Background: Joyful and healthy eating is not in the focus in dietary treatment of PKU but rather the controlled intake of natural protein or phenylalanine. This is a burden on the families and the social life of the patients. Even relaxed diets due to sapropterin therapy are still complex. A working group of German, Austrian and Swiss Dieticians for relaxed PKU diet developed a new patient education system for strict and relaxed PKU diet. Results: The system indicates graphically in pyramidal form food categories that should be consumed during a day, e.g. foods from plant sources (fruit, vegetables, cereal based foods), foods from animal sources replaced by the corresponding amount of protein supplement and a few phenylalanine-rich extras. Portion sizes using a hand model for each food group are specified. The pyramid system emphasizes analogies to recommendations for healthy children. Patients are enabled to control their diet proactively and prospectively with regard to portions still allowed for the day instead of recording yet consumed and metabolized foods. The system was hitherto successfully applied in two metabolic centres. Conflict of Interest declared. Background: Exclusion of diary products in the treatment of inborn errors of amino acid (AA) metabolism results in a risk of deficit of absorbable calcium (Ca), phosphate ( P) and vitamin D with consequences of decreased bone mineral density (BMD). Patients and Methods: The following data from 28 patients (9 MSUD, 8 HT1, 4 MMA, 3 IVA, 2 MCC and 2 HOGA), in the age range 4-23 yrs: biochemical parameters of Ca-P metabolism, anthropometry, whole skeleton and lumbar spine BMD by DXA and nutritional status (based on diet records) were analyzed. Results: Body weight and height were within ±2SD in 90% of patients. Laboratory Ca-P markers were normal except decreased 25OHD3 (below 20 ng/ml) in 35% of patients. DXA examination revealed physiological results in 48% of patients, Z-score for whole skeleton BMD was below normal value in 32%, at lumbar spine low-normal/below normal BMD in 20% of patients. Analysis of diet records showed decreased vitamin D intake (mean 41% of RDA) with unfavourable Ca/P ratio. Introduction: There is little information about dietary practices in nonpyridoxine responsive homocystinuria (NPR-HCU) across Europe. Methods: 29 NPR-HCU dietary management questionnaires were returned from the SSIEM D-G network: (14 UK, 5 Germany, 3 Netherlands, 2 Switzerland, 2 Portugal, 1 France, 1 Norway, 1 Belgium). Results: 182 patients with NPR-HCU (57% (n=103) on a methionine/ intact protein restriction) were identified. Age distribution was: <1y, n=1; 1-10y, n−20; 11-16y, n=29; >16y, n=132. The use of diet with betaine increased with age: <1y, <1%; 1-10y, 10%; 11-16y, 12%; and >16y, 34%. The median intact protein intake (g\d) on diet only was <1y, 8 g; 1-10y, 12 g; 11-16y, 12 g; and >16y, 27 g. With diet and betaine, median intact protein intake (g\d) was: 1-10y, 14 g; 11-16y, 18 g; and >16y, 38 g. 41% (n=12) of centres used food methionine analysis for intact protein allocation; methionine exchange systems (primarily10mg or 20 mg) were common. 94% of patients on diet were prescribed methionine-free protein substitute. 48% of centres recommended cystine supplements for low plasma concentrations. Target concentrations for homocystine/homocysteine (free/total) and frequency of monitoring was inconsistent. Conclusion: In NPR-HCU, the use of dietary restriction declined with age. There is a need for European consensus guidelines on the management of NPR-HCU. Conflict of Interest declared. Background: Patients submitted to low protein diet (LPD) are susceptible to develop nutritional deficits, namely of vitamins, minerals and essential fatty acids, like docosahexaenoic acid (DHA). DHA is found mostly in fish, which isn't available in these patients' diet. Objectives: evaluate plasma DHA concentration in individuals submitted to LPD due to an inherited disorder, comparing with a control group and assess evolution after DHA supplementation. Methods: 20 patients (2 organic aciduria, 2 UCD, 10 PKU, 2 homocystinuria, 3 MSUD and 1 tyrosinemia type I), aged less than 18 years were submitted to analytic determination of plasma DHA in three moments (T0, T1 and T2), with 60 days intervals. In this period, patients