S. Evans

Breast feeding in IMD

SummaryBreast feeding has proven benefits for many infants with inherited metabolic disorders (IMDs) but, with the exception of phenylketonuria, there are few reports in other conditions. A questionnaire, completed by dietitians and clinicians from 27 IMD centres from 15 countries (caring for a total of over 8000 patients with IMDs on diet) identified breast feeding experience in IMD. Successful, demand breast feeding (in combination with an infant amino acid formula free of precursor amino acids) was reported in 17 infants with MSUD, 14 with tyrosinaemia type I, and 5 with homocystinuria. Eighty-nine per cent were still breast fed at 16 weeks. Fewer infants with organic acidaemias were demand breast fed (7 with propionic acidaemia; 6 with methylmalonic acidaemia and 13 with isovaleric acidaemia) (usually preceded by complementary feeds of a protein-free infant formula or infant amino acid formula free of precursor amino acids). Only 12 infants with urea cycle disorders were given demand breast feeds, but this was unsuccessful beyond 8 days in CPS deficiency. Further work is needed in developing guidelines for feeding and for clinical and biochemical monitoring for breast-fed infants with IMDs.

Fibre content of enteral feeds for the older child

BACKGROUND There is currently a lack of clinical data on fibre requirements in UK children. Subsequently, the ideal fibre profile for enteral formulae designed to meet the requirements of older children is unknown. The present study aimed to investigate the effect of fibre supplementation on gastrointestinal function of children aged 7-12 years (or weight 21-45 kg) receiving an age-specific high-energy enteral feed. METHODS In this double-blind randomised crossover study, 25 home enterally tube-fed children with a range of medical conditions (including cystic fibrosis, neurological conditions, liver transplant and bone marrow transplant) were given a 1.5 kcal mL(-1) formula with or without added dietary fibre (1.13 g per 100 mL). Each formula was taken for 6 weeks, followed by 6 months on the second randomly assigned formula. Anthropometry, blood biochemistry, stool characteristics, tolerance and oral dietary intake were assessed. RESULTS Despite a higher median fibre intake on the fibre-containing formula (84% versus 26% of recommended intake; P = 0.003), most children did not meet existing international recommendations for fibre as a result of small feed volumes (median 800 mL day(-1); 9 g fibre day(-1)). There was some evidence of reduced constipation, laxative reliance and abdominal pain on the fibre-containing formula. CONCLUSIONS Given the poor fibre intakes and absence of adverse effects, the use of fibre-containing formulae should become standard practice for the majority of children on enteral feeds. Larger trials in children are required to further evaluate the effect of amount and blend of fibre in enteral formulae for older children. However, it is likely that current formulae require higher levels of fibre.

Accuracy of home enteral feed preparation for children with inherited metabolic disorders

BACKGROUND Many children with rare chronic disorders require home enteral tube feeds (HETF) consisting of multiple modular ingredients. Feeds are often complex and the risk of errors during their preparation is high. The consequences of over- or under-concentration can be critical. The aim of the present prospective observation study was to assess the accuracy, skills and technique of caregivers when preparing and administering HETF. METHODS Fifty-two HETF patients (median age 7.5 years, range 0.7-18.0 years) with inherited metabolic disorders (IMD) requiring special feeds were recruited. Using observation and a structured questionnaire, a practical assessment of feed preparation and storage by the main caregiver was undertaken by an independent dietitian and nurse in the childs home, including hygiene practices, accuracy of measuring recipe ingredients, and storage of both ingredients and prepared feeds. RESULTS The majority (85%; n = 44) of feeds were based on >1 ingredient (median 3; range 1-6). Almost half (48%; n = 25) of caregivers measured feed ingredients inaccurately. Of the 31% (n = 16) using scoops, 31% used incorrect measuring spoons and 25% did not level scoops appropriately. Some 45% (n = 20/44) of carers measured liquid ingredients inaccurately. Hygiene practices during feed preparation were poor, including a lack of hand washing (31%: n = 16) and incorrect storage procedures for unused feed ingredients (56%; n = 29). CONCLUSIONS Practices in the preparation of modular HETF for children with IMD were not ideal. A combination of inaccuracy, poor hygiene, inappropriate storage, and long feed hanging times increases both metabolic and microbial risk. Better education, regular monitoring and the development of ready-to-use or preweighed ingredients would be beneficial.

The challenge of nutritional profiling of a protein-free feed module for children on low protein tube feeds with organic acidaemias

BACKGROUND Enteral tube feeding for children with organic acidaemias (OA) is recommended. Protein restriction, providing minimum safe levels of protein intake, is advocated. Standard paediatric tube feeding formulae provide more than the minimum safe protein requirements and are unsuitable in OA without modification. Modified paediatric enteral feeds consist of several modular ingredients. The aim of this prospective longitudinal interventional study was to assess the efficacy of a premeasured novel protein-free module developed for children aged over 12 months compared to conventional practice. METHODS In total, 15 children with OA (11.6-31 kg) needing enteral feeding were recruited. The protein-free module, from either a protein-free infant feed or modular ingredients, was replaced by the study feed. To ensure metabolic stability, energy and protein intake were unchanged. Dietary intake, anthropometry and nutritional biochemistry were recorded at baseline and week 26. RESULTS Dietary intakes of magnesium (P = 0.02), sodium (P = 0.005), vitamin D (P = 0.04), docosahexaenoic acid (P = 0.01) and arachidonic acid (P = 0.001) significantly improved; plasma selenium (P = 0.002) and whole blood glutathione peroxidase (P = 0.02) significantly increased. Feed preparation accuracy as measured by composition analysis showed consistent errors both in pre- and study feeds. CONCLUSIONS A protein-free module improved nutritional intake and biochemistry, although feed preparation errors remained a common finding.

Preoperative antibiotics for septic arthritis in children: delay in diagnosis

Purpose. To review the records of 50 children who underwent open joint washout for septic arthritis with (n=25) or without (n=25) preoperative antibiotics. Methods. Records of 50 children who underwent open joint washout for presumed septic arthritis with (n=25) or without (n=25) preoperative antibiotics were reviewed. 17 boys and 8 girls aged 3 weeks to 16 years (median, 1.5 years) who were prescribed preoperative antibiotics before joint washout were compared with 12 boys and 13 girls aged one month to 14 years (median, 2 years) who were not. Following arthrotomy and washout, all patients were commenced on high-dose intravenous antibiotics. Patients were followed up for 6 to 18 months until asymptomatic. Results. Patients who were referred from places other than our emergency department were twice as likely to have been prescribed preoperative antibiotics (p=0.0032). Patients prescribed preoperative antibiotics had a longer median (range) time from symptom onset to joint washout (8 [2–23] vs. 4 [1–29] days, p=0.05) and a higher mean erythrocyte sedimentation rate (93.1 vs. 54.3 mm/h, p=0.023) at presentation. Nonetheless, the 2 groups were comparable for weight bearing status, fever, and positive culture, as well as the mean (range) duration of antibiotic treatment (4.9 [4–7] vs. 4.7 [1–8] weeks, p=0.586). Conclusion. Preoperative antibiotics should be avoided in the management of septic arthritis in children. Their prescription delays diagnosis and definitive surgery, and leads to additional washouts and complications. A high index of suspicion and expedite referral to a specialist paediatric orthopaedic unit is needed if septic arthritis is suspected.