NUTRITIONAL ASSESSMENT OF PATIENTS WITH METABOLIC DISORDERS Thompson SM 1 , Yip Q 2 , Dennison B 1 , Watson P 1 , Coakley J 1 , Alexander I 3 , Bhattacharya K 3 , Ellaway C 3 , Christodoulou J 3 1 The Children's Hospital at Westmead, Sydney, Australia 2 Univ of Newcastle, Newcastle, Australia 3 Children's Hosp Westmead and Univ of Syd, Sydney, Australia Background: Patients with metabolic disorders may have inadequate, excessive or unbalanced micronutrient intake. A protocol assessing biochemical markers for 15 micronutrients was implemented in 2009. Methods: A chart review of 67 patients, fully or partially tested per protocol, compared blood results to normal ranges. Forty-six (69%) were on protein-restricted diets, of which 35 (52% total) were supplemented with amino acid medical food. Ten (15%) were on unrestricted diets and 11 (16%) on fat modified diets. Results: The most common deficiency across diet groups was docosahexaenoic acid (DHA) (76% of 58 tested). Two patients on protein-restricted diets had low plasma vitamin B12. Low plasma levels of magnesium, zinc and vitamins D and E were more common when a medical food was not prescribed. Plasma folate was elevated in 93% patients on medical foods; elevated levels of vitamin A, E and riboflavin were also reported. Low levels of plasma manganese were found across all diet groups. Vitamin D status was of concern in unrestricted diets. Discussion: Both elevated and low levels of micronutrients and long chain fatty acids were found in metabolic patients, despite regular assessment of nutrient intake and advice on suitable supplements. Regular monitoring under a revised protocol is recommended. Background: We performed a study to test the hypothesis that psychoeducation of patients and parents has an influence on compliance for patients with a metabolic disease. Methods: Thirty-five patients with different metabolic diseases were enrolled in the study. During an interview the following information was recorded: background of the patient, perception and knowledge of the disease and diet. Knowledge about disease, diet and consequences of noncompliance were scored on a Likert scale (1 to 5). Compliance was obtained by summing the scores, 0 (insufficient), 1 (acceptable) or 2 (preferable) of four items: 'number of contacts with the dietician (CD)', 'frequency of control of biochemical parameters (FC)', 'results of controls (RC)' and 'quantity of ordering diet products (QP)'.