S122 Poster presentations

100,000 live births) characterized by blistering of the skin andmucous membranes in response to minor mechanical trauma.The involvement of the oral mucosa and oesophagus stenosis issuggested to be responsible for severe nutritional deficiencies,but few studies have till now considered this aspect. Thisobservational study aimed to evaluate homocysteine (Hcy)status by assessing Hcy and metabolically related vitamins (B6,B12, Folate).Methods: Twenty EB patients (12M; age range 0.5 19 years)were enrolled at the Pediatric Unit of our Hospital. Peripheralblood samples were drawn to assess the levels of: plasmaHcy and serum B12 (immunoenzymatic assay on AIA 600IIanalyser, Tosoh Bioscience, Tokyo, Japan); serum active B12,the biologically active fraction of B12, and Folate, serumand erytrocyte (immunoenzymatic assay on Architect analyseri2000SR Abbott Diagnostics, Abbott Park, IL, USA); plasmaB6 (HPLC; Chromsystems Instruments & Chemicals, Munich,Germany). For statistical analysis was used MedCalc software.Results: Our EB patients showed:

slight hyperhomocysteinemia: 55% of cases (10.9±1.2mM;mean cut-off <9mM [1]);B6 deficiency: 75% of cases (22.05±12.35 nM; mean cut-off>40 nM [2]);active B12 levels: 10% of cases (27.2±0.7 pM) under cut-off(>40 pM);B12, s-Fol and ery-Fol levels: within the relevant referenceinterval.

Conclusion: This is the first study which evaluated homocys-teine status in EB patients. Interestingly, most of them showedhyperhomocysteinemia due to impaired transulphuration via(B6); only 10% of patients had low active B12. Our preliminarydata suggest the importance of monitoring nutritional statusalso in EB patients and the potential role of dietarysupplements related to EB severity.

References

[1] Bailey D et al. Clinical Chemistry 2013; 59(9): 1393 405.[2] Kerr MA, et al. Pediatrics 2009; 123(2): 627 35.

Disclosure of Interest: None Declared.

PP275-SUNORAL BUDESONIDE IN PROTEIN LOOSING ENTEROPATHYPATIENTS POST FONTAN SURGERY: A SINGLE CENTEREXPERIENCE

A. Almehaidib1. 1Pediatrics, KFSHRC, Riyadh, Saudi Arabia

Rationale: Protein Loosing Enteropathy (PLE) is a well-knowncomplication of Fontan cardiac operation. It occurs in 1 15%post Fontan with a survival rate of 50% and 30%at 5 and 10years respectively. Budesonide is a locally acting steroid. Ithas been reported to treat children who developed PLE postFontan operation with promising results.Methods: This is a retrospective chart review of chil-dren <14 years who developed PLE post Fontan operation. Thedata collected included: patients’ demographics, time fromFontan operation to developing PLE; previous PLE treatments,Budesonide dose and duration. Serum albumin values pre andpost treatment. PLE was defined as an albumin level <30 g/Lwith clinical manifestation of edema in the form of facialswelling, lower limb swelling or ascites and positive albumintagged scan. The outcome was measured by improvement

in serum albumin level >30 g/L and reduction in albumintransfusion requirements.Results: Nine patients were identified with a median age of108 months (48 192 months). Six were males (66.6%). Themedian age for developing PLE post Fontan is 53 months(20 143 months). All patients presented with edema withlow serum albumin while 2 patients only had diarrhea.The median serum Albumin value is 16 g/L (range 12 21 g/l)before and 39 g/L (range 31 44 g/L) after treatment. Theincrease in serum albumin above 30 g/L post treatment wasstatistically significant (P-value = 0.002). No side effectsfor Budesonide were reported. Only 2 patients relapsed onweaning Budesonide below 3 mg/day.Conclusion: Budesonide is effective in reducing albumininfusion requirements in patients who develop PLE postFontan surgery. Relapse occurs once it is weaned below 3 mgper day independent of age & weight. Follow up studiesare needed to evaluate long-term effects and potential sideeffects.

Disclosure of Interest: None Declared.

