Development of a Dutch intervention for obese young children.

The objective of this article is to provide insight in the five-step development process of the best evidence, best practice intervention for obese young children 'AanTafel!'. A set of requirements for intervention development was developed to guide the data inquiry: the use of theory, influencing factors, tailoring, multi-disciplinarity, duration/frequency and evaluation and monitoring. Step I retrieved evidence from clinical guidelines, followed by a systematic review with meta-analysis (Step II) and an extended literature review (Step III). Evidence was consistent with regard to parent-focus, targeting family level, including diet, physical activity and behaviour change techniques and tailoring to age. However, no evidence or inconsistent evidence emerged from the theory-basis, group-versus-individual sessions, face-to-face contact versus Internet-mediated contact, which disciplines to involve and how to involve them, as well as intervention duration and intensity. Hence, practice-based insights from parental interviews (Step IV) and involved therapists were added and subsequently integrated to the intervention 'AanTafel!' (Step V). 'AanTafel!' is a multi-component, multi-disciplinary, family-based, parent-focused, age-specific intervention, which is tailored to individual children and families with a duration of 1 year, and using a combination of individual and group sessions as well as a Web-based learning module. Changes in scientific working principles with regard to data collection, reporting and translation to guidelines are required. Practice and science may benefit from close collaboration in designing, implementing and evaluating interventions.

Clinical and biochemical effects of zileuton in patients with the Sjögren-Larsson syndrome.

UNLABELLED: The Sjögren-Larsson syndrome (SLS) is an inborn error of lipid metabolism, characterised clinically by congenital ichthyosis, mental retardation and spasticity. Patients also suffer from severe pruritus. The degradation of leukotriene (LT) B4 is one of the defective metabolic routes in SLS. Zileuton inhibits the synthesis of LTB4 and the cysteinyl leukotrienes. Five SLS patients were treated with zileuton for 3 months. Favourable effects were found on pruritus score (P = 0.006), general well-being, and background activity of electroencephalographic studies. Neuropsychological test results did not change significantly. There was, however, a clinically important trend towards improvement in the speed of information processing. Results of cerebral MRI and proton magnetic resonance spectroscopy did not change during therapy. Urinary concentrations of LTB4 and omega-OH-LTB4 decreased significantly (P=0.02 and P=0.003 respectively), while their concentrations in CSF were normal at baseline and remained so after therapy. CONCLUSION: Patients with Sjögren-Larsson syndrome might benefit from treatment with zileuton, especially with respect to the agonising pruritus. The findings reported here, point to a crucial role for leukotriene B4 in the pathogenesis of pruritus.