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Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0–6 months; 7–12 months; 1–10 years; 11–16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice.

It has been known for centuries that fasting can suppress seizure activity in patients with epilepsy.1 In 1921, it was suggested that a ketogenic diet, designed to induce and sustain the metabolic effects of fasting, might have the same beneficial effects.2 This diet, high in fat and low in carbohydrates and proteins, was popular until the introduction of anticonvulsants such as phenytoin.3 In the 1990s, interest in the diet was renewed, following its successful use to treat a child with drug-resistant epilepsy, and the establishment of an awareness raising support group by the father of the child.3 This article provides a brief overview of ketogenic diets and then focuses on the issues relating to the use of medicines in patients on these diets. Approximately 25% of children with epilepsy have an inadequate response to antiepileptic drugs.4 Consideration of ketogenic diet use has been recommended under the following circumstances: 1. In children who have failed 2–3 anticonvulsant therapies, regardless of age and gender, and particularly in those with symptomatic generalised epilepsies (International Ketogenic Diet Study Group (IKDSG), 2008).5 2. In children and young people with epilepsy whose seizures have not responded to appropriate antiepileptic drugs (AEDs) and who have been referred to a tertiary paediatric epilepsy specialist (National Institute for Health and Care Excellence guidance, 2012).6 Contraindications to the ketogenic diet include pyruvate carboxylase deficiency, β-oxidation defects, primary carnitine deficiency and porphyria.3 Currently, four types of ketogenic therapy are in use: the classical ketogenic diet, the medium-chain triglyceride (MCT) diet, the modified Atkins diet (MAD) and the low glycaemic index (LGI) treatment. For the classical ketogenic diet, the ratio of fat to carbohydrate and protein ranges from 2:1 to 4:1 and it is predominantly long-chain fat sources that are used. As the name suggests, the …

This review seeks to explore the change and expansion in roles and responsibilities experienced by acute unit based paediatric dietitians over the past decade. The conventional role of providing advice to patients on a one to one basis following individual referral from a medic has been expanded to include extended cross discipline roles within hospital specialities, greater involvement in nutrition strategic development, best practice and higher degree level nutrition education on a local, regional and national level and closer involvement in the transformation of nutritional care provision across the health care setting. This reflection seeks to illustrate how the innovative use of dietitians can be used to increase both the effectiveness and efficiency of nutrition support services within the financial constraints of the present health system.

We aimed to assess and quantify parental beliefs regarding ketogenic dietary therapies (KDTs). We also aimed to determine whether beliefs were related to response to KDTs. Adapted versions of the Beliefs about Medicine Questionnaire were completed by parents of children following KDTs for epilepsy. Demographic and clinical data were collected from hospital records. Ketogenic dietary therapy response was defined as ≥50% seizure reduction compared to baseline. Many parents had a positive perception of KDTs and were convinced of the necessity of KDTs for their children, although beliefs were wide-ranging. Over half of parents reported concerns about the potential long-term effects of KDTs. Parental beliefs about KDTs were significantly correlated with patient response. This was an attempt to quantify parents' beliefs regarding the use of KDTs for their child's epilepsy. The questionnaire may be used to identify individuals with a less positive attitude towards KDTs and who may be less likely to report a favorable response to KDTs. It is unknown whether people who have positive beliefs about KDTs engage in less nonadherent behavior or whether beliefs regarding KDTs simply reflect outcomes. The evidence behind the long-term side effects of KDTs should be emphasized when counseling patients and their families.