PP276-SUNEXCESS WEIGHT IN CHILDREN INCREASES HOSPITALIZATIONDAYSM.T. Bechere Fernandes1, A.A. Ferraro1, G.V. Danti1,D.M.L. Garcia1. 1Pediatrics, Universidade de Sao Paulo,Sao Paulo, Brazil

Rationale: Excess weight (EW) in children is widely consideredin Primary Care settings. More recently studies withinSecondary Care units have suggested the importance of it aswell. This last statement generated the present work. Its aimsare: to verify the prevalence of EW in the pediatric ward of aGeneral University Hospital; to verify the association betweenthe nutritional status at admission in hospital with the numberof hospitalization days; to evaluate the evolution of weightstatus during hospitalization.Methods: A retrospective cohort study was executed basedon the patient’s medical records. A convenience sample madeof 91 subjects from all in-patients in the year 2009 wasevaluated. Inclusion criterion was being in the age rangeof 1 10 years. Nutritional status classification had the WHOcurves as reference. Weight-for-age for children under 3 yearsof age and body-mass-index for children older than 3 yearswere chosen as index. First a description of the data wasperformed and then a multivariate linear regression modeladjusted for age and sex. Local Ethics committee approvedthe present study. No conflict of interest is declared.Results: initial nutritional assessment revealed that 87.8%of the children were eutrophic, 3.3% malnourished and8.9% presented EW. The average age among this lastgroup was 5.55 years (±3.04); among eutrophic oneswas 3.37 (±2.58) and among malnourished children was7.29 (±3.45); the main diagnoses for admission werepulmonary diseases. EW children presented greater hos-pitalization days (5.5±6.23 days) and higher weight lossduring hospitalization ( 0.38 z-score ±0.81). Adjusted linearregression models found that EW children had greater weightloss (in average 0.48 z-score; p = 0.018) and longer hospital-ization (in average +2.37 days; p = 0.047) than eutrophic andmalnourished children.

Protein and amino acid metabolism S123

Conclusion: EW children may present longer hospitalizationand greater weight loss.

Disclosure of Interest: None Declared.

PP277-SUNIMPLEMENTATION OF NUTRITION SUPPORT GUIDELINES FORTHE CRITICALLY ILL CHILD: A QUALITY IMPROVEMENTPROGRAMU.G. Kyle1, J.C. Silva1, J. Lusk1, L.A. Lucas2, N. Maldonado3,G. Dardon3, L.S. Shekerdemian1, J.A. Coss-Bu1. 1Pediatrics,Crit Care Med, Baylor College of Medicine, 2ClinicalNutrition Services, 3Pediatrics, Crit Care Med, TexasChildrens Hospital, Houston, United States

Rationale: Critically ill children are at high risk of developingnutritional deficiencies, and hospital undernutrition is knownto be a risk factor for morbidity and mortality in children.Clinical guidelines are used to standardize and provide bettercare in the pediatric intensive care unit (PICU). The study aimswere to examine nutrition support practices before and afterimplementation of nutrition support guidelines in the PICU.Methods: Chart review of dietary intake for first 8 days ofPICU admission prior to (Oct-Dec 2012) and after (Oct-Dec2013) educational interventions and training completed duringAugust 2013 by the nutrition support team to all nursing andclinical staff. IRB approved.Results: 191 patients: age 1.4 (0.3 7.5) yrs (median (25 75th

percentile) and PICU length of stay (LOS) 6 (4 10) days; and193 patients, 1.8 (0.5 8.2) yrs and PICU LOS 6 (4 10) dayspre- and post-implementation, respectively, were included.

Implementation Day 1 4 Day 5 8

Pre Post Pre Post

kcal, % BMR<2 yrs 77 (28 121) 82 (31 128) 116 (64 146) b 120 (81 156) b

�2 yrs 30 (19 67) 30 (17 94) 88 (38 123) b 105 (79 129) a,b

Protein, % ASPEN<2 yrs 31 (0 56) 37 (0 76) a 50 (31 77) b 79 (35 101) a,b

�2 yrs 0 (0 51) 0 (0 79) a 68 (12 104) b 100 (45 134) a,b

Values are median (25 75th percentile). a Post vs pre-implementation MannWhitney p < 0.05; b Day 5 8 vs day 1 4 Wilcoxon Signed Rank test, p < 0.05.