were submitted to DHA supplementation (15 mg/Kg/day). Control group was randomly constituted by 33 individuals, aged matched and under normal diet. Results: in T0, patients' group presented a significantly lower DHA value than controls (27,65±13,69 μg/ml vs 55,23±21,84 μg/ml). Between T0 and T2, there was a significant improvement of patients' plasma values to 59,71±22,61 μg/ml, similar to control group. Conclusion: Daily supplementation of DHA allows plasma levels normalization and should be included in the nutritional schedule of these patients, in order to avoid deficits and optimize their growth and neurodevelopment. Methods: An LCMSMS method for quantification of DHAwas developed and fully validated. The assay was applied to establish a reference range (n=40), and to assess plasma DHA in 22 PKU patients. Results: DHA reference range was 131 μM-589 μM. PKU patients who were following a protein restricted diet demonstrated significantly lower plasma DHA than both the reference group and those PKU patients who were on a restricted diet and receiving DHA supplements (p<0.05). Conclusion: The method developed here is clinically useful in identifying patients deficient in DHA, and will be useful to clinicians who wish to supplement and monitor the DHA status of their patients in the future. Introduction: Propionic acidemia (PA) is caused by a congenital deficiency of propionyl-CoA carboxylase. Therapy includes a protein modified diet (with or without substitution of a precursor free amino acid mixture). Aim: Aim of this retrospective study was to investigate a possible correlation between protein intake (total and natural) and isoleucine concentrations in patients with PA. Methods: Laboratory parameters (e.g. concentrations of amino acids, n= 280) and up to 4 nutritional records (period of time 01/01-07/10, 3 days record estimated or weighted by parents, 2 days telephone record by a dietitian) of 7 patients with PA (diagnosis molecularly and enzymatically confirmed) were evaluated. Results: Isoleucine concentrations were in low or below reference range in all patients (mean: 33 μmol/l; median: 31 μmol/l, (range 7-67) n=25). They did not depend on total protein intake (mean: Background: Gastrointestinal symptoms and pancreatitis are recognised complications of Methylmalonic Acidaemia (MMA). Objective: Investigate incidence and review management strategies of feeding problems, gastrointestinal symptoms and pancreatitis. Case series: Fourteen patients with vitamin B12 non-responsive MMA. Age at presentation: neonatal (n=9) to 1.5y. Twelve patients with vitamin B12 responsive MMA. Age at presentation: neonatal (n=2) to 4y. Results: B12 non-responsive: 12 have feeding problems. Eleven are long term tube fed (from presentation n=4, by 6 months from diagnosis n=3 and all by 3 years) due to inadequate oral intake. 50% of those with feeding problems are on anti-emetics/anti-reflux medications and need feed slowly administered via feeding pump. Four have chronic diarrhoea and receive hydrolysed/elemental feeds. Four patients have presented with pancreatitis; isolated episodes n=3, acute on chronic n=1. Latter is now on anti-emetics and elemental feed with MCT via gastrostomy. During acute illness regimen is changed to slow jejunal feeds with increased fluid volume. B12 responsive group: one is tube fed (elemental). Conclusion: Feeding problems are frequent, presenting from diagnosis or acquired. Tube feeding becomes essential to meet prescribed diet and fluid requirements. Modifying feed rate, mode and 'type' may reduce gastrointestinal symptoms and enable enteral feeding in acute pancreatitis. Treatment of long chain fatty acid oxidation disorders (LCFAOD) is primarily by diet, but defining the ideal nutritional composition of a generic formula to treat all conditions remains challenging. Aim: To investigate the safety of a new MCT-containing (MCTF), nutritionally complete formula (Lipistart: Vitaflo International) in children with LCFAOD. Methods/Subjects: 5 well-controlled children (VLCADD, n = 2; LCHADD, n=1; CACTD, n=2; median age 9y; range 7-14y; 3 boys), who take daily MCT oil, completed a 21 day, phase 2 trial, on the safety of MCTF. MCTF (per 100 ml) contained 3.3% fat (2.6 g MCT); it replaced their usual formula (median volume 720 ml/d; range 500 ml-1900 ml/d).