase Hand X-rays severity of osteodystrophy and growth potential Dual-energy X-ray absorptiometry (DEXA)- to assess low bone mass Lateral spine X-ray vertebral fractures in older children

12 VITAMIN D DEFICIENCY Nutrition factor Vitamin D Assessment tools Deficiency: 25-OH vitamin D level <30 ng/ml Calcium, phosphorus levels Deficiency Toxic effects Recommendations Hypocalcaemia Hypophosphataemia Muscle hypotonia Poor dentition Rickets: Bowed legs Epiphyseal enlargement Rachitic rosary Craniotabes Frontal bossing Delayed fontanelle closure Hypercalcaemia Pseudotumor cerebri Nephrocalcinosis Ergocalciferol 3-10 times RDI Cholecalciferol based on weight and vitamin D levels Weight <40 kg Weight >40kg <10 ng/ml <10 ng/ml 100 U/kg/day U/day ng/ml ng/ml 75 U/kg/day 4000 U/day ng/ml ng/ml 50 U/kg/day 3000 U/day Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607

13 VITAMIN E DEFICIENCY Common in cholestatic liver disease. Peripheral neuropathy Spino-cerebellar degeneration Ataxia Reversible early in disease, permanent if treatment is delayed In presence of hyperlipidaemia, vitamin E levels falsely normal Ratio to tocopherol: total lipids or tocopherol: cholesterol should be used to assess deficiency

14 Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607 VITAMIN E DEFICIENCY Nutrition factor Assessment tools Deficiency Toxic effects Recommendations Vitamin E Deficiency: Vitamin E : total lipid ratio <0.6 mg/g (<1 y) <0.8 mg/g ( >1 y) Poor nerve conduction Hypo/areflexia Ataxia Peripheral neuropathy Loss of vibratory sense Myopathy Vision loss Haemolytic anaemia Impaired neutrophil chemotaxis Potentiation of vitamin K deficiency Coagulopathy Diarrhoea Hyperosmolality Alpha-Tocopherol (acetate) IU/kg/day TPGS (Luiqi E) IU/kg/d

15 VITAMIN K DEFICIENCY Prothrombin time/inr used to assess These are influenced by liver synthetic function Response to vitamin K supplementation will determine the difference between liver dysfunction or vitamin K deficiency Bone disease secondary to carboxylation defects in vitamin K deficiency

16 VITAMIN K DEFICIENCY Nutrition factor Assessment tools Deficiency Recommendations Vitamin K Deficiency: Prolonged PT/INR Elevated PIVKA-II Haemorrhagic disease Excessive bruising Vitamin K mg, 2-7 times/wk Intravenous vitamin K 2-10 mg may be required Multivitamin preparation providing 1-2 times RDI

17 MINERALS AND TRACE ELEMENTS Nutrition factor Assessment tools Deficiency Toxic effects Recommendations Iron Deficiency: Decreased iron level Increased total iron-binding capacity Pallor Koilonychia Stomatitis Teeth staining Haemorrhagic gastroenteritis Metabolic acidosis Coma Liver failure Elemental iron 5-6 mg/kg/d Zinc Deficiency: Plasma zinc level <60 μg/dl Alopecia,thin sparse hair Acrodermatitis enterohepatica Decreased intestinal absorption copper and iron Zinc sulphate solution 10 mg/ml elemental zinc Elemental zinc 1 mg/kg/d Selenium Deficiency: Plasma selenium <40 μg/l Dermatological changes Diarrhoea Dyspepsia Anorxia 1-2 μg/kg/d oral sodium selenite 1-2 μg/kg/d selenium in TPN Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607

18 MINERALS AND TRACE ELEMENTS Nutrition factor Magnesium Assessment tools Deficiency: Serum magnesium <0.7 mmol/l Deficiency Toxic effects Recommendations Respiratory depression Lethargy Coma Magnesium oxide mmol/kg daily po Magnesium Sulphate (50%) mmol/kg IV over 3-6 h Calcium Deficiency in steatorrhoea despite corrected vitamin D status Poor dentition Hypercalcaemia Hypercalciuria mg/kg/d up to mg/d Phosphorus Low serum phosphorus level despite corrected vitamin D and calcium status Gastrointestinal intolerance mg/kg/d up to 500mg/d Adapted from Nel E, et al: SAMJ, v105 n7 (201507): 607

19 NUTRITIONAL SUPPORT GOALS - normal growth - normal body composition - prevention of vitamin deficiency - prevention of trace element deficiency

20 NUTRITIONAL SUPPORT Should not be delayed Review intake regularly Involve a paediatric dietician early High energy intake % of normal Increase fat intake MCT s not dependant on bile salts - Should not exceed 80% of total fat - Long chain fatty acids essential for normal growth and brain development e.g. egg yolk and veg oils

21 NUTRITIONAL SUPPORT Carbohydrates important energy source Adequate amount without causing osmotic diarrhoea Formula with glucose polymers and not sugars may be necessary Galactose free diet in galactossaemia Protein restriction should be avoided Branch-chain amino acid supplements improve lean body mass but are expensive. Fat soluble vitamin supplementation Trace element supplementation

22 ROUTES OF FEEDING Most children can be fed orally. Preterm formulas- high energy, mineral content and mediumchain fatty acids. Often used if infants unable to breastfeed Breastfed infants - fortification and/or supplementation with medium-chain fatty acids More frequent feeds for children with advanced liver disease Overnight nasogastric tube feeds if energy requirements not met during the day Enteral feeds fail TPN in consultation with paeds GI and paeds dietician Placement of percutaneous endoscopic gastrostomy (PEG) usually avoided

23 TAKE HOME MESSAGES Assess frequently Skin fold thickness and MUAC more accurate Act early Involve a paediatric dietician Increase energy requirements % Monitor for vitamin and trace element deficiencies and treat. DO NOT protein restrict unless in liver failure Increase MCT intake Encourage oral feeds Nasogastric tube feeds if not meeting energy requirements AIM FOR NORMAL GROWTH

24 THANK YOU