Fibre is an essential nutrient in the human diet that is crucial for human health. It provides a range of functional benefits, including stool bulking, and physiological benefits through fermentation of diverse fibre types by the gut microbiome including cholesterol lowering, glycaemic control and weight control. The by-products of the fermentation of fibre in the gut confer health benefits that extend beyond the gut to the immune system and organs such as the liver, kidneys and the brain. A lack of fibre in the diet has been associated with several disorders in children including constipation, irritable bowel syndrome, allergies and immune-related disorders. In paediatric practice, concerns exist over tolerance of dietary fibre which may lead to unnecessary restrictions, especially for children receiving nutritional support. One reason for this may be the terminology which has historically been used. Fibre is often described in terms of its physico-chemical properties (solubility, viscosity), rather than its physiological effects/functionality (fermentability, bulking effects). To describe fibre in these latter terms represents more clearly the important role it plays. Most international guidelines recommend a daily quantity of fibre, failing to mention the quality aspect of the fibre required for health. Here we consider the evidence base for the current recommendations for daily fibre intakes for healthy children, those requiring nutritional support and those with functional gastrointestinal disorders. We also consider the importance of the gut microbiome and the role of fibre in maintaining gut microbial health and its role in health beyond the gut.

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Objectives Deoxyguanosine kinase (DGUOK) deficiency is one of the leading causes of the mitochondrial DNA-depletion syndromes (MDDS) associated with hepatocerebral involvement. Herein, we present four cases of DGUOK deficiency to emphasize the clinical variability of disease and the challenges in the diagnosis of DGUOK deficiency. Case presentation Hepatomegaly, hyperlactatemia, elevated alpha fetoprotein (AFP), alanine, and transaminase levels were detected in all patients, and cholestasis, coagulopathy, and hypotonia were common findings. All patients had a low birth weight, one patient underwent liver transplantation (LT). Clinical and laboratory findings of two patients and one patient suggested neonatal hemochromatosis and type 1 tyrosinemia, respectively. All patients were diagnosed with DGUOK deficiency by performing molecular genetic analysis. Conclusions Mitochondrial DNA-depletion syndromes should be kept in mind in cases in which hypotonicity, lactic acidosis, and neonatal cholestasis are observed. DGUOK deficiency may present in different clinics suggesting neonatal hemochromatosis or tyrosinemia type 1.

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Purpose: To obtain information on characteristics, management, current objective nutritional status and perception of nutritional status of children with cerebral palsy (CP) from healthcare professionals (HCPs) and caregivers. Materials and methods: A detailed survey of several items on eight main topics (general characteristics, motor function, comorbidities, therapies, anthropometry, feeding mode and problems and perceived ARTICLE HISTORY