Protein intake increased significantly post-implementation in<2 and �2 yrs old children. Energy intake was 6.2 and 27.2%and protein intake 20.0 and 57.2% higher post- than pre-implementation in children <2 and �2 yrs, respectively.Conclusion: Implementation of clinical guidelines in con-junction with educational efforts had a positive effect onthe adequacy of nutrition support in critically ill children.Continued efforts by all the practitioners are needed to ensuresustained results.

Disclosure of Interest: None Declared.

Protein and amino acid metabolism

PP278-SUN Outstanding abstractVITAMIN D DEFICIENCY REDUCES FASTED MUSCLE PROTEINSYNTHESIS RATE IN OLD RATS THROUGH ECTOPIC FATDEPOSITION AND INCREASED EIF2a PHOSPHORYLATIONA. Chanet1, J. Salles1, C. Giraudet1, A. Berry1, M.-L. Collin1,V. Patrac1, M. Jourdan2, Y. Luiking2, S. Verlaan2, Y. Boirie1,S. Walrand1. 1INRA, Clermont-Ferrand, France; 2NutriciaResearch, Nutricia Advanced Medical Nutrition, Utrecht,Netherlands

Rationale: We have previously shown that vitamin D (vitD)deficiency was responsible for a 40% reduction in fastedmuscle protein synthesis in old rats. The aim was toinvestigate the role of ectopic lipid accumulation in thereduced muscle protein synthesis induced by vitD deficiencyin old rats.Methods: 15-month-old Wistar rats were fed a vitD-deficient(VDD group) or a control diet for 9 months (n = 7 9/group). Af-ter 6 months of vitD depletion, a set of rats was supplementedwith vitD (5000UI/kg; VDS group) for the last 3 months.Muscle lipid content was analyzed by chromatography. Thephosphorylation state of Akt/mTOR (positive controllers ofprotein translation) and eIF2a (negative controller) pathwayswas assessed by Western blot. ANOVA was used for statisticalanalysis.Results: After 9 months under VDD, triglycerides and ce-ramides significantly accumulated in plantaris muscle com-pared to controls (+73 and +63% respectively, p < 0.05); thesemetabolites did not differ from controls rats following theVDS diet. VitD deficiency did not affect the phosphorylationof Akt or downstream effectors p70S6K and 4EBP1, whileVDS increased Akt activation when compared to controls. Thephosphorylation state of eIF2a was increased in VDD animalscompared to controls (+128%, p < 0.05), potentially explainingthe decrease in protein translation rate.Conclusion: Long-term vitD deficiency reduces muscle proteinsynthesis through an over-phosphorylation of eIF2a withoutaffecting the Akt/mTOR pathway. Therefore, intramuscularlipid deposition with vitD depletion may affect proteinsynthesis through eIF2a phosphorylation.Disclosure of Interest: A. Chanet: None Declared, J. Salles: NoneDeclared, C. Giraudet: None Declared, A. Berry: None Declared, M.-L.Collin: None Declared, V. Patrac: None Declared, M. Jourdan Other:Employee of Nutricia Research, Y. Luiking Other: Employee of NutriciaResearch, S. Verlaan Other: Employee of Nutricia Research, Y. Boirie:None Declared, S. Walrand: None Declared.

PP279-SUNCYSTINE IMPROVES SURVIVAL RATE IN LPS-INDUCED SEPSISMOUSE MODELK.A.K. Tanaka1, S. Kurihara1, T. Shibakusa1, Y. Chiba2,T. Mikami3. 1Institute for Innovation, Ajinomoto Co., Inc.,Kawasaki, 2Nutrition Care Dept., Ajinomoto Co., Inc.,Tokyo, 3Research Institute for Bioscience products & FineChemicals, Ajinomoto Co., Inc., Kawasaki, Japan

Rationale: Controlling excessive inflammation is consideredimportant for suppressing severe sepsis. Immune systemsuppression by trauma-induced excessive inflammatory cy-tokines was reported. We previously reported that cystinesuppressed lipopolysaccharide (LPS)-induced IL-6